complex ventricular arrhythmia and death follows overt congestive heart failure ( chf ) , and it is one of the poor prognostic sign of acromegaly .
though prevalence of biventricular hypertrophy present in 90% of autopsied older and 20% of younger patients with acromegaly , presence of overt chf is less common . the estimated frequency of overt chf ranges from 1% to 4% in the literature
the literature on prevalence of overt chf and their outcomes is sparse and none from india till date and hence , this study was planned .
this is a retrospective analysis of hospital records of consecutive patients with acromegaly between 1996 and 2007 from the acromegaly registry of department of endocrinology , postgraduate institute of medical education and research , chandigarh , india .
records of the patients with the diagnosis of acromegaly and presence of overt heart failure were retrieved from the departmental acromegaly registry .
acromegaly was diagnosed based on elevated igf-1 compared to age and sex - matched normative data and/or failure to suppress growth hormone ( gh ) following 75 gm anhydrous glucose load in a patient with signs and symptoms suggestive of acromegaly .
these patients were treated as per the standard management of acromegaly including transnasal sphenoid surgery followed by medical and/or radiotherapy for persistent or recurred disease .
as per the departmental protocol and pre - operative assessment , all the patients with active acromegaly were subjected for ecg and echocardiography to rule out cardiomyopathy in accordance with standard guidelines .
patients diagnosed to have cardiomyopathy were followed up at regular interval at endocrine and cardiology clinics of the institute , and therapy for cardiomyopathy was decided by the cardiologist .
the diagnosis of overt congestive heart failure ( chf ) was based on clinical signs as described by framingham heart study group and the new york heart association ( nyha ) classification and echocardiography showing a left ventricular ejection fraction ( lvef ) of  45% .
detailed history , hormonal and radiological investigations and cardiovascular procedures , treatment modalities , and outcomes were recorded separately and analyzed .
m - mode , two - dimensional and pulsed doppler echocardiography were done by commercial ultrasound system using 2.5  mhz transducer in three to five consecutive cardiac cycles .
left ventricular end diastolic diameter end systolic diameter , interventricular septal thickness , left ventricular posterior wall thickness , and left ventricular ejection fraction ( lvef ) were recorded .
decision for cardiac catheterization and coronary angiography with left ventricular angiography were taken by the opinion of the cardiologist , and their data were analyzed by them ( rbv and ksr ) .
this is a retrospective analysis of hospital records of consecutive patients with acromegaly between 1996 and 2007 from the acromegaly registry of department of endocrinology , postgraduate institute of medical education and research , chandigarh , india .
records of the patients with the diagnosis of acromegaly and presence of overt heart failure were retrieved from the departmental acromegaly registry .
acromegaly was diagnosed based on elevated igf-1 compared to age and sex - matched normative data and/or failure to suppress growth hormone ( gh ) following 75 gm anhydrous glucose load in a patient with signs and symptoms suggestive of acromegaly .
these patients were treated as per the standard management of acromegaly including transnasal sphenoid surgery followed by medical and/or radiotherapy for persistent or recurred disease . as per the departmental protocol and pre - operative assessment ,
all the patients with active acromegaly were subjected for ecg and echocardiography to rule out cardiomyopathy in accordance with standard guidelines .
patients diagnosed to have cardiomyopathy were followed up at regular interval at endocrine and cardiology clinics of the institute , and therapy for cardiomyopathy was decided by the cardiologist .
the diagnosis of overt congestive heart failure ( chf ) was based on clinical signs as described by framingham heart study group and the new york heart association ( nyha ) classification and echocardiography showing a left ventricular ejection fraction ( lvef ) of  45% .
detailed history , hormonal and radiological investigations and cardiovascular procedures , treatment modalities , and outcomes were recorded separately and analyzed .
m - mode , two - dimensional and pulsed doppler echocardiography were done by commercial ultrasound system using 2.5  mhz transducer in three to five consecutive cardiac cycles .
left ventricular end diastolic diameter end systolic diameter , interventricular septal thickness , left ventricular posterior wall thickness , and left ventricular ejection fraction ( lvef ) were recorded .
decision for cardiac catheterization and coronary angiography with left ventricular angiography were taken by the opinion of the cardiologist , and their data were analyzed by them ( rbv and ksr ) .
a total of 150 patients were diagnosed to have acromegaly during the period of 11 years . of these
, 20 had evidences for left ventricular hypertrophy and/or left ventricular diastolic dysfunction ( 13.3% ) , 1 had lv systolic dysfunction due to previous coronary artery disease , and 6 patients had evidence of overt congestive heart failure ( 4% ) . out of 6 patients , in 4 patients , congestive heart failure ( chf ) was presenting manifestation of acromegaly and 2 developed chf during the course of the illness .
there were 5 males and 1 female with the mean age of 41  17.3 years ( range 24 - 50 years ) .
mean gh level following oral glucose load was 41  17.5 ng / dl ( range 12 - 64 ng / dl ) .
three patients were hypothyroid , and 2 were hypocortisolic at the time of diagnosis and were on replacement therapy .
mri showed macroadenoma ( size > 1 cm ) with evidence of apoplexy in 1 case ( case 3 ) , and 1 developed apoplexy during hospital admission ( case 4 ) . in 1 patient , pituitary was not separately visualized due to fibrous dysplasia involving sphenoid sinus ( case 5 ) .
four patients had chf at the time of presentation and 2 developed it during course of illness ( case- 2 and 6 ) .
four patients had nyha class iv dyspnea while 2 had class iii , left ventricular ejection fraction was 30 or less with echocardiographical features suggestive of global biventricular hypokinesia in all patients .
two patients had history of hypertension and controlled with medications , and 1 had diabetes who was on high dose of insulin ( 120 units / day ) to control his blood glucose .
data on coronary angiography was available in 3 cases , which showed dilated left ventricles with normal coronary arteries in all these cases [ figure 1 ] and confirmed by autopsy in 1 patient .
out of these 6 patients , the patient with fibrous dysplasia lost to follow up , 1 patient died before planned surgery , and 2 underwent transsphenoid surgery for removal of pituitary macroadenoma .
one patient , already underwent transfrontal surgery for pituitary macroadenoma 6 year back , had recurrence of disease and was presented to chf after 6 years , he was given somatostatin analogues in interim period and after stabilization of general condition , he underwent transphenoidal surgery ( case 2 ) .
another patient underwent tss 1 year back before he presented with chf , and he died before investigation and management was contemplated .
of 5 cases available for outcome analysis , only 1 patient is alive ( case 3 ) .
he was young with short duration of acromegaly and treated timely and did not have other risk factors like hypertension and diabetes , furthermore , his gh levels were lowest compared to other 5 patients after 75 gm oral glucose load . after trans - sphenoidal surgery , gh normalized , and at 3 month , left ventricular ejection fraction improved to 35% from 25% at baseline and furthermore , there was normalization of cardiac size on serial chest x - ray [ figure 2 ] .
three patients died of ventricular arrhythmias and 1 due to sepsis and csf over drainage from lumber drain .
one patient died of apoplexy followed by ventricular arrhythmia , and autopsy was performed in this case ( case 4 ) .
autopsy confirmed normal coronaries , severe concentric biventricular hypertrophy , myofibrillar necrosis , and interstitial fibrosis [ figure 3 ] .
hormonal , radiological , cardiovascular characteristics and outcomes of acromegaly patients with over congestive heart failure ( n=6 ) ( a ) coronary angiography showing normal epicardial coronaries and ( b ) left ventricular angiography showing dilated left ventricle ( a ) chest x - ray pa view showing cardiomegaly at the time of presentation ( b ) chest x ray pa view of same patient three months after transsphenoid surgery showing reduction of cardiac size ( a ) autopsy specimen of the heart showing the concentric left ventricular hypertrophy at apical slice .
( b ) low power photomicrograph of the left ventricle showing variation in the myofibre size with anisonucleosis and interstitial fibrosis .
( h and e , 20).(c ) low power photomicrograph of the heart in special stain to highlight the interstitial fibrosis , seen as bluish green .
we report 6 cases of overt congestive heart failure out of 150 cases of acromegaly ( 4% ) .
clinical symptoms and signs of heart failure were found in about 10% of newly diagnosed acromegaly , and they were characterized by normal systolic function implying high output failure .
however , the overt chf were reported as less than 1% in one study while another recent study estimated its prevalence as 4% .
our study finds prevalence of overt chf as 4% , which is in consonance with later study .
diabetes mellitus and systemic hypertension are independent risk for morbidity and mortality , and they aggravate the heart failure .
however , in present study , only 1 had diabetes and 2 had hypertension suggesting elevated gh is most important factor for overt chf .
normalization of gh and igf levels either with medical or surgical management have been shown to reverse the cardiac manifestation effectively and reduce morbidity and mortality .
somatostatin analogues normalize gh and igf-1 levels in 60 - 70% of cases and with short treatment of 1 - 3 months have been shown to reduce lv posterior and interventricular wall thickness , and more significant effect is achieved with long - term treatment .
we used somatostatin analogue in one patient to normalize gh and stabilization of chf before surgery .
biopsy and autopsy studies indicate that interstitial fibrosis is the main histological features that gradually impair architecture and function .
other histological features are increased extra cellular collagen deposition , myofibrillar derangement , and areas of lymphocytic infiltration gradually impairing whole architecture .
generally , coronary arteries remain uninvolved as is evident by normal epicardial coronaries in all our 3 patients who underwent angiography and 1 patient at autopsy .
echocardiography shows initial cardiac chamber enlargement followed by diastolic dysfunction and at the end , global hypokinesia with component of systolic dysfunction .
the cardiac function can be restored to normal with control of the disease . in all studies , diastolic dysfunction improved , but ejection fraction and exercise performance remained variable .
the prevalence of cardiac arrhythmias in acromegaly was reported between 3.7% in one study and 48% in another . in our study ,
the increased frequency is thought to be due to the abnormal remodeling of the ventricular wall .
this is the first study from india to examine prevalence of chf in large number of acromegaly patients with complete work up including angiography data and autopsy finding in one .
these are the strength of the study . however , retrospective nature and lack of comparative group are the limitations . in conclusion ,
overt chf is associated with high mortality and hence , this complication should be recognized at earliest and medical management to normalized cardiac function should be given utmost priority .
 background : though cardiac involvement is common in acromegaly , overt congestive heart failure is uncommon.materials and methods : this is retrospective analysis of hospital record between 1996 and 2007 . 
 we analyzed records of 150 consecutive patients with acromegaly . 
 we included the patients with acromegaly those who had overt congestive heart failure either at presentation or during the course of illness for the present analysis . 
 the diagnosis of acromegaly and congestive cardiac failure were based on standard criteria.results:out of 150 patients with acromegaly , 6 patients had overt chf ( 4.0% ) , of which 4 presented with the features of chf and 2 developed during the course of illness . 
 three patients had hypertension and 1 had diabetes . 
 baseline echocardiography showed severe biventricular dysfunction and global hypokinesia in all . 
 angiography showed dilated hypokinetic left ventricle with normal coronaries in 3 , it was confirmed at autopsy in 1 . 
 three underwent trans - sphenoidal surgery , 1 received somatostatin analogue as primary treatment modality . 
 normalization of growth hormone and igf-1 led to improvement in cardiac function in 1 , 1 patient lost to follow up , and 4 died during the course of illness . in 1 patient , 
 autopsy was performed and cardiac specimen revealed normal coronaries , concentric ventricular hypertrophy , and dilatation with myofibrolysis and interfascicular fibrosis.conclusion:prevalence of overt chf is 4% in present series . 
 overt chf carries poor prognosis and hence , this complication should be recognized at earliest , and medical management to normalized cardiac function should be given utmost priority . 
I
M
Study design
Patient selection
Cardiology assessment
R
D