Background
Unroofed coronary sinus syndrome (URCSS) is a spectrum of cardiac anomalies in which
part (partial type) or the entire common wall (complete type) between the coronary
sinus (CS) and left atrium is absent. It is commonly associated with a persistent
left superior vena cava (PLSVC). The PLSVC can even anomalously connect to the left
atrium in complete type anomaly. URCSS has been reported to be associated with delayed
diagnosis and life-threatening cerebral injury. The purpose of the present study was
to review our experience with surgical correction of this often-concealed malformation
and discuss methods for reducing diagnostic omission.
Methods
Thirty-six patients with URCSS who underwent surgical treatment from 1998 to 2015
in our institution were enrolled in this retrospective analysis.
Results
The mean age of the patients was 21.8 ± 20.9 years. Type I anomalies were found in 15 (41.7%) patients, type II in 4 (11.1%),
type III in 6 (16.7%), and type IV in 11 (30.6%). A patient presenting with URCSS
without other defects was identified in six (16.7%). One patient (2.8%) had a history
of paradoxical embolisation. Twenty-five patients (69.4%) were diagnosed before surgery.
Ten patients (27.8%) were diagnosed with URCSS intraoperatively. One patient (2.8%)
was diagnosed with URCSS by follow-up echocardiography after device occlusion for
atrial septal defect. Coronary sinus ostium dilation was the most important clue leading
to partial type lesion disclosure intraoperatively. The anomalous PLSVC of 15 patients
was repaired using roof tunnel technique in four (26.7%) cases, baffle redirection
in eight (53.3%) cases, posterior left atrial wall plicating in two (13.3%) cases,
and extracardiac bypass in one (6.7%) case. Postoperative complete atrioventricular
block occurred in one patient (2.8%). One patient died postoperatively (2.8%). All
patients are doing well after discharge from hospital.
Conclusions
Diagnostic suspicion can contribute to lesion disclosure. Surgical correction has
good long-term outcomes and low risks.
Keywords
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Article info
Publication history
Published online: June 22, 2017
Accepted:
May 27,
2017
Received in revised form:
February 13,
2017
Received:
November 17,
2016
Identification
Copyright
© 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
