Clinical Investigations
Echocardiography in Infant Congenital Heart Disease
Association of Bulboventricular Foramen Size and Need for Early Intervention in Infants with Tricuspid Atresia or Double-Inlet Left Ventricle with Normally Related Great Arteries

https://doi.org/10.1016/j.echo.2022.11.009Get rights and content

Highlights

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    iBVFA is associated with pulmonary outflow adequacy.

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    iBVFA < 1.8 cm2/m2 is associated with need for intervention before Glenn operation.

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    The highest risk patients have iBVFA < 1 cm2/m2.

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    Flow acceleration/outflow narrowing can be associated with need for intervention.

Background

The association of bulboventricular foramen (BVF) size and systemic outflow adequacy has been studied in patients with tricuspid atresia (TA) or double-inlet left ventricle (DILV) with transposed great arteries. The aim of this study was to determine the relationship between initial BVF size and risk for progressive pulmonary outflow obstruction requiring intervention to increase pulmonary blood flow in patients with TA or DILV with normally related great arteries.

Methods

Patients with TA or DILV with normally related great arteries were identified by retrospective chart review at a single center from 2005 to 2021. Patients were stratified by indexed BVF area (iBVFA) to determine the relationship of iBVFA size and the need for intervention before the Glenn operation to establish supplemental pulmonary blood flow with either a Blalock-Taussig-Thomas shunt (BTTS) or patent ductus arteriosus (PDA) stent. Patients were followed through the time of their Glenn operations. Logistic regression analysis was performed to determine optimal iBVFA cut points.

Results

Thirty-seven patients with TA or DILV with normally related great arteries were included. Sixteen had iBVFA < 1 cm2/m2, with all 16 (100%) requiring either a BTTS or PDA stent to increase pulmonary blood flow before the Glenn operation. Seventeen had iBVFAs of 1 to 2 cm2/m2, with 10 (59%) requiring either a BTTS or PDA stent. Nine of those 10 demonstrated flow acceleration across the BVF and/or pulmonary outflow tract. Four had iBVFA > 2 cm2/m2, with only one patient (25%) requiring a BTTS. Among our cohort, an iBVFA of <1.8 cm2/m2 provided sensitivity of 96% with good positive and negative predictive values (81% and 80%, respectively) for requiring intervention with a BTTS or PDA stent before the Glenn operation.

Conclusions

An iBVFA of ≤1.8 cm2/m2 on initial postnatal echocardiography is associated with the development of subpulmonary obstruction requiring intervention with a BTTS or PDA stent before the Glenn operation, with the highest risk noted in those with iBVFA of ≤1 cm2/m2. Factors such as BVF flow acceleration or pulmonary outflow tract narrowing should also be considered in the decision to augment pulmonary blood flow.

Section snippets

Methods

A retrospective, single-center study was conducted at Vanderbilt University Medical Center to determine indexed BVFA (iBVFA) size and patient outcomes. Patients chosen for inclusion had TA or DILV with normally related great arteries, with transthoracic echocardiography performed in the neonatal period from 2005 to 2021. Neonates with TA or DILV with pulmonary atresia were excluded. iBVFA measurements were performed by cardiologists blinded to patient outcomes. To evaluate interobserver

Results

Forty patients with TA or DILV with normally related great arteries were identified from 2005 to 2021. Three patients were excluded from analysis: two (one with TA, one with DILV) died before the Glenn operation from causes that were assumed unrelated to their cardiac diagnoses, and one (with TA) was lost to follow-up. Among the 37 patients included in the analysis, 30 had TA and seven had DILV. Table 1 describes the characteristics of the 37 patients included in the analysis. There were no

Discussion

Echocardiographic measurements of iBVFA in patients with TA or DILV with TGA to determine the adequacy of systemic output have been previously reported.1 In this study we characterized iBVFA and its relation to postnatal course and the need for intervention in patients with TA or DILV with normally related great arteries before the Glenn operation (in the form of a BTTS or PDA stent). Among our cohort, 73% underwent BTTS or PDA stent placement before the Glenn operation, and those with TA were

Conclusion

An iBVFA of ≤1.8 cm2/m2 on initial postnatal echocardiography is associated with the development of subpulmonary obstruction requiring intervention with a BTTS or PDA stent before the Glenn operation, with the highest risk patients having iBVFA of ≤1 cm2/m2. Factors such as pulmonary valve size and evidence of flow acceleration across the BVF and/or pulmonary outflow tract may be helpful in the decision algorithm to determine which patients at intermediate risk may require intervention to

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Conflicts of interest: None.

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2022 by the American Society of Echocardiography.