Two-way right ventricular malformation in children

Dual-outlet right ventricle is a rare and very dangerous congenital heart disease. This disease is caused by an abnormality in the aorta's connection to the heart. The condition of the right ventricle with two outflow tracts can co-occur with other congenital heart defects, making the baby face worse.

In a normal heart structure, the pulmonary artery is attached to the right ventricle and the aorta is attached to the left ventricle. Dual-outlet right ventricle is a condition in which both the aorta and pulmonary artery attach to the right ventricle. This pathology is almost always accompanied by a ventricular septal defect.
In children with dual-outlet right ventricle, oxygen-rich blood from the lungs enters the left atrium and then the left ventricle, but cannot directly enter the aorta to supply oxygen-rich blood to the body. In this case, the only way is to go through the ventricular septal defect into the right ventricle. Because of the hole, it mixes with the oxygen-poor blood in the right ventricle to form mixed blood. Therefore, the right ventricle will simultaneously pump blood into both the pulmonary artery and the aorta, causing increased pressure on the right ventricle. In addition, this mixed blood does not contain much oxygen, so it is not enough to supply the body's needs, so the ventricles must work more than usual. Since then, causing many serious complications for children.
Classify the two-outlet right ventricle into 3 types depending on the location of the ventricular septal defect and the presence of pulmonary stenosis.
Type with pulmonary stenosis and subaortic septal defect. This condition closely resembles another congenital heart disease, tetralogy of fallot, except that the shunting of the ventricular septum is always a left-right shunt. Non-pulmonary stenosis and subaortic or muscular septal septal defect. The last type is the non-pulmonary stenosis as the ventricular septal defect is located below the pulmonary artery.

In most cases we cannot determine the true cause of this congenital heart disease. The risk of this congenital heart defect may be increased in children with trisomy 13 (an extra chromosome 13), trisomy 18 (an extra chromosome 18) or DiGeorge syndrome (deletion of 22q11.2).

Babies born with severe congenital heart defects will often have obvious symptoms soon after birth or within the first few weeks of life. The same is true of the right ventricle with two outflow tracts, which is a severe malformation, so functional manifestations often appear early. Depending on the clinical classification, there are different manifestations in each child.
3.1 Type with pulmonary stenosis and subaortic septal defect This condition is severe, so there are early signs of cyanosis, shortness of breath, and tachypnea. A systolic murmur of pulmonary stenosis is heard, T1 is small or sometimes normal. T2 is usually seen alone, may appear T4 heart sound. Subclinical: Electrocardiogram is suggestive of the disease with manifestations such as sharp P, long PR interval, right axis deviation, Q wave in lead D1, avL. Straight chest x-ray showed signs of systemic arterial accessory circulation, dilated ascending and transverse aorta, no pulmonary artery trunk. 3.2 Non-pulmonary stenosis and subaortic septal defect Manifested as heart failure with dyspnea, cyanosis. Cyanosis at birth is mild, but gets worse and may not be reversible. Recurrent lung infections. Examination: Chest deformity is seen, normal T1 and pulsating T2 are heard. Subclinical: Left axis deviation, PR is often long, but high P is right atrial thickening, can be combined with 2 peaks of left atrial thickening, always shows right ventricular thickening and sometimes left ventricular thickening.

3.3 Non-pulmonary stenosis and subpulmonary ventricular septal defect Atrial fibrillation will appear cyanotic at birth and progressively worsen. Shortness of breath, cough, fatigue, dizziness, chest pain. Repeated lung infections. Examination revealed a high rib cage, high systolic fibrillation, full systolic murmur in the fibrillation site, strong T2, small or normal T1. Subclinical: Electrocardiogram shows that the PR is not usually long, the P is large in both atrial, medial axis or right deviation, signs of right ventricular thickening. In all three cases above, echocardiography is a subclinical measure with high diagnostic value. Through echocardiography, it is possible to see the correlation between the arteries and the ventricles, the valve stenosis, and the associated lesions.

Cases confirmed by echocardiography with right ventricular two outflow tracts need surgery to treat.
For infants, it is necessary to conduct temporary repair surgery, depending on the type of injury, different surgical steps are needed.
For children over 6 months of age, a comprehensive repair can be performed if:
The two ventricles are almost normal in size. The atrioventricular valves are of near-normal size and function. The pulmonary artery is of normal size. There are not many muscular ventricular septal defects. There are many different repair techniques in the surgical approach. Depending on the specific case, the doctor will choose the appropriate technique.
Dual-outlet right ventricular malformation is a severe, rapidly progressive malformation. Therefore, children need to be detected as early as possible. If not treated early, the disease progresses to heart failure, affecting the muscles of life and increasing the risk of death in infants and young children.
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