APV stands for anomalous pulmonary veins. The anomalous pulmonary venous connection represents a malfunction of the lungs. The blood is usually pumped from the veins of the lungs into the atrium on the left side. In the context of an anomalous pulmonary vein, however, the blood wrongly reaches the right side of the heart, disrupting the usual flow.
What is an anomalous pulmonary vein junction?
If people suffer from an abnormal pulmonary vein confluence, the blood is transported to the right atrium of the heart. In principle, it is blood that has already been enriched with oxygen through the lungs. Normally, the blood goes to the left side. From there it flows into the so-called aorta and is finally pumped back into the circulatory system. See AbbreviationFinder for abbreviations related to APV.
In the anomalous pulmonary vein, however, the blood flows into the right side. Basically, there are different forms of anomalous pulmonary veins, which primarily refer to the different degrees of severity of the disease. There is partial and total anomalous pulmonary vein confluence and the so-called scimitar syndrome.
The anomalous pulmonary vein is a congenital disorder that develops in fetuses in the womb. However, in some cases the disease is only discovered later or only by chance.
Symptoms, Ailments & Signs
The symptoms and complaints that occur as part of the anomalous pulmonary vein connection vary primarily depending on the respective form and characteristics of the anomalous pulmonary vein connection present in the individual case. Total anomalous pulmonary vein confluence is a very severe form of the disease.
The disease is also referred to as Total Anomalous Pulmonary Venous Connection. The abbreviation TAPVC, which may also be used in medical jargon in this country, is derived from this term. In the case of total anomalous pulmonary veins, all the veins in the lungs connect to the circulatory system, which is on the right side.
There are four pulmonary veins. For this reason, the pulmonary veins are completely abnormal. Depending on the individual severity of the disease, the veins flow into different areas. Thus, openings into the right atrium, the upper or lower vena cava and the so-called hepatic vein are possible.
If there is a partial anomalous pulmonary vein confluence, only a certain proportion of the veins in the lungs end in the wrong area. In addition, in the case of partial anomalous pulmonary veins, only one lung is usually affected by the disease. It turns out that partial anomalous pulmonary veins occur far more frequently on the left side than on the right, with a ratio of about 1:2.
The veins flow either into the right atrium, the superior vena cava and, in rare cases, into the inferior one. In addition, there is often a defect in the atrial septum. This disorder may also be referred to by the abbreviation ASD. Because some of the veins in the lungs drain into the wrong areas, too much blood is pumped to the right side of the heart.
This is oxygen-enriched blood, which puts a great strain on the right heart. The impairment affects in particular the ventricle and the corresponding atrium. The volume of blood at this point is too large, putting a considerable strain on the heart. In addition, the lungs are supplied with more blood than usual.
If the anomalous pulmonary vein confluence occurs in childhood, the affected patients usually do not suffer from any symptoms and show normal resilience. The defect of the heart is usually only discovered by chance at a doctor’s appointment. Depending on the form and type of anomalous pulmonary vein confluence, the therapeutic measures that are used also change.
Diagnosis & course of disease
The diagnosis of anomalous pulmonary veins is based on various examination techniques, which the attending physician uses after taking the anamnesis. First of all, the conversation with the patient plays an important role, with the description of the complaints and lifestyle being the focus of consideration.
Once the doctor has received enough information about the patient and the symptoms shown, he begins to carry out further examination measures. An EKG examination, for example, is often used. The result usually shows an excessively heavy load on the right heart. During an echocardiography, there is often an enlargement of the ventricle on the right side.
In addition, the results indicate an expansion of the pulmonary trunk. X-rays show increased blood flow to the lungs and an enlarged heart. However, this is often only weakly pronounced. Other possible examination methods consist of a cardiac and a heart catheter examination. The veins of the lungs and the areas of their mouths can be displayed more clearly.
In the worst case, an anomalous pulmonary vein can lead to the death of the person concerned. It must therefore be treated by a doctor in any case, since this disease will not heal itself. The heart is put under a lot of strain by the high oxygen content in the blood, which can lead to heart failure. The patient usually dies.
Furthermore, the resilience of the patient is significantly reduced due to the anomalous pulmonary vein confluence and most of those affected suffer from constant fatigue. In children, this complaint can therefore lead to delayed and restricted development. Due to the significant overloading of the heart, without treatment, the life expectancy of those affected is reduced.
However, the diagnosis is relatively easy to make. The further course of the disease depends heavily on the severity of the symptoms. As a rule, an operation is necessary in the case of anomalous pulmonary veins in order 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 the treatment is successful.
When should you go to the doctor?
People who suffer from respiratory disorders or irregular heart rhythms should consult a doctor. A general malaise, a feeling of illness or an inner weakness are signs of discrepancies that need to be clarified. Reduced performance, lack of energy or problems in performing daily tasks must be examined and treated. In many cases, the anomalous pulmonary vein remains symptom-free for a long time. This makes diagnosis difficult and delays the start of treatment.
If the affected person’s usual resilience drops or if the usual physical activities and sporting activities can only be carried out to a limited extent, a doctor should be consulted. In the case of sleep disorders, tachycardia or abnormalities in blood pressure, it is advisable to seek medical advice for the symptoms. If there is a feeling of pressure in the chest, breathing becomes difficult or it is difficult for the person concerned to breathe deeply, a doctor should be consulted. Consultation with a doctor can also take place with a vague feeling of disagreement.
If perceptions cannot be classified, if fears arise or if there are circulatory disorders, a visit to the doctor is advisable. In the event of interruptions in attention, an increased need for sleep, exhaustion or easy fatigue, it is necessary to discuss the symptoms with a doctor. Inner restlessness, headaches or an incomprehensible feeling of heat should also be examined by a doctor.
Treatment & Therapy
The treatment of the anomalous pulmonary veins depends primarily on the type of disorder. A total anomalous pulmonary vein requires surgery, which is usually performed as soon as possible after the diagnosis is made.
A heart-lung machine is used. Surgery is also usually necessary for partial anomalous pulmonary veins. The blood from the lungs is channeled through patches into the atrium on the left side.
Outlook & Forecast
The pulmonary vein malformation can usually be successfully treated by surgery. Affected children recover after intensive medical therapy and continue to develop without any abnormalities in 90 percent of all cases. The disease is congenital. Scientific advances allow for very good prospects of a cure. As part of the aftercare, regular check-ups are necessary, which can also be followed in adulthood.
In about every tenth case, the first surgical intervention is unsuccessful. First, the doctors will try to widen the vessels again. A heart catheter is suitable for this. If this does not succeed, the prospect is very bad. Another operation involves significant 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 anomalous pulmonary vein confluence is decisive for life expectancy. The total form of expression often leads to early infant death due to respiratory weakness and susceptibility to infections. The partial pulmonary vein malformation can go undetected for years. Subjectively, there are no signs at all for the person concerned. Minor progressive forms are usually diagnosed as part of routine examinations or other complaints.
There are no options for preventing anomalous pulmonary veins because the disease is congenital.
As a rule, the anomalous pulmonary vein is a serious and, above all, life-threatening disease that must be examined and treated by a doctor in any case. If unusual symptoms and signs appear outside of the regular check-up visits, a doctor must be consulted immediately. Most of those affected suffer from severe breathing difficulties due to the anomalous pulmonary vein opening, which can lead to permanent tiredness and exhaustion. It is therefore not possible for most of those affected to actively participate in everyday life. They are encouraged to avoid stressful or physically demanding activities. Sometimes this unwanted shutdown leads to psychological upsets. Encouraging conversations with family or friends can help easier to deal with the disease. It cannot be universally predicted whether this will lead to complete healing.
You can do that yourself
Patients with an anomalous pulmonary vein suffer from a significantly limited physical resilience and therefore pay particular attention to maintaining sufficient and regular rest periods and not to physically overexerting themselves. The practice of sporting activities must always be coordinated with the doctor treating you and certain types of sport should be avoided altogether. It is important for those affected to protect their lungs and avoid respiratory infections. This applies in particular in preparation for a surgical intervention to correct the anomalous pulmonary vein confluence.
In order to support the success of the operation, the patient tries to strengthen his immune system and eat a balanced diet before the operation. Contact with possibly contaminated air and smoking must be avoided. Immediately after the operation to correct the anomalous pulmonary veins, the patients receive intensive medical care and must follow all the doctors’ instructions.
As soon as the patients are back home after the surgical procedure, there is hardly anything standing in the way of a life without the disease. During the healing, however, those affected pay attention to the prescribed rest phases and avoid physical stress or even overexertion. Lifelong cessation of smoking is recommended and reduces the risk of complications.