Single ventricle palliation relies on the pulmonary vasculature accommodating non-pulsatile systemic venous return. Mean pulmonary artery pressure (MPAP) and indexed pulmonary vascular resistance (PVRi) are two measures that impact pulmonary blood flow following bidirectional cavopulmonary connection (BCPC). The purpose of the study was to determine which hemodynamic features are associated with adverse outcomes after BCPC. Pre-operative hemodynamic data and post-operative morbidity and mortality in 250 patients undergoing BCPC at a single center from 2008 to 2014 were reviewed. Patients were then separated into 5 physiologic states based on MPAP, PVRi, and degree of pulmonary to systemic blood flow (Qp:Qs). There were 9 (3.6%) deaths, and 49 patients (20%) sustained major morbidity. An ROC curve identified MPAP > 16 mmHg as an inflection point. Pre-BCPC sildenafil and oxygen use, ventricular dysfunction, and MPAP > 16 mmHg (OR 4.1 [95% CI 1.8–9.2]) were independently associated with morbidity. MPAP > 16 mmHg (OR 6.7 [95% CI 1.6–28]) and pre-BCPC oxygen use were associated with hospital mortality. PVRi was not associated with morbidity or mortality. Of the five physiologic states, patients with high MPAP, low PVRi, and low Qp:Qs fared the worst, with the highest risk of major morbidity (OR 8.6 [3.0–24.9]) and highest risk of mortality (OR 8.0 [1.5–41.3]) when compared to their reference groups (low MPAP, low PVRi). Elevated MPAP, need for pre-operative oxygen support, sildenafil use, and systemic ventricular systolic dysfunction predict morbidity following BCPC. Specifically, patients with elevated MPAP not due to elevated PVRi or pulmonary blood flow had the highest risk of morbidity and mortality.
Glenn procedure Risk factor Morbidity Mortality
Mean pulmonary artery pressure
Indexed pulmonary vascular resistance
Bidirectional cavopulmonary connection
Pulmonary to systemic blood flow
Atrioventricular valve regurgitation
Extracorporeal membrane oxygenation
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This study was funded by the Paige Foundation. There is no grant number. Grant recipient is Susanna Tran.
Compliance with Ethical Standards
Conflict of interest
All authors declare that they have no conflict of interest.
This article does not contain any studies with human participants performed by any of the authors.
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