The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries
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...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2022-04-01
|
| Series: | Children |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2227-9067/9/4/515 |
| _version_ | 1797436312305795072 |
|---|---|
| author | Matteo Trezzi Enrico Cetrano Sonia B. Albanese Luca Borro Aurelio Secinaro Adriano Carotti |
| author_facet | Matteo Trezzi Enrico Cetrano Sonia B. Albanese Luca Borro Aurelio Secinaro Adriano Carotti |
| author_sort | Matteo Trezzi |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T10:59:50Z |
| format | Article |
| id | doaj.art-10d70de32bf542b6b29ee00c909a4047 |
| institution | Directory Open Access Journal |
| issn | 2227-9067 |
| language | English |
| last_indexed | 2024-03-09T10:59:50Z |
| publishDate | 2022-04-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj.art-10d70de32bf542b6b29ee00c909a40472023-12-01T01:18:50ZengMDPI AGChildren2227-90672022-04-019451510.3390/children9040515The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral ArteriesMatteo Trezzi0Enrico Cetrano1Sonia B. Albanese2Luca Borro3Aurelio Secinaro4Adriano Carotti5Units of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, ItalyUnits of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, ItalyUnits of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, ItalyAdvanced Cardiothoracic Imaging Unit, Department of Imaging, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, ItalyAdvanced Cardiothoracic Imaging Unit, Department of Imaging, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, ItalyUnits of Cardiac Surgery, Department of Cardiac Surgery, Cardiology, Heart and Lung Transplantation, Bambino Gesù Children’s Hospital IRCCS, 00165 Rome, ItalyPulmonary 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.https://www.mdpi.com/2227-9067/9/4/515Fallot’s tetralogypulmonary atresia with ventricular septal defectmajor aortopulmonary collateral arteriesunifocalizationrehabilitation |
| spellingShingle | Matteo Trezzi Enrico Cetrano Sonia B. Albanese Luca Borro Aurelio Secinaro Adriano Carotti The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries Children Fallot’s tetralogy pulmonary atresia with ventricular septal defect major aortopulmonary collateral arteries unifocalization rehabilitation |
| title | The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries |
| title_full | The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries |
| title_fullStr | The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries |
| title_full_unstemmed | The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries |
| title_short | The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries |
| title_sort | modern surgical approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries |
| topic | Fallot’s tetralogy pulmonary atresia with ventricular septal defect major aortopulmonary collateral arteries unifocalization rehabilitation |
| url | https://www.mdpi.com/2227-9067/9/4/515 |
| work_keys_str_mv | AT matteotrezzi themodernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT enricocetrano themodernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT soniabalbanese themodernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT lucaborro themodernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT aureliosecinaro themodernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT adrianocarotti themodernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT matteotrezzi modernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT enricocetrano modernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT soniabalbanese modernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT lucaborro modernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT aureliosecinaro modernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries AT adrianocarotti modernsurgicalapproachtopulmonaryatresiawithventricularseptaldefectandmajoraortopulmonarycollateralarteries |