Everything we are told in this case is typical of COLLAGENOUS COLITIS. In principle, the clinical and endoscopic findings may leave us a little as at the beginning, but histology is little more than the definition of this pathology. The specific criterion for the diagnosis of collagenous colitis is the additional presence of an irregular band of collagen just below the surface epithelium of the colonic mucosa in continuity with the basement membrane, visible with trichrome staining (stains type I collagen fibers), which traps superficial capillaries producing lesions in the surface epithelium. In addition, it is accompanied by a chronic inflammatory infiltrate in the lamina propria, composed mainly of lymphocytes, plasma cells and eosinophils.

A 59-year-old woman presents with chronic watery diarrhea of 4 months' duration. On endoscopy, the mucosa showed no relevant features. In particular, no ulcers or friable areas were observed. A biopsy of the transverse colon was performed. Histopathology revealed a thickened area below the superficial lining epithelium, which was more evident by Masson's trichrome technique and involved epithelial atrophy and denudation. There was also a clear increase in intraepithelial lymphocyte density. The diagnosis of the intestinal lesion is?

The role of mucosal biopsy in the diagnosis of chronic diarrhea: value of multiple biopsies when colonoscopic finding is normal or nonspecific.

There are controversies about taking routine mucosal biopsy when the gross colonoscopic finding is normal. This study was conducted to determine the frequency of clinically important histological abnormalities, prospectively, in chronic diarrhea patients with grossly normal or nonspecific colonoscopic findings. One hundred and eighteen patients suffering from nonbloody diarrhea with average frequency of more than two times a day for more than 4 weeks were included. Multiple biopsies (cecum, ascending colon, mid-transverse colon, descending colon, sigmoid colon and rectum) were taken during colonoscopic examinations and each biopsy specimen was reviewed by one pathologist after H&E and Masson-trichrome staining. Clinically significant abnormalities (2 collagenous colitis, 1 lymphocytic colitis, 1 eosinophilic enterocolitis, 1 ulcerative colitis and 4 melanosis coli) were observed in 9 patients (7.6%). Sixteen cases (13.6%) of borderline histological abnormalities were observed (8 cases of possible collagenous colitis and 8 cases showing some features of lymphocytic colitis). Ninety two cases (78.8%) showed nonspecific inflammation only. Clinically important histological lesions can exist in significant percentage in spite of normal or nonspecific colonoscopic findings, which can justify routine mucosal biopsy in the evaluation of chronic diarrhea patients. The clinical significance of borderline histological abnormalities needs to be determined by careful follow-up studies.

Microscopic colitis

Diagnosis Colonoscopic appearances are normal or near normal. As the changes are often patchy, an examination limited to the rectum may miss cases of microscopic colitis, and so a full colonoscopy is necessary. Multiple colonic biopsies are taken in order to make the diagnosis. Histological features of colonic biopsies indicating microscopic colitis are: greater than 20 intraepithelial lymphocytes per 100 epithelial cells and, additionally, 10-20 μm of a thickened subepithelial collagen band in collagenous colitis. Inflammation of the lamina propria, with mainly mononuclear cells, may be observed in collagenous colitis.

Lymphocytic, collagenous and other microscopic colitides: pathology and the relationship with idiopathic inflammatory bowel diseases.

Collagenous colitis and lymphocytic colitis are the two major conditions characterized by chronic watery diarrhoea, without endoscopic or radiological lesions, but with histological abnormalities and therefore considered as "microscopic colitis". The histology of colonic biopsies shows inflammation of the mucosa, and either thickening of the subepithelial collagen band or an increase of lymphocytes in the surface epithelium. Different variant forms have been reported under separate names. These are probably not specific entities. The incidence of microscopic colitis is slightly less than the incidence of chronic idiopathic inflammatory bowel diseases (IBD). Microscopic colitis and IBD are clearly different entities. The relation between both entities is weak but double. Biopsy samples from patients with IBD may mimic the features of lymphocytic or collagenous colitis, both in the initial onset and during follow-up. In the large majority of these cases, endoscopy shows or has shown mucosal lesions. In rare cases, however, a double diagnosis was made. Certain patients, usually of older age, presented first with a microscopic, usually collagenous colitis and developed subsequently genuine ulcerative colitis.

[Microscopic colitis - review].

Microscopic colitis (MC) is an encompassing term for two diseases; collagenous colitis and lymphocytic colitis. The colon appears normal by colonoscopy and a diagnosis is only obtained with a biopsy. The histopathology of collagenous colitis is mainly characterized by a thickening of the subepithelial basement membrane of the colonic mucosa with a band of collagen. Lymphocytic colitis is mainly characterized by an intraepithelial lymphocytosis without the collagen thickening. Even though the two diseases have a distinctive pathology their clinical symptoms are characterized by chronic watery diarrhea without bleeding. Microscopic colitis is thought to cause about 4-13% of all chronic diarrhea but their relative frequency is much higher among older people. The mean annual incidence for collagenous and lymphocytic colitis has been increasing. Steroids are the most effective treatment for microscopic colitis and budesonide is the most studied and effective therapy for MC. The aim of this paper is to give a review of two relatively new diseases which are among the most common cause of chronic diarrhea, especially among older people.

Histopathological diagnosis of microscopic colitis.

A typical symptom of microscopic colitis (MC) is chronic watery diarrhea with normal endoscopic findings and characteristic inflammatory changes in histopathology. Treatment of the disease is mainly empiric. MC has two main subtypes: lymphocytic colitis and collagenous colitis. There are also untypical histopathological forms of MC: MC with giant cells, MC not otherwise specified (NOS) and cryptal lymphocytic coloproctitis. Some other histopathological changes in MC have been observed, especially Paneth cell hyperplasia or epithelial degeneration. Eosinophilic colitis, acute colitis, amyloidosis, ulcerative colitis and Crohn's disease should be taken into consideration in differential diagnosis. The most reliable biopsy material for histopathological examination are samples obtained from transverse colon. Some studies proved that treatment of MC makes it possible to reduce not only clinical, but also histopathological, manifestations.

Microscopic colitis

Pathology Microscopic colitis is characterized by an increase in inflammatory cells, particularly lymphocytes, in colonic biopsies with an otherwise normal appearance and architecture of the colon. Inflammatory cells are increased both in the surface epithelium ("intraepithelial lymphocytes") and in the lamina propria. The key feature is more than 20 intra-epithelial lymphocytes per 100 epithelial cells. These are the principal features of lymphocytic colitis. An additional distinguishing feature of collagenous colitis is a thickened subepithelial collagen layer, which may be up to 30 micrometres thick, that occurs in addition to the features found in lymphocytic colitis. The fact that the two types of microscopic colitis share many features including epidemiology, risk factors and, response to therapy has led to the suggestion that they are actually subtypes of the same disease.

[Microscopic colitis: pathogenesis].

Microscopic colitis (MC) is a chronic inflammatory process observed in colon biopsies of patients with chronic aqueous diarrhea. It is called microscopic because diagnosis is determined by histological studies since the microscopic characteristics of the colon endoscopy are normal. Two patterns exist: Lymphocytic Microscopic Colitis and Collagenous Microscopic Colitis. Etiology is unknown, and the proposed pathogenic mechanisms indicate an immunological phenomenon. Based on this, the authors of this study hypothesize that lymphocytic infiltration of the lamina propria could be related to cytotoxic lymphocytes CD8 as causative agents of colon tissue damage. Prove hypothesis of immunological pathogenesis of MC. APPARATUS AND METHODS: Thirty eight (38) patients with diagnosed MC were recruited for the last four years in the Pathology Laboratory at Ricardo Palma University. Twenty two (22) colon biopsies with the most severe histological lesions were selected. These biopsies were obtained from 17 patients: 5 patients had 2 biopsies in 2 colonoscopy sessions. Biopsies were fixed in neutral formaldehyde, processed through the paraffin inclusion method, and stained with hematoxiline-eosine and Masson trichromic to distinguish collagenous tissue. Immunohistochemistry was conducted in 4- or 5-micron-thick histological sections processed through the immunoperoxidase method. Nineteen (19) biopsies corresponded to Lymphocytic MC and 3 to Collagenous MC. Lymphocytic MC showed intraepithelial lymphocytosis, dystrophic epithelial damage in the areas of lymphocytic infiltration, lamina propria inflammation with lymphocytes and plasma cells, and normal basement membrane. Collagenous MC showed thickened basement membrane due to the presence of a collagenous band, mild to moderate intraepithelial lymphocytosis, vacuolization,and frequent detachment of protective epithelium. Twenty two (22) biopsies were positive in the immunohistochemical studies.

Ulcerative colitis

The simple clinical colitis activity index was created in 1998 and is used to assess the severity of symptoms. Endoscopic The best test for diagnosis of ulcerative colitis remains endoscopy, which is examination of the internal surface of the bowel using a flexible camera. Initially, a flexible sigmoidoscopy may be completed to establish the diagnosis. The physician may elect to limit the extent of the initial exam if severe colitis is encountered to minimize the risk of perforation of the colon. However, a complete colonoscopy with entry into the terminal ileum should be performed to rule out Crohn's disease, and assess extent and severity of disease. Endoscopic findings in ulcerative colitis include: erythema (redness of the mucosa), friability of the mucosa, superficial ulceration, and loss of the vascular appearance of the colon. When present, ulcerations may be confluent. Pseudopolyps may be observed.

[Evolution of ideas on microscopic colitis].

The literature review gives the present-day views of the definition, etiology, pathogenesis, diagnosis, and treatment of microscopic colitis (MC). In the present view, MC is an inflammatory bowel disease of unknown etiology, which is characterized by chronic watery diarrhea, no macroscopic signs of large bowel involvement in the presence of specific pathomorphological changes. There are two major forms of MC, which are similar in its clinical picture, yet, heterogeneous in histological criteria: collagenous colitis (CC) and lymphocytic colitis (LC). As of now, the prevalence of MC is about 100 cases per 100,000 population, which is similar with that in other inflammatory bowel diseases, such as ulcerative colitis and Crohn's disease. MC generally prevails in women aged over 50 years. The etiology and pathogenesis of MC have not fully investigated. Watery diarrhea is as a predominant pathognomonic symptom in all the patients with MC. The major histological criterion for the diagnosis of CC is subepithelial collagen lining thickening (more than 10 pm) and that for LC is higher intraepithelial lymphocyte counts (more than 20 intraepithelial lymphocytes/100 epitheliocytes). The topical glucocorticosteroid budesonide is currently the only agent, the efficacy of which has been proven in both inducing and maintaining remission in patients with MC in many clinical trials.

[Are lymphocytic and collagenous colitis two forms of a single disease? Arguments taken from a biopsy quantitative study].

Collagenous colitis and lymphocytic colitis are defined by a clinicopathologic syndrome with chronic watery diarrhea, microscopic lesions of colonic biopsies, and normal barium enema and colonoscopy. A histopathological study was performed on multiple colorectal biopsies to compare 12 cases of collagenous colitis (defined by a subepithelial collagen thicker than 10 microns) and 7 cases of lymphocytic colitis (defined by a number of intraepithelial lymphocytes more than 20 per 100 epithelial cells at least in one biopsied site). The study included a semiquantitative analysis of inflammatory infiltrate in the lamina propria, crypts distortion and epithelial detachment. The number of intraepithelial lymphocytes per 100 epithelial cells was determined in surface epithelium and crypts. The subepithelial collagen thickening was studied by computerised morphometry. The intraepithelial lymphocytes, villous atrophy and thickness of the subepithelial collagen were also determined in gastric and duodenal biopsies. In collagenous colitis, the subepithelial collagenous thickness ranged from 10 to 40 microns in the colon (median 20.99 microns). In 4 cases of collagenous colitis, no thickening of the collagen plate was seen in the rectum. We found constant epithelial detachment and mucosal distortion. In lymphocytic colitis, the thickness of the subepithelial collagen ranged from 6 to 10 microns in 4 cases and was less than 6 microns in 3 cases (median 6.24 microns). The median number of intraepithelial lymphocytes in surface epithelium was 22.35 (range 18.2 to 40) in lymphocytic colitis versus 12.22 (range 4.6 to 24.4) in collagenous colitis. In conclusion, we observed an overlap of both the collagenous plate thickness and the number of intraepithelial lymphocytes in collagenous colitis and lymphocytic colitis. This result favours a unified histogenesis for these two entities.

Lymphocytic colitis

Lymphocytic colitis is a subtype of microscopic colitis, a condition characterized by chronic non-bloody watery diarrhea. Causes No definite cause has been determined. The peak incidence of lymphocytic colitis is in persons over age 50; the disease affects women and men equally. Some reports have implicated long-term usage of NSAIDs, proton pump inhibitors, and selective serotonin reuptake inhibitors, and other drugs. Associations with other autoimmune disorders suggests that overactive immune responses occur. Diagnosis The colonoscopy is normal but histology of the mucosal biopsy reveals an accumulation of lymphocytes in the colonic epithelium and connective tissue (lamina propria). Collagenous colitis shares this feature but additionally shows a distinctive thickening of the subepithelial collagen table.

[Microscopic colitis: histopathological review with a clinicopathological correlation].

Microscopic colitis is a clinicopathological entity which, in addition to typical symptoms such as watery diarrhea, is characterized by its specific histopathology. Since colonoscopy yields normal findings, microscopic colitis belongs in a histological domain. The term encompasses two forms: lymphocytic and collagenous colitis. Histologically, lymphocytic colitis shows an increase in intraepithelial lymphocytes of more than 20 lymphocytes per 100 surface colonocytes, while collagenous colitis is characterized by a thickened subepithelial collagen layer of more than 10 µm. Specific stains help in the quantification of both. Since microscopic colitis does not always affect the entire colon and the number of intraepithelial lymphocytes varies physiologically, obtaining stepwise biopsies of the colon (with information on location where possible) is recommended. A thickened collagen layer is relatively specific for collagenous colitis, whereas intraepithelial lymphocytosis is also found in other diseases. Therefore, to make a correct diagnosis, it is important to correlate histological findings with clinical symptoms, including the main symptom of watery diarrhea.

Colitis

An important investigation in the assessment of colitis is biopsy. A very small piece of tissue (usually about 2mm) is removed from the bowel mucosa during endoscopy and examined under the microscope by a histopathologist. It can provide important information regarding the cause of the disease and the extent of bowel damage. Types There are many types of colitis. They are usually classified by the cause. Types of colitis include: Autoimmune Inflammatory bowel disease (IBD) – a group of chronic colitides. Ulcerative colitis (UC) – a chronic colitis that affects the large intestine. Crohn's disease (CD) – another type of IBD that often leads to colitis. Unknown Microscopic colitis – a colitis diagnosed by microscopic examination of colonic tissue; macroscopically ("to the eye") it appears normal. Lymphocytic colitis Collagenous colitis Treatment-caused Diversion colitis Chemical colitis Chemotherapy-induced colitis Radiation colitis Checkpoint inhibitor induced colitis

Segmental colitis associated with diverticulosis

Types There are four types of SCAD, based on endoscopic appearance. Pattern A is characterized by involvement of crescentic folds and is the most common type of SCAD (52%). Pattern B has an appearance similar to mild-to moderate ulcerative colitis (30.40%), whereas pattern C appears similar to Crohn's disease (10.90%). Pattern D is the least common, and appears similar to severe ulcerative colitis (6.50%). Diagnosis SCAD is diagnosed via colonoscopy, often incidentally during examination for unrelated concerns. Colonoscopy shows erythema of the colonic mucosa, which may be characterized by friability and exudate. The descending and sigmoid colon are typically involved. Biopsies of the affected area and the unaffected rectum confirm the diagnosis. Biopsies of SCAD show evidence of chronic inflammation. Rectal biopsies show normal mucosa.

Ulcerative colitis

Ulcerative colitis is usually continuous from the rectum, with the rectum almost universally being involved. Perianal disease is rare. The degree of involvement endoscopically ranges from proctitis (rectal inflammation) to left sided colitis (extending to descending colon), to extensive colitis (extending proximal to descending colon). Histologic Biopsies of the mucosa are taken during endoscopy to confirm the diagnosis of UC and differentiate it from Crohn's disease, which is managed differently clinically. Histologic findings in ulcerative colitis includes: distortion of crypt architecture, crypt abscesses, and inflammatory cells in the mucosa (lymphocytes, plasma cells, and granulocytes). Unlike the transmural inflammation seen in Crohn's disease, the inflammation of ulcerative colitis is limited to the mucosa.

Microscopic colitis

Microscopic colitis refers to two related medical conditions which cause diarrhea: collagenous colitis and lymphocytic colitis. Both conditions are characterized by the presence of chronic non-bloody watery diarrhea, normal appearances on colonoscopy and characteristic histopathology findings of inflammatory cells. Signs and symptoms The main symptom is persistent non-bloody watery diarrhea, which may be profuse. People may also experience abdominal pain, fecal incontinence, and unintentional weight loss. Microscopic colitis is the diagnosis in around 10% of cases investigated for chronic non-bloody diarrhea.

Histology of microscopic colitis-review with a practical approach for pathologists.

Microscopic colitis has emerged as a major cause of chronic watery non-bloody diarrhoea, particularly in elderly females. The term is used as an umbrella term to categorize a subgroup of colitides with distinct clinicopathological phenotypes and no significant endoscopic abnormalities. Lymphocytic colitis is defined by an increased number of surface intraepithelial lymphocytes, and collagenous colitis by a thickened collagen band underneath the surface epithelium. There is increased inflammation in the lamina propria, but only little or no crypt architectural distortion. Incomplete and variant forms showing less characteristic features have been reported under different names. The differential diagnosis mainly includes resolving infectious colitis and changes related to the intake of drugs such as non-steroidal anti-inflammatory drugs. Substantial clinical and histological overlap between lymphocytic and collagenous colitis has been described, raising the suspicion that the conditions are two histological manifestations of the same entity, possibly representing different manifestations during the disease course or different stages of disease development. In this review, we provide a practical approach for pathologists, with a focus on diagnostic criteria and differential diagnosis, and discuss recent insights into the pathogenesis of disease and the relationship with classic chronic inflammatory bowel disease, i.e. Crohn's disease and ulcerative colitis.

Collagenous colitis and lymphocytic colitis: clinical and endoscopic findings.

collagenous colitis (CC) and lymphocytic colitis (LC) are two entities of unknown cause, characterized by chronic watery diarrhea, grossly normal-appearing colonic mucosa and abnormal histopathological findings in colonic biopsies. The clinical features of the disease are based mainly on case reports or small uncontrolled series. Although normal colonoscopic findings are, as a rule, part of the diagnosis of CC, several cases of macroscopic colitis associated with CC have been reported, and the spectrum of endoscopic mucosal changes has not been described in large series. we present a retrospective study of all patients who underwent total colonoscopy and mucosal biopsy in our Endoscopy Unit between 1991 and 1997. Clinical and endoscopic findings in patients diagnosed as having CC or LC were recorded. of 676 patients studied, 398 suffered from chronic diarrhea. Collagenous colitis was diagnosed in 22 patients and LC in 10. Eleven per cent of the patients with CC and 20% of those with LC did not have diarrhea. Macroscopic colitis was observed in 6 out of 22 patients with CC (27%) and in 4 out of 10 with LC (40%). Macroscopic lesions included edema, erythema, abnormal vascular pattern, superficial erosions or ulcerations and hemorrhagic lacerations. In this series 7.03% of the patients with chronic diarrhea were diagnosed as having CC or LC. collagenous colitis and LC are two entities that should be considered in the differential diagnosis of chronic diarrhea. Total colonoscopy with multiple biopsies that include the right colon are mandatory. The presence of macroscopic lesions on endoscopy does not rule out a diagnosis of either entity. We identified patients who fulfilled the histopathological criteria for CC or LC but who did not have diarrhea.

Inflammatory bowel disease

No disease specific markers are currently known in the blood, enabling the reliable separation of Crohn's disease and ulcerative colitis patients. The way doctors can tell the difference between Crohn's disease and UC is the location and nature of the inflammatory changes. Crohn's can affect any part of the gastrointestinal tract, from mouth to anus (skip lesions), although a majority of the cases start in the terminal ileum. Ulcerative colitis, in contrast, is restricted to the colon and the rectum. Microscopically, ulcerative colitis is restricted to the mucosa (epithelial lining of the gut), while Crohn's disease affects the full thickness of the bowel wall ("transmural lesions"). Lastly, Crohn's disease and ulcerative colitis present with extra-intestinal manifestations (such as liver problems, arthritis, skin manifestations and eye problems) in different proportions.

Ulcerative colitis

The clinical presentation of ulcerative colitis depends on the extent of the disease process. Up to 15% of individuals may have severe disease upon initial onset of symptoms. A substantial proportion (up to 45%) of people with a history of UC without any ongoing symptoms (clinical remission) have objective evidence of ongoing inflammation. Ulcerative colitis is associated with a generalized inflammatory process that can affect many parts of the body. Sometimes, these associated extra-intestinal symptoms are the initial signs of the disease. Extent of involvement In contrast to Crohn's disease, which can affect areas of the gastrointestinal tract outside of the colon, ulcerative colitis is usually confined to the colon. Inflammation in ulcerative colitis is usually continuous, typically involving the rectum, with involvement extending proximally (to sigmoid colon, ascending colon, etc). In contrast, inflammation with Crohn's disease is often patchy, with so-called "skip lesions."

Microscopic colitis in routine colonoscopies.

Lymphocytic colitis (LC) and collagenous colitis (CC), both known as microscopic colitis (MC), are uncommon entities with increasing incidence as more clinicians take biopsies from macroscopically normal colons and as pathologists use more rigorous diagnostic criteria to be confident of the diagnosis. Information on the incidence of this type of colitis is limited and based on reported cases. The purpose of this work is to estimate the incidence of LC and CC in reviewed routine colonoscopies. We reviewed 2815 colonoscopies performed at a tertiary referral center with an open-access service using restricted histological criteria in order to establish the frequency rate of LC and CC in routine colonoscopic biopsy material. Cases suspicious for MC were stained with Masson's trichrome or Congo red stain and immunohistochemically for lymphocytes, where appropriate. Review of routine colonoscopic biopsies showed that MC is underreported in our colonoscopic material. Incidence rates of LC and CC (0.9 and 0.4, respectively) were based on morphological assessment of colonoscopic biopsies using stringent criteria together with clinical data and after differentiation with other lesions which can mimic MC. The 10.2% rate of this type of colitis in patients with chronic watery diarrhea indicates the necessity to consider these lesions in older individuals with diarrhea and normal endoscopical colonic mucosa.

Microscopic colitis in patients presenting with chronic diarrhea.

To investigate the prevalence of microscopic colitis among patients presenting with chronic watery diarrhea. Colonic biopsies from 400 patients presenting with chronic watery diarrhea and other symptoms pertaining to lower gastrointestinal tract were studied. After a detailed clinical history and thorough physical examination full length colonoscopy was done using flexible colonoscope. Colonic biopsies were taken from abnormal and normal areas. Three to five micron thick sections were cut and stained with hematoxylin and eosin and Masson's trichrome stain to highlight sub epithelial collagen. Fifteen out of 400 (3.7%) colonic biopsies from patients presenting with chronic diarrhea had evidence of microscopic colitis. Five out of fifteen biopsies (33%) were diagnosed as collagenous colitis, 10 biopsies (67%) had evidence of lymphocytic colitis; 14/400(3.5%) histologically normal biopsies were taken as controls to compare various demographic and risk factors. Ten out of 15 patients (67%) were clinically diagnosed as irritable bowel syndrome. In the remaining five an infective etiology was suspected. On colonoscopy12/15 (80%) had no abnormality and 3/15 (20%) had mild hyperemia. A possibility of microscopic colitis should be considered while examining colonoscopic biopsy of a patient with chronic watery diarrhea and normal colonoscopy to avoid the misdiagnosis that may affect the treatment of patients.

Endoscopic findings and colonic perforation in microscopic colitis: A systematic review.

Microscopic colitis (MC) is a clinical syndrome of severe watery diarrhea with few or no endoscopic abnormalities. The incidence of MC is reported similar to that of other inflammatory bowel diseases. The need for histological confirmation of MC frequently guides reimbursement health policies. With the advent of high-definition (HD) coloscopes, the incidence of reporting distinct endoscopic findings in MC has risen. This has the potential to improve timely diagnosis and cost-effective MC management and diminish the workload and costs of busy modern endoscopy units. Publications on distinct endoscopic findings in MC available until March 31st, 2017 were searched systematically (electronic and manual) in PubMed database. The following search terms/descriptors were used: collagenous colitis (CC) OR lymphocytic colitis (LC) AND endoscopy, colonoscopy, findings, macroscopic, erythema, mucosa, vasculature, scars, lacerations, fractures. An additional search for MC AND perforation was made. Eighty (n=80) articles, predominantly single case reports (n=49), were found. Overall, 1582 (1159F; 61.6±14.1 years) patients (pts) with MC and endoscopic findings were reported. The majority of articles (n=62) were on CC (pts 756; 77.5% females). We identified 16 papers comprising 779 pts (69.2% females) with LC and 7 articles describing 47 pts (72.3% females) diagnosed as MC. The youngest patient was 10 and the oldest a 97-year-old. Aside diarrhea, symptoms included abdominal pain, weight loss, bloating, flatulence, edema and others. In the study group we found 615 (38.8%) persons with macroscopic lesions in gut. Isolated linear ulcerations were identified in 7 pts (1.1%) while non-ulcerous lesions i.e. pseudomembranes, a variable degree of vasculature pruning & dwindling, mucosal lacerations and abnormalities such as erythema/edema/nodularity, or surface textural alteration in 608 pts (98.1%). The location of endoscopic findings was not reported in 27 articles. The distinct endoscopic findings were described in the left (descending, sigmoid, rectum - 10/21/11 studies), right (cecum, ascending - 7/7 studies), transverse colon (n=12), as well as duodenum (n=4), and terminal ileum (n=2). In 17 (1.1%) pts colonic perforation occurred. Endoscopic findings are recognized with increased frequency in pts with MC. This could improve MC diagnosis by prompting a more extensive biopsy protocol in such cases and an earlier initiation of treatment. Procedure-related perforation has been reported in this group; therefore, cautious air insufflation is advisable when endoscopic findings are recognised.

Collagenous colitis

Collagenous colitis is an inflammatory bowel disease affecting the colon specifically with peak incidence in the 5th decade of life, affecting women more than men. Its clinical presentation involves watery diarrhea in the absence of rectal bleeding. It is often classified under the umbrella entity microscopic colitis, that it shares with a related condition, lymphocytic colitis. Signs and symptoms Microscopic colitis causes chronic watery diarrhea with greater than 10 bowel movements per day. Some patients report nocturnal diarrhea, abdominal pain, urgency, fecal incontinence, fatigue, dehydration and weight loss. Patients report a significantly diminished quality of life. Causes The cause of collagenous colitis is unknown.

Microscopic colitis: Common cause of unexplained nonbloody diarrhea.

Microscopic colitis (MC) is characterized by chronic, watery, secretory diarrhea, with a normal or near normal gross appearance of the colonic mucosa. Biopsy is diagnostic and usually reveals either lymphocytic colitis or collagenous colitis. The symptoms of collagenous colitis appear most commonly in the sixth decade. Patients report watery, nonbloody diarrhea of a chronic, intermittent or chronic recurrent course. With collagenous colitis, the major microscopic characteristic is a thickened collagen layer beneath the colonic mucosa, and with lymphocytic colitis, an increased number of intraepithelial lymphocytes. Histological workup can confirm a diagnosis of MC and distinguish the two distinct histological forms, namely, collagenous and lymphocytic colitis. Presently, both forms are diagnosed and treated in the same way; thus, the description of the two forms is not of clinical value although this may change in the future. Since microscopic colitis was first described in 1976 and only recently recognized as a common cause of diarrhea, many practicing physicians may not be aware of this entity. In this review, we outline the epidemiology, risk factors associated with MC, its etiopathogenesis, the approach to diagnosis and the management of these individuals.

[Ulcerative colitis with segmental involvement].

Ulcerative colitis is a chronic inflammatory disease affecting areas of the colon or the full length. From the endoscopic point of view, ulcerative colitis presents lesions that stretch continuously from the rectum to variable colon segments, a characteristic that is of great value when distinguishing it from Crohn's disease. Continuous involvement, without healthy patches, justifies ending endoscopic exploration once the distal end of the lesion has been reached. To retrospectively study the frequency of segmental lesions in the colonoscopies performed in patients with ulcerative colitis. Diagnosis of ulcerative colitis and proctitis was established by clinical, endoscopic, histologic, analytical, and radiological criteria. The indication and number of endoscopies was made on the basis of the clinical criteria of diagnosis, acute episodes, refractoriness or dysplasia screening. The extent of the examination also depended on clinical criteria: the severity of the episode, tolerance to colonoscopy or the degree of cleansing. A total of 155 coloscopies were performed. In 113 colonoscopies (73%) the distal end of the lesion was reached and in 70 (45%) the cecum was reached. Of the 80 patients, 27 (33%) presented ulcerative proctitis at diagnosis. Nine of the 80 patients (11.3%) biopsies were performed in healthy colonic patches, which confirmed histological normality. Six of the 9 patients were receiving no treatment. In all patients except two, the cecum was reached in one or more of the colonoscopies. The distribution of the segmental lesions varied but these were mainly found in the periappendicular region and in the cecum in 6 of the 7 patients in whom the cecum was reached. Of the 80 patients, endoscopic evidence of rectal sparing was found in 5 (6.3%); of these, 4 were receiving systemic or topical treatment. Histological analysis confirmed the absence of inflammatory lesions in these patients. The only patient who was not receiving treatment presented microscopic lesions compatible with ulcerative colitis. Endoscopic segmental lesions in ulcerative colitis were present in 11.3% of patients. Segmental lesions were most frequently found in the cecum and periappendicular region. Endoscopic and histologic evidence of rectal sparing may be the result of systemic or topical treatment.

Pitfalls in the diagnosis of collagenous colitis: experience with 75 cases from a registry of collagenous colitis at the Johns Hopkins Hospital.

Collagenous colitis is a relatively rare disorder presenting mainly in middle-aged women as watery diarrhea. Endoscopic and radiographic studies of the colon are usually normal, and diagnosis must be made by biopsy. The characteristic biopsy findings are a combination of increased mucosal inflammation (collagenous colitis) as well as subepithelial collagenous thickening. The mucosal inflammatory changes include increased lamina propria plasma cells, prominent intraepithelial lymphocytes, and in some cases, numerous eosinophils. The collagenous thickening has qualitative as well as quantitative differences from normal, and may be highlighted by Masson trichrome stains. Simply quantitating the thickness of a subepithelial collagen layer is neither adequate nor necessary for the diagnosis of collagenous colitis. Major problems in diagnosing collagenous colitis arise from focusing solely on the subepithelial region without attention to inflammatory changes. For example, tangential sectioning of normal colon results in an artifactually thickened basement membrane, and such cases have been wrongly interpreted as collagenous colitis. If biopsies lack the characteristic inflammatory pattern, a tangentially cut thick basement membrane should be ignored. The key to correct diagnosis of collagenous colitis is analyzing the summation of various inflammatory changes plus subepithelial collagenization, rather than focusing on any single feature in isolation.

[Clinical significance of erosive and/or small ulcerative lesions in the colon and terminal ileum--short-term follow-up study].

Various etiologies and diseases may be related to erosions and/or small ulcers without gross inflammatory changes in the surrounding mucosa found in the colon and terminal ileum during colonoscopy. However, studies on follow-up of these lesions are rare. Thus, we investigated the clinical significance of these lesions and their characteristics helpful for differential diagnosis. We reviewed the data of 183 patients with colonoscopically observed erosive or small ulcerative lesions (<2 cm), and analyzed them according to the location, number, and size of lesions, histopathologic findings, chief complaints, laboratory findings, changes of symptoms, and changes in lesions during 4-12 week follow-up period. Histopathologic findings of these lesions included acute nonspecific inflammation, chronic nonspecific inflammation, Crohn's disease, tuberculous colitis, ischemic colitis, Behcet's disease, cytomegalovirus infection, eosinophilic colitis, ulcerative colitis or pseudomembranous colitis, but most of them were nonspecific (84%). In patients with nonspecific inflammation, histopathologic findings, symptoms, location and multiplicity of the lesions were not prognostic factors for the persistency of symptoms and lesions during follow-up period. Two patients with acute inflammation, who showed no improvement in symptoms and lesions, were later diagnosed as Crohn's disease. Erosive or small ulcerative lesions without macroscopic inflammatory changes in the surrounding mucosa during colonoscopy, are mainly nonspecific. However, careful follow-up is required when the symptoms and/or lesions are not improved.

Pancolitis

Pancolitis, in its most general sense, refers to inflammation of the entire colon. This can be caused by a variety of things. Pancolitis or universal colitis is frequently used in a more specific fashion to denote a very severe form of ulcerative colitis. This form of ulcerative colitis is spread throughout the entire large intestine including the right colon, the left colon, the transverse colon, descending colon, and the rectum. A diagnosis can be made using a number of techniques but the most accurate method is direct visualization via a colonoscopy. Symptoms are similar to those of ulcerative colitis but more severe and affect the entire large intestine. Patients with ulcerative colitis generally exhibit symptoms including rectal bleeding as a result of ulcers, pain in the abdominal region, inflammation in varying degrees, and diarrhea (often containing blood). Pancolitis patients exhibit these symptoms and may also experience fatigue, fever, and night sweats. Due to the loss of

Microscopic colitis

Epidemiology Incidence and prevalence of microscopic colitis nears those of ulcerative colitis and Crohn's disease. Studies in North America found incidence rates of 7.1 per 100,000 person-years and 12.6 per 100,000 person-years for collagenous colitis for lymphocytic colitis, respectively. Prevalence has been estimated as 103 cases per 100,000 persons. People who develop microscopic colitis are characteristically, though not exclusively, middle-aged females. The average age of diagnosis is 65 but 25% of cases are diagnosed below the age of 45. History The condition of microscopic colitis was first described as such in 1982. Lymphocytic colitis was described in 1989. Collagenous colitis was recognised earlier, in 1976. References External links MayoClinic.com Colitis Steroid-responsive inflammatory conditions

Microscopic colitis

Differential diagnosis Differential diagnoses, which should be ruled out, include bile acid diarrhea, lactose malabsorption, celiac disease, Crohn's disease, ulcerative colitis, and infectious colitis. Treatment Lymphocytic and collagenous colitis have both been shown in randomized, placebo-controlled trials to respond well to budesonide, a glucocorticoid. Budesonide formulated to be active in the distal colon and rectum is effective for both active disease and in the prevention of relapse. However, relapse occurs frequently after withdrawal of therapy. Studies of a number of other agents including antidiarrheals, bismuth subsalicylate (Pepto-Bismol), mesalazine/mesalamine (alone or in combination with cholestyramine), systemic corticosteroids, cholestyramine, immunomodulators, and probiotics have shown to be less effective than budesonide for treating both forms of microscopic colitis.

List of MeSH codes (C06)

– gastroenteritis – appendicitis – cholera morbus – colitis – colitis, ischemic – colitis, microscopic – colitis, collagenous – colitis, lymphocytic – colitis, ulcerative – proctocolitis – dysentery – dysentery, amebic – dysentery, bacillary – enteritis – duodenitis – ileitis – pouchitis – enterocolitis – enterocolitis, necrotizing – enterocolitis, neutropenic – enterocolitis, pseudomembranous – esophagitis – esophagitis, peptic – gastritis – gastritis, atrophic – gastritis, hypertrophic – inflammatory bowel diseases – colitis, ulcerative – crohn disease – mucositis – proctitis – proctocolitis


Aortic stenosis.

A 78-year-old patient with no past history who consults for asthenia and dyspnea of 3 months of evolution, which has progressed to rest in the last few days. In the previous days he also refers chest pain with anginal characteristics with small efforts. On examination, blood pressure of 110/80 mmHg, heart rate of 85 bpm and auscultation with a rough systolic murmur in the right second intercostal space and crackles in both lung bases. Which of the following is the most likely diagnosis?

Dilated cardiomyopathy of ischemic origin.

[An autopsied case with a bicuspid aortic valve who had progressive angina pectoris and heart failure during follow-up of 27 years].

A Japanese man who died at age 85 had been followed since the age of 59, when he first presented. He had hypertension of 162/102 mmHg and a loud systolic murmur on his first visit. He had had an active daily life without any medication for the next 10 years. At the age of 72 he complained of mild chest discomfort on exercise. Although electrocardiography showed no abnormalities, echocardiogram showed calcified bicuspid aortic valve with mild stenosis. At the age of 81 the dyspnea and chest oppression were exacerbated, associated with marked ST depression on exercise electrocardiogram and restriction of aortic valve opening on echocardiograms. In the following years a gradual increase in QRS voltage and ST depression with T wave inversion were recorded on resting electrocardiograms and sharp increases in both left ventricular end-diastolic diameter and flow velocity at the aortic root were observed on echocardiograms. At the age of 85 he died of intractable heart failure with massive pleural effusion. Autopsy revealed marked hypertrophy and moderate dilatation of the heart (weight: 580 g). The bicuspid aortic valve had anterior-posterior cusps with a raphe on the anterior cusp. The mobility of the cusps was almost lost because of severe calcification and thickening. Severe stenosis was found near the orifice of the right coronary artery, but there were no significant ischemic myocardial lesions.

Cases from the Osler Medical Service at Johns Hopkins University.

PRESENTING FEATURES: A 70-year-old African American man was admitted with a history of fever, chills, and malaise of several days' duration. His past medical history was notable for end-stage renal disease requiring hemodialysis, coronary artery disease, and aortic stenosis requiring a bioprosthetic aortic valve replacement. On the day of admission, the patient was noted to have a shaking chill while undergoing dialysis through his catheter and was admitted to the hospital. He complained of pain at the catheter insertion site, shortness of breath, and dyspnea on exertion, but denied chest pain. On physical examination, the patient had a temperature of 100.4 degrees F, with a heart rate of 64 beats per minute, blood pressure of 127/72 mm Hg, and an oxygen saturation of 97% on room air. He was a mildly obese man in no apparent distress. He had shotty cervical lymphadenopathy and a right subclavian dialysis catheter in place, with erythema and pus at the entry site. His jugular venous pressure was 10 cm H(2)O. Lung examination showed bibasilar rales. Heart sounds were normal, with no rub or gallop. He had a 2/6 systolic ejection murmur best heart at the left sternal border as well as a 3/6 holosystolic murmur at the apex that radiated to his left axilla. Examination of the abdomen and extremities was unremarkable. The patient's neurological examination was unremarkable, and he was alert and oriented to person, place, and time. Laboratory studies showed an elevated white blood cell count of 16,700 cells/microL. His blood urea nitrogen level was 43 mg/dL and his serum creatinine level was 4.9 mg/dL. Multiple blood cultures grew methicillin-resistant Staphylococcus aureus. An admission, chest radiograph showed no infiltrate. An admission electrocardiogram showed normal sinus rhythm with first degree atrioventricular block, left anterior fascicular block, and left ventricular hypertrophy. shows rhythm strips from lead II electrocardiograms 5 months before admission (top), on admission (middle) and 5 days after admission (bottom). What is the diagnosis?

Dyspnoea on exertion in a 53-year-old woman.

A 53-year-old woman with no previous medical history complained of easy fatigue over the last 6 months. She had a positive family history for coronary artery disease but no other risk factors. On physical examination, a 3/6 pansystolic murmur was heard over the apex, and the lung auscultation was unremarkable. Her ECG showed a left anterior fascicular block, with poor R wave progression in the anterior leads (see online supplementary image A). A subsequent echocardiogram revealed a slightly dilated for the patient's body surface area (BSA) (1.73 m<sup2</sup) left ventricle (55/35 mm), with preserved systolic function and a moderate functional mitral regurgitation. The estimated pulmonary artery pressure was 45 mm Hg. During treadmill radionuclide scintigraphy, her exercise tolerance was normal, with good inotropic response, and 96% oxygen saturation at rest and at peak exercise. A 2 mm ST segment depression was noted at peak effort, which persisted well into recovery (see online supplementary image B). The scintigraphy scan showed extensive reversible anteroapical wall ischaemia (see online supplementary image C). At this point she was referred to us for right and left heart catheterisation. Intracardiac pressures and saturations were: right atrium (RA)RA=3 mm Hg, right ventricle (RV)=26/3 mm Hg, Pulmonary artery (PA)=26/10/mean 16 mm Hg, pulmonary capillary wedge pressure (PCWP)=11 mm Hg, left ventricle (LV)=110/10 mm Hg, Aorta (Ao)=110/60/mean 80 mm Hg, Superior vena cava saturation (SVCsat)=62%, RAsat=62%, PAsat=78%, Aosat=96% and estimated pulmonary to systematic flow ratio (Qp/Qs)=1.8. Her coronary angiography and CT angiography are shown in figure 1A,B.DC1SP110.1136/heartjnl-2017-311256.supp1Supplementary material 1 DC2SP210.1136/heartjnl-2017-311256.supp2Supplementary material 2 DC3SP310.1136/heartjnl-2017-311256.supp3Supplementary material 3 heartjnl;103/17/1390/F1F1F1Figure 1Coronary and CT angiograms. What is the most likely diagnosis?Right coronary fistula to right ventricleKawasaki disease with fistulaAnomalous origin of the left coronary artery from the pulmonary arteryPersistent truncus arteriosus.

Valvular heart disease

Medical signs of aortic stenosis include pulsus parvus et tardus, that is, diminished and delayed carotid pulse, fourth heart sound, decreased A2 sound, sustained apex beat, precordial thrill. Auscultation may reveal a systolic murmur of a harsh crescendo-decrescendo type, heard in 2nd right intercostal space and radiating to the carotid arteries. Aortic regurgitation Patients with aortic regurgitation may experience heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, and angina pectoris. In acute cases patients may experience cyanosis and circulatory shock.

Valvular heart disease

Medical signs of aortic regurgitation include increased pulse pressure by increased systolic and decreased diastolic blood pressure, but these findings may not be significant if acute. The patient may have a diastolic decrescendo murmur best heard at left sternal border, water hammer pulse, Austin Flint murmur, and a displaced apex beat down and to the left. A third heart sound may be present Mitral stenosis Patients with mitral stenosis may present with heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, chest pain, hemoptysis, thromboembolism, or ascites and edema (if right-sided heart failure develops). Symptoms of mitral stenosis increase with exercise and pregnancy

Chronic dissecting aneurysm of the ascending aorta developed in a patient who had rejected surgical treatment for type II acute ascending aortic dissection three years earlier.

A 66-year-old male patient was admitted to our clinic because of shortness of breath and chest pain. A grade 4/6 diastolic murmur was heard on auscultation. Physical examination revealed signs of congestive heart failure and poor peripheral perfusion. There was a diagnosis of type II ascending aortic dissection in the history of the patient. He had refused emergency surgical intervention three years earlier. Computed tomography revealed that the ascending aorta was dilated to about 10 cm in diameter, and there was a chronic aortic type II dissection. The patient had second- to third-degree aortic insufficiency and he had a calcified bicuspid aortic valve on echocardiography. Two-vessel disease and a 90-mmHg aortic gradient were detected on angiography. Graft replacement of the ascending aorta, serape aortic valve replacement with a mechanical valve, and coronary arterial bypass grafting were performed successfully under cardiopulmonary bypass with an open aortic technique. The patient was discharged on the 10th postoperative day with no problems.

Aortic Valve Disease -- History and Physical

Aortic regurgitation when acute and/or severe can be suspected when the patient has a wide pulse pressure, and a low pitched early diastolic murmur is auscultated again over the right sternal border at the second intercostal space. Accentuated P2 may also be noticed due to elevated pressures in the pulmonary vasculature. Chronic aortic regurgitation may illicit a blowing diastolic decrescendo murmur with a positive correlation between the duration of murmur and the severity of the disease. Often auscultation of a laterally and inferiorly displaced apical impulse is present and sustained. Often there is the phenomenon of Corrigan pulse (water hammer), a bounding and forceful pulse that rapidly increases and collapses. Other less common physical exam findings include the de Musset sign, which is subtle head bobbing with a pulse, as well as the Quincke sign and Muller sign (pulsations on the fingernails and uvula, respectively). Similar physical exam findings to aortic stenosis can also be seen late in disease progression. Acute aortic regurgitation will present differently depending on the etiology. If a patient presents with tearing severe chest pain along with physical exam findings such as variation in blood pressure between the right and left extremities, consider aortic dissection as a cause. If the patient presented with a history of streptococcal infection along with fevers, swollen and tender joints, skin nodules, new onset of rash, then rheumatic heart disease would be high on the differential.

Case 1/2013: 69-year-old male patient with sudden back and lower right limb pain and shock.

The patient, MSM, a 69-year-old man, sought medical care due to left dorsal and right lower limb pain. The chest x-ray showed mediastinal enlargement. He was undergoing examination when he lost consciousness and went into shock. Subcutaneous emphysema was observed in the left hemithorax, as well as abolition of breath sounds at auscultation. Tracheal intubation was performed with draining of blood-tinged fluid from the left hemithorax. Echocardiography showed left ventricle with 44/29 mm; septum, 12 mm; posterior wall, 13 mm; mild aortic root dilation, dissection of the lamina and periaortic hematoma. The valves and pericardium were normal. The patient was transferred to Instituto do Coraçao - InCor. Physical examination (21 Oct 2004: 10:45) showed that the patient was sedated with tracheal intubation, pale, heart rate at 90 bpm, blood pressure 130 x 80 mmHg, bloody drainage in the chest tube. Electrocardiogram - frequency 90 bpm, sinus rhythm, low voltage in the frontal plane and decreased voltage in left leads (Fig. 1). Computed tomography showed bilateral subcutaneous emphysema, thoracic aorta with inaccurate borders in its descending portion (from the subclavian artery to the middle portion), collapsed left lung and extensive collection of hematic characteristics in same hemithorax and middle and posterior mediastinum. Small right pneumothorax; small right pleural effusion with underlying parenchymal alterations. The analysis of the heart was impaired by the presence of hemothorax. While undergoing computed tomography, the patient showed no pulse, mydriasis, with asystole unresponsive to resuscitation and died (21 Oct 2011; 15:00 h).

Aortic regurgitation

If there is increased stroke volume of the left ventricle due to volume overload, an ejection systolic 'flow' murmur may also be present when auscultating the same aortic area. Unless there is concomitant aortic valve stenosis, the murmur should not start with an ejection click. There may also be an Austin Flint murmur, a soft mid-diastolic rumble heard at the apical area; it appears when a regurgitant jet of blood from severe aortic regurgitation partially closes the anterior mitral leaflet. Peripheral physical signs of aortic regurgitation are related to the high pulse pressure and the rapid decrease in blood pressure during diastole due to blood returning to the heart from the aorta through the incompetent aortic valve, although the usefulness of some of the eponymous signs has been questioned: Phonocardiograms detect AI by having electric voltage mimic the sounds the heart makes. Characteristics- indicative of aortic regurgitation are as follow:

Chest pain

Cocaine use: This condition is suspected when a person with few or no risk of arteriosclerosis presented with non-traumatic chest pain. Ingestion of cocaine can cause vasoconstriction of coronary arteries, thus producing chest pain similar to heart attack. Symptoms can appear within one hour of cocaine use. Aortic stenosis: This condition happens when the person has underlying congenital bicuspid valve, aortic sclerosis, or history of rheumatic fever. Chest pain usually happens during physical activity. Syncope is a late symptom. Signs and symptoms of heart failure may also present. On auscultation, loud ejection systolic murmur can be best heard at the right second intercostal space and radiates to the carotid artery in the neck. Splitting of second heart sound is heard in severe stenosis.

Austin Flint Murmur -- History and Physical

The Austin Flint murmur is a rumbling diastolic murmur best heard at the apex of the heart that is associated with severe aortic regurgitation and is usually heard best in the fifth intercostal space at the midclavicular line. Younger patients are more likely to have a history of a bicuspid aortic valve or rheumatic heart disease, while older patients are more likely to suffer from calcific valvular disease. A blood pressure reading on the patient will show an increased pulse pressure due to the backflow of blood through the aortic valve during diastole. An astute clinician may be able to palpate a "water hammer" pulse, which is also known as "Corrigan's pulse." This finding demonstrates arterial swelling followed by a brisk diastolic fall. Patients may endorse a history of syncope or lightheadedness associated with an inability to maintain forward flow through the aortic valve and the significant difference between systolic and diastolic pressure. Decreasing exercise tolerance and the inability to perform activities of daily living should prompt screening for this condition.

Aortic Stenosis -- History and Physical

The acquired aortic stenosis manifests with exertional dyspnea, syncope, angina, and, ultimately, heart failure. [17] [18] Typically, symptoms begin at the age of 50 to 70 years in patients with the bicuspid aortic valve and in greater than 70 years in patients with tri-leaflet valve calcific stenosis. Patients progressively experience a gradual decrease in exercise tolerance, dyspnea on exertion, and fatigue. Severe exertional dyspnea, paroxysmal nocturnal dyspnea, orthopnea, and pulmonary edema show various degrees of pulmonary venous hypertension. Angina results from the combination of the need for increased oxygen in hypertrophied myocardium and reduction of oxygen delivery secondary to the excessive compression of coronary vessels. Syncope is caused by the decrease in cerebral perfusion occurring during exertion when the arterial pressure declines due to systemic vasodilation and an inadequate increase in cardiac output related to stenosis. It is also due to the malfunction of the baroreceptor mechanism in severe aortic stenosis. Non-cardiac symptoms include gastrointestinal (GI) bleeding and cerebral emboli. GI bleeding is observed in patients with severe aortic stenosis and is often associated with angiodysplasia or other vascular malformations. It manifests from shear stress-induced platelet aggregation and reduction in the von Willebrand factor. [19] Cerebral emboli occur due to microthrombi formation on thickened bicuspid valves. There is also an observed increase in the risk of infective endocarditis in patients with aortic valve disease, especially with a bicuspid valve. On examination, carotid upstroke can be observed on palpation. A slow-rising, late-peaking, and a low-amplitude carotid impulse, pulsus parvus et tardus, is an expected finding in severe aortic stenosis and, when present, is specific to aortic stenosis. On auscultation, the second heart sound may lack a split and can be heard as a single sound during inspiration. It can also become paradoxical when the closure of the aortic valve gets delayed than the pulmonic valve. A mid-systolic ejection murmur, heard best over the right second intercostal space, with radiation into the right neck. However, high-frequency components may radiate to the apex in calcified aortic valves, and this phenomenon is called the Gallavardin phenomenon. The murmur becomes softer in LV failure and when there is a fall of stroke volume.

Twenty-three year-old with pleuritic chest pain.

A 23-year-old woman followed at another medical centre for congenital heart disease (CHD) presented to our emergency clinic with 3 weeks of bilateral pleuritic chest pain. She returned from holiday in Greece 6 weeks earlier where a tattoo and nasal piercing had been performed. There was no history of night sweats or fever.Her temperature was 37.5°C, heart rate 120 beats/min, oxygen saturations 94% on room air and blood pressure 110/74. Her chest was clear and there was systolic murmur on auscultation. The chest radiograph showed peripheral bilateral lower zone atelectasis. The ECG demonstrated sinus tachycardia. The haemoglobin was 11.2 g/dL, white cell count 10.18×10<sup9</sup/L, C-reactive protein 67 mg/L (normal <5 mg/L) and D dimer=430 ng/mL (normal <230 ng/mL).A pulmonary embolus was suspected and a CT pulmonary angiogram was performed (figure 1). Based on the CT findings, what is the most likely underlying congenital heart lesion in this patient?Bicuspid aortic valveCoarctation of the aortaFontan circulationParachute mitral valveVentricular septal defect heartjnl;105/6/464/F1F1F1Figure 1CT pulmonary angiogram (coronal views).

Cardiac examination

Finally the sacrum and ankles are checked for pitting edema which is caused by right ventricular failure in isolation or as part of congestive cardiac failure. Auscultation One should comment on S1 and S2 – if the splitting is abnormal or louder than usual. S3 – the emphasis and timing of the syllables in the word Kentucky is similar to the pattern of sounds in a precordial S3. S4 – the emphasis and timing of the syllables in the word Tennessee is similar to the pattern of sounds in a precordial S4. If S4 S1 S2 S3 Also known as a gallop rhythm. diastolic murmurs (e.g. aortic regurgitation, mitral stenosis) systolic murmurs (e.g. aortic stenosis, mitral regurgitation) pericardial rub (suggestive of pericarditis) The base of the lungs should be auscultated for signs of pulmonary oedema due to a cardiac cause such as bilateral basal crepitations.

InternalMed_Harrison

Death in patients with severe AS occurs most commonly in the seventh and eighth decades. Based on data obtained at postmortem examination in patients before surgical treatment became widely available, the average time to death after the onset of various symptoms was as follows: angina pectoris, 3 years; syncope, 3 years; dyspnea, 2 years; congestive heart failure, 1.5–2 years. Moreover, in >80% of patients who died with AS, symptoms had existed for <4 years. Among adults dying with valvular AS, sudden death, which presumably resulted from an arrhythmia, occurred in 10–20%; however, most sudden deaths occurred in patients who had previously been symptomatic. Sudden death as the first manifestation of severe AS is very uncommon (<1% per year) in asymptomatic adult patients. Calcific AS is a progressive disease, with an annual reduction in valve area averaging 0.1 cm2 and annual increases in the peak jet velocity and mean valve gradient averaging 0.3 meters/s and 7 mmHg, respectively

[Silent aortic insufficiency mimicking dilated cardiomyopathy].

The authors report 6 cases of severe and silent aortic insufficiency having simulated in all aspects the picture of a dilated cardiomyopathy at the stage of cardiac insufficiency with primary manifestations. They insist on signs leading to the diagnosis of this clinical entity: past history of rheumatism, signs of electrical left ventricular hypertrophy in the absence of arterial hypertension, aortic calcifications and mostly presence of a discrete mitral diastolic fluttering during echocardiographic examination. Supra-sigmoid aortic angiography confirms the diagnosis of severe aortic regurgitation. In order to explain the non-perception of the murmur, they invoke the alteration of the transmission secondary to an air cushion (3 cases the association of another valvulopathy with murmur (1 case) and mainly the decrease of the leakage by increase of the left ventricular telediastolic pressure and the decrease of the diastolic aortic pressure, with diminution of the turbulences. The advantage of knowing this entity rests on the possibility of valve replacement, even at the stage of myocardial failure, since the satisfactory long-term post-operative evolution in 4 patients stands in contrast with the dangerous nature of a spontaneous evolution.

For aortic stenosis, a systolic ejection murmur can often be heard best over the right sternal border at the second intercostal space. This murmur will peak early during systole when the disease is mild, and as severity increases will peak later, it also tends to radiate to the carotid arteries along with a slowly rising carotid upstroke. Auscultation often reveals a sustained apical impulse as well. In severe disease states, a thrill can sometimes be palpated over the carotid arteries and aortic area. Additional physical exam findings due to complications and sequelae of aortic stenosis include those associated with heart failure and left ventricle remodeling, such as third and fourth heart sounds, pulmonary crackles, jugular venous distention, and pedal edema.

First_Aid_Step1

Auscultation of the heart (eg, physiologic murmur) Aortic valve sclerosis Where to listen: APT M 111234567777777 Left sternal border: Aortic regurgitation Pulmonic regurgitation cardiomyopathy PA M T Aortic area: Systolic murmur Pulmonic area: Tricuspid area: Mitral area (apex): Crescendo-decrescendo systolic ejection murmur and soft S2 (ejection click may be present). LV >> aortic pressure during systole. Loudest at heart base; radiates to carotids. “Pulsus parvus et tardus”—pulses are weak with a delayed peak. Can lead to Syncope, Angina, and Dyspnea on exertion (SAD). Most commonly due to age-related calcification in older patients (> 60 years old) or in younger patients with early-onset calcification of bicuspid aortic valve.

InternalMed_Harrison

Palpation may reveal cardiac enlargement and abnormal contraction of the cardiac impulse (left ventricular dyskinesia). Auscultation can uncover arterial bruits, a third and/or fourth heart sound, and, if acute ischemia or previous infarction has impaired papillary muscle function, an apical systolic murmur due to mitral regurgitation. These auscultatory signs are best appreciated with the patient in the left lateral decubitus position. Aortic stenosis, aortic regurgitation (Chap. 283), pulmonary hypertension (Chap. 304), and hypertrophic cardiomyopathy (Chap. 287) must be excluded, since these disorders may cause angina in the absence of coronary atherosclerosis. Examination during an anginal attack is useful, since ischemia can cause transient left ventricular failure with the appearance of a third 1582 and/or fourth heart sound, a dyskinetic cardiac apex, mitral regurgitation, and even pulmonary edema. Tenderness of the chest wall, localization of the discomfort with a single

Exertional dyspnoea in a 28-year-old woman.

A 28-year-old woman with a history of critical pulmonic stenosis, status postsurgical valvotomy and subsequent pulmonary valve replacement, presented to the cardiology clinic with 1 year of progressive exertional dyspnoea. She has a heart rate of 75 bpm and blood pressure of 110/55 mm Hg. Cardiac auscultation reveals a 1/6 systolic ejection murmur along the left sternum and an early 3/6 diastolic decrescendo murmur. A transthoracic echocardiogram is obtained (figure 1). Which of the following would be most likely found during right heart catheterisation?Ratio of pulmonary to systemic blood flow (Qp:Qs) >1.5Pulmonary vascular resistance >3 Wood unitsRight atrial pressure >10mm HgPulmonary artery systolic pressure >45mm Hg E. Pulmonary artery diastolic pressure <10mm Hg.

ECG Case of the Month: Heart Failure in a Man from Chile.

A 49-year-old man visiting New Orleans from Chile comes to the hospital complaining of exertional dyspnea for 2 months with the more recent onset of ankle edema. He is a slender man with a blood pressure of 91/60 mmHg, crackles at both lung bases, and markedly distended neck veins. His cardiac rhythm is irregular. A soft murmur of mitral regurgitation and a soft S-3 are heard at the left ventricular apex. All four pedal pulses are easily palpable. Epigastric tenderness is noted on palpation; although the patient has a history of peptic ulcer disease, the tenderness is probably due to an enlarged left lobe of his liver, the result of hepatic congestion from his heart failure. A chest radiograph shows generalized cardiomegaly with an especially large left ventricle. Pulmonary congestion and small bilateral pleural effusions also are noted. The Figure shows the electrocardiogram recorded on admission.

Aortic Regurgitation -- History and Physical -- Physical Examination

AR is associated with widened pulse pressure as a result of systolic hypertension and decreased diastolic pressure. The apical LV impulse is hyperdynamic and displaced laterally and inferiorly. A prominent systolic thrill may be palpable at the base of the heart or suprasternal notch and over the carotid arteries. It is caused by the large forward stroke volumes and low aortic diastolic pressure. S1 is normal, but S2 is increased (with a dilated aortic root) or decreased (when the aortic leaflets are thickened). A high frequency, blowing, decrescendo, diastolic murmur is heard best third intercostal space along the left sternal border. It is easier to appreciate the murmur of AR at the end of expiration while the patient is leaning forward.  The murmur increases with squatting or isometric exercise and decreases with maneuvers that decrease blood pressure. This murmur is early diastolic with mild AR and becomes holodiastolic with severe AR.

Cardiogenic Pulmonary Edema -- History and Physical -- Physical Examination

Cardiovascular Findings Tachycardia and hypotension may be present along with jugular venous distention. Auscultation of the heart helps to differentiate between the various causes of valvular lesions causing pulmonary edema. Auscultation typically reveals an S3 gallop in volume overload states, which may be associated with accentuation of the pulmonic component of S2. Several different types of murmurs can be heard depending on the cause of the valvular lesion. Mitral stenosis produces a low-pitched, rumbling diastolic murmur associated with an opening snap at the apex, which becomes accentuated on expiration and produces loud S1. Mitral regurgitation produces a blowing, high-pitched pan-systolic murmur best heard at the apex, radiating to the left axilla and accentuating on expiration, producing soft S1. Aortic stenosis produces a harsh crescendo-decrescendo ejection systolic murmur at the aortic area, increasing on expiration, usually radiating towards the right side of the neck. Aortic regurgitation produces a high-pitched blowing early diastolic murmur best heard in the aortic area, greatest during expiration.

Supravalvular aortic stenosis

Pathophysiology Supravalvular aortic stenosis is due to diffuse or discrete narrowing of ascending aorta. The murmur associated with it is systolic murmur and is similar in character to valvular aortic stenosis murmur but commonly present at 1st Intercostal space (ICS) on the right. Individuals with this anomaly may have unequal carotid pulses, differential blood pressure in upper extremities and a palpable thrill in Suprasternal notch. Individuals with significant supravalvular AS chronically may develop left ventricular hypertrophy and also are at risk of developing coronary artery stenosis. With increased metabolic demands (e.g. exercise) such individuals may develop subendocardial or myocardial ischemia due to increased myocardial oxygen demand and seek medical help with symptoms of exercise induced angina. Diagnosis Treatment References Diseases of the aorta

Chest pain in a young woman.

Chest pain in a young person is often caused by chest wall tenderness, associated with mitral valve prolapse, or attributed to psychologic factors. Ischemic cardiac pain may be overlooked because of its rare occurrence in this age group. A 35-year-old woman complained of substernal chest pressure precipitated by exertion and relieved by rest. The symptom had been noted for 15 years. Worsening of the symptom during dancing prompted her to seek medical advice. She had no other illnesses, was taking no medications, was a nonsmoker, and had no family history of coronary disease. Physical examination disclosed a grade 1 (on the basis of 1 to 6) systolic ejection murmur, an ejection click, and a grade 2 diastolic murmur. An exercise test produced symptoms at 4 minutes. Coronary arteriography showed the absence of a left coronary ostium and filling of the entire coronary system from the right ostial injection through collateral vessels from the right coronary artery. Surgical repair was recommended. Operative intervention showed a dysplastic bicuspid aortic valve with a membrane that covered the left coronary ostium. Excision of the membrane reestablished antegrade blood flow to the left coronary system. A follow-up exercise test revealed normal findings. Because chest pain in a young person is rarely ischemic in origin, benign or noncardiac causes are usually considered; however, if the history suggests ischemic pain, the possible presence of unusual cardiovascular abnormalities should not be disregarded.

List of MeSH codes (C14)

– heart failure, congestive – cardiomyopathy, dilated – dyspnea, paroxysmal – edema, cardiac – heart neoplasms – heart rupture – heart rupture, post-infarction – ventricular septal rupture – heart valve diseases – aortic valve insufficiency – aortic valve stenosis – aortic stenosis, supravalvular – williams syndrome – aortic stenosis, subvalvular – cardiomyopathy, hypertrophic – discrete subaortic stenosis – heart murmurs – heart valve prolapse – aortic valve prolapse – mitral valve prolapse – tricuspid valve prolapse – mitral valve insufficiency – mitral valve stenosis – pulmonary atresia – pulmonary valve insufficiency – pulmonary valve stenosis – leopard syndrome – pulmonary subvalvular stenosis – tricuspid atresia – tricuspid valve insufficiency – tricuspid valve stenosis

InternalMed_Harrison

A medical professional should be present throughout the exercise test. It is important to measure total duration of exercise, the times to the onset of ischemic ST-segment change and chest discomfort, the external work performed (generally expressed as the stage of exercise), and the internal cardiac work performed, i.e., by the heart rate–blood pressure product. The depth of the ST-segment depression and the time needed for recovery of these ECG changes are also important. Because the risks of exercise testing are small but real—estimated at one fatality and two nonfatal complications per 10,000 tests—equipment for resuscitation should be available. Modified (heart rate–limited rather than symptom-limited) exercise tests can be performed safely in patients as early as 6 days after uncomplicated myocardial infarction (Table 293-2). Contraindications to exercise stress testing include rest angina within 48 h, unstable rhythm, severe aortic stenosis, acute myocarditis, uncontrolled

Heart murmur

Timing refers to whether the murmur is a systolic or diastolic murmur. Shape refers to the intensity over time; murmurs can be crescendo, decrescendo or crescendo-decrescendo. Crescendo murmurs progressively increase in intensity. Decrescendo murmurs progressively decrease in intensity. With crescendo–decrescendo murmurs (diamond- or kite-shaped murmurs), a progressive increase in intensity is followed by a progressive decrease in intensity. Location refers to where the heart murmur is usually heard best. There are four places on the anterior chest wall to listen for heart murmurs; each of the locations roughly corresponds to a specific part of the heart and should be listened to (through the stethoscope) with the patient lying down, face up. The four locations are: Aortic region - the 2nd right intercostal space. Pulmonic region - the 2nd left intercostal spaces. Tricuspid region - the 4th left intercostal space.

Mitral Valve Insufficiency -- History and Physical

The majority of patients suffering from chronic MR are asymptomatic, but serial quantitative assessment and follow-up of a patient with asymptomatic MR have shown a significant impact on the survival and clinical outcome. [28] Due to the insidious and slowly progressive nature of chronic MR, patients may not report any specific symptoms and may not notice a progressive decline in functional status during the early phase of the disease by gradual modification of their activities. Bedside evaluation for valvular heart disease is very important as management highly depends on symptoms as well as the severity of valvular lesions. Patients with MR are usually referred due to symptoms of chest pain, shortness of breath, palpitations, leg swelling, or just incidental finding of systolic murmur on physical examination. A thorough history of prior bacterial or viral infections, rheumatic fever, trauma, ischemic events, invasive cardiac/noncardiac procedures and family history with a comprehensive review of systems should be obtained. A detailed physical examination with particular attention to the cardiovascular system, including pulse, apical impulse, jugular veins, edema, signs of congestive heart failure, and assessment of characteristic systolic murmur with other pertinent findings should be documented. [29] [3] A characteristic holosystolic murmur present at the apex and radiating to the left axilla can be present most of the time except in cases of MVP where the murmur appears in mid-late systole with or without midsystolic click. The murmur should not be confused with other systolic murmurs such as aortic stenosis, tricuspid regurgitation, pulmonic stenosis, ventricular septal defect, benign flow murmurs, and other cardiac disorders in the spectrum. [30]

Aortic Valvular Atresia -- History and Physical

On physical examination, a precordial bulge can be present, though it is not a reliable indicator. Over 50% of patients, however, have a pulmonary systolic ejection murmur, the intensity of which can vary from grade 1 to grade 3. The murmurs can be heard clearest along the left sternal border. The second heart sound at the cardiac base is mostly single, though sometimes can have a very close split and rarely have an ejection click. Heart failure is quick to develop in about 60% of these patients, with the average age of development being 2.5 days. It is almost always associated with hepatomegaly, with about 3cm of protrusion beyond the right costal margin. An S3 heart sound can sometimes be heard, along with chest rales, edema, and palpable spleen. Blood pressure as well can be lower than average, hovering around 65/45 mmHg, and pulse pressure is generally narrow. No clinically significant pressure difference is noted between upper and lower extremity measurements. [5]

[Rapid onset aortic stenosis and clinical course in adults. Clinico-hemodynamic correlations].

In 8 patients aged 41-66 years, a second left heart catheterisation done 27-98 months after the first study, demonstrated a pressure gradient across the aortic valve, that had not previously existed, or had been trivial. No significant change of the cardiac output had occurred. All but 1 patient were hypertensive. The etiology was rheumatic in 4, degenerative in 4. Electrocardiographic, radiographic, and echocardiographic evolution could not separate the patients with a gradient greater than or equal to 70 mmHg from those whose gradient was less than or equal to 40 mmHg. The intensity of the aortic component of the second heart sound, however, decreased in all former patients, and in only 1 of the latter. Aortic valvular stenosis can arise and rapidly develop in adult patients. Concomitant rheumatic mitral valve disease, coronary artery disease and hypertension can mask and/or modify symptoms, signs and laboratory findings. Changes of the aortic component of the second sound may suggest its occurrence.

MYL4

Clinical significance MYL4 expression in ventricular myocardium has shown to abnormally persist in neonates up through adulthood in patients with the congenital heart disease, tetralogy of Fallot. Altered ALC-1 expression is also altered in other congenital heart diseases, Double outlet right ventricle and infundibular pulmonary stenosis. Moreover, in patients with aortic stenosis or aortic insufficiency, ALC-1 expression in left ventricles was elevated, and following valve replacement decreased to lower levels; ALC-1 expression also correlated with left ventricular systolic pressure. Additionally, in patients with ischemic cardiomyopathy, dilated cardiomyopathy and hypertrophic cardiomyopathy, ALC-1 protein expression is shown to be reactivated, and ALC-1 expression correlates with calcium sensitivity of myofilament proteins in skinned fiber preparations, as well as ventricular dP/dtmax and ejection fraction. Interactions ALC-1 interacts with: ACTC1 MYH7 References