title: First report of atretic coronary sinus stenting in a 5-kg infant resulting in dramatic improvement of ventricular function in functional single ventricle.
abstract: The patient was a male infant with L-transposition of great arteries (L-TGA), Ebstein's anomaly of the tricuspid valve, subvalvar aortic stenosis, ventricular septal defect (VSD), hypoplastic right ventricle, arch hypoplasia, and congenital complete heart block. He underwent a Norwood procedure, aortic arch repair, permanent pacemaker implantation, and a 3.5-mm aortopulmonary shunt at 4 days of age. At the time of his surgery, left ventricular function was in the normal range (ejection fraction [EF] = 67%). However by 3 months of age, he was noted to have developed moderate-severe biventricular dysfunction (left ventricular ejection fraction [LVEF] = 34%). Atresia of the coronary sinus with a small left superior venacava (LSVC) and a bridging vein was discovered during cardiac catheterization at this time. The coronary sinus mean pressure was 17 mm Hg, and the common atrial mean pressure was 6 mmHg. We opened the atretic coronary sinus ostium using radiofrequency ablation and stent placement. There was dramatic improvement in ventricular function observed over a 2-month period. Follow-up cardiac catheterization 5 months later revealed the stent in the coronary sinus to be widely patent with no intimal buildup, and the ventricular function was normal (LVEF = 58%). The patient had a bidirectional Glenn procedure with an uncomplicated postoperative course and is currently awaiting Fontan completion.
authors.affiliation: Department of Pediatric Cardiology, Rady Children's Hospital, University of California San Diego, San Diego, Calif, USA.
authors.forename: Howaida
authors.initials: H
authors.lastname: El-Said
authors.forename: Sanjeet
authors.initials: S
authors.lastname: Hegde
authors.forename: John
authors.initials: J
authors.lastname: Moore
mesh_terms: D000015:Abnormalities, Multiple; D015906:Angioplasty, Balloon, Coronary / Q000295:instrumentation*; D001835:Body Weight; D002932:Cineangiography; D017023:Coronary Angiography; D054326:Coronary Sinus / Q000002:abnormalities* / Q000503:physiopathology; D003330:Coronary Vessel Anomalies / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015641:Radiography, Interventional / Q000379:methods; D020127:Recovery of Function; D015607:Stents; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right
keywords: Atretic Coronary Sinus; Functional Single Ventricle; Stent Implantation