Background: Anomalous coronary artery originating from the opposite sinus of Vasalva with interarterial course (ACAOS-IAC) is associated with sudden cardiac death (SCD) in young athletes. If identified in adulthood prognosis is usually more benign, resulting in a dilemma regarding revascularization.
Methods: This is a retrospective observational single-center study, including adults with ACAOS-IAC. Medical records between 2012 and 2019 were reviewed for management approach, mortality, cardiac death and coronary related adverse events. Coronary computed tomographic angiography (CCTA) were reviewed. We provide a literature review in regard to clinical outcome.
Results: We identified 40 patients with ACAOS-IAC (mean age 51). Presentation was acute in 7/40 (18%). Ischemia detection with single photon emission tomography (SPECT), cardiac magnetic resonance (CMR) or dobutamine stress echocardiography were performed in 25/40 (63%) patients. Ischemia in the vascular territory of the anomaly was present in 2/25 (8%). In 39/40 (98%) patients were treated expectative. During median follow-up of 2.7 years (IQR 1.5-5.3) no cardiovascular death was observed. Mortality occurred in 1/40 (3%) and coronary related adverse events in 2/40 (5%). We identified 20 studies describing 1194 patients. Revascularization was performed in 376/1154 (32.6%) patients. Mortality stratified for clinical management was 23/431 (5.3%) in the non-revascularization versus 16/253 (6.3%) in the revascularization group during 4.0 years follow-up (weighted median). Cause of death was cardiovascular in 10/596 (1.7%) in 4.2 years (weighted median) follow up.
Conclusions: Both revascularization and non-invasive management have good prognosis in adults with ACAOS-IAC during early follow up. There is need for guidelines and long-term surveillance.
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http://dx.doi.org/10.1016/j.ijcard.2021.04.005 | DOI Listing |
Ann Thorac Surg Short Rep
December 2024
Children's Heart Institute, Children's Memorial Hermann Hospital, Houston, Texas.
A patient with known pulmonary atresia and intact ventricular septum and ductal stent presented with low cardiac output and arrythmia. Intraoperatively, the patient was found to have an anomalous left coronary artery arising from the pulmonary artery. After reimplantation of the left coronary artery to the aortic root and placement of a central shunt, the patient progressed well and was discharged home.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan.
Undetected coronary anomalies at Norwood procedure are associated with poor prognosis due to inadequate myocardial protection. We report a case of anomalous origin of the right coronary artery from the main pulmonary artery trunk with hypoplastic left heart syndrome and aortic atresia. Although, during bilateral pulmonary artery banding as initial palliation, the proximity between the right coronary artery origin and the aortic root made a visual diagnosis difficult, it was diagnosed using computed tomography before the Norwood procedure.
View Article and Find Full Text PDFJTCVS Open
December 2024
Division of Pediatric Cardiac Surgery, Heart, Vascular and Thoracic Institute, Cleveland Clinic Children's Hospital, Cleveland, Ohio.
Objective: The study objective was to assess longitudinal postoperative health-related quality of life among patients with adult congenital heart disease facilitated by a novel electronic medical record-based patient-reported outcomes follow-up platform.
Methods: From January 2022 to October 2023, 559 patients with adult congenital heart disease underwent cardiac surgery; 491 (88%) completed a 23-element health-related quality of life questionnaire covering 3 domains (physical, mental, and social) yielding 911 assessments. Automated questionnaires via electronic medical record were sent at 7 days preoperatively and postoperatively at 1, 3, 6, and 12 months.
Pediatr Cardiol
January 2025
Department of Pediatrics, Cincinnati Children's Hospital Medical Center, The Heart Institute, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Cardiac-Urogenital Syndrome (CUGS) is a recently identified genetic disease characterized by urogenital, diaphragmatic, ophthalmic, and cardiac abnormalities caused by heterozygous pathogenic variants in the Myelin Regulatory Factor (MYRF) gene. The complete spectrum of disease characteristics and prevalence is not yet defined. This report documents the first known cases of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in MYRF-associated Cardiac-Urogenital Syndrome (MYRF-CUGS).
View Article and Find Full Text PDFPediatr Cardiol
January 2025
Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München (DHM), Technische Universität München (TUM), Lazarettstr. 36, 80636, Munich, Germany.
Anomalous origin of coronary arteries from the pulmonary artery (ACAPA) are rare but clinically significant condition with high mortality if left untreated. Even more rarely, ACAPA is associated with other congenital heart defects. From 1974 to 2024, 120 patients with anomalous coronary arteries connected to the pulmonary artery were retrospectively analyzed.
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