endocarditis of the right side of the heart is uncommon by virtue of the low hemodynamic pressure and lack of isolated or significant right - sided valvular deformities .
the valves fall prey to an infective process in settings of congenital heart disease or endothelial injury .
pulmonary arterial endarteritis is a rare event , even in patients with congenital heart disease ( chd ) .
further , a combination of pulmonary endarteritis with a systemic suppuration in chd invites greater attention .
a 4-year - old male child , born from a non - consanguineous marriage , was admitted with the history of fever and progressive right - sided weakness for 1 week and cyanosis noted since 6 months of age .
the patient 's mother had noted dyspnea on feeding since 1 year of age . on admission ,
the patient had dyspnea on exertion ( grade iii / iv , ats grading ) .
the cardiac apex was at left 5 intercostal space , 3 cm outside the mid - clavicular line . an ejection systolic murmur ( grade iii / vi ) was audible in the pulmonary area . a right - sided complete hemiparesis with extensor plantar was noted .
an ecg revealed right atrial enlargement , extreme right axis , and poor progression of r wave .
chest x - ray revealed cardiomegaly with reduced pulmonary vascular markings [ figure 1 ] .
a full - segmental echocardiography revealed a single ventricle of the double - inlet left ventricle type [ figure 2 ] .
a rudimentary right ventricle was connected to the main ventricle by a non - restrictive foramen .
the aorta arising from the rudimentary chamber and pulmonary artery from the main chamber [ figure 3 ] .
a 7 mm  5 mm fixed structure on the wall of the main pulmonary artery with erratic movement , indicative of a vegetation , suggested pulmonary endarteritis [ figure 4a and b ] .
a small osteum - secundum atrial septal defect was present , but patent ductus arteriosus was not present .
ct scan of the brain revealed bilateral multiple cerebral abscesses [ figure 5 ] , with the largest one present superficially in the left parietal region .
chest x - ray showing cardiomegaly with decreased pulmonary flow echocardiography ( subcostal anatomically corrected view )  showing both mitral and tricuspid valves opening into a single ventricle of lv morphology .
a rudimentary rv type of ventricle is also visualized echocardiography ( subcostal anatomically corrected view )  showing aorta arising from a rudimentary right ventricle which is connected to the left ventricle through a nonrestrictive foramen ( a ) and ( b ) shows the pulmonary vegetation suggesting pulmonary endarteritis ct scan of the brain showing bilateral cerebral abscesses the child was started on parenteral ceftriaxone 
80 mg/ kg / day ( plus , gentamicin  3 mg / kg / day for 2 weeks ) for the endarteritis and was continued for 6 weeks for optimal treatment of the cerebral abscess .
an attempted drainage of the largest cerebral abscess resulted in some improvement of the hemiparesis .
the patient became afebrile in 2 weeks and was able to walk after 4 weeks of therapy .
repeat echo before discharge showed substantive decrease in size of the pulmonary artery vegetation and cardiac symptoms .
the patient has been referred to the cardiac surgery department for corrective surgery for single ventricle .
the risk of infective endocarditis is considerably increased in children with congenital heart disease , and the disease can occur with almost any lesion , more so with cyanotic congenital heart disease ( cchd ) .
right - sided infective endocarditis is rare by virtue of the low hemodynamic pressure and lack of isolated or significant right - sided valvar deformities . in adults
, right - sided infective endocarditis usually follows prolonged intravenous catheterization or intravenous drug - abuse , affecting the tricuspid valve alone or in combination with the pulmonary valve .
a similar valvar involvement also occurs in infants and in children , but here congenital cardiac anomalies with their accompanying interventions are important pre - disposing factors .
pulmonary endarteritis as an entity of right - sided endocarditis is rare , even in patients of congenital heart disease .
most of the reported pulmonary endarteritis cases have been associated with a patent ductus arteriosus ( pda ) .
there are less than 30 reported cases of pulmonary endarteritis associated with pda in the adult , and overall incidence has dramatically decreased over the last 30 years .
bilge et al . , in their case report of pulmonary endarteritis in pda , underlines the importance of echocardiography in not only making this rare diagnosis but also as an effective means of following up such a case .
apart from pda , a single report exists of pulmonary endarteritis occurring after anatomical correction of complete transposition of the great arteries . to the best of our knowledge and available resources , our case report of pulmonary endarteritis in a child of single ventricle is probably the first such so far .
the onset of pulmonary endarteritis and the formation of vegetation can be explained by several factors , including 
an obstacle of a valve or bloodstream jet and venturi effect , the formation of a platelet fibrin thrombus , transient bacteremia , and adhesion factor for infection . in the present case
, an untreated infection in the child could have led to multiple episodes of bacteremia .
the presence of multiple , bilateral cerebral abscesses is a corroborative evidence to the bacteremia .
the pulmonary artery in this case arose from the main ventricle ( morphological double - inlet lv ) and was hypoplastic .
turbulent blood flow distal to the stenotic lesion may increase the risk of developing infective endarteritis in an already hypoplastic pulmonary artery .
the intima is also damaged in these areas , predisposing to formation of platelet fibrin thrombus , and thus making the region more susceptible to colonization by pathogens .
congenital heart disease ( chd ) has a reported incidence of 9596/million live births ( 0.95% ) , out of which 14.5% ( 1391/million live births ) have cyanotic congenital heart disease ( cchd ) .
single ventricle ( 106/million live births ) accounts for 7.6% of cchds and 1.1% of all chds .
meticulous full - segmental echocardiographic approach is required to diagnose and delineate the specific type of single ventricle .
single ventricle is a complex cchd and is considered a high risk condition for an infective endocarditis .
trans - thoracic echocardiography ( tte ) is more sensitive in the pediatric population than in the adult population for detection of vegetation .
tte is more likely to identify vegetations in children with normal anatomy or isolated valvular pathology than in those with complex cchd .
the importance of a carefully - performed tte or trans - esophageal echocardiography is , therefore , essential in a complex cchd like single ventricle states .
cyanotic heart disease accounts for 12.8%-69.4% of all cases of brain abscesses , with the incidence being higher in children .
the incidence of brain abscess in patients with cyanotic heart disease has been reported to range between 5 and 18.7% . in our case ,
bacteremia from an unknown source could have bypassed the pulmonary circulation by entering the double - inlet lv ( the dominant ventricle ) and moved through the aorta and seeded in the brain .
patients with cyanotic heart disease could have low - perfusion areas in the brain due to chronic severe hypoxemia and metabolic acidosis as well as increased viscosity of blood due to secondary polycythemia .
these low - perfusion areas commonly occur in the junction of gray and white matter , and they are prone to seeding by microorganisms that may be present in the bloodstream . the advent of ct scans and their use in the management of these abscesses has resulted in a fourfold decrease in the mortality rate in patients with brain abscesses , secondary to cyanotic heart disease - from 40%-60% in the pre - ct era to ~10% . the threshold for ct scans in a cchd should be low , and regular ct scans should be obtained to monitor the size of the abscess . in conclusion , this case report aims to highlight the considerable and varied morbidity associated with cyanotic congenital heart diseases ( cchd ) . the presence of a relatively common infectious complication ( cerebral abscesses ) and a rare infectious complication ( pulmonary endarteritis ) in the same child with a cchd ( single ventricle ) validates the need for a heightened clinical awareness and a prompt and meticulous investigational approach for diagnosis and therapy in this group of patients .
 endocarditis of the right side of the heart is otherwise uncommon in children . 
 pulmonary endarteritis as a complication of congenital heart disease is even rarer . 
 herein , we report the case of pulmonary endarteritis with a 7 mm 5 mm vegetation , involving the main pulmonary artery in a 4-year - old male child , with cyanosis and a 1-week history of fever and rapidly - progressive hemiparesis . a full segmental echocardiography demonstrated a double inlet left ventricle with left - sided subaortic 
 hypoplastic right ventricle ( van praagh 's a - iii type  
 single ventricle ) . additionally , ct scan of the brain revealed bilateral cerebral abscesses . to the best of our knowledge , 
 the occurrence of pulmonary endarteritis and cerebral abscesses in a case of single ventricle is hitherto unreported . 
 this article underlines the importance of heightened clinical awareness and meticulous echocardiography in cases of congenital heart disease so that relatively rare complications may not be missed . 
INTRODUCTION
CASE REPORT
DISCUSSION