[Pediatric case of congenital coronary artery fistula; surgical result and late changes in coronary artery aneurysm].

Congenital coronary artery fistula is an uncommon heart anomaly involving the coronary arteries. We report here a case of a 4-year-old boy who had a coronary fistula from the right coronary artery to the right ventricle, with a coronary aneurysm. He was asymptomatic, but the calculated ratio of pulmonary blood flow to systemic blood flow was shown to be high [pulmonary flow (Qp)/systemic flow(Qs)=1.78]. The coronary angiography showed that the right coronary artery was dilated beginning at the ostium and had an aneurysm at the acute marginal portion. A large spherical aneurysm approximately 20 mm in diameter was found to have been connected with coronary fistula opening into the right ventricle. Surgical repair by closure of the fistula under direct vision, partial resection and suture closure of the aneurysm was performed. Plication of the proximal portion of the right coronary artery was not performed, and the diffusely dilated artery was left untouched. After this operation, he recovered well under anticoagulant treatment with warfarin and aspirin. Postoperative angiography was performed 17 months after the surgery to evaluate morphological changes in the coronary artery. The angiography confirmed the closure of the fistula and the regression of coronary artery dilatation.

Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.

BACKGROUND AND OBJECTIVE: Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. PATIENTS AND METHODS: Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods. RESULTS: Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications. CONCLUSIONS: Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.

Endovascular Transjugular Occlusion of Congenital Intrahepatic Portosystemic Venous Shunt Using Simultaneous Fluoroscopy and Transabdominal Ultrasound Guidance: Report of 2 Cases.

Congenital intrahepatic portosystemic venous shunts (CIPVS) are rare anomalies that can be detected before birth or in early infancy or later in life. Symptomatic shunts are treated as they carry high risk of complications like hepatic encephalopathy. Various treatment options include surgery, endovascular embolization, and percutaneous closure devices. We treated 2 infants with CIPVS successfully by endovascular embolization of the shunt using vascular plug through transjugular route. Transabdominal ultrasound guidance in addition to fluoroscopy was used at the time of vascular plug placement. We emphasize that the use of transabdominal ultrasound during endovascular occlusion enhances the safety and technical success rate.

Successful correction of congenital giant right coronary artery aneurysm with fistula to left ventricle.

A 26-year-old male presented with radiographical evidence of enlargement of the right side of the heart. Echocardiography and computed tomography-scan revealed a diffuse, hugely enlarged right coronary artery (RCA) aneurysm, with the distal portion flowing directly into the left ventricle (LV). A radical correction operation was performed successfully. The RCA was reconstructed with an autograft of a greater saphenous vein after the aneurysm was removed and the RCA-to-LV fistula was closed. The postoperative course was uneventful and the patient was discharged on 12th day after operation.

Absent pulmonary valve, imperforate tricuspid valve, and dysplastic right ventricle.

We report a very rare case involving an absent pulmonary valve, an imperforate tricuspid valve, a ventricular septal defect (VSD), and a dysplastic right ventricle in a patient who underwent a Fontan operation successfully. Upon performing a bidirectional cavopulmonary anastomosis, we closed the VSD and coronary artery-to-right ventricle fistulae, and plicated the right ventricular free wall in order to obliterate the right ventricular cavity.

Congenital right pulmonary artery-to-left atrial fistula.

A 23-year-old man presented with cyanosis since birth. Precordial echocardiography failed to detect any structural heart abnormality except for enlarged left atrium. Pulmonary angiography revealed a type I right pulmonary artery-to-left atrial fistula. Simple ligation of the fistula without cardiopulmonary bypass was performed successfully, and the cyanosis disappeared postoperatively.

The congenital porto-caval fistula: a unique presentation and novel intervention.

Congenital porto-caval fistulas are uncommon vascular malformations with a varied clinical presentation beginning in infancy and spanning late adulthood. We report a 14-year-old male who presented with dyspnea and cough. His past medical history was significant for a chronic non-immune hemolytic anemia, thrombocytopenia, coagulopathy and a learning disability. He was found to have severe pulmonary hypertension and hyperammonemia associated with a large congenital porto-caval fistula. The abnormal vessel was occluded via endovascular covered stent placement in the vena cava. His pulmonary hypertension has improved remarkably while his chronic anemia, thrombocytopenia and ammonia have normalized to allow improved cognitive performance.