Fig. 55.1
Diagrammatic representation of HLHS. On the left is aortic valve atresia and on the right is mitral valve atresia. Note the hypoplastic left ventricle in both cases. Abbreviations: SVC superior vena cava, IVC inferior vena cava, RA right atrium, RV right ventricle, PT pulmonary trunk (artery), RPA right pulmonary artery, LPA left pulmonary artery, LA left atrium, LPV left pulmonary vein, LV hypoplastic left ventricle, A aorta (Diagram the courtesy of Patrick O’Leary, MD, Mayo Clinic College of Medicine)
55.2 Pathologic Physiology
At birth, systemic blood flow is supplied by the right ventricule pumping blood into the pulmonary artery and then both to the lungs and, via a patent ductus arteriosus, to the aorta. When the ductus closes, significant organ ischemia and death occurs. If the ductus does not close significantly, pulmonary edema occurs leading to death. There are very rare reports of patients surviving to childhood because of the development of early pulmonary vascular obstructive disease that limits pulmonary blood flow and pulmonary edema.
55.3 Clinical Presentation
Most infants with HLHS will present with cyanosis. Occasionally, the cyanosis will be mild and missed. These infants then present at 3–7 days of age in shock when the ductus arteriosus closes.
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