Background - Hypoplastic Left Heart Syndrome consists of a spectrum of congenital defects including hypoplasia or atresia of the aortic and mitral valves and hypoplasia of the left ventricle and ascending aorta. Hypoplastic Left Heart Syndrome is a rare congenital heart disease and has been notorious in causing neonatal fatality until timely diagnostic methods and palliative surgical measures were developed. Echocardiography has been the mainstay diagnostic modality in evaluating the intricacies of Hypoplastic Left Heart Syndrome. We present a case of Hypoplastic Left Heart Syndrome to underscore the value of non-invasive Cardiac Computed Tomography Angiography in the diagnosis and in resolving clinical disparities in cases of complicated congenital heart disease.
Case - The patient is a 23 day old female who presented with dyspnea and on physical examination had S1 N, S2 single and a Grade 3/6 pan systolic murmur at the left mid sternal border. Echocardiography was done with an initial interpretation of Congenital Heart Disease, Truncus Arteriosus type I, Atrial Septal Defect secundum, Patent Ductus Arteriosus, Interrupted aortic arch type C. Her abating course in the ward prompted for further evaluation; thus, cardiac computed tomography angiography was done with findings consistent with Hypoplastic Left Heart Syndrome.
Conclusion - Hypoplastic left heart syndrome is one of the rare congenital heart diseases that has been known to cause high fatality. It necessitates prompt diagnosis to plan the suitable surgical procedure. The importance of Cardiac Computed Tomography Angiography in resolving complex congenital heart diseases such as hypoplastic left heart syndrome is underscored in this case report.