Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation

AbstractThe objective of this study was to review the echocardiographic findings and autopsy correlates of pulmonary atresia with an intact ventricular septum when diagnosed during fetal life. All cases with this lesion (nearly 60) seen in the Perinatal Cardiology unit of Guy's Hospital since 1983 are part of this study. More than half of the cases were detected within the last two years, and all bar three were referred because of suspicion of a cardiac malformation detected on routine obstetric scanning. The cases were divided into two distinct groups. On the one hand were those with dilatation of the chambers of the right heart. These had a uniformly poor prognosis. Severe regurgitation of the tricuspid valve was a characteristic feature. On the other hand were the cases with cavitary hypoplasia due to mural overgrowth. Direct measurements of pressure in one such case revealed suprasystemic values in the right ventricle. Although contemplated, fetal intervention proved impossible. Autopsy revealed cases with either valvar or infundibular atresia. In most cases diagnosed prior to 24 weeks of gestation, mothers opted for termination of pregnancy. All cases with dilatation of the right ventricle died. Of pregnancies which continued, only 30% of children are still alive, all but one being less than two years of age at present. The use of cross-sectional and Doppler echocardiography permits the accurate diagnosis of pulmonary atresia with an intact ventricular septum during fetal life. Autopsied cases showed strong correlation with the echocardiographic findings. The poor prognosis in most cases warrants consideration in the future of intervention during fetal life.

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Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum

Cardiology in the Young ◽

10.1017/s1047951100000214 ◽

1991 ◽

Vol 1 (2) ◽

pp. 105-113 ◽

Cited By ~ 31

Author(s):

Robert H. Anderson ◽

Christine Anderson ◽

James R. Zuberbuhler

Keyword(s):

Right Ventricle ◽

Right Ventricular ◽

Tricuspid Valve ◽

Coronary Arteries ◽

Pulmonary Atresia ◽

Outflow Tract ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Ventricular Cavity ◽

The Right

SummaryAtresia of the outflow tract of the right ventricle in the presence of an intact ventricular septum poses major problems for surgical management. In the light of known problems, 43 autopsied hearts with this congenital abnormality were evaluated to note the arrangement of the myocardium and the cavity of the right ventricle, the state of the tricuspid valve, the precise substrate for pulmonary atresia, and the presence offistulous communications between the ventricular cavity and the coronary arteries. The specimens could be divided into two groups. The first group (38 hearts) had hypoplasia of the cavity of the right ventricle with mural hypertrophy while the second group (5 hearts) had a dilated right ventricular cavity with thinning of the wall. The larger group could be divided into two subgroups on the basis of the substrate for pulmonary atresia. Twenty hearts had muscular atresia of the right ventricular outflow tract and 18 had a potentially patent outflow tract blocked by an imperforate pulmonary valve. A comparison of these two subgroups revealed that mural hypertrophy and cavitary hypoplasia were more severe in hearts with muscular atresia. The tricuspid valve was hypoplastic and its leaflets were not dysplastic in this group but the leaflets were dysplastic in hearts with valvar pulmonary atresia. Fistulous communications between the ventricular cavity and the coronary arteries were only seen in the cases with muscular atresia. In this series, recognition of muscular pulmonary atresia would have identified those patients with the worst surgical prognosis.

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