21 research outputs found
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