Type your tag names separated by a space and hit enter

Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome is a topic covered in the 5-Minute Pediatric Consult.

To view the entire topic, please or purchase a subscription.

Pediatrics Central™ is an all-in-one application that puts valuable medical information, via your mobile device or the web, in the hands of clinicians treating infants, children, and adolescents. Explore these free sample topics:

Pediatrics Central

-- The first section of this topic is shown below --

Basics

Description

Hypoplastic left heart syndrome (HLHS) is a continuum of congenital cardiac defects resulting from severe underdevelopment of the structures of the left side of the heart (left atrium, mitral valve, left ventricle, aortic valve, and ascending aorta).

Epidemiology

  • 0.16 to 0.36 per 1,000 live births
  • 8% of congenital heart disease (CHD); 3rd most common cause of critical CHD in the newborn
  • 23% of all neonatal mortality from CHD
  • Male predominance (67%)

Risk Factors

Genetics

Familial inheritance: Sibling recurrence risk ranges from 8% to 21%, with higher recurrence observed when cardiovascular malformations are present in either parent. In addition, rare kinships have a frequency approaching autosomal dominant transmission.

Commonly Associated Conditions

  • Increased mortality when associated with definable genetic disorders, which comprise 10–28% of HLHS patients:
    • Turner syndrome, Noonan syndrome, Smith-Lemli-Opitz syndrome, Holt-Oram syndrome
    • Trisomy 13, 18, 21, or microdeletion syndromes
  • Major extracardiac anomalies (diaphragmatic hernia, omphalocele)

Pathophysiology

  • The etiology appears multifactorial, most likely resulting from an in utero reduction of left ventricular inflow or outflow (mechanisms postulated include premature closure of the foramen ovale and fetal cardiomyopathy).
  • As a result, the right ventricle (RV) must supply both the pulmonary and systemic circulations (via the ductus arteriosus) before and after birth.
  • The reduction in pulmonary vascular resistance that occurs with lung expansion at birth reduces the proportion of RV output to the systemic circulation. If the ductus arteriosus closes, shock occurs.

-- To view the remaining sections of this topic, please or purchase a subscription --

Citation

Cabana, Michael D., editor. "Hypoplastic Left Heart Syndrome." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617285/all/Hypoplastic_Left_Heart_Syndrome.
Hypoplastic Left Heart Syndrome. In: Cabana MD, ed. 5-Minute Pediatric Consult. 8th ed. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617285/all/Hypoplastic_Left_Heart_Syndrome. Accessed March 24, 2019.
Hypoplastic Left Heart Syndrome. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult. Available from https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617285/all/Hypoplastic_Left_Heart_Syndrome
Hypoplastic Left Heart Syndrome [Internet]. In: Cabana MD, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2019 March 24]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617285/all/Hypoplastic_Left_Heart_Syndrome.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Hypoplastic Left Heart Syndrome ID - 617285 ED - Cabana,Michael D, BT - 5-Minute Pediatric Consult UR - https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617285/all/Hypoplastic_Left_Heart_Syndrome PB - Wolters Kluwer ET - 8 DB - Pediatrics Central DP - Unbound Medicine ER -