The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries
Matteo Trezzi,
Enrico Cetrano,
Sonia B. Albanese,
Luca Borro,
Aurelio Secinaro,
Adriano Carotti
Affiliations
Matteo Trezzi
Units of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, Italy
Enrico Cetrano
Units of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, Italy
Sonia B. Albanese
Units of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, Italy
Luca Borro
Advanced Cardiothoracic Imaging Unit, Department of Imaging, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, Italy
Aurelio Secinaro
Advanced Cardiothoracic Imaging Unit, Department of Imaging, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, Italy
Adriano Carotti
Units of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, Italy
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.
