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  • • 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

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Epidemiology

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  • • 5-7% of congenital cardiac anomalies

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

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Symptoms and Signs

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

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Imaging Findings

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  • • Echocardiography: Sufficient to make diagnosis

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When to Refer

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

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  • • Evaluate for other cardiac and extracardiac anomalies

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  • • Physical exam

    • Echocardiography

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

    • Maintain ductal patency with PGE1

    • If inadequate mixing: Balloon atrial septostomy

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Surgery

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  • • 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

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Indications

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  • • Surgical intervention necessary for survival

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Treatment Monitoring

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  • • Frequent echocardiography and angiography

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Prognosis

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

    • Most deaths occur soon after first stage

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References

Bove EL, Ohye RG, Devaney EJ: Hypoplastic left heart syndrome: conventional surgical management. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004;7:3.  [PubMed: 15283346]
Bove EL et al: Tricuspid valve repair for hypoplastic left heart syndrome and the failing right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007;10:101.
Hirsch JC et al: The lateral tunnel Fontan procedure for hypoplastic left heart syndrome: results of 100 consecutive patients. Pediatr Cardiol 2007;28:426.  [PubMed: 17676374]
Pizarro C et al: Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome. Circulation 2003;108(Suppl 1):II155.
Stasik CN et al: Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 2006;131:412.  [PubMed: 16434272]

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