Two wrongs make a right: A case report on the anesthetic management of a pediatric patient with congenitally corrected transposition of great arteries

@#Congenitally corrected transposition of the great arteries (ccTGA) or ventricular inversion, is a rare form of congenital heart disease (CHO) representing approximately 0.5% of all CHO. It is characterized by atrioventricular and ventriculoarterial discordance, in which the atria are connected to the opposite ventricle, and the ventricles are connected to the incorrect great artery. The defect is termed "corrected" because of the physiologic flow of blood through the body despite the malformation. ccTGA can be associated with other cardiac anomalies like ventricular septa! defect (VSO), pulmonary outflow tract (LVOT) obstruction, tricuspid valve lesions, and coronary artery anomalies. This paper aims to discuss the anesthetic management unique to patients with ccTGA in which the ultimate goal is to prevent hemodynamic instability that could potentially lead to cardiac failure. Here, we report the anesthetic management of a 6 year old child with ccTGA with mild tricuspid regurgitation who underwent plastic repair of cleft lip under general endotracheal tube anesthesia (inhalational). With use of balanced anesthesia to produce minimal to no cardiovascular effects, the operation concluded successfully.

The clinical research of double switch operation with Glenn and hemi-Mustard in CC-TGA in children / 中华胸心血管外科杂志

Objective To avoid complications associated with the complete Senning procedures and to assist right-heart hemodynamics, this study was undertaken to assess the risks and benefits of the double-switch operation using a hemi-Mustard atrial switch procedure and the bidirectional Glenn operation for congenitally corrected transposition of the great arteries. we fa-vor a modified atrial switch procedure. Methods Between January 2014 and March 2016, anatomic repair was achieved in 26 patients. A hemi-Mustard procedure was the atrial switch procedure for 26. 9％(7/26). The Rastelli-atrial switch procedure was performed in 5 patients( RAS) and the arterial-atrial switch procedure was performed in 2 patients( AAS) . Results There was no in-hospital death after anatomic repair. There were no late deaths in a median follow-up of 7-21 months. Glenn opera-tion complications were uncommon limited to the perioperative period, atrial baffle-related reoperations or sinus node dysfunc-tion had not been observed. Tricuspid regurgitation decreased to mild for 1 case and mild to moderate for 6 cases. Right ventri-cle-pulmonary artery conduit longevity was significantly improved. Conclusion We recentely describe an experience with the double-switch operation using a modified atrial switch procedure with favorable earlyterm results. The risks of the hemi-mustard and bidirectional Glenn operation are minimal and are limited to a well-defined patient subset. The benefits include reduced baffle-and sinus node-related complications, technical simplicity and inspiring results.

Repair of Corrected Transposition of the Great Arteries

PURPOSE: To assess the clinical characteristics and surgical approaches in different anatomical and hemodynamic types of corrected transposition of great arteries(TGA) and learn the surgical results of those patients. METHODS: All 52 patients who were diagnosed as corrected TGA between December 1987 and November 1999 and their medical records were reviewed. Three groups were identified according to associated anomalies', Group 1: TGA with intact ventricular septum(n=7), Group 2: TGA with ventricular septal defect(n=6), Group 3: TGA with ventricular septal defect and pulmonary stenosis(n=39). RESULTS: The clinical manifestations and managements according to associated anomalies were different. The average ages at operation were 190, 8.8 and 47 months in Groups 1, 2, and 3, respectively. The five patients underwent double switch operation and the remainder were managed conventionally without correcting discordant connection. Four patients died and 5 patients were re-operated. Eleven patients developed complete heart block, and 7 of them had permanent pacemakers. Progressive systemic tricuspid valve regurgitation developed in 15 patients and progressive systemic right ventricle dysfunction developed in 3 patients. CONCLUSION: The results of conventional management were disappointing, with significant morbidity of tricuspid failure, right ventricle failure and conduction system failure. Anatomical repair of corrected TGA can be achieved with favorable immediate surgical results but long-term follow up will be necessary.

Double switch operation for congenital corrected transposition of great arteries with heart anomaly / 中华胸心血管外科杂志

Objective To report the outcomes of double switch operation as the primary approach for congenital corrected transposition of great arteries (cTGA) with heart anomaly. Methods From April 2002 to June 2004, seven patients ranged 4 to 15 years with cTGA underwent double switch operation. Six patients were situs solitus (SLL segmental anatomy) while one patient was situs inversus (IDD segmental anatomy). The heart defects included ventricular septal defect in 6 cases, secondary atrial septal defect in, double outlet of right ventricle in 1, pulmonary stenosis in 6, dextrocardia in 3 and levocardia in 1. The operative procedures comprised of 4 modified Senning+Rastelli, Mustard +Rastelli +bidirectional Glenn, 1 Senning +Rastelli, 1 modified Senning+switch. Results There was one early operative death after modified Senning+switch operation. The cause of death was left ventricular failure. The postoperative complications included severe low cardiac output syndrome in 1, temporary atrioventricular block in 1, pleurisy and low plasma protein in 2. The survivors were followed up from 2 to 24 months, all of them were in sinus cardiac rhythm except one case with junction cardiac rhythm. All were in NYHA I class. Conclusion Anatomic correction of cGTA by double switch operation can be performed with lower operative mortality and good medial-term outcome. In the SLL cases, modified Senning operation may yield better outcomes than Mustard operation.