| disease | Double Outlet Left Ventricle |
Double outlet left ventricle (DOLV) refers to a condition where both great arteries originate from the left ventricle, with their orifices located in the same plane. It is characterized by bilateral conus and conal muscle hypoplasia, as well as continuity between the aortic and pulmonary valves, semilunar valves, and mitral valve. This is an extremely rare congenital cardiovascular malformation. It is often associated with ventricular septal defect, pulmonary stenosis, Ebstein's anomaly of the tricuspid valve, right ventricular hypoplasia, atrioventricular discordance, and atrial and visceral situs solitus or inversus. The hemodynamic changes in DOLV resemble those of severe tetralogy of Fallot or complete transposition of the great arteries with ventricular septal defect.
bubble_chart Clinical Manifestations
Cyanosis is often accompanied by pulmonary congestion or ischemia. Cardiac auscultation reveals ejection or systolic murmurs. Electrocardiogram examination: Left ventricular hypertrophy is commonly observed. Right heart catheterization, selective left ventricular angiography, and cross-sectional echocardiography are the primary diagnostic methods. The oxygen saturation in the main {|###|} stirred pulse and the pulmonary {|###|} stirred pulse is almost equal. Left ventricular angiography shows simultaneous opacification of both major {|###|} stirred pulses.
bubble_chart Treatment Measures
The indications for surgery are the same as for double outlet right ventricle, and surgical treatment should be performed once the diagnosis is confirmed. In infants with double outlet left ventricle accompanied by pulmonary congestion, early surgery or initial pulmonary artery banding is recommended. For cases with pulmonary stenosis and pulmonary ischemia, a systemic-pulmonary shunt should be performed first, followed by corrective surgery using a valved extracardiac conduit. However, if severe pulmonary vascular obstructive disease has already developed, it becomes a contraindication for surgery.
Radical surgical methods: The procedure is performed through a median sternotomy incision under extracorporeal circulation combined with hypothermia, and the approach depends on the location of the ventricular septal defect and the presence or absence of pulmonary stenosis:1. Ventricular septal defect located below the pulmonary valve without pulmonary stenosis: A right ventricular incision is made, and a patch is used to construct an internal tunnel connecting the pulmonary artery to the right ventricle. If pulmonary stenosis is present, the stenosis must be relieved. If necessary, an outflow tract enlargement plasty is performed.
2. Ventricular septal defect located below the aortic valve, with or without pulmonary stenosis: A right ventricular incision is made, the ventricular septal defect is closed with a patch, the pulmonary valve orifice is closed or the proximal pulmonary artery is ligated, and a valved extracardiac conduit is used to connect the right ventricle to the pulmonary artery.
3. Close the ventricular septal defect while enlarging the right ventricular outflow tract.
4. Close the ventricular septal defect, suture the pulmonary valve, resect the hypertrophic muscle of the infundibulum, reconstruct and enlarge the right ventricular outflow tract with a patch, and implant an artificial valve membrane in the pulmonary artery.
The surgical mortality rate remains high.
