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Review
. 2022 Apr 5;9(4):515.
doi: 10.3390/children9040515.

The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

Matteo Trezzi  1 , Enrico Cetrano  1 , Sonia B Albanese  1 , Luca Borro  2 , Aurelio Secinaro  2 , Adriano Carotti  1
Affiliations
Review

The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

Matteo Trezzi et al. Children (Basel). .

Abstract

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches to its management. Unifocalization and rehabilitation focus on mobilization of collateral arteries and growth of native pulmonary arteries, respectively, with the ultimate surgical goal of achieving separated systemic and pulmonary circulations with the lowest possible right ventricular pressure. Regardless of the strategy, outcomes have altered the natural history of the disease, with a complete repair rate of approximately 80% and low early and late mortality rates. Given this heterogeneity of pulmonary vasculature, a tailored approach should be adopted for each patient, using all diagnostic methods currently offered by technical developments.

Keywords: Fallot’s tetralogy; major aortopulmonary collateral arteries; pulmonary atresia with ventricular septal defect; rehabilitation; unifocalization.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Childrens’ Hospital Bambino Gesù algorithm for PA/VSD/MAPCAs treatment in infancy according to the presence, size and distribution of native pulmonary arteries.
Figure 2
Figure 2
Surgical results of PA/VSD/MAPCAs patients treated at Bambino Gesù Children Hospital.
Figure 3
Figure 3
Survival by the era of all treated patients with PA/VSD/MAPCAs.
Figure 4
Figure 4
CT imaging of the patient who underwent pulmonary artery rehabilitation and subsequent unifocalization with a central shunt. Colored rendering for Frontal (A), Posterior (B), Lateral (C), and Top (D) views obtained from CT images with semi-automatic 3D segmentation process.
Figure 5
Figure 5
Preoperative CT imaging of the patient who underwent one-stage unifocalization and repair. Colored rendering for Frontal (A), Posterior (B), Lateral (C), and Top (D) views obtained from CT images with semi-automatic 3D segmentation process.
See this image and copyright information in PMC

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