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

Christine H. Attenhofer Jost, Heidi M. Connolly, Gordon K. Danielson, Joseph A. Dearani, Carole A. Warnes, A. Jamil Tajik

Research output: Contribution to journalArticle

22 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)592-600
Number of pages9
JournalCardiology in the Young
Volume17
Issue number6
DOIs
StatePublished - Dec 2007

Fingerprint

Coronary Sinus
Venae Cavae
Cyanosis
Atrial Heart Septal Defects
Veins
Tricuspid Atresia
Pulmonary Atresia
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Brain Abscess
Transient Ischemic Attack
Cardiac Catheterization
Heart Atria
Reoperation
Dyspnea
Survivors
Echocardiography
Mouth
Heart Diseases
Lung

Keywords

  • Congenital heart disease
  • Diagnosis
  • Echocardiography
  • Heart surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Attenhofer Jost, C. H., Connolly, H. M., Danielson, G. K., Dearani, J. A., Warnes, C. A., & Jamil Tajik, A. (2007). Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus. Cardiology in the Young, 17(6), 592-600. https://doi.org/10.1017/S1047951107001412

Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus. / Attenhofer Jost, Christine H.; Connolly, Heidi M.; Danielson, Gordon K.; Dearani, Joseph A.; Warnes, Carole A.; Jamil Tajik, A.

In: Cardiology in the Young, Vol. 17, No. 6, 12.2007, p. 592-600.

Research output: Contribution to journalArticle

Attenhofer Jost, CH, Connolly, HM, Danielson, GK, Dearani, JA, Warnes, CA & Jamil Tajik, A 2007, 'Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus', Cardiology in the Young, vol. 17, no. 6, pp. 592-600. https://doi.org/10.1017/S1047951107001412
Attenhofer Jost CH, Connolly HM, Danielson GK, Dearani JA, Warnes CA, Jamil Tajik A. Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus. Cardiology in the Young. 2007 Dec;17(6):592-600. https://doi.org/10.1017/S1047951107001412
Attenhofer Jost, Christine H. ; Connolly, Heidi M. ; Danielson, Gordon K. ; Dearani, Joseph A. ; Warnes, Carole A. ; Jamil Tajik, A. / Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus. In: Cardiology in the Young. 2007 ; Vol. 17, No. 6. pp. 592-600.
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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.",
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AU - Warnes, Carole A.

AU - Jamil Tajik, A.

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N2 - 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.

AB - 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.

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KW - Echocardiography

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