Introduction:
Congenital coronary fistulas (CCF) are rare (0.002% in general population) however under-diagnosis is frequent. Patient and fistula characteristics vary among different populations, treatment is controversial and the impact in survival is not well established.
Purpose:
This study aimed to describe clinical characteristics, treatment received and long-term mortality among different options of treatment for CCF.
Methods:
We identified all cases of CCF diagnosed from January 2009 to January 2019 in a single center. Clinical data was obtained from medical records and mortality was assessed according to clinical checkups or by phone call. Long-term mortality was defined after at least one-year follow-up. Descriptive statistics were applied to all cases.
Results:
22 cases of CCF were diagnosed, medium age was 53.6 years, 65% were male and 31% had smoking history. At presentation 36% had angina and 27% had dyspnea. Only 13.6% had no significant past medical history, 20% had another congenital anomaly associated, 13.6 % had chronic heart failure and 45.4% were associated to coronary artery disease (CAD), usually anterior descending artery (ADA) was affected. Most frequent origin of fistula was from the ADA in 42.3% of the cases and drainage was frequent to the pulmonary artery in 53.4%, 80% had a single fistula and 45% presented small fistulas. Overall mortality was 13.6% (n=3) with median follow-up of 3.6 (1-9.9) years. Among patients treated, 72.8% of the patients received medical treatment with a 12.5% rate of long-term mortality, however only one patient died from cardiovascular cause (worsening heart failure). None of the patients who received interventional treatment (N=3) died at long-term, 13.6% had coronary artery bypass grafting for CAD (none had fistula ligation), only one patient died several years later of neurological cause.
Conclusions:
The most common origin of CCF was from the ADA and the drainage was common to the pulmonary artery. Long term mortality is low in patients with CCF despite treatment received, only one patient in the entire cohort died of cardiac disease even when 55% had medium to large coronary fistula and 86.4% had previous cardiovascular disease. Further studies are needed to establish treatment guidelines.
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