Double inlet left ventricular main chamber, subaortic small left sided right ventricle and interrupted aortic arch...

A case of a 23 year old female patient who suffered from the complex congenital heart lesion of a double inlet left ventricular main chamber, subaortic small left sided right ventricle and interrupted aortic arch type A is reported. With equally high blood pressures, the perfusion in the upper half of the body was maintained through the ascending aorta while the lower half and the lungs were supplied through the pulmonary artery and a patent ductus arteriosus (PDA). Angiographically, the bulbo-ventricular foramen appeared to be nonrestrictive. However, distinct signs of muscular subaortic stenosis were detected. The hemodynamic status principally allowed surgical correction when this became necessary because of increasing left heart failure. Treatment for this complex lesion undoubtedly required reduction of pulmonary perfusion, even when associated with the danger of increasing cyanosis. Various forms of surgical treatment (functional correction, palliative procedures) were discussed. The most elegant was performed without cardiopulmonary bypass: this consisted in connection of the pulmonary artery with the descending aorta using a 16 mm Dacron tube, reconstruction of the aortic arch by a prostheso-subclavian synthetic graft, suture ligation of the PDA, and banding of the pulmonary artery trunk distal to the origin of the prosthesis. One year after the operation, the patient's physical performance has improved. Moreover, despite the disappearance of cardiac failure she has not become more cyanotic during exertion.