Elsevier

American Heart Journal

Volume 152, Issue 1, July 2006, Pages 163.e1-163.e7
American Heart Journal

Clinical Investigation
Valvular and Congenital Heart Disease
Borderline left ventricles in prenatally diagnosed atrioventricular septal defect or double outlet right ventricle: Echocardiographic predictors of biventricular repair

https://doi.org/10.1016/j.ahj.2006.04.018Get rights and content

Background

Atrioventricular septal defect (AVSD) and double outlet right ventricle (DORV) with normally related great arteries and normal ventricular sizes are associated with a good long-term prognosis after biventricular (BV) repair. The outcome of cases with a borderline small left ventricle (bLV) is unclear. The purpose of the study was to retrospectively determine echocardiographic predictors of successful BV repair in fetuses with AVSD or DORV with a bLV.

Methods and Results

From 1991 to 2004, 24 fetuses with AVSD plus bLV and 24 with DORV plus bLV were identified. Fetal echocardiographic parameters comparing BV repair versus single ventricle (SV) palliation were obtained, including the presence or absence of an apex-forming bLV was recorded. A bLV was defined as a right ventricular/left ventricular end-diastolic dimension ratio between 2 and 4 SDs for gestational age. The overall survival from fetal diagnosis was 21% (5/24) for AVSD/bLV and 13% (3/24) for DORV/bLV. Of 11 liveborns with AVSD/bLV and 8 liveborns with DORV/bLV, 6 underwent BV repair (5 survivors), 7 SV palliation (3 survivors), and 1 cardiac transplant. Five infants receiving compassionate care only were excluded from the analysis. Parameters such as ratio of valve annuli, ventricular end-diastolic dimensions, degree of valve regurgitation, and the presence of endocardial fibroelastosis were not too predictive of outcome. The presence of an apex-forming bLV was the only predictor of BV repair (6/6 BV repair vs 2/8 SV palliation, P < .05).

Conclusions

Prenatally diagnosed AVSD or DORV with bLV has a very poor prognosis. An apex-forming bLV predicts successful BV repair and is an important prognostic indicator.

Section snippets

Patient inclusion criteria

A bLV was defined as an RV/LV end-diastolic diameter ratio between 2 and 4 SDs for gestational age.18 All cases of unbalanced AVSD or DORV with a hypoplastic LV diagnosed in the fetus from the Hospital for Sick Children, Toronto, Canada, were identified from the cardiac database between 1991 and 2004.

Patient exclusion criteria

Cases were excluded if the AVSD or DORV was associated with mitral or aortic atresia, intact ventricular septum, hypoplastic pulmonary arteries, a heterotaxy syndrome, total anomalous pulmonary

Results

Between 1991 and 2004, 1481 fetuses were diagnosed with structural congenital heart disease at the Hospital for Sick Children. Of those, a total of 152 (10%) and 77 (5%) fetuses were diagnosed with AVSD and DORV, respectively. Of the 151 cases of AVSD, 27 (18%) were found to have a bLV, and of the 78 cases of DORV, 27 (34%) had a bLV. Six of these cases were excluded from the analysis including 2 with severely hypoplastic pulmonary arteries and 4 for different postnatal diagnoses (1 VSD and

Discussion

Fetal echocardiography has evolved significantly with both improvements in image resolution and the diagnosis of most forms of congenital heart disease. We are now beginning to focus on details that will accurately predict the outcome of affected pregnancies to provide appropriate and accurate counseling and plan for perinatal management. One of the most critical limitations encountered in this field is the ability to predict when a bLV is going to be sufficient to sustain the systemic

References (24)

  • E. Belli et al.

    Biventricular repair for double-outlet right ventricle. Results and long-term follow-up

    Circulation

    (1998)
  • V. Fesslova et al.

    Spectrum and outcome of atrioventricular septal defect in fetal life

    Cardiol Young

    (2002)
  • Cited by (0)

    c

    Current affiliation: Division of Cardiology, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland.

    d

    Current affiliation: Fetal Cardiovascular Program, Pediatric and Fetal Treatment Centers, University of California, San Francisco, CA.

    View full text