Isolated Biventricular Noncompaction in an adult with severe pulmonary hypertension : an association reviewed.

Biventricular noncompaction is a recently recognized rare form of cardiomyopathy. It is characterized by altered structure of myocardial wall as a result of intrauterine arrest of compaction of the myocardial fibers in absence of coexisting congenital lesion. Left ventricle is the most affected site for noncompaction, but right ventricular involvement has been reported in a few cases. Diagnosis is made with 2-dimensional echocardiography or cardiac magnetic resonance imaging. While major clinical manifestations are heart failure, arrhythmias and embolic events,pulmonary artery hypertension ( PAH)has not been well elaborated in the literature. We present a 13- year old boy who had Biventricular noncompaction complicated by severe pulmonary hypertension. Pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic left ventricular dysfunction secondary to noncompaction. This article reviews the literature particularly with reference to PAH in the context of this case.peer-reviewe

Symptomatic giant left atrial aneurysm in a child : a rare entity

Isolated left atrial aneurysms are rare entities in clinical practice. Usually the condition is diagnosed in the second to fourth decades of life. The presence of such lesions in the pediatric age group is scantily described. We present a 2 year boy who presented with complaints of excessive irritability, respiratory distress and swelling of the feet. On examination, child was tachypnoeic with irregularly irregular rhythm. Echo showed a huge aneurysmal LA appendage with severe left ventricle dysfunction. The child underwent surgical resection for same. Findings were confirmed intraoperatively but he continued to have low cardiac output state after the surgery, with frequent arrhythmias and expired on day 7 of surgery. The case is reviewed and compared with the available English literature.peer-reviewe

Submitral aneurysm: An antenatal diagnosis

We describe the echocardiographic features of submitral aneurysm in a 26 week old fetus. The diagnosis was confirmed on neonatal echocardiography

Double orifice tricuspid valve in an infant with partial atrioventricular septal defect

Double orifice Tricuspid Valve is an extremely rare malformation with few case reports. We report such a case in an infant associated with atrioventricular septal defect

Giant coronary cameral fistula with coarctation of aorta in a neonate

A coronary cameral fistula (CCF) involves a sizable communication between a coronary artery and a cardiac chamber. We present a case of giant coronary cameral fistula associated with coarctation of aorta in a 13 days old neonate

Calvarial tuberculosis: Review of six cases

Calvarial tuberculosis is a very rare entity scantily described in literature. It forms 0.2-1.3% of the bone tuberculosis. This is a rare entity even in the endemic areas. Primary calvarial tuberculosis with no evidence of tuberculosis is still rarer of these cases. We describe six cases of calvarial tuberculosis. The relevant clinicoradiological features and management are discussed. A high index of suspicion and awareness of this condition may lead to early diagnosis