Top

Published in:

01-08-2010 | Case Report

Hypoplastic Left Heart Syndrome Associated with Large Omphalocele and Hypoplastic Left Bronchus

Authors: Michael Hainstock, Dianna Bardo, Mary Rice, Stephen Langley

Published in: Pediatric Cardiology | Issue 6/2010

Abstract

We present a patient with the combination of hypoplastic left heart syndrome (HLHS), large omphalocele, and hypoplastic left bronchus who underwent a modified Norwood procedure and was successfully discharged home. The child required tracheostomy with home ventilation and we elected to delay the primary repair of the omphalocele. To our knowledge, this is the first reported case with this combination of anomalies surviving till hospital discharge. Our experience demonstrates the potential viability of patients with concurrent HLHS, large abdominal wall defects, and airway anomalies and may additionally advocate for delayed repair of large omphaloceles in patients with complex congenital heart disease.

Brantberg A, Blass HG, Haugen SE, Eik-Nes SH (2005) Characteristics and outcome of 90 cases of fetal omphalocele. Ultrasound Obstet Gynecol 26:527–537CrossRefPubMed

Hoffman JE, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 9(12):1890–1900CrossRef

Kim JB (2007) A case of PAGOD syndrome with hypoplastic left heart syndrome. Int J Cardiol 114(2):270–271CrossRefPubMed

Lee SL et al (2006) Initial nonoperative management and delayed closure for treatment of giant omphaloceles. J Pediatr Surg 41:1846–1849CrossRefPubMed

Mabogunje OA, Mahour GH (1984) Omphalocele and gastroschissi. Trends in survival across two decades. Am J Surg 148:679–686CrossRefPubMed

Nishimura M, Taniguchi A, Imanaka H, Taenaka N (1992) Hypoplastic left heart syndrome associated with congenital right-sided diaphragmatic hernia and omphalocele. Chest 100(1):263–264CrossRef

Wheeler DS, St Louis JD (2007) Pentalogy of Cantrell associated with hypoplastic left heart syndrome. Pediatr Cardiol 28:311–313CrossRefPubMed

Title: Hypoplastic Left Heart Syndrome Associated with Large Omphalocele and Hypoplastic Left Bronchus
Authors: Michael Hainstock
Dianna Bardo
Mary Rice
Stephen Langley
Publication date: 01-08-2010
Publisher: Springer-Verlag
Published in: Pediatric Cardiology / Issue 6/2010
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-010-9716-7

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Hypoplastic Left Heart Syndrome Associated with Large Omphalocele and Hypoplastic Left Bronchus

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 6/2010

Polysplenia and Interrupted Inferior Vena Cava With Azygos Continuation: 64-Slice Multidetector Row Computed Tomography Findings

Unusual Cardiac Associations with Tetralogy of Fallot—A Descriptive Study

No Demonstrable Effect of Benzathine Penicillin on Recurrence of Rheumatic Fever in Pacific Island Population

Endomyocardial Disease Related to Idiopathic Hypereosinophilic Syndrome: A Cardiac Magnetic Resonance Evaluation

Acute Myocardial Infarction Caused by Transient Coronary Vasospasm in a Child With Kawasaki Disease and No Coronary Aneurysms

Electrocardiographic Strain Pattern in Children With Left Ventricular Hypertrophy: A Marker of Ventricular Dysfunction