Double outlet right ventricle refers to various malformations of the heart. In this case, the large arteries, pulmonary artery and aorta, originate exclusively in the right ventricle.
What is double outlet right ventricle?
Double outlet right ventricle (DORV) is the English name for right double outlet ventricle. It refers to congenital malformations of the heart. A double outlet right ventricle occurs when both the aorta and the pulmonary artery originate in the right ventricle, resulting in a heart defect. Normally, oxygenated blood is supplied to the body by the aorta from the left ventricle. The pulmonary artery, or pulmonary artery, on the other hand, carries deoxygenated blood from the right ventricle toward the lungs. The two major blood vessels are located side by side. In rare cases, the origin of the aorta and pulmonary artery can also be the left ventricle. Physicians then speak of a double outlet left ventricle (DOLV), which leads to the same complaints as a double outlet right ventricle. A double outlet right ventricle is always accompanied by a ventricular septal defect (VSD) with a hole in the cardiac septum. This hole forms the single outlet of the ventricle. It is also not uncommon for pulmonary stenosis to occur. Furthermore, transposition of the great arteries is conceivable.
Causes
The development of the double outlet right ventricle occurs during embryonic development. The reason is an impairment of the rotation of the cardiac outflow tract, which in turn results from a malformation. In medicine, the double outlet right ventricle is classified as a sign of heterotaxy (displacement of organs in the chest and abdominal cavity). These malformations are also considered lateralization defects. The reasons for the development of such a defect could not be determined so far. Heterotaxies are rare and occur in one out of 15,000 people. Often, a double outlet right ventricle presents together with other malformations. These primarily include a persistent ductus arteriosus, an atrial septal defect, and aortic isthmus stenosis. The position of the ventricular septal defect or ventricular septal defect plays an important role in the clinical manifestation of the right double-outflow ventricle. The same applies to the existence of pulmonary stenosis. Thus, physicians distinguish between a subpulmonary ventricular septal defect and a subaortic ventricular septal defect or a mixed form of both. Furthermore, a ventricular septal defect that does not have spatial proximity to the aorta or pulmonary artery is also possible. A special form of double outlet right ventricle is the Taussig-Bing syndrome. In this case, the ventricular septal defect is located under the pulmonary artery. The increased blood flow that results causes the lungs to become truly flooded, and affected children exhibit cyanosis because the important aorta is too far from the ventricular septal defect.
Symptoms, complaints, and signs
Symptoms of double outlet right ventricle vary. For example, 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 by weak drinking, accelerated breathing or failure to thrive. In the worst case, there is a risk of fatal heart failure. In the case of a double outlet right ventricle without pulmonary stenosis, the flow of arterialized blood is toward the lungs, while venous blood is largely transported into the body. This in turn results in cyanosis. Without correction, pulmonary hypertension is imminent. The symptoms in this mixed form depend on the position of the ventricular septal defect. A double outlet right ventricle with pulmonary stenosis resembles tetralogy of Fallot, which accounts for about ten percent of all congenital heart defects. If a subaortic VSD is present, there is no cyanosis, which is seen in all other forms. Arterial and venous blood mix across the ventricular septal defect, resulting in cyanosis.
Diagnosis
The main diagnostic procedures used to investigate a double outlet right ventricle include noninvasive echocardiography and Doppler ultrasonography.These procedures allow the short-circuiting of the blood via ventricular septal defect to be perceived acoustically and visually. The same applies to the malformation of the blood vessels. To obtain a precise picture of the right double-outflow ventricle, the physician also performs an examination with a cardiac catheter. If the heart defect can be corrected with surgery, the double outlet right ventricle usually takes a favorable course. However, in complex cases, additional heart surgery may be needed because additional problems arise.
Complications
Double outlet right ventricle is a congenital malformation of the heart that may develop within the embryonic period when the organs are displaced. The symptom has a ventricular septal defect under the pulmonary artery as an associated condition. For this purpose, the aorta and pulmonary artery run out of the right ventricle. Consequently, the lungs are flooded by the excessive inflow of blood. This can lead to life-threatening situations, especially in young children. The resulting complications should not be ignored. In general, children with a double outlet right ventricle tend to be weak drinkers, short of breath, general developmental disorders, visible cyanosis due to the much too deoxygenated blood and heart failure. In addition to circulatory problems, other malformations such as a narrowing of the thoracic artery, an occlusion defect or a hole in the cardiac septum are usually present. In cases of hardship, there is a risk of lung damage with resulting heart-lung transplantation. Parents in particular are faced with great challenges in a child with double outlet right ventricle. Corrective surgical measures are initiated at an early stage. Depending on the extent of the heart defect, with the help of the heart-lung machine. To ensure that oxygen-rich blood can be pumped into the circulation again, a so-called redirection with restoration of the ventricular septum is performed. Affected patients require lifelong checkups and medication as needed, even after surgery.
When should you see a doctor?
A physician should always be consulted if there is weakness in drinking, accelerated breathing, and other signs of double outlet right ventricle. If there is shortness of breath, visible cyanosis, and general developmental problems, the child is best taken immediately to the appropriate physician. The same applies in the case of prolonged cramps or visible malformations of the limbs. Medical advice is required at the latest if the malformations lead to accidents or falls. In case of circulatory collapse or heart failure, the emergency services must be alerted immediately. If the doublet outlet right ventricle has been diagnosed, the affected child must be closely monitored by a physician. In severe cases, a longer hospital stay is advisable, although this depends on the nature of the 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 see a doctor and have the symptoms clarified and, if necessary, treated. If problems occur again after treatment has been completed, the pediatrician or family doctor must be consulted. Other contacts are the cardiologist and the angiologist. If symptoms and complaints are nonspecific, the emergency medical service can be contacted.
Treatment and therapy
The goal of treatment for double outlet right ventricle is to produce a largely normal anatomy. A heart-lung machine is used as part of the necessary surgery. An essential part of the therapy is redirecting blood from the left ventricle toward the aorta. In this way, the oxygen-rich blood gets back into the body’s circulation. Furthermore, the ventricular septal defect is also repaired by closing the hole. It is also important to have an unobstructed outflow of blood from the right ventricle to the pulmonary artery. This results in closure of the ventricular septal defect as well as creation of an aortic tunnel. However, the operation must be performed as soon as possible to counteract damage to the lungs due to pulmonary hypertension. If this does not succeed, only a heart-lung transplantation can help. Even after a positive outcome of the operation, lifelong check-ups of the patient must take place.
Outlook and prognosis
Because double outlet right ventricle is a malformation of the heart, this condition must be treated in all cases. In doing so, it can lead to the death of the affected person without treatment, thereby considerably reducing life expectancy. The condition itself has a very negative effect on the child’s development and can lead to developmental disorders, breathing difficulties and further to heart failure. The internal organs and the brain are not supplied with enough oxygen, which causes lasting damage to them. This damage cannot be reversed. Those affected also suffer from circulatory problems and a significantly reduced ability to cope with stress. In most cases, children die from the symptoms of double outlet right ventricle if the condition is not treated early. Treatment of double outlet right ventricle usually involves surgery. In most cases, this surgery is successful and completely relieves the symptoms. However, in severe cases, transplants of the heart and lungs are necessary to cure the disease. However, these cannot always be performed, so these patients usually die.
Prevention
There are no preventive measures that can be taken to prevent double outlet right ventricle. For example, the heart defect is already congenital.
Follow-up care
In most cases of double outlet right ventricle, the patient has very few options and measures of aftercare. In this case, the affected person is in any case dependent on an intervention that can solve the malformation of the heart, so that it does not come to further complications or in the worst case to the death of the affected person. The earlier the disease is recognized and treated, the better the further course of the disease usually is, so that the early recognition of the disease is in the foreground in the case of double outlet right ventricle. As a rule, a surgical procedure is then performed on the heart to alleviate the symptoms. Even after the operation, the affected person is dependent on permanent and lifelong examinations of the heart in order to detect and treat further damage. Since transplantation of the heart or a lung may also be necessary in some cases, Double outlet right ventricle usually significantly reduces the life expectancy of the affected person. Sufferers should generally take it easy on their bodies and not subject themselves to unnecessary exertion. A healthy lifestyle with a healthy diet, as well as abstaining from alcohol and tobacco, can also alleviate the condition.
Here’s what you can do yourself
Double outlet right ventricle is a congenital heart defect that forms during embryonic development. There are no self-help measures that the patient or his or her parents can take to combat the condition causally. However, it is important that the malformation is recognized quickly and treated professionally. Unless the disorder is detected before or immediately after birth, parents of affected infants must respond promptly. Infants suffering from a double outlet right ventricle often do not drink properly and breathe heavily or at an accelerated rate. Such symptoms should always be discussed promptly with a physician. Infants diagnosed with double outlet right ventricle almost always must undergo multiple complex surgeries. Until the disorder is corrected, there is also a risk of sudden cardiac death. If the surgeries are unsuccessful, lung damage usually results. The child can then only be saved by a heart-lung transplant. The parents and family members of an affected child must prepare for a long period of regular hospital stays, doctor visits and home nursing. In addition, the child’s life is often in danger. Parents and relatives should not bear this enormous burden alone, but should seek psychotherapeutic care in good time.
