A rare lethal combination of premature closure of the foramen ovale and d-transposition of the great arteries with intact ventricular septum

doi:10.1016/j.ijcard.2007.11.055

International Journal of Cardiology

Volume 130, Issue 2, 12 November 2008, Pages e57-e59

https://doi.org/10.1016/j.ijcard.2007.11.055 Get rights and content

Abstract

We report an infant who presented with cyanosis after birth and was unresponsive to all resuscitative efforts. Emergent echocardiogram showed d-transposition of the great arteries (d-TGA) with an intact ventricular septum. Attempts to perform a bedside balloon atrial septostomy failed due to inability to traverse the atrial septum. Autopsy revealed d-TGA with intact atrial and ventricular septa and patent ductus arteriosus. Though no survivors have been reported for this rare lethal combination of lesions thus far in the literature, we speculate that recent advances in fetal treatment may soon be applicable to infants with this condition.

Introduction

D-transposition of the great arteries (d-TGA) is the most common cyanotic congenital heart disease in neonates. The discordant ventriculo-arterial arrangement results in parallel circulation. Unless there is an avenue for adequate mixing of deoxygenated and oxygenated blood, this cardiovascular malformation is not compatible with extrauterine life. Complete premature closure of the foramen ovale in d-TGA with intact ventricular septum is extremely rare, with only three case reports in the literature [1], [2], [3]. All reported cases resulted in early neonatal demise despite aggressive treatment including extracorporeal membrane oxygenation (ECMO) in one [3]. In this paper, we report a fourth case of premature closure of the foramen ovale in an infant with d-TGA and intact ventricular septum who became cyanotic and decompensated immediately after birth.

Section snippets

Case report

A 2880 gm, 38 weeks' gestation female infant was born to a healthy mother. Prenatal fetal ultrasound at 32 weeks' gestation showed, by report, normal four-chambered cardiac anatomy with two ventricular outflow tracts identified. Cardiac size and position were reported as normal. The infant was delivered vaginally after an unremarkable labor. The infant cried spontaneously immediately after birth. Apgar scores were 7 and 8 at 1 and 5 min, respectively. The infant subsequently became dusky, limp

Discussion

Infants with d-TGA have parallel systemic and pulmonary circulations, and are dependent on shunting of oxygenated blood to the systemic circulation to survive postnatally. In 1979, Bhatt first reported a rare lethal combination of premature closure of the foramen ovale and d-TGA with intact ventricular septum [1]. Since then, antenatal restriction of the foramen ovale and constriction of the ductus arteriosus have been recognized to be associated with increasing risk of morbidity and mortality

References (6)

D.R. Bhatt et al.
Prenatal closure of the foramen ovale in complete transposition of great vessels. Observations in a case with intact ventricular septum and normally developed left heart chambers
Am J Cardiol
(1979)
W. Tworetzky et al.
Fetal interventions for cardiac defects
Pediatr Clin North Am
(2004)
L.M. Berry et al.
Premature "closing" of the foramen ovale in transposition of the great arteries with intact ventricular septum: rare cause of sudden neonatal death
Pediatr Cardiol
(1998)

There are more references available in the full text version of this article.

Cited by (11)

Prenatal diagnosis of foramen ovale premature closure and hydrops fetalis
2013, Diagnostico Prenatal
Citation Excerpt :
Image was obtained with a Toshiba Power Vision 6000 ultrasound with 2D and color Doppler mode. The PCFO has been reported to be associated a great variety of cardiac defects: transposition of great arteries, giant atrium septum aneurysm, aortic stenosis, left ventricular dilatation, mitral atresia, double-outlet right ventricle, prenatal treatment with indomethacin, non-immune hydrops fetalis or gene defects.2–9 The Doppler application is useful to detect flow changes and permeability of foramen ovale, tricuspid regurgitation, abnormalities of tricuspid and pulmonary valves or ductus arteriosus, associated with hydrops fetalis.10,11
A case of premature closure of foramen ovale (PCFO) is presented. The patient was sent to the Fetal Maternal Unit after 30 weeks of gestation due to hydrops fetalis. A Doppler ultrasound performed after 34 weeks of gestation showed no interatrial flow and led to the confirmation of PCFO, associated to pleural effusion and ascitis. No evidence of hydrops, pleural effusion, ascitis, cardiac failure, cardiac defects nor chromosomal abnormalities were present in the newborn baby. Since prenatal diagnosis of PCFO is a life threatening condition, detection improves fetal and neonatal life expectancy.
Se reporta el cierre prematuro de foramen oval (CPFO) en un embarazo de 30 semanas de gestación, enviado con hidrops fetal a Unidad de Medicina Materno Fetal. A las 34 semanas se evaluó con Ultrasonido Doppler y se observó falta de flujo entre aurículas, confirmando el CPFO, presencia de derrame pleural y ascitis. Después del nacimiento, al neonato se le descartó la presencia de hidrops, derrame o ascitis, insuficiencia cardiaca, defectos cardiacos o cromosomopatía, y egresó sano. El CPFO pone en riesgo la vida. Cuando es detectado prenatalmente mejora la expectativa de vida fetal y neonatal.
A recurrent patent ductus arteriosus
2011, International Journal of Cardiology
Citation Excerpt :
Patent ductus arteriosus (PDA) [1–7] can occur as part of more congenital cardiac malformations or as an isolate finding.
Patent ductus arteriosus (PDA) can occur as part of more congenital cardiac malformations or as an isolate finding. Spontaneous, transcatheter, surgical closure, and pharmacological treatment have been reported. We present a case of recurrent PDA after the initial course of a pharmacological treatment. Also this case focuses on recurrent PDA after the initial course of a pharmacological treatment.
Interatrial defect and patent ductus arteriosus
2010, International Journal of Cardiology
We greatly appreciated the letter to the Editor by Rana Olgunturk and Serdar Kula. We can say that his interpretation coincides with our interpretation and with the knowledge about patent ductus arteriosus (PDA) and secundum atrial septal defects. Although functional closure of PDA usually occurs in the first few hours of life in healthy infants born at term (about 15 h of life in healthy infants born at term), true anatomic closure, in which the ductus loses the ability to reopen, may take several weeks. Persistent, patency for up to 10 days after birth is encountered in premature infants. The male Italian newborn infant of the cited case report was referred at the age of 2 days to the Cardiology Unit for a cardiovascular check-up (in the text: “in a 3-day-old newborn infant” must be comprehended as “in a 2-day-old newborn infant”) and was mature. Interatrial septal shunts in newborns are frequently encountered. Since there are no definitive diagnostic criteria for both patent foramen ovale and secundum atrial septal defects, it is difficult to differentiate these two lesions by echocardiography, particularly in the early newborn period. Some authors, called these two lesions as “interatrial septal openings”. The diagnosis, in the case report cited, was made not only on the basis of the first observation but also on the basis of the persistence of abnormalities during the long-term follow-up and therefore the infant was referred to a pediatric heart surgical unit were the abnormalities were confirmed.
MRI shows limited mixing between systemic and pulmonary circulations in foetal transposition of the great arteries: A potential cause of in utero pulmonary vascular disease
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Letter to the EditorA rare lethal combination of premature closure of the foramen ovale and d-transposition of the great arteries with intact ventricular septum

Abstract

Introduction

Section snippets

Case report

Discussion

Am J Cardiol

Pediatr Clin North Am

Premature "closing" of the foramen ovale in transposition of the great arteries with intact ventricular septum: rare cause of sudden neonatal death

Pediatr Cardiol

Letter to the Editor
A rare lethal combination of premature closure of the foramen ovale and d-transposition of the great arteries with intact ventricular septum