An unusual form of double outlet right ventricle/Cift cikisli sag ventrikulun nadir bir formu.

A 20-year-old male was admitted to the hospital with cyanosis, exercise intolerance and failure to thrive. Physical examination revealed cyanosis, clubbing and 3/6th grade systolic murmur in the second left and right intercostal spaces. Transthoracic echocardiographic examination demonstrated a hypoplastic left ventricle with atretic mitral valve (Fig. 1). Both great vessels were arising from the right ventricle, which was communicated with hypoplastic left ventricle via a subaortic ventricular septal defect (VSD). The aorta was anterior to the main pulmonary artery. There was also a wide secundum atrial septal defect (ASD). A 16 mmHg gradient was measured by the pulmonary valve and a persistent left superior vena cava was identified. Cardiac magnetic resonance imaging confirmed the hypoplastic left ventricle, ASD, VSD and the spatial relationship of the great vessels (Fig. 2-3). The patient underwent cardiac catheterization, which showed Eisenmenger physiology, and the patient was referred to heart-lung transplantation list. Double outlet right ventricle (DORV) represents a continuum of congenital heart diseases that ranges from VSD with significant override of the aorta to a common arterial trunk arising completely from the right ventricle. Patients rarely present with varying degrees of left ventricular hypoplasia and mitral valve anomalies such as stenosis or atresia. To our knowledge, a case of DORV with mitral atresia and left ventricular hypoplasia who survived beyond 20 years without surgery has not been reported.

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Mehmet Yokusoglu, Ali Cevat Tanalp, Mehmet Uzun, Nail Bulakbasi *, Ersoy Isik From Departments of Cardiology and Radiology *, Gulhane Military Medical Academy, Ankara, Turkey

Address for Correspondence: Mehmet Yokusoglu, MD, Gulhane Askeri Tip Akademisi Kardiyoloji Anabilim Dali, 06018 Etlik, Ankara, Turkiye Tel.: [phone] Faks: [phone] E-mail: [e-mail]