Hypoplastic Left Heart Syndrome: A Case Report

Abstract

Hypoplastic left heart syndrome is a rare critical congenital heart defect constituting a prevalence of 1/3,500 to 1/12,500 live births in which there is obstructions to the blood flow within the left heart. Here we present a case of a full-term 38 weeks male baby with a birth weight of 3.5 kg, and no obvious physical deformity referred to our centre at 22 hours of life for respiratory distress and cyanosis. At 23 hours of life, he was diagnosed with hypoplastic left heart syndrome and kept on Prostaglandin E1 infusion till the 12th day of life. The baby had a large ventricular septal defect and atrial septal defect with a severely hypoplastic left ventricle, hypoplastic aortic root, ascending aorta and post-ductal coarctation of the aorta. The diagnosis was reconfirmed by computed tomography cardiac angiography on the 10th day of life with the same cardiac findings suggestive of hypoplastic left heart syndrome.

An Uncommon Finding in an Adult: A Case Report of Hypoplastic Left Heart Syndrome and Protein-Losing Enteropathy

“…PLE is a rare cause of hypoalbuminemia. It is usually caused by the loss of proteins from the GI mucosa through abnormal venous or lymphatic flow [ 1 ]. Patients who underwent the Fontan procedure have high central venous pressure, leading to enteric congestion and loss of proteins through the bowel [ 4 ].…”

“…Hypoplastic left heart syndrome (HLHS) is a rare and serious congenital heart defect that affects approximately 1 in 3,500 to 1 in 12,500 live births [ 1 ]. This condition involves impaired blood flow to the heart due to an underdeveloped left heart that precludes reconstructive surgery (Norwood, Glenn, and Fontan surgery).…”

“…Protein-losing enteropathy (PLE) involves the abnormal loss of proteins through the GI tract, a phenomenon attributed to various underlying causes. Individuals exhibiting low serum protein levels, after excluding alternative causes of hypoproteinemia, should be considered potential PLE suspects, according to the available medical literature [ 1 - 3 ]. In this case report, we discuss the connection between HLHS and PLE.…”