pediatrics
Hypoplastic Left Heart Syndrome
Diagnosis of Hypoplastic Left Heart Syndrome
Chest x-ray and ECG Echocardiography Diagnosis of hypoplastic left heart syndrome is suspected clinically, particularly in neonates with Metabolic Acidosis that worsens after receiving oxygen; oxygen lowers pulmonary vascular resistance and thus increases the relative proportion of the right ventricle output that flows to the lungs rather than through the patent ductus arteriosus to the body. Diagnosis is confirmed by emergency echocardiography. Metabolic acidosis that worsens when supplemental oxygen is administered is characteristic of hypoplastic left heart syndrome. Cardiac catheterization is rarely required for diagnosis. Chest x-ray shows cardiomegaly and pulmonary venous congestion or pulmonary edema. ECG shows right ventricular hypertrophy and diminished left ventricular forces, though it may be within normal limits for a neonate. 