Abstract
OBJECTIVES: We conducted a systematic review to evaluate the clinical features, diagnosis, and treatment of coronary artery to pulmonary artery fistula (CPAF) and its associated aneurysms. METHODS: Our search encompassed MEDLINE/PubMed, Scopus, Google Scholar, and the Cochrane Collaboration Database from 1970 to 2025. RESULTS: A total of 461 cases were analysed. The volume of publications steadily increased after 2010. The left anterior descending coronary artery was the most common site of origin for CPAF. A total of 457 cases underwent coronary angiography confirming CPAF, with computed tomography coronary angiography being the most frequently used non-invasive imaging modality (190 cases, 41.2%). Stress tests were conducted in 78 cases (16.9%), revealing a coronary steal phenomenon associated with CPAF. Particularly, when comparing aneurysm CPAF to non-aneurysm CPAF, we found that older age is an independent risk factor for developing an aneurysm. For interventions, the symptomatic patients were more likely to close the fistula. We identified that 218 cases (49.7%) underwent surgical treatment, while 121 cases (27.6%) received percutaneous embolization. There were 8 reported deaths, resulting in a mortality rate of 1.8%. CONCLUSIONS: The clinical presentations of CPAF are often non-diagnostic. Computed tomography coronary angiography is the preferred method for visualizing the fistulas and their surrounding structures. Surgical treatment is beneficial in cases of aneurysm CPAF or with other surgical indications. Transcatheter closure of CPAF may be considered for selected individuals with favourable anatomy (PROSPERO Number CRD42025643603). CLINICAL REGISTRATION NUMBER: PROSPERO Number CRD42025643603.