Modified techniques for orthotopic heart transplantation are mandatory when complex congenital anomalies are associated in adult patients. An unusual case of a heterotaxy syndrome and dilated cardiomyopathy following mitral ring annuloplasty is presented in a 62-year-old male. Orthotopic cardiac transplantation was performed by using a modified operative strategy: selective peripheral and central venous cannulation according to the thoraco-abdominal venous challenges, biatrial technique, and preservation of venous drainage via the native coronary sinus. We discuss the anatomical features of heterotaxy in adult patients and surgical approaches when heart transplantation is needed.

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hypertrophy or subpulmonary muscle bundles. 1,2 An isolated fibrous subpulmonary membrane is rarely observed in adults and was even more rarely reported as a cause of RVOT obstruction, especially in the absence of ventricular septal defects or pulmonary valve abnormalities. 1,2 It usually causes RVOT and RV hypertrophy and is characterized by the presence of an anomalous fibrous ring bundle that protrudes from the free wall of the RV. 1-5 Symptoms appear during childhood. The subsequent course is silent until patients progressively develop obstruction followed by RV hypertrophy. 1-3,5 Adult patients are rarely asymptomatic because severe RVOT obstruction manifests itself as low cardiac output and RV failure. 1-3,5 Charcot-Marie-Tooth disease is an inherited neurologic disorder affecting peripheral nerves. Type 1 disease affects the myelin sheath of peripheral nerves, while type 2 is less common and affects the axon rather than myelin sheath. Generally, CMT has been associated with conduction disturbances or dilated myocardiopathy. No structural cardiac abnormalities have been previously described in relation to this polyneuropathy. To the best of our knowledge, a subpulmonary membrane has never been described before in the context of CMT disease. It is possible that due to physical limitations patients with CMT disease avoid physical effort, which contributes to a delayed diagnosis. 1-3,5 A high clinical suspicion is necessary to ensure a prompt diagnosis and clinical management. An accurate