pediatrics
Hypoplastic Left Heart Syndrome
Symptoms and Signs of Hypoplastic Left Heart Syndrome
Symptoms of hypoplastic left heart syndrome appear when the ductus arteriosus begins to close during the first 24 to 48 hours of life. Subsequently, the clinical picture of Cardiogenic and obstructive shock (eg, tachypnea, dyspnea, weak pulse, pallor, cyanosis, hypothermia, metabolic acidosis, lethargy, oliguria or anuria) rapidly develops. When systemic circulation is compromised, coronary and cerebral perfusion may be reduced, leading to symptoms of myocardial or cerebral ischemia. Perfusion of the kidneys, liver, and mesentery is also inadequate, and oliguria or anuria are common. If the ductus arteriosus is not reopened, death rapidly ensues. The patient often presents with a history of poor feeding, increased work of breathing, pale or gray coloration, and lethargy. Physical examination shows a very active precordium with a marked parasternal lift associated with very poor peripheral perfusion, cool extremities, bluish gray skin color, and absent or barely palpable pulses. The finding of a marked parasternal lift in a patient with critically low perfusion and near-absent peripheral pulses is a key to distinguishing cardiogenic shock from severe left heart obstruction and non-cardiac–related shock (eg, sepsis, metabolic, toxic). The second heart sound (S2) is loud and single. A soft, nonspecific systolic murmur is often present, as is hepatomegaly. Severe metabolic acidosis, often worsening if supplemental oxygen is administered, is characteristic of hypoplastic left heart syndrome. 