Clinical InvestigationValvular and Congenital Heart DiseaseImpact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle
Section snippets
Inclusion and exclusion criteria
All cases with a prenatal or postnatal diagnosis of DORV between January 2000 and December 2007 at the Hospital for Sick Children, Toronto, Canada, were identified. The study was approved by the institutional Research Ethics Board. No extramural funding was used to support this work. The authors are solely responsible for the design and conduct of this study, all study analyses, the drafting and editing of the paper, and its final contents.
Patients with absent pulmonary valve, atresia of any
Patient population
Two hundred sixty-seven DORV patients were identified; 160 were identified prenatally and 107 postnatally. Of the 160 fetal cases, 67 were excluded (Figure 1). There were 24 pregnancy terminations, 10 of which had abnormal chromosomes (Trisomy 13 [n = 2], Trisomy 18 [n = 3], and chromosome 22q11 microdeletion [n = 5]), 7 had complex extracardiac anomalies, and 7 were terminated for unknown or personal reasons. Two patients had structurally normal hearts on postnatal echocardiography (both were
Prenatal versus postnatal diagnosis of DORV
There was no significant difference in overall mortality between the prenatal and postnatal groups in this DORV series, which is unlike other lesions such as TGA and hypoplastic left heart syndrome.21, 22, 23, 24 However, a prenatal diagnosis did confer a shorter intensive care unit stay and less postoperative cardiac complications.
Previously reported series have demonstrated a very poor outcome in fetuses with DORV.4, 25 These cohorts had a high proportion of fetuses with heterotaxy and/ or
Conclusions
This series investigates risk factors for demise and need for complex postnatal care in DORV patients without heterotaxy. Prenatally diagnosed DORV had a higher proportion of chromosomal abnormalities, and few of these patients achieved a single, primary complete repair compared with the postnatal group. Even when not associated with heterotaxy, DORV incurs significant pre and postnatal morbidity and mortality. Morphologic subtype, irrespective of pre or postnatal diagnosis, is a major
Disclosures
There are no conflicts of interest to disclose.
References (28)
- et al.
A concept of double-outlet right ventricle
J Thorac Cardiovasc Surg
(1972) - et al.
Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect
Ann Thorac Surg
(2006) - et al.
Surgical anatomy of double-outlet right ventricle – a reappraisal
Am J Cardiol
(1983) - et al.
Malaligned outlet septum with sub-pulmonary ventricular septal defect and abnormal ventriculoarterial connection: a morphologic spectrum defined echocardiographically
J Am Coll Cardiol
(1990) - et al.
Surgical anatomy of double-outlet right ventricle with situs solitus and atrioventricular concordance
J Thorac Cardiovasc Surg
(1981) - et al.
Doube outlet right ventricle: anatomic types and associated anomalies
Chest
(1975) - et al.
Comparison of outcome when hypoplastic left heart syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of these two conditions is made only postnatally
Am J Cardiol
(1999) - et al.
Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies
J Am Coll Cardiol
(1999) - et al.
Borderline left ventricles in prenatally diagnosed atrioventricular septal defect or double outlet right ventricle: echocardiographic predictors of biventricular repair
Am Heart J
(2006)
Clinical features, management and outcome of children with fetal and postnatal diagnosis of isomerism syndromes
Circulation
Diagnosis and prognosis in double-outlet right ventricle
Am J Perinatol
Biventricular repair in patients with heterotaxy syndrome
J Thorac Cardiovasc Surg
Pathology of congenital heart disease
Cited by (0)
- a
Principal coinvestigators.