Elsevier

American Heart Journal

Volume 160, Issue 4, October 2010, Pages 692-700
American Heart Journal

Clinical Investigation
Valvular and Congenital Heart Disease
Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle

https://doi.org/10.1016/j.ahj.2010.07.009Get rights and content

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.

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.

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