A double outlet right ventricle is understood to mean different malformations of the heart. The large arteries pulmonary artery and aorta have their origin exclusively in the right ventricle.
What is the double outlet right ventricle?
According to abbreviationfinder.org, double outlet right ventricle (DORV) is the English name for right double outlet ventricle. This means congenital malformations of the heart. A right double outflow ventricle is when both the main artery (aorta) and the pulmonary artery (pulmonary artery) arise from the right ventricle, which leads to a heart defect.
Normally, oxygen-rich blood is supplied to the body from the aorta from the left ventricle. The pulmonary artery, on the other hand, transports oxygen-poor blood from the right ventricle towards the lungs. The two large blood vessels are side by side. In rare cases, the origin of the aorta and pulmonary artery can also originate from the left ventricle.
Doctors then speak of a double outlet left ventricle (DOLV), which leads to the same symptoms as a double outlet right ventricle. A right double outflow ventricle is always associated with a ventricular septal defect (VSD) with a hole in the septum of the heart. This hole is the only exit from the ventricle. It is also not uncommon for pulmonary stenosis to occur. A transposition of the large arteries is also conceivable.
Causes
The formation of the double outlet right ventricle occurs during embryonic development. The reason for this is an impairment of the rotation of the cardiac outflow path, which in turn is caused by a malformation. In medicine, the right double outflow ventricle is classified as a sign of heterotaxia (displacement of organs in the chest and abdomen).
These malformations are also considered to be lateralization defects. So far it has not been possible to determine the reasons for such a disruption. Heterotaxies are rare, affecting 1 in 15,000 people. A double outlet right ventricle often shows up together with other malformations. These primarily include a persistent ductus arteriosus, an atrial septal defect and aortic coarctation.
The position of the ventricular septal defect or ventricular septum defect plays an important role in the clinical manifestation of the right double outflow ventricle. The same applies to the existence of pulmonary stenosis. Doctors differentiate between a subpulmonary ventricular septal defect and a subaortic ventricular septal defect or a mixture of both. A ventricular septal defect that is not in close proximity to the main artery or pulmonary artery is also possible.
The Taussig-Bing syndrome represents a special form of the double outlet right ventricle. In this case, the ventricular septum defect is located under the pulmonary artery. The resulting increased blood flow leads to a veritable flooding of the lungs and the affected children show blue rash (cyanosis) because the important aorta is too far away from the ventricular septum defect.
Symptoms, ailments & signs
The symptoms of a double outlet right ventricle vary. The symptoms depend on whether the pulmonary artery valve is narrowed or not. If there is a narrowing of the pulmonary artery, this is noticeable in the affected babies through poor drinking, accelerated breathing or failure to thrive. In the worst case scenario, there is a risk of fatal heart failure.
With a double outlet right ventricle without pulmonary stenosis, the arterialized blood flows in the direction of the lungs, while the venous blood is largely transported into the body. This in turn leads to cyanosis. Without correction, there is a risk of pulmonary hypertension. The complaints with this mixed form depend on the position of the ventricular septal defect.
A double outlet right ventricle with pulmonary stenosis resembles a Fallot tetralogy, which accounts for around ten percent of all congenital heart defects. If there is a subaortic VSD, the cyanosis that is noticeable in all other forms does not occur. Arterial and venous blood mix via the ventricular septal defect, causing cyanosis.
Diagnosis
The most important diagnostic methods for examining a double outlet right ventricle include non-invasive echocardiography and Doppler sonography. With this method, the short-circuit connection of the blood via the ventricular septal defect can be perceived acoustically and visually. The same applies to the malformation of the blood vessels.
In order to get a precise picture of the right double outflow ventricle, the doctor also carries out an examination with a cardiac catheter. If the heart defect can be corrected with an operation, the double outlet right ventricle usually takes a favorable course. In complex cases, however, additional heart surgery may be required because of additional problems.
Complications
The double outlet right ventricle is a congenital malformation of the heart that can develop during the embryonic phase when the organs are relocated. The symptom is accompanied by a ventricular septum defect under the pulmonary artery. The aorta and pulmonary artery run out of the right ventricle.
As a result, the lungs are flooded by the excessively high influx of blood. This can lead to life-threatening situations, especially with small children. The resulting complications must not be ignored. In general, children with a double outlet right ventricle tend to have poor drinking, shortness of breath, general developmental disorders, visible blue rash due to the excessively deoxygenated blood, and heart failure.
In addition to relevant circulatory problems, other malformations such as a narrowing of the thoracic artery, an occlusive defect or a hole in the heart septum are usually found. In the case of hardship, there is a risk of lung damage with the resultant heart-lung transplant. Parents in particular are faced with major challenges in a child with a double outlet right ventricle.
Corrective operative measures are initiated at an early stage. Depending on the severity of the heart defect, with the help of the heart-lung machine. So that oxygen-rich blood can be pumped back into the circulation, a so-called redirection with restoration of the ventricular septum is carried out. Even after the operation, those affected require lifelong check-ups and appropriate medication.
When should you go to the doctor?
In the event of poor drinking, rapid breathing and other signs of a double outlet right ventricle, a doctor should always be consulted. If shortness of breath, visible blue rash and general developmental disorders occur, the child should be brought to the responsible doctor immediately. The same applies to prolonged cramps or visible malformations in the limbs. At the latest, if the malformations lead to accidents or falls, medical advice is required. In the event of a circulatory collapse or heart failure, the emergency services must be alerted immediately.
If the right ventricle doublet outlet has been diagnosed, the affected child must be closely monitored by a doctor. In severe cases, a longer stay in the clinic makes sense, although this depends on the type of symptoms and the general course of the disease.
In general, however, the following applies: if there are signs of a double outlet right ventricle, it is essential to go to a doctor and have the symptoms clarified and, if necessary, treated. Should occur, must be the same after completion of treatment again problems children – or family doctor are spoken. Other contact persons are the cardiologist and the angiologist. The medical emergency service can be contacted in the event of unspecific symptoms and complaints.
Treatment & Therapy
The aim of treating the double outlet right ventricle is to restore a largely normal anatomy. A heart-lung machine is used as part of the required operation. An essential part of the therapy is diverting the blood from the left ventricle towards the aorta. In this way, the oxygen-rich blood gets back into the body’s circulation.
The ventricular septal defect is also repaired by closing the hole. It is also important that the blood flows freely from the right ventricle to the pulmonary artery. This closes the ventricular septum defect and creates an aortic tunnel. However, the operation must be carried out as soon as possible in order to counteract damage to the lungs due to pulmonary hypertension.
If this does not succeed, only a heart-lung transplant can help. Even if the operation is positive, lifelong follow-up examinations of the patient must take place.
Outlook & forecast
Since the double outlet right ventricle is a malformation of the heart, this disease must be treated in any case. Without treatment, it can lead to the death of the person affected and thereby significantly reduce life expectancy.
The symptoms themselves have a very negative effect on the child’s development and can lead to developmental disorders, breathing difficulties and furthermore to heart failure. The internal organs and the brain are insufficiently supplied with oxygen, which permanently damages them.
This damage can no longer be reversed. Those affected also suffer from circulatory problems and significantly reduced resilience. In most cases, if the disease is not treated early, the children die from the symptoms of the double outlet right ventricle.
The treatment of double outlet right ventricle is usually carried out by surgery. In most cases, this procedure is successful and completely relieves the symptoms. In severe cases, however, heart and lung transplants are necessary to cure the disease. However, these cannot always be carried out, so that most of these patients die.
Prevention
There are no preventive measures that can be used to prevent a double outlet right ventricle. So the heart defect is already congenital.
Aftercare
In the case of the double outlet right ventricle disease, in most cases the patient has very few options and measures for follow-up care. The affected person is definitely dependent on an intervention that can resolve the malformation in the heart so that there are no further complications or, in the worst case, death of the affected person.
The earlier the disease is recognized and treated, the better the further course is usually, so that the early detection of the disease is in the foreground with Double outlet right ventricle. As a rule, an operation on the heart is then carried out to alleviate the symptoms.
Even after the procedure, the person concerned is dependent on permanent and lifelong examinations of the heart in order to identify and treat further damage. Since a heart or lung transplant may also be necessary in some cases, the life expectancy of the person affected is usually significantly reduced with the double outlet right ventricle. In general, those affected should take care of their bodies and not expose themselves to unnecessary exertion. A healthy lifestyle with a healthy diet, as well as abstaining from alcohol and tobacco, can also alleviate the disease.
You can do that yourself
The double outlet right ventricle is a congenital heart defect that develops during embryonic development. There are no self-help measures that the patient or their parents can take to combat the cause of the disease.
However, it is important that the malformation is recognized quickly and treated professionally. If the disorder is not recognized before or immediately after the birth, parents of affected children must react promptly. Infants with double right ventricle outlet often do not drink properly and breathe heavily or quickly. Such symptoms should always be discussed with a doctor in a timely manner.
Children diagnosed with double outlet right ventricle almost always have to undergo several complex surgeries. There is also a risk of sudden cardiac death until the malfunction has been rectified. If the operations are unsuccessful, lung damage usually occurs. The only way to save the child is through a heart-lung transplant.
The parents and family members of an affected child have to be prepared for a long period of regular hospital stays, visits to the doctor and home nursing. In addition, the child’s life is often at risk. Parents and relatives should not bear this enormous burden alone, but should take care of psychotherapeutic care in good time.
