A 35y.o. with dextrocardia and transposition of the great vessels is sp a Mustard procedure and now presents with hemoptysis. What could be wrong?
Our patient had a narrowing of the subclavian baffle done when he was a child leading to collaterals developing in the lung; some of which were now bleeding.
In this congenital anomaly venous circulation drainsinto the right ventricle but from this chamber blood goes into the aorta and the L ventricle is connected to the pulmonary artery. The patient could not survive this arrangement when the normal prenatal shunts close after birth. Historically, the first procedure tried was a hole in the atrial septum to allow at least some mixing of oxygenated blood . In the mustard procedure this was refined by creating a conduit from thevena cava to the mitral valve and thus to the pulmonary circulation. This conduit or baffle is usually made of pericardium with a patch on the vena cave shaped like an elephant’s trunk. The more standard procedure done now, since bypass is available, is an atrial switch where the arteries are switched to the correct ventricles and the coronary arteries are reimplanted.
Other complications include thrombosis of the baffle, R heart failure and sudden death from arrhythmia. Follow up now extends to over 30 years.
He was admitted to the ICU and had a second episode of massive hemoptysis. His aorto pulmonary artery collaterals were coiled and he had no further episodes of bleeding.
Teaching point: 100 cc of blood will fill the trachea and cause hypoxia and death in someone not able to cough. Worry about hemoptysis. Other causes are listed below.
Williams, WG, Trusler GA, Kirklin JW, et al. Early and late results of a protocol for simple transposition leading to antrial swith repair. J Thorac Cardiovasc Sug 1988 .95;717-726.
Gelatt M, Hamilton RM, McCrindle BW, et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. 1997J Am Coll Cardiol. 29;194-201.