ABC | Volume 112, Nº2, February 2019

Case Report Large Bilateral Coronary Artery Fistula: 10-year Follow-up in Clinical Treatment Rodrigo Melo Kulchetscki, 1 Luka David Lechinewski, 2 Luciana Oliveira Cascaes Dourado, 1 Whady Armindo Hueb, 1 Luiz Antonio Machado César 1 Instituto do Coração (InCor) do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), 1 São Paulo, SP – Brazil Hospital da Irmandade da Santa Casa de Misericórdia de Curitiba, 2 Curitiba, PR – Brazil Mailing Address: Rodrigo M. Kulchetscki • Av. Dr. Eneas de Carvalho Aguiar, 44 andar AB, Unidade Clínica de Coronariopatia Crônica. Postal Code 05403-000, São Paulo, SP – Brazil E-mail: r.kulchetscki@hc.fm.usp.br , r.kulchetscki@gmail.com Manuscript received March 19, 2018, revised manuscript July 02, 2018, accepted July 02, 2018 Keywords Arterio-Arterial Fistula/diagnosis; Coronary Angiography; Diagnostic, Imaging; Radionuclide Imaging; Coronary Vessel Anomalies; Mitral Valve Insufficiency; Myocardial Ischemia DOI: 10.5935/abc.20180267 We report on the 10-year evolution of an asymptomatic patient with a large bilateral coronary artery-pulmonary artery fistula for whom clinical treatment was chosen. Published previously, 1 the report reinforces the need for treatment individualization in patients with moderate coronary fistulas. Case Report A 59-year-old asymptomatic female patient, with a diagnosis of a large bilateral coronary-pulmonary artery fistula made in 2007 was investigated after a cardiac murmur was identified on a routine examination. At the time, conservative treatment was chosen. Cardiac auscultation showed a more audible systolic-diastolic murmur in the upper left sternal border, with a more audible component in systole. There were no other findings in the cardiological physical examination or even the overall segmental examination. The patient had no comorbidities at the time, except for a prior history of smoking (10-pack-years). During the evolution, at the annual outpatient follow-up, she had diagnoses of dyslipidemia, glucose intolerance and depression. At the last consultation, in 2017, the patient was asymptomatic. She used atenolol 25 mg/ day, metformin 850 mg/day, atorvastatin 20 mg/day and sertraline 50 mg/day. The examinations performed after 10 years of follow‑up were compared with those at the time of diagnosis. The current echocardiogram showed right coronary (RC) with 4 mm of diameter at the origin and 7 mm in the middle third; the left main coronary artery (LMCA) with 8 mm. The patient had a fistulous trajectory with tortuous flow communicating both coronaries with the pulmonary trunk, without the presence of pulmonary hyperflow. Additionally, the evolution of mitral regurgitation showed to be of an important degree. Table 1 shows the echocardiographic parameters during follow-up. Myocardial scintigraphy with dipyridamole and 99m-technetium-sestamibi showed no changes in perfusion, as well as the previous examinations performed in 2007 and 2011. The ergospirometry treadmill test (modified Balke protocol, 3.4mph), lasting 7minutes and 38 seconds, was maximal (109% of maximal HR), with VO 2 peak of 22.4 mL/kg/min (87% of predicted VO 2 ). The angiotomography of the coronary arteries was performed in 2017 and the comparison with the 2007 examination can be seen in Figure 1. The finding of a systemic-pulmonary fistula persists, in the RC + ADA with the LMCA, described as the presence of a high-caliber branch emerging from the right coronary artery origin, with a tortuous trajectory, surrounding the pulmonary trunk anteriorly and communicating with the proximal third of the anterior descending artery. It shows communication with the pulmonary trunk, associated with two aneurysms along its trajectory, measuring 19x16 mm and 14x13 mm. There is no pulmonary dilation or other signs suggesting hemodynamic repercussion. Total coronary calcium score of 246 (Agatston), corresponding to the 99 th percentile for the age group and gender, and absence of significant coronary luminal reduction were also observed. Discussion Coronary fistulas (CFs), abnormal communications between one or more coronary arteries with some cardiac or thoracic structure, usually congenital in origin, 2 have a prevalence of 0.05% to 0.88%, depending on the diagnostic method used. 3 They originate from one or more branches of the coronary arteries, and the pulmonary trunk is the most frequent termination of bilateral CFs. 2,4 They may be associated with mitral regurgitation/mitral valve disease – a finding present in this case – atrial and/or ventricular septal defect, pulmonary stenosis and atresia. 5 In the adult population, 75% are symptomatic, with chest pain and dyspnea being the most frequently complaints. Heart murmur is observed in 37% of patients at clinical examination. 5 Patient evolution seems to be quite variable and depends on the size and hemodynamic repercussion of the CF, in addition to associated malformations. Long-term follow-up 2,4 shows that patients can progress from being asymptomatic to symptoms of heart failure due to decreased ejection fraction, left atrial enlargement and pulmonary hypertension, and a few with coronary aneurysm, which is associated mainly with unilateral fistulas. Coronary aneurysms may favor coronary rupture and may also generate ischemia through the flow steal mechanism. 5,6 The ideal treatment of CFs remains uncertain, especially regarding the moderate and asymptomatic cases. 211