Double outlet right ventricle

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Classification according to ICD-10
Q20.1 Right double outflow ventricle
ICD-10 online (WHO version 2019)

The double outlet right ventricle (DORV) is a congenital malformation of the heart in which the large arteries aorta and pulmonary artery arise exclusively from the right ventricle . In parallel, there is always a ventricular septal defect (VSD), which is the only exit of the left ventricle, and often pulmonary stenosis . There may be a transposition of the great arteries , which in this case are referred to as d or l malposition.

The origin of the aorta and pulmonary artery from the left ventricle ( double outlet left ventricle , DOLV) also occurs in rare cases; the clinical picture and therapy are similar to that of DORV.

Emergence

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Since the malformation is caused by a disturbed rotation of the outflow path of the heart during embryonic development , it is regarded as a sign of heterotaxia . This heart defect is associated with other malformations such as atrial septal defect , persistent ductus arteriosus or coarctation of the aorta to varying degrees .

Classification

The location of the VSD and the presence and severity of pulmonary stenosis have an important influence on the hemodynamics and clinical characteristics.

  1. subaortic VSD (ventricular septal defect shape)
  2. subpulmonary VSD (transposition form)
  3. Mixed form of 1 + 2
  4. VSD without physical proximity to the aorta or pulmonary artery

Anatomically, the subaortic form of the DORV with pulmonary stenosis is only differentiated from the Fallot tetralogy by the displacement of the aorta by at least 50% to the side of the right ventricle.

clinic

In general, there are differently pronounced signs of heart failure . Furthermore, general signs of congenital heart defects such as poor drinking and failure to thrive .

DORV without pulmonary stenosis

Via the subaortic VSD , arterialized , oxygen-rich blood flows from the lungs mainly into the body's circulation, while the venous blood mainly flows via the pulmonary artery into the lungs. The clinical picture and the hemodynamics largely correspond to those of a non-pressure separating VSD.

In subpulmonary VSD , the arterialized blood flows into the lungs, while the venous blood flows mainly into the body, causing cyanosis. The picture is similar to a transposition of the great arteries with VSD . If not corrected, the pressure load on the lungs leads to pulmonary hypertension .

The clinic of the mixed form depends on the exact location of the VSD.

If the VSD is not in close proximity to the aorta or the pulmonary artery , the arterial and venous blood is mixed. Clinically, there is cyanosis. The patients are often dependent on a persistent ductus arteriosus (ductus dependence).

DORV with pulmonary stenosis (Fallot form)

If pulmonary stenosis is present, the picture of a Fallot tetralogy is largely found . With subaortic VSD without, with all other forms with cyanosis. Depending on the severity of the pulmonary stenosis, there may be ductus-dependent.

The severity of this heart defect can be very different, depending on which malformation combination is present. In some cases the clinical picture in the distinction to Fallot's tetralogy depends only on the more or less strongly riding aorta . Venous and arterial blood is mixed via the VSD. The result is a cyanosis .

Diagnosis

therapy

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Surgical correction with the help of the heart-lung machine depends on the severity of the heart defect. The aim is to create a normal anatomy or an anatomy that largely approximates the norm . The following is possible:

  • one-sided correction for "simple variants"
  • Closure of the VSD and correction of the pulmonary stenosis by enlarging or implanting a homograft (human donor valve)
  • If the VSD is below the pulmonary valve or below both large arteries or far from the vessels in the musculature of the cardiac septum, a Rastelli operation is used.
  • If the right ventricle is assigned to the body artery and the left ventricle is assigned to the pulmonary artery through the closure of the VSD, and if the large arteries are transposed , an "arterial switch operation" is performed.
  • In the Damus-Kaye-Stensel operation, the transposition of the large arteries is corrected by connecting the pulmonary artery to the side of the body artery
  • The Fontan operation is rare in very complicated anatomical conditions

Sometimes the corrective operation is preceded by the creation of an aorto-pulmonary shunt (in the case of severe cyanosis) or the creation of a pulmonary banding (subpulmonary VSD without pulmonary stenosis).

Treatment outcomes and prognosis

Correction can be relatively easy with very good surgical results. Problems can arise later with complex shapes. A slight continued narrowing or leakage of the pulmonary stenosis can become more important and make a later operation necessary. A small remaining VSD is usually of no significance. A narrowing in the outflow path of the left ventricle (aortic valve) usually requires a quick renewed intervention. Since this operation is often close to the conduction system, permanent cardiac arrhythmias can occur. Given the diversity of this heart defect, general prognoses are not possible.

Life-long check-ups are necessary. Likewise, compliance with endocarditis prophylaxis .

Literature and Sources