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Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus

Published online by Cambridge University Press:  24 October 2007

Christine H. Attenhofer Jost ,
Heidi M. Connolly ,
Gordon K. Danielson ,
Joseph A. Dearani ,
Carole A. Warnes  and
A. Jamil Tajik
Christine H. Attenhofer Jost
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
Heidi M. Connolly*
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
Gordon K. Danielson
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
Joseph A. Dearani
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
Carole A. Warnes
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
A. Jamil Tajik
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
*
Correspondence to: Heidi M. Connolly MD, Division of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester MN 55905, USA. E-mail: connolly.heidi@mayo.edu
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Abstract

Objective

To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with partial fenestrations of the coronary sinus, a rare congenital disorder.

Background

Partial fenestrations of the walls that usually separate the coronary sinus from the left atrium, also known as partial unroofing of the coronary sinus, may result in increased flow of blood to the lungs, cyanosis, or bidirectional shunting. The diagnosis is important, but difficult to confirm.

Methods

We reviewed retrospectively the clinical, echocardiographic, operative, and follow-up data on 25 patients with partial fenestration of the coronary sinus. The patients had a median age of 8 years, and underwent cardiovascular surgery at Mayo Clinic between 1958 and 2003.

Results

The initial diagnosis of a fenestration of the coronary sinus was made by the surgeon at repair of other congenital cardiac anomalies, by cardiac catheterization, or by echocardiography. In 14 patients, fenestration was missed during previous cardiovascular surgery. The most common associated cardiac lesions were atrial septal defects within the oval fossa, persistent left or right superior caval veins, and pulmonary or tricuspid atresia. In 7 patients, the symptoms were at least partially attributable to the fenestration, and included dyspnea, cerebral abscess, transient ischaemic attacks, and cyanosis. The fenestration was addressed surgically in 23 patients, and consisted of closure of the mouth of the coronary sinus, creation of an intra-atrial baffle, or direct repair of the fenestration. Death occurred in 1 patient due to complications unrelated to the repair. In the 24 early survivors, who have been followed up for a median of 85 months, 1 patient has died after a third reoperation for complex congenital cardiac disease.

Conclusions

Fenestrations of the coronary sinus are often missed in the preoperative evaluation of congenitally malformed hearts. When associated with right heart hypoplasia, atrial septal defect, or persistent superior caval vein, fenestrations of the coronary sinus should be considered as a possible additional finding. Once the diagnosis has been made, repair is straightforward.

Keywords

Congenital heart diseasediagnosisechocardiographyheart surgery
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Issue 6 , December 2007 , pp. 592 - 600
Copyright
Copyright © Cambridge University Press 2007

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