title: Isolated right ventricular hypoplasia caused by giant aneurysm of right coronary artery to left ventricle fistula in an adult: a case report.
abstract: BACKGROUND
  Right ventricular hypoplasia (RVH) is often caused by tricuspid valve atresia and pulmonary valve atresia. this condition leads to low right ventricular blood volume and right ventricular maldevelopment. But, in adults, the main cause of RVH may also be associated with alloplasia of the right coronary artery, which results in an insufficient blood supply to the right ventricular myocardium. Isolated RVH caused by a right coronary artery fistula is very rare and requires immediate treatment.
  
  
  METHODS
  We herein report a case involving a 45-year-old man who presented with isolated RVH caused by a giant aneurysm from the right coronary artery to a left ventricle fistula. Echocardiography showed that the right coronary artery was extremely tortuous and obviously dilated with a huge aneurysm. A fistula drained from the right coronary artery into the left ventricle. Moreover, the right heart chamber was significantly collapsed due to extrinsic compression of multiple tortuous, dilated vascular structures. The patient was referred to cardiac surgery. The giant aneurysm was resected, and the proximal and distal openings were closed directly. The fistula was also closed directly, and bypasses were constructed sequentially from the ascending aorta to three branches of the right coronary artery.
  
  
  CONCLUSIONS
  Although standard therapeutic strategies of isolated RVH secondary to a right coronary artery fistula are not well established because of the rarity of this condition, our clinical results show that diagnostic echocardiography, coronary artery angiography, and cardiac computed tomography angiography followed by surgical treatment may be an effective management option.
authors.affiliation: Echocardiography and Vascular Ultrasound Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, China.
authors.forename: Fawang
authors.initials: F
authors.lastname: Zhu
authors.affiliation: Echocardiography and Vascular Ultrasound Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, China.
authors.forename: Zhelan
authors.initials: Z
authors.lastname: Zheng
authors.affiliation: Echocardiography and Vascular Ultrasound Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Yao
authors.affiliation: Echocardiography and Vascular Ultrasound Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, China. xuyuntin@163.com.
authors.forename: Yun
authors.initials: Y
authors.lastname: Mou
authors.affiliation: Echocardiography and Vascular Ultrasound Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Cheng
authors.affiliation: Echocardiography and Vascular Ultrasound Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, China.
authors.forename: Huanhuan
authors.initials: H
authors.lastname: Gao
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods; D003323:Coronary Aneurysm / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D017023:Coronary Angiography; D003331:Coronary Vessels / Q000000981:diagnostic imaging* / Q000601:surgery; D004452:Echocardiography; D005402:Fistula / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging* / Q000209:etiology; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging* / Q000601:surgery; D006801:Humans; D021621:Imaging, Three-Dimensional; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed
keywords: Cardiac computed tomography angiography; Coronary artery angiography; Echocardiography; Left ventricle; Right coronary artery fistula; Right ventricular hypoplasia