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Reports of 2 Rare Associations of Hypoplastic Left Heart Syndrome

K Anitha Jayakumar1, Gouri Tilak2, Bibhuti B Das1

  • 1Pediatric Heart Institute, Joe DiMaggio Children's Hospital, Hollywood, Florida.

JACC. Case Reports
|July 28, 2021
PubMed

Insights

Hypoplastic left heart syndrome outcomes vary significantly due to rare anatomical differences. One infant with pulmonary valve stenosis had successful surgery, while another with unique venous stenosis received comfort care.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Medical Case Reports

Background:

  • Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect.
  • HLHS involves underdevelopment of the left side of the heart.
  • Rare anatomical variations can influence HLHS presentation and management.

Observation:

  • Two HLHS cases with rare, contrasting associations are presented.
  • Case 1: HLHS with pulmonary valve stenosis, surgically palliated.
  • Case 2: HLHS with intact atrial septum and stenotic bilateral levoatriocardinal veins, managed with comfort care.

Findings:

  • Outcomes in HLHS are highly dependent on associated anomalies.
  • Successful surgical palliation is possible in some HLHS variants.
  • Complex venous stenosis in HLHS may preclude surgical intervention, necessitating comfort care.

Implications:

  • HLHS management requires careful consideration of unique anatomical and physiological factors.
  • These cases highlight the spectrum of HLHS presentations and treatment possibilities.
  • Understanding rare HLHS associations is crucial for personalized patient care and prognosis.

Keywords:
CHD, congenital heart diseaseHLHS, hypoplastic left heart syndromeIUGR, intrauterine growth retardationPA, pulmonary arterycongenital heart diseasehypoplastic left heart syndromelevoatriocardinal veinspalliative carepulmonary valve dysplasia

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