Skip to Main Content

+

  • • 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

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]

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.