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

Dual communication of confluence of total anomalous pulmonary venous connection: An interesting but a rare entity

Here, we describe case of a 9-month-old male child who was diagnosed with a very unusual and rare variety of mixed total anomalous pulmonary venous connection

Ductal aneurysm masquerading as nonresolving pneumonia: A challenging differential!

We report here, the case of a six-and-a-half-month-old boy investigated for persistent respiratory distress and homogeneous opacity in the left upper lobe. Echocardiography revealed a giant ductal aneurysm compressing the left pulmonary artery and upper lobe division of the left bronchus. Computerized tomography angiogram delineated the exact anatomy and prompt surgical resection provided a successful cure to this lesser known entity

Constriction of juxta-ductal aorta and rapid progression of obstruction in a newborn

A 13-day-old baby girl presenting with features of congestive cardiac failure was found to have coarctation of the aorta (CoA) and patent ductus arteriosus (PDA) by echocardiography. Doppler spectral display revealed moderate CoA. Echocardiogram, 12 hours later, showed severe juxtaductal aortic coarctation with spontaneous closure of PDA. This case emphasises the need to keep a close watch on the progress of CoA in the neonatal period, even if the duct has narrowed to a small size thus demonstrating the role of constriction of juxtaductal aorta in pathogenesis of coaractation. Closure of even asmall PDA can cause acute progression CoA in the presence of posterior shelf

Hypocalcemic cardiomyopathy presenting as cardiogenic shock

Hypocalcemia is a less known but treatable cause for dilated cardiomyopathy, leading to severe heart failure in children. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. We describe 5 infants presenting with cardiogenic shock over 3 years, who were found to have severe hypocalcemia as a sole cause of myocardial dysfunction. The patients responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized within months of treatment. In any case of cardiogenic shock, hypocalcemia should be included in the differential diagnosis and must be investigated