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Published on: October 28, 2020
Unexpected Severe Cyanosis Following Ventricular Septal Defect Closure and Repair of Right Ventricular Outflow Tract
Daniel Mohammadi1, Keaton Dubois2, Preeti Ramachandran3
1Department of Cardiothoracic Surgery, University of Kentucky, Lexington, KY, USA.
Insights
A congenital heart defect case highlights an undiagnosed unroofed coronary sinus defect causing cyanosis after ventricular septal defect repair. Prompt diagnosis and closure of this defect enabled successful patient recovery.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Echocardiography
Background:
- Ventricular septal defect (VSD) is a frequent congenital heart anomaly necessitating surgical intervention.
- Complex congenital heart disease can present diagnostic challenges, particularly during intraoperative management.
Purpose of the Study:
- To report a rare case of intraoperative cyanosis in an infant following ventricular septal defect (VSD) repair.
- To highlight the diagnostic utility of intraoperative transesophageal echocardiography in identifying an unroofed coronary sinus defect.
Main Methods:
- Intraoperative transesophageal echocardiography with agitated saline contrast study.
- Surgical exploration and repair of an unroofed coronary sinus defect.
Main Results:
- An infant with a large perimembranous VSD and pulmonary stenosis experienced severe cyanosis post-VSD closure.
- Intraoperative echocardiography revealed a significant atrial-level right-to-left shunt attributed to an unroofed coronary sinus defect.
- Successful closure of the coronary sinus defect resolved the shunt and enabled weaning from cardiopulmonary bypass.
Conclusions:
- An unroofed coronary sinus defect can be a critical, previously unrecognized cause of intraoperative shunt and cyanosis in infants undergoing VSD repair.
- Intraoperative echocardiography is invaluable for diagnosing such anomalies, guiding timely surgical correction and improving patient outcomes.
Abstract:
Ventricular septal defect (VSD) is a common congenital heart defect that often requires surgical closure. We present the case of a seven-month-old infant with a large perimembranous VSD, moderate pulmonary valve stenosis, and hypertrophied right ventricular muscle bundles who failed to wean from cardiopulmonary bypass due to severe cyanosis after VSD closure and muscle bundle resection. An intraoperative transesophageal echocardiogram was performed with an agitated saline test, and a large atrial-level right-to-left shunt was seen. Return to cardiopulmonary bypass revealed an unroofed coronary sinus defect, which had not previously been identified. There was no evidence of a persistent left superior vena cava. Closure of the orifice of the coronary sinus eliminated the shunt, enabling successful weaning from cardiopulmonary bypass, and the patient was subsequently discharged home.
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