Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect
The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease’s rarity. We reviewed 69 cases from 42 studies to reveal...
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MDPI AG
2023-01-01
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| Series: | Journal of Cardiovascular Development and Disease |
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| Online Access: | https://www.mdpi.com/2308-3425/10/1/17 |
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| author | Naofumi F. Sumitomo Kazuki Kodo Tadashi Inoue Takayuki Oyanagi Hiroyuki Yamagishi |
| author_facet | Naofumi F. Sumitomo Kazuki Kodo Tadashi Inoue Takayuki Oyanagi Hiroyuki Yamagishi |
| author_sort | Naofumi F. Sumitomo |
| collection | DOAJ |
| description | The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease’s rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease. |
| first_indexed | 2024-03-09T12:13:50Z |
| format | Article |
| id | doaj.art-678424435107469c9ee72cf32d06c2c1 |
| institution | Directory Open Access Journal |
| issn | 2308-3425 |
| language | English |
| last_indexed | 2024-03-09T12:13:50Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj.art-678424435107469c9ee72cf32d06c2c12023-11-30T22:49:11ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252023-01-011011710.3390/jcdd10010017Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal DefectNaofumi F. Sumitomo0Kazuki Kodo1Tadashi Inoue2Takayuki Oyanagi3Hiroyuki Yamagishi4Department of Pediatrics, Keio University School of Medicine, Tokyo 1608582, JapanDepartment of Pediatrics, Keio University School of Medicine, Tokyo 1608582, JapanDepartment of Pediatrics, Keio University School of Medicine, Tokyo 1608582, JapanDepartment of Pediatrics, Keio University School of Medicine, Tokyo 1608582, JapanDepartment of Pediatrics, Keio University School of Medicine, Tokyo 1608582, JapanThe existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease’s rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease.https://www.mdpi.com/2308-3425/10/1/17coronary-to-pulmonary artery fistulapulmonary atresia with ventricular septal defecttetralogy of Fallotmajor aortopulmonary collateral arteries |
| spellingShingle | Naofumi F. Sumitomo Kazuki Kodo Tadashi Inoue Takayuki Oyanagi Hiroyuki Yamagishi Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect Journal of Cardiovascular Development and Disease coronary-to-pulmonary artery fistula pulmonary atresia with ventricular septal defect tetralogy of Fallot major aortopulmonary collateral arteries |
| title | Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect |
| title_full | Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect |
| title_fullStr | Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect |
| title_full_unstemmed | Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect |
| title_short | Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect |
| title_sort | clinical characteristics of coronary to pulmonary artery fistula in patients with pulmonary atresia and ventricular septal defect |
| topic | coronary-to-pulmonary artery fistula pulmonary atresia with ventricular septal defect tetralogy of Fallot major aortopulmonary collateral arteries |
| url | https://www.mdpi.com/2308-3425/10/1/17 |
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