Pulmonary vein malocclusion is a disorder of the function of the lungs. Blood is usually pumped from the veins of the lung into the atrium on the left side. However, in pulmonary vein malocclusion, the blood mistakenly goes to the right side of the heart, so the usual flow is disrupted.
What is pulmonary vein malperfusion?
If individuals suffer from pulmonary vein malperfusion, blood is transported to the right atrium of the heart. In principle, this is blood that has already been oxygenated by the lungs. Normally, the blood enters the left side. From there, it flows into the so-called aorta and is eventually pumped back into the circulatory system. In pulmonary vein malformations, however, the blood enters the right side. Basically, there are different forms of pulmonary vein malformations, which primarily refer to the different degrees of severity of the disease. Thereby, there are partial and total pulmonary vein malocclusion as well as the so-called scimitar syndrome.
Causes
Pulmonary vein malocclusion is a congenital disorder that develops in fetuses while they are still in the womb. However, in some cases, the condition is discovered later or only by chance.
Symptoms, complaints, and signs
The symptoms and complaints that occur as part of pulmonary vein malocclusion vary primarily depending on the particular form and severity of pulmonary vein malocclusion present in each case. Total pulmonary vein malocclusion represents a very severe form of the disease. The disease is also called Total Anomalous Pulmonary Venous Connection. From this term is derived the abbreviation TAPVC, which may also be used in medical jargon in this country. In total anomalous pulmonary venous connection, all the veins of the lungs connect to the circulation, which is on the right side. It involves four pulmonary veins. For this reason, there is a total pulmonary vein mis-orifice. Depending on the individual manifestation of the disease, the veins open into different areas. Thus, orifices into the right atrium, the superior or inferior hollow vein, and the so-called hepatic vein are possible. If partial pulmonary vein malocclusion is present, only a certain proportion of the veins of the lung end in the wrong area. In addition, in partial pulmonary vein malocclusion, usually only one lung is affected by the disease. This shows that partial pulmonary vein malocclusion is far more common on the left side than on the right, with a ratio of about 1:2. In this case, the veins either open into the right atrium, the superior vena cava, and in rare cases, the inferior vena cava. In addition, there is often a defect in the atrial septum. This disorder may also be referred to by the abbreviation ASD. Since some of the veins of the lungs open into the wrong areas, too much blood is pumped to the right side of the heart. This is oxygen-enriched blood, which places a great strain on the right heart. The impairment affects the ventricle and the corresponding atrium in particular. The volume of blood is too large at this point, so the heart is put under considerable strain. In addition, the blood supply to the lungs is greater than usual. If the pulmonary vein defect occurs in childhood, the affected patients usually suffer from no symptoms and show normal exercise capacity. The defect of the heart is usually discovered only by chance during a doctor’s appointment. Depending on the present form and type of pulmonary vein malocclusion, the therapeutic measures that are applied also change.
Diagnosis and disease progression
Diagnosis of pulmonary vein malocclusion is based on various examination techniques that the treating physician uses after taking a medical history. First, the interview with the patient plays an important role, focusing on the description of symptoms and lifestyle. Once the physician has obtained sufficient information about the patient and the symptoms shown, he begins to carry out further examination measures. Often, for example, an ECG examination is used. The result usually shows excessive stress on the right heart. An echocardiogram often reveals an enlargement of the ventricle on the right side.In addition, the results indicate enlargement of the pulmonary trunk. X-ray examinations show increased blood flow to the lungs and enlargement of the heart. However, this is often only mild. Other possible examination methods include cardiac and cardiac catheterization. This allows the veins of the lungs as well as the areas of their orifices to be more clearly visualized.
Complications
In the worst case, pulmonary vein malocclusion can lead to the death of the affected person. Therefore, it must be treated by a physician in any case, since there will be no self-healing in this disease. The heart is severely stressed by the high oxygen content in the blood, so that heart failure can occur. In this case, the patient usually dies. Furthermore, the patient’s exercise tolerance is also significantly reduced due to the pulmonary vein malfunction, and most sufferers experience constant fatigue. In children, this complaint can therefore lead to delayed and restricted development. Without treatment, the significant overload on the heart results in a reduced life expectancy for the affected individual. However, the diagnosis is relatively easy to make. The further course of the disease depends largely on the severity of the symptoms. As a rule, surgical intervention is necessary in pulmonary vein malocclusion to resolve the symptoms. There are no particular complications and the course of the disease is usually positive. The patient’s life expectancy is also not reduced if treatment is successful.
When should one go to the doctor?
People suffering from respiratory disorders or irregularities in heart rhythm should see a doctor. A general feeling of malaise, a feeling of illness or internal weakness are signs of inconsistencies that need to be clarified. Decreased performance, lack of strength, or problems performing daily tasks, should be investigated and treated. In many cases, pulmonary vein dysfunction remains asymptomatic for a long time. This makes it difficult to make a diagnosis and delays the start of treatment. If the usual resilience of the affected person decreases or the usual physical activities as well as sports activities can only be performed to a limited extent, a check-up visit to a doctor should be made. In the event of sleep disturbances, palpitations or abnormalities in blood pressure, it is advisable to seek medical clarification of the symptoms. If there is a feeling of pressure in the chest, breathing seems difficult or the person concerned finds it difficult to breathe deeply, a doctor should be consulted. Consultation with a physician can also take place in the case of a diffuse feeling of incongruity. If perceptions cannot be classified, if anxiety sets in, or if circulatory disturbances occur, a visit to the doctor is advisable. In case of interruptions of attention, an increased need for sleep, fatigue or easy fatigability, it is necessary to discuss the symptoms with a doctor. An inner restlessness, headaches or a sensation of warmth that cannot be understood should also be examined by a doctor.
Treatment and therapy
Treatment of pulmonary vein malocclusion depends primarily on the nature of the disorder. Total pulmonary vein malocclusion requires surgical intervention, which is usually performed as soon as possible after the diagnosis is made. This involves the use of what is known as a heart-lung machine. Surgery is also usually required for partial pulmonary vein malperfusion. Blood from the lungs is directed through so-called patches into the atrium on the left side.
Outlook and prognosis
Pulmonary vein malformation can usually be successfully treated with surgery. Affected children recover after intensive medical therapy and continue to develop without abnormalities in 90 percent of all cases. The disease is congenital. Scientific advances allow very good prospects of cure. Regular check-ups are necessary as part of follow-up care, which should also be followed in adulthood. In about one in ten cases, the first surgical intervention is not successful. First, the doctors will try to widen the vessels again. A cardiac catheter is suitable for this purpose. If this does not succeed, the outlook is very poor. Another operation entails considerable risks. A fatal outcome is usually foreseeable.In principle, cases are also conceivable in which a diagnosis does not take place. Then, the extent of the pulmonary vein malformations is decisive for life expectancy. The total form of expression often leads to early infant death in view of respiratory weakness and susceptibility to infections. The partial pulmonary vein malformation may exist undetected for years. Subjectively, there are then no signs at all for the affected person. Most often, minor progressions are diagnosed during routine examinations or when other complaints are present.
Prevention
Options for prevention of pulmonary vein malocclusion do not exist because the condition is congenital.
Follow-up care
As a rule, pulmonary vein malocclusion is a serious and, more importantly, life-threatening disease that must be examined and treated by a physician in all cases. If any unusual symptoms or signs develop outside of regular check-up visits, a physician should be consulted immediately. Most affected individuals suffer from severe breathing difficulties due to pulmonary vein malocclusion, which can lead to permanent fatigue and exhaustion. As a result, most sufferers are unable to actively participate in everyday life. They are advised to avoid stressful or physically demanding activities. Sometimes this involuntary shutdown leads to psychological upsets. Encouraging conversations with relatives or friends can help to make it easier to cope with the disease. Whether this can lead to a complete cure cannot be universally predicted.
What you can do yourself
Patients with pulmonary vein malocclusion suffer from significantly reduced physical capacity and therefore take special care to maintain adequate and regular rest periods and to avoid overexerting themselves physically. The exercise of sporting activities should always be coordinated with the attending physician and certain types of sport should be avoided altogether. For those affected, it is important to take care of their lungs and avoid respiratory infections. This is especially true in preparation for surgery to repair the pulmonary vein malocclusion. To support the success of the surgery, patients try to strengthen their immune system and eat a balanced diet in advance of the procedure. Contact with potentially contaminated air as well as smoking must be avoided at all costs. Immediately after the surgery to correct the pulmonary vein malocclusion, patients receive intensive medical care and must follow all instructions given by the doctors. As soon as patients return to live at home after the surgical procedure, there is little in the way of a life without the disease. During the healing process, however, the patients pay attention to the prescribed rest periods and avoid physical stress or even overexertion. The lifelong renunciation of smoking is recommended and reduces the risk of complications.
