Hypoplastic Left Heart Syndrome

• • An obstructive congenital heart lesion

  • Spectrum of underdevelopment of left-sided structures (mitral and aortic valves, left ventricle, ascending aorta, and arch)

  • Commonly associated with the following:

  • -Mitral and aortic valve atresia

    -LV hypoplasia

    -Ascending aorta measures 2-3.5 mm

  • Coronary arteries may be stenotic

  • Other common anomalies:

  • -Ventricular septal defect

    -Double-outlet right ventricle

  • 25% may have CNS anomalites

  • Uniformly fatal without operative treatment

  • Entire cardiac output delivered from right heart, maintaining oxygenation through pulmonary flow and systemic oxygen delivery

  • Ductus closure/inadequate interarterial communication worsens disease

Epidemiology

• • 5-7% of congenital cardiac anomalies

  • Accounts for 25% of cardiac mortality in first few days of life

Symptoms and Signs

• • With closure of ductus: Respiratory failure, hemodynamic failure, acidosis, multisystem organ failure

Imaging Findings

• • Echocardiography: Sufficient to make diagnosis

When to Refer

• • These patients should be referred to tertiary center with expertise

• • Evaluate for other cardiac and extracardiac anomalies

• • Physical exam

  • Echocardiography

• • Initial management: Mechanical ventilation, fluid, pressor and HCO3 resuscitation

  • Maintain ductal patency with PGE1

  • If inadequate mixing: Balloon atrial septostomy

Surgery

• • 2 operative choices:

  • 1. Staged procedure: Norwood, followed by Glenn or hemi-Fontan at 6-8 mos, followed by Fontan at 1-1.5 yrs

    2. Cardiac transplantation

Indications

• • Surgical intervention necessary for survival

Treatment Monitoring

• • Frequent echocardiography and angiography

Prognosis

• • Improved recently: 5-year survival, 40-70%

  • Most deaths occur soon after first stage

References