Pulmonary atresia with intact ventricular septum in the fetus

1992 ◽  
Vol 2 (4) ◽  
pp. 367-376 ◽  
Author(s):  
Lindsey D. Allan ◽  
Andrew Cook
Keyword(s):  
Right Ventricle ◽  
Poor Prognosis ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Fetal Life ◽  
Intact Ventricular Septum ◽  
Cross Sectional ◽  
The One ◽  
The Right ◽  
Echocardiographic Findings

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.

Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life

2002 ◽  
Vol 12 (5) ◽  
pp. 436-444 ◽  
Author(s):  
George G. S. Sandor ◽  
Andrew C. Cook ◽  
Gurleen K. Sharland ◽  
S. Yen Ho ◽  
James E. Potts ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Fetal Echocardiography ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Fetal Life ◽  
Intact Ventricular Septum ◽  
Z Scores ◽  
Definition Of ◽  
The Right

Objectives: To establish the prevalence of coronary arterial abnormalities in mid-trimester fetuses with pulmonary atresia with intact ventricular septum, and whether their presence correlates with right ventricular morphology. Background: The presence of coronary arterial fistulas significantly alters the surgical options and prognosis for patients with pulmonary atresia with intact ventricular septum. The lesion can reliably be diagnosed using fetal echocardiography, and further definition of the prognosis is important for counselling parents. Methods: We examined the hearts of 39 pathological specimens diagnosed during fetal life, 3 of whom died postnatally. Coronary arterial abnormalities were defined as non-connection of the left or right coronary arteries to the aorta, ostial stenosis, marked tortuosity, dilation, thickening or abnormal myocardial branching. Mild tortuosity, or myocardial bridging, were considered normal. We measured the dimensions of the tricuspid valve along with the inlet and outlet portions of the ventricles. Ebstein's malformation, tricuspid valvar dysplasia, and the presence or absence of the infundibulum, were especially noted. We examined also 12 normal hearts as controls. Results: Coronary arterial abnormalities were found in 14/39 (36%). The dimensions of the right ventricle and tricuspid valves, and the gestational ages of the fetuses, were compared for these 14 with the 25 having no abnormalities using independent t-tests. The gestational ages were similar, 21.9 vs 21.1 weeks. The mean dimensions of the tricuspid valve, median z-scores, and right ventricle were smaller, 2.9 vs 7.2 mm; p < 0.002; −4.46 vs 0.23; p < 0.03; and 6.9 vs 13.7 mm; p < 0.002, for those with coronary arterial abnormalities. Ebstein's malformation, or dysplasia of the tricuspid valve, was present in 4 of 14 with, vs 15 of 25 without, coronary arterial abnormalities. A patent infundibulum was noted in 34 of 39 specimens. Conclusions: Mid-trimester fetuses with pulmonary atresia with intact ventricular septum already exhibit coronary arterial abnormalities, with a prevalence of 36%. The presence of a patent infundibulum confirms that atresia of the pulmonary valve is an acquired process. Coronary arterial abnormalities are seen in 50% of those with hypoplastic right ventricles, but less frequently in the presence of well developed ventricles. This is important information for those involved in counselling parents.

scholarly journals Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum

1991 ◽  
Vol 101 (2) ◽  
pp. 222-229 ◽  
Author(s):  
William G. Williams ◽  
Patricia Burrows ◽  
Robert M. Freedom ◽  
George A. Trusler ◽  
John G. Coles ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
The Right

Balloon Valvuloplasty Through the Right Ventricle: Another Treatment of Pulmonary Atresia With Intact Ventricular Septum

2013 ◽  
Vol 95 (5) ◽  
pp. 1670-1674 ◽  
Author(s):  
Qian-zhen Li ◽  
Hua Cao ◽  
Qiang Chen ◽  
Gui-Can Zhang ◽  
Liang-Wan Chen ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Balloon Valvuloplasty ◽  
Intact Ventricular Septum ◽  
The Right

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

1991 ◽  
Vol 1 (2) ◽  
pp. 105-113 ◽  
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.

Right ventricular “overhaul”—an intermediate step in the biventricular repair of pulmonary atresia with intact ventricular septum

1995 ◽  
Vol 5 (2) ◽  
pp. 161-165 ◽  
Author(s):  
Ash Pawade ◽  
Roger B. B. Mee ◽  
Tom Karl
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Fontan Operation ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intermediate Step ◽  
Intact Ventricular Septum ◽  
Biventricular Repair ◽  
Antegrade Flow ◽  
The Right

SummaryThe management of pulmonary atresia with intact ventricular septum remains controversial. The plan for management at our institution is based on the echocardiographic identification of a well-developed infundibulum. Neonates with such a well-developed infundibulum are prepared for a biventricular repair, whereas those without an infundibulum are groomed for a Fontan operation. In all, 48 neonates with the lesion were admitted to our unit between 1980 and 1992. In 31 neonates with a well-formed infundibulum, the initial palliation consisted mainly of pulmonary valvotomy without cardiopulmonary bypass and construction of a polytetrafluoroethylene shunt from the left subclavian artery to the pulmonary trunk. In most of these patients, growth of the right ventricle could be promoted by establishing antegrade flow through the ventricle. In seven patients in this group, nonetheless, the right ventricle failed to grow satisfactorily. In this subgroup, additional intermediate procedures were performed on the apical component and infundibulum of the ventricle together with the tricuspid and pulmonary valves which could collectively be termed as “right ventricular overhaul.” Five of these patients have gone on to further successful biventricular repairs at a median interval of 24 months after the overhaul. There was no operative mortality in this group. Following the final biventricular repair, there have been no late deaths or reoperations over a total follow-up of 145.4 patient months (mean 22.2 months).

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