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Hypoplastic left heart syndrome in PAGOD syndrome
Author(s) -
Takahashi Kazuhiro,
Miyake Akira,
Nakayashiro Mami
Publication year - 2014
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12280
Subject(s) - medicine , hypoplastic left heart syndrome , omphalocele , hypoplasia , pulmonary hypoplasia , congenital diaphragmatic hernia , diaphragmatic hernia , cardiology , atresia , diaphragm (acoustics) , surgery , lung , diaphragmatic breathing , fontan procedure , heart disease , hernia , pregnancy , pathology , fetus , physics , alternative medicine , biology , acoustics , loudspeaker , genetics
Chromosomal abnormalities as well as non‐cardiac anomalies have been identified as independent risk factors for surgical morbidity and mortality in F ontan palliation. The combination of malformations consisting of pulmonary hypoplasia, agonadism (sex reversal), omphalocele, and diaphragmatic defect is compatible with pulmonary artery and lung hypoplasia, agonadism, omphalocele, and diaphragmatic defect ( PAGOD ). Most cases have been associated with cardiac disease, particularly hypoplastic left heart syndrome ( HLHS ) that is potentially destined for F ontan palliation. Reported herein is the case of a J apanese female infant diagnosed with PAGOD syndrome along with HLHS (mitral atresia and aortic atresia), in whom intractable respiratory failure manifested as bilateral eventration of the diaphragm and presumed right lung hypoplasia. These characteristic pulmonary lesions associated with the syndrome precluded use of the F ontan pathway.