What is Double Outlet Right Ventricle?

The right ventricle double outlet (DORV) is a series of complex congenital and complicated cardiac malformations. Its broad morphological manifestations can range from ventricular condyle defect (VSD) with aortic straddling, to aortic transposition with VSD. The main cardiac malformations include: pulmonary stenosis, open ductus arteriosus, atrial septal defect, atrioventricular septal defect, complete pulmonary vein ectopic connection, left ventricular outflow tract obstruction, atrioventricular valve malformation (stenosis, straddling), etc. Related symptoms include palpitations, right ventricle double-track, and cardiac murmurs.

Basic Information

English name

double outlet of right ventricle

Visiting department

Thoracic and Cardiovascular Surgery

Common causes

Congenital complex heart malformation

Common symptoms

Palpitation, shortness of breath, colds since childhood, recurrent pneumonia, slow weight gain, etc.

Causes of right ventricular double exit

The etiology of right ventricular double outlets can be divided into 6 major areas:

1. Atrial ventricle connection, there may be various conditions in the atrial ventricular connection relationship;

2. Macrovascular relationship. In most cases, the position of the two arteries is normal, and the aorta is in the right rear of the pulmonary artery.

3. The aorta is on the right side of the pulmonary artery;

4. The aorta is in the left anterior position of the pulmonary artery;

5. The conduction beam distribution of the two ventricles is similar to that of tetralogy of Fallot.

6. Coronary artery development can also be abnormal.

Clinical manifestations of right ventricle double exit

The clinical symptoms of this disease are diverse. Those with no pulmonary artery stenosis have more pulmonary blood. Pulmonary hypertension occurs early. Symptoms can be similar to large ventricular septal defects, palpitation, shortness of breath, colds from childhood, recurrent pneumonia, slow weight gain, and early congestive heart failure. Ventricular defects are located under the pulmonary valve or with severe pulmonary hypertension. If accompanied by pulmonary stenosis, the clinical symptoms can be similar to those of tetralogy of Fallot, with cyanosis since childhood, progressive progressive, clubbing fingers (toes) obvious, dyspnea, headache, dizziness, restricted mobility, and like squatting.

Right ventricular hypertrophy can cause the anterior cardiac area to bulge forward, and the second heart sound in the pulmonary valve area can be hyperactive or weakened, which is mainly determined by the presence of pulmonary artery stenosis. The left margin of the sternum 2 to 4 intercostal space can often hear 3 to 4/6 full systolic hairy murmur, and can touch fine tremor. May be accompanied by palate and clubbing fingers (toes).

Double ventricle exit examination

1. X-ray inspection

In the right ventricle double outlet without pulmonary artery stenosis, the X-ray showed enlarged heart shadows, prominent pulmonary artery trunks, bloody lungs, similar to large ventricular septal defect with signs of pulmonary hypertension. Those with pulmonary arterial stenosis have reduced texture, similar to the Fallot tetralogy, and the heart shadow is usually slightly increased, but it does not appear as a typical wooden boot.

2. Echocardiography

The parasternal left ventricular long-axis view shows that two parallel aorta originate from the right ventricle, the connection between the anterior wall of the aorta and the ventricle is interrupted, and the fiber connection between the posterior aortic valve and the anterior mitral valve is interrupted. Echoes of the muscular cone. On the apical and xiphoid four-chamber views, the upper position of the inferior vena cava can be used to determine the atrial position. According to the position of the mitral and tricuspid valves and the trabecular muscles of the ventricle, it can be seen that the visceral atrial relationship and the atrial and ventricular connection relationship can be The relationship between ventricular septal defect and the two major arterial openings was shown, and the right ventricular double outlets were classified.

3. ECG

The electrocardiogram shows right axis deviation, right ventricular hypertrophy, left and right ventricular hypertrophy, prolonged PR, common indoor block or right bundle branch block. Patients with pulmonary stenosis may have right atrial hypertrophy, right ventricular hypertrophy, and intraventricular block, and may also have left ventricular hypertrophy and prolonged PR interval.

4. Cardiac catheterization

In patients without pulmonary artery stenosis, the left and right ventricular pressures are equal, the pulmonary pressure is equal to the body arterial pressure, the pulmonary resistance is severely increased, and the ventricular septal defect is smaller, the left ventricular pressure may be higher than the right ventricular pressure. The right ventricular blood oxygen saturation increases, but the arterial and venous blood is not uniformly mixed, and the arterial blood oxygen saturation is slightly lower or normal, which has a certain relationship with the ventricular defect site. With pulmonary stenosis, the left and right ventricular pressures are equal, and there is a pressure step between the right ventricle and the pulmonary artery. Right ventricular blood oxygen saturation was higher than that in right atrium.

5. Cardiovascular angiography

Right ventricle angiography usually shows the aorta and pulmonary arteries at the same time, or they are slightly different in sequence, juxtaposed, and the tongue-shaped filling defect of the supraventricular wall bundle separates the outflow tract into the aorta and the pulmonary artery. The aortic valve and the pulmonary valve originate from the same cross section, or the pulmonary valve plane is slightly higher. Left ventricle angiography showed that the contrast agent passed through the ventricle to the right ventricle and the aortic root. Pulmonary arteries are dilated or have different degrees of pulmonary valve and / or funnel stenosis.

Diagnosis of right ventricle double exit

The lesions of the right ventricle double exit are complicated, there are many types, and the clinical diagnosis is difficult. No symptoms, signs, or electrocardiograms were characteristic. Two-dimensional echocardiography can prompt diagnosis. Selective right and left ventricular angiography is the best way to make a diagnosis, but it can only make the correct diagnosis in most cases.

Double Ventricle Outlet Therapy

There is no possibility of self-healing in the right ventricle double outlet, and surgery should be performed after the diagnosis is established. The method of operation was to open the chest in the middle, and perform the operation under low temperature extracorporeal circulation.