An increasing number of patients with transposition of great arteries reaches adult age after an atrial redirection operation. The well known late sequelae of the Mustard and Senning operations include supraventricular brady-tachyarrhythmias, dilatation and failure of the systemic ventricle, severe tricuspid valve incompetence, obstruction of either the systemic or pulmonary venous return as well as left ventricular outflow tract obstruction, and baffle leaks. The case of a 26-year-old woman with a rarer postoperative course, namely severe aneurysmal dilatation of the pulmonary artery and pulmonary arterial hypertension 22 years after a Mustard operation is described. The pros and contras of the available therapeutic alternatives are discussed.

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