Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle

Maria E. Lagopoulos, Cedric Manlhiot, Brian W. McCrindle, Edgar T. Jaeggi, Mark K. Friedberg, Lynne E. Nield

Research output: Contribution to journalArticlepeer-review

Abstract

Background: We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy. Methods: Patients with a pre or postnatal diagnosis of DORV from 2000 to 2007 were identified and classified into 3 subgroups: subaortic ventricular septal defect (VSD) and normal great artery (GA) arrangement (=VSD type), tetralogy of Fallot type, and transposition of the GA type (=TGA type). Patients with heterotaxy, atrioventricular septal defect, valve atresia, and ventricular hypoplasia were excluded. Complex postnatal care was defined as prematurity, need for prostaglandins, surgical repair <2 months, or univentricular palliation. Risk factors for complex postnatal care and demise were sought in multivariable logistic regression models. One hundred fort-five patients were included (93 prenatal, 52 postnatal). Results: There were 24 pregnancy terminations and 7 in utero deaths. Fetal demise was associated with abnormal karyotype (odds ratio [OR] 1.9, P = .01), any tricuspid valve regurgitation (OR 10.6, P = .01), and hydrops (OR 23.8, P = .02). Of 114 liveborn patients, 23 were tetralogy-type, 67 VSD-type, and 24 TGA-type patients. Postnatal survival of liveborn patients at 1 year was similar in pre- versus postnatally diagnosed patients (84% vs 85%). Abnormal GA relationship (OR 2.9, P = .02), subpulmonary VSD (OR 6.0, P = .001), unobstructed pulmonary blood flow at birth (OR 2.8, P = .05), and aortic coarctation (OR 9.0, P = .007) were associated with suboptimal postsurgical outcomes. Conclusion: Double outlet right ventricle, even without heterotaxy, is associated with complex postnatal care and high risk of early demise. Morphologic subtype, irrespective of pre- or postnatal diagnosis, is a major determinant of outcome.

Original languageEnglish (US)
Pages (from-to)692-700
Number of pages9
JournalAmerican heart journal
Volume160
Issue number4
DOIs
StatePublished - Oct 2010
Externally publishedYes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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