Truncus Arteriosus - modified Van Praagh’s Type 3A and Anesthesia: a case report.

Background
One of the rare complex congenital anomalies is truncus arteriosus—modified Van Praagh’s type 3A. Survival of this type of truncus arteriosus child beyond infancy without surgical treatment is unreported. Anesthesiologists do anesthetize children with complex congenital heart disease during the cardiac catheterization study. The final diagnosis of such children is often made after the anesthesia and cardiac catheterization study. We report a 12-year-old with truncus arteriosus with absent right pulmonary artery and main pulmonary artery with multiple Major Aorto-Pulmonary Collateral Arteries. (MAPCAs) for the right lung, who is surviving without surgical treatment.
Case presentation
A 12-year-old girl was brought by her parents to Meenakshi Hospital at Thanjavur (India) with complaints of shortness of breath during respiratory infection. The patient was diagnosed to have congenital heart disease at 6 years of age and not on any treatment. There was no history of cyanotic spell. Her echocardiography revealed tetralogy of Fallot, situs solitus, levocardia, large mal-aligned ventricular septal defect with bidirectional shunt, VSD size 12 mm, pulmonary atresia, moderate tricuspid regurgitation (TR pressure gradient, 103 mmHg), thickened aortic valve, grade II aortic regurgitation, right ventricular hypertrophy, intact interatrial septum, dilated right atrium/right ventricle, dilated coronary sinus, and persistent left superior vena cava, good biventricular function 65%, multiple MAPCAs, no coarctation of aorta, normal veno atrial, atrio-ventricular connections, normal pulmonary venous drainage, and no pericardial effusion. She underwent cardiac catheterization study for further evaluation under anesthesia. Her final diagnosis was truncus arteriosus with absent right pulmonary artery and main pulmonary artery with multiple MAPCAs for right lung, (truncus arteriosus—modified Van Praagh’s type 3A).
Conclusion
An anesthesiologist may be encountering such patients during cardiac catheterization study or emergency non-cardiac surgery, where an understanding of the complex anatomy (the aorta, left pulmonary artery, coronary artery, all arising from the common arterial trunk, the truncus arteriosus) and the physiology of their circulation would help in safe anesthesia. From our report, we conclude intra venous ketamine along with regional analgesia would be safe for sedating such patients coming for cardiac catheterization study.