Congenital Coronary Artery Fistula in Children:A Review of 28 Cases with Clinical and Imaging Outcomes

Background:Congenital coronary artery fistula(CCAF)is a rare anomaly.Treatment strategies tend to close the defect with a symptomatic and significant shunt,primarily based on expert consensus and case series.Results for long-term follow-up in children are limited Methods:We conducted a retrospective study to assess clinical and imaging outcomes of children with CCAF at Siriraj Hospital,Thailand during 2000–2020.Patients with single ventricle were excluded.Treatment strategies[surgical closure(SC),and percutaneous closure(PC)]were classified and the clinical outcomes at the follow-up in 2021,including coronary thrombosis,myocardial ischemia,and the results of cardiovascular imaging were reviewed.Results:Twenty-eight children with CCAF were included in the study.The median age at diagnosis was 2.5 years(2 days–18 years).Presenting symptoms were audible murmur(82%)and heart failure(35%).Most of fistulae arose from the right coronary artery(12/28)and exited at the right atrium(11/28).In recent visits(0.5–14 years follow-up),six patients with asymptomatic small CCAF were managed by watchful follow-up without complications.PC was primarily treated in 11 children:7 underwent successful procedures;1 had a residual shunt and required re-intervention;1 had ischemic symptoms immediately after the procedure with left coronary occlusion that required device removal plus SC and 2 were technically unable to place the device,requiring SC.Four patients were waiting for interventions(1 PC and 3 SC).Cardiovascular imaging surveillance that followed closure demonstrated asymptomatic thrombus formation in three patients(1 PC and 2 SC).No mortality presented.Conclusion:CCAF with significant shunt is indicated to close either SC or PC.Ischemic events are rare but have been reported after closure.In addition,thrombus formation should be watched for post-intervention.Surveillance with cardiovascular imaging is recommended after defect closure(ideally 1–5 years post closure),or at interval follow-ups in patients with symptoms to evaluate possible recanalization,thrombus,or ischemia.Life-long clinical and echocardiographic follow-up is warranted.Watchful follow-up is acceptable for hemodynamically insignificant fistula without complication in the series.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

AIM To compare the behavior of pulmonary hypertension(PHT) associated with coronary artery fistulas(CAFs) between the Asian and Caucasian subjects.METHODS CAFs may be complicated with PHT secondary to leftto-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9(range 19-83) and 49.9 years(range 16-85), respectively. In both groups, right heart catheterization was the most commonly(95%) used method for determining pulmonary artery pressure. RESULTS From all of the reviewed subjects, PHT was found in 49 patients(23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization(27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.CONCLUSION The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Surgical Correction of Coronary Artery Ectasia Combining Congenital Coronary Artery Fistula

Background:Coronary artery ectasia(CAE)complicated with concomitant congenital coronary artery fistula(CCAF)is rare.This study characterizes the clinical characteristics of CAE combining CCAF,and reports a single-institution experience with surgical correction of CAE combining CCAF.Methods:A total of 24 symptomatic patients(8 males,median 52.5 years old)who underwent surgical correction of CAE combining CCAF in this center were reviewed.Based on the size of ectatic segment,the CAE were classified as a giant CAE(>20 mm,n=14)and a non-giant CAE(≤20 mm,n=10).Individualized surgical approaches were chosen.The patients were followed up for a median of 3.8 years.Results:The overwhelming majority of CAEs were solitary,and only 4.2%of CAEs were associated with multiple lesions.CAEs were predominantly located in the right coronary artery with predilection to women more than to men(2:1).95.8%of patients with the CCAF had single fistula defect.The right atrium was the most frequent drainage site(33.3%)followed by the left ventricle(25.0%).Surgical mortality was 4.2%.All 22 follow-up patients survived with recovery from symptoms and New York Heart Association(NYHA)functional class I-II.In 10 patients with non-giant CAEs undergoing closure of fistula alone,favorable in-hospital outcomes were recorded,but residual fistula(one patient)and acute inferior wall myocardial infarction related to intracoronary thrombosis(one patient)were observed at follow-up.In 11 patients with giant CAEs undergoing aneurysm resection plus distal bypass grafting at the time of closure of fistula,favorable in-hospital outcomes and encouraging midterm results were recorded.Additionally,in 3 patients with giant CAEs undergoing closure of fistula plus aneurysmal plication,adverse events occurred,including surgical death related to rupture of the ectatic segment(one patient),perioperative myocardial infarction caused by acute thromboembolism(one patient),nonfatal inferior wall myocardial infarction related to intracoronary thrombosis(one patient)at follow-up.Conclusion:Individualized surgical approaches based on the size and the location of ectatic coronary artery as well as fistula should be offered to symptomatic patients with CAE combining CCAF.

Surgical treatment of 52 patients with congenital coronary artery fistulas

Abstract:Objective To evaluate the outcome of surgical treatment for congenital coronary artery fistulas (CAF) in 52 patients seen between May 1988 and July 1999.Methods Fifty-two patients ranging in age from 9 months to 58 years (mean 15.7±16.4 years) were studied. Thirty-six patients had no other cardiac defects, 9 of those patients were more than 20 years old and presented with symptoms. Only one of 36 patients less than 20 years old had clinical findings before surgery. Sixteen patients had associated cardiac lesions. The site of fistula origin was the right coronary artery in 37 patients (71.2%), and the left coronary artery in 15 patients (28.8%). The sites of CAF drainage were the right ventricle, right atrium, left ventricle, left atrium and pulmonary artery in 22 (42.3%), 16 (30.8%), 6 (11.5%), 3 (5.8%), and 5 (9.6%) patients, respectively. The mean diameter of the fistula in 43 patients with single ostium was 7.34±4.12?mm.Results Cardiopulmonary bypass was used in all patients and no patient died. An arteriotomy was made on the anomalous coronary artery and the proximal opening of a fistula was closed within the vessel in 10 patients. Closure of the distal opening of a fistula draining into a cardiac chamber or pulmonary artery was performed in 26 patients. In 16 patients, both the proximal and distal opening were closed. Two and 3 distal opening of a fistula were found in 6 and 3 patients, respectively. No residual shunt was found before patients were discharged from the hospital. Forty patients were followed up for a mean period of 3.14±1.84 years. The remaining 12 patients could not be contacted during follow-up. No clinical symptoms were found in those patients during follow-up but one patient still presented with ST-T change. Conclusion Early and properly surgical management is safe and effective for congenital coronary fistula.