Hypoplastic left heart syndrome with intact atrial septum: case report

Cester, Maddalena and Nanhorngue, Kimta Nanhorngue and Pascoli, Irene and Garofolo, Greta and Surico, Nicola and Paternoster, Delia Maria (2007) Hypoplastic left heart syndrome with intact atrial septum: case report. Journal of prenatal medicine, 1 (2). pp. 32-34. ISSN 1971-3290

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Abstract

Objectives. Hypoplastic left heart syndrome (HLHS) with an intact atrial septum (IAS) is a rare finding, reported in only 1% of pathologic specimens with hypoplasia of the aortic tract complex. In newborns with left heart obstruction, the existence of an interatrial communication is very important for oxygenated blood to be distributed to the body and to prevent pulmonary congestion. The ability to predict prenatally restriction of the atrial defect may allow earlier surgery to be planned. Methods. We report a case of prenatal diagnosis of HLHS with a complete premature closure of the foramen ovale that was not detected by prenatal echocardiography. Results and conclusion. The management of neonates with HLHS in the first days of life is crucial to the results of the first stage of the Norwood procedure. We suggest that delivery of the mother close to surgical centre and avoiding neonatal transfer improve the results, but stabilisation with prostaglandins and balancing of the systemic and pulmonary resistances are also important. A restrictive or closed atrial septal defect may contribute to haemodynamic instability in the first days of life. The ability to predict this complication prenatally may help in the immediate postnatal management of the affected infant.

Item Type: Article
Additional Information:
Uncontrolled Keywords: Hypoplastic left heart syndrome, intact atrial septum, fetal echocardiography
Subjects: 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 618 Altri rami della medicina; Ginecologia e ostetricia, Pediatria, Geriatria > 618.3 Malattie e complicazioni della gravidanza
Depositing User: Gianni Aiello
Date Deposited: 10 Feb 2014 14:15
Last Modified: 10 Feb 2014 14:15
URI: http://eprints.bice.rm.cnr.it/id/eprint/5583

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