Double-outlet right ventricle with left malposition of the great arteries and total anomalous pulmonary venous connection

Abstract

The patient was diagnosed with double-outlet right ventricle with left malposition of the great arteries, total anomalous pulmonary venous connection, and pulmonary stenosis at 2 months of age. Because the progression of pulmonary venous obstruction was suspected at 2 years of age, the patient was catheterized and underwent a definitive repair. The patient had a right aortic arch and left juxtaposition of the atrial appendages; the intraventricular anatomy exhibits malalignment between the conal septum and the muscular intraventricular septum, and the conal septum is well developed and dextroposed, resulting in pulmonary obstruction and multiple ventricular septal defects (VSDs). Because a supracardiac type (Darling's type Ib) of total anomalous pulmonary venous connection was also combined with such an anomaly, total correction consisted of an anastomosis of the left atrium and the common pulmonary vein, creation of an intraventricular tunnel, direct closures of muscular VSDs, pulmonary valvotomy, and infundibular resection. To our knowledge, this is the first description of the successful surgical correction of this type of anomaly in one stage.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in “Double Outlet Right Ventricle

“…As previously mentioned, while levo-position of the great arteries in the concept of DORV is already a rare anomaly, the juxtaposition of both atrial appendages is seen even more rarely. Although some reports demonstrate their association with different other anomalies, there are only a few case reports of detection of both anomalies in the same patient [ 2 , 5 , 6 ] . Our case report is important due to the rarity of this anatomical type of DORV.…”