Double outlet left ventricle

Double outlet left ventricle (DOLV) is an extremely rare congenital heart defect in which both great arteries — the aorta and the pulmonary artery — arise predominantly or entirely from the morphological left ventricle. This is in contrast to the normal anatomy where the aorta arises from the left ventricle and the pulmonary artery from the right ventricle. DOLV is classified under conotruncal heart malformations and is one of the rarest forms of ventriculoarterial connection abnormalities. The condition affects the cardiovascular system and results in abnormal blood flow patterns that can lead to cyanosis (bluish discoloration of the skin due to low oxygen levels), heart failure, and failure to thrive in infancy. Clinical presentation depends on the associated cardiac anomalies, which are almost always present. These commonly include ventricular septal defect (VSD), which provides the only outlet for the right ventricle, as well as pulmonary stenosis, transposition or malposition of the great arteries, and various degrees of right ventricular hypoplasia. Symptoms typically manifest in the neonatal or early infantile period and may include respiratory distress, cyanosis, poor feeding, and signs of congestive heart failure. The severity of symptoms is largely determined by the presence and degree of pulmonary stenosis and the size of the ventricular septal defect. Diagnosis is established through echocardiography, cardiac catheterization, and advanced imaging such as cardiac MRI or CT angiography. Treatment is surgical and must be individualized based on the specific anatomy of each patient. Surgical options may include biventricular repair when feasible, involving rerouting of the right ventricular outflow through the VSD to one of the great arteries, or univentricular palliation (such as the Fontan procedure) when biventricular repair is not possible due to right ventricular hypoplasia or other complex associated anomalies. Early diagnosis and intervention are critical for improving outcomes, though long-term prognosis varies depending on the complexity of the cardiac anatomy and the success of surgical repair.

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