Coronary artery fistula between single right coronary artery and right pulmonary artery: a case report and literature review

Xie et al. BMC Cardiovascular Disorders (2015) 15:171 DOI 10.1186/s12872-015-0166-2 CASE REPORT Open Access Coronary artery fistula between single right coronary artery and right pulmonary artery: a case report and literature review Li-Jian Xie, Li Zhang, Ting-Ting Xiao and Jie Shen* Abstract Background: Coronary artery fistula and single coronary artery are two different rare congenital anomalies. The cases with co-existed the two anomalies are more rare. To the best of our knowledge with literature review, the coronary artery fistula between single right coronary artery and right pulmonary artery has not been previously reported. Case presentation: In the present article, we report a Chinese patient (a 8-month-old male) who presented cyanosis when cried and heart murmur. The cardiac angiography confirmed coronary artery fistula between single coronary artery arising from the right aortic sinus and right pulmonary artery. Furthermore, the right pulmonary artery was interrupted with main pulmonary artery and the pulmonary blood supplied by single right coronary artery. Following the surgical procedure, the anomalous fistula vessel was cut and sutured. The right pulmonary artery was reconstructed to connect with main pulmonary artery. The patient had an uneventful postoperative course and discharged. Then we reviewed the related literature with Medline and Pubmed databases for further details. Conclusion: We believe our patient is the very particular case about coronary artery fistula combined with single coronary artery, and it is first reported with our literature review. As other coronary anomalies, coronary or aortic root angiography is the gold standard method for the diagnosis. Furthermore, early surgery is an optimal treatment in our case. Keyword: Coronary artery fistula, Single coronary artery, Angiography Background Coronary artery fistula (CAF) has been described as a direct connection between a coronary artery and one of the cardiac chambers, large vessels or other vascular structures [1]. This abnormality accounts for 0.27–0.40 % of all congenital cardiac defects [2]. Congenital anomalies of the coronary arteries occur in 0.4–2 % of the population [3]. It is well known that certain coronary artery anomalies, including single coronary artery (SCA), Bland-WhiteGarland syndrome, coronary aneurysm, and CAF, can be associated with fatal complications [4]. However, SCA combined with CAF is very rare and easily misdiagnosed. Here we report a rare case of a SCA arising from the right aortic sinus associated with CAF * Correspondence: Department of Cardiology, Shanghai Children’s Hospital, Shanghai Jiaotong University, Postal address: No. 355 Luding Road, Postcode: 200062 Shanghai, China with right pulmonary artery (RPA). Furthermore, RPA is interrupted with main pulmonary artery (MPA) and directly connected with SCA, combined with patent duct arteriosus (PDA) and atrial septal defect (ASD). The anomalies were successfully corrected with operation in our hospital. The case is first reported as we reviewed the literature with Medline and Pubmed databases. Case presentation A 8-month-old Chinese boy (Han race) was admitted with heart murmur and cyanosis when cried or exercised. The boy was the mother’s first child from her first pregnancy. He was breech delivered and his birth weight was 3.05 kg. His Apgar scores were not known. His development was nearly normal and the body weight was 8.0 kg until 8 month old. Physical examination found a grade 3/6 continuous murmur in the second to fourth intercostal space at the left sternal border. The electrocardiogram showed © 2015 Xie et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Xie et al. BMC Cardiovascular Disorders (2015) 15:171 sinus rhythm, right atrium and ventricle hypertrophy. The chest X-ray showed heart shade enlargement, cardiothoracic ratio was 66 %. Transthoracic echocardiography revealed SCA arising from the right aortic sinus and CAF with RPA, RPA was interrupted with MPA, ASD and PDA. Heart catheterization demonstrated left pulmonary artery (LPA) capillary wedge pressure of 13/2 mm Hg (mean 9), MPA pressure 80/35 mm Hg (mean 55), right ventricular (RV) pressure 88/2 mm Hg (mean 12), left ventricular (LV) pressure 81/5 mm Hg (mean 38), descending aorta artery pressure 74/41 mm Hg (mean 58). Oxygen saturation was 86 % in aorta artery, 62 % in superior vena cava and 64 % in MPA. The ratio of Qp/Qs was 1.09. The total pulmonary resistance was 4.2 wood and the pulmonary arteriolar resistance was 3.6 wood. Selective aortic root angiography showed a SCA arising from the right aortic sinus and a dilated and tortuous fistula between SCA and RPA (Fig. 1.1–2). Furthermore, the blood flow of RPA was supplied by SCA arising from the right aortic sinus (Fig. 1.1–2). Selective RV angiography showed the dilated MPA was connected with LPA and interrupted Page 2 of 5 with RPA (Fig. 1.3). So we made a diagnosis of SCA arising from the right aortic sinus with a CAF draining into the RPA, pulmonary hypertension and surgical correction was recommended (Additional file 1, avi style). Following median sternotomy, the pericardium was opened. The right atrium and ventricle were enlarged and MPA was dilated. An isolated SCA was found arising from the right aortic sinus and a tortuous anomalous vessel that ran to the RPA was identified, also, RPA was interrupted with MPA and RPA blood flow was supplied by CAF. After the cardiopulmonary bypass established, first,arterial duct was separated and sutured. Then the anomalous fistula vessel connected between SCA and RPA was separated and cut. The fistula was continuously sutured by 6/0 Prolene line. The RPA and MPA vascular anastomosis was successfully made. At last, the right atrium was opened and ASD was closed with continuous suture. The total time of bypass was 67 min. The patient had an uneventful postoperative course and discharged. On follow-up echocardiography, performed 2 weeks, 1 and 3 months after surgical treatment, no residual fistulous communications Fig. 1 1 and 2: Selective aortic root angiography showed a right SCA arising from the right coronary sinus and a dilated and tortuous fistula between SCA and RPA, with the LCx and LAD arising separately from the common trunk, and, the blood flow of RPA was supplied by right SCA (A-P and lateral position). 3: Selective RV angiography showed the dilated MPA was connected with LPA and (...truncated)