The Eisenmenger syndrome is a congenital heart defect. It causes severe lung and heart damage as a result of pulmonary arterial hypertension (PAH). A heart-lung transplant is the only curative therapy. Most patients only live up to the third decade of life.
According to PERCOMPUTER.COM, Eisenmenger Syndrome is also called Eisenmenger Reaction or Eisenmenger Complex. It is based on a congenital heart defect. This is usually diagnosed in childhood. This enables the blood from the left half of the heart to get back into the right half of the heart via a shunt, a hole in the wall of the heart sheath.
The resulting change in pressure in the heart creates pathological vascular pressure in the pulmonary arteries. This change manifests itself in pulmonary arterial hypertension (PAH), which is also called pulmonary hypertension. The lung function is restricted and leads to shortness of breath. Cardiovascular complaints arise and lead to life-threatening complications.
The cause of the iron quantity syndrome is a defect in the heart septum. The heart septum divides the heart into the right and left halves of the heart. The oxygen-poor, venous blood from the tissues collects in the right half of the heart. The blood now flows into the lungs, where it is enriched with oxygen. The oxygen-rich blood then reaches the left half of the heart and is brought back into the body’s circulation via the main artery, the aorta.
The force that is needed to bring the blood into our body is greater than the force that is needed to pump it into the lungs. If the blood now reaches the right half of the heart from the left half of the heart through a left-right saunt in the heart septum, stronger pressure builds up. Blood collects in the left heart. This puts a strain on the heart. It has to work harder so that the heart muscle grows and becomes insufficient.
The risk of thrombosis increases due to the low flow rate. Now the pressure in the left ventricle increases steadily. This gradually becomes greater than the pressure in the right ventricle. There is a stunt reversal. The oxygen-poor blood now enters the body’s circulation directly without going through the pulmonary circulation to become saturated with oxygen. An oxygen deficit arises. This is usually shown by a blue discoloration (cyanosis) of the fingers and lips. This is known as the amount of iron reaction. Inadequate oxygen supply to organs is fatal.
Symptoms, ailments & signs
The symptoms of Eisenmenger syndrome are caused by heart failure. Typical symptoms of heart failure are cyanosis, exertion-dependent shortness of breath, fatigue, dizziness, headache and syncope. Many patients perceive their heart beating faster and stronger. This is known as palpitation.
Further consequences of insufficient oxygen supply are sensations of misery on arms and legs. These are caused by nerve fibers that go under, as these also need oxygen to survive. The eye also needs a lot of oxygen, so visual disturbances manifest themselves early. Another sensitive organ is the kidney. Even under physiological conditions it has a low oxygen content in the tissue.
If this saturation drops further, the kidney tissue dies. Since the kidneys are responsible for regulating the water balance as well as regulating blood pressure, high blood pressure develops in many patients. This can also damage blood vessels and the heart muscle.
A heart defect can be discovered during the physical examination by the doctor. The doctor listens to the lungs and heart with his standing head. If the suspicion of a heart defect solidifies, an electrocardiogram (EKG) and an echocardiogram are performed. Here the enlargement of the heart (right heart hypertrophy) and the shunt are determined.
Polyglobulia, an increased number of erythrocytes or hemoglobin concentration in the blood can be found in the blood count. It is a physiological adaptation to the internal lack of oxygen. However, it is accompanied by changes in the viscosity of the blood.
Eisenmenger syndrome is already a complication of an undetected heart defect. As a result of the syndrome, accumulations of blood and mucus in the lungs, which are accompanied by coughing fits and inflammation, are acute. It can also lead to attacks of weakness and, as a result of the loss of blood, acute anemia with feelings of weakness and circulatory collapse.
If the syndrome is left untreated, the underlying heart failure inevitably leads to further heart and lung complications. Typical complaints are tiredness, dizziness, headache and shortness of breath when moving. As a result of the accelerated pulse, palpitation can also occur, in which the own heartbeat is perceived as particularly loud and annoying.
The lack of oxygen also leads to concentration disorders and thus increases the risk of accidents and falls. As a result, there are sometimes abnormal sensations in the limbs, severe visual disturbances and kidney problems. In the long term, Eisenmenger syndrome causes permanent damage to the blood vessels and internal organs.
The heart, lungs and brain are stressed the most. The changed viscosity of the blood affects other body-internal processes and is usually associated with a sharp decrease in general well-being.
When should you go to the doctor?
If the typical symptoms of heart failure are noticed, a doctor should be consulted immediately. Eisenmenger syndrome manifests itself, for example, through fatigue, dizziness and headaches. If these symptoms occur and do not go away within a few days, this indicates a heart defect. A doctor must determine whether this is Eisenmenger syndrome or another condition. If there is indeed a heart defect, regular visits to the cardiologist are indicated.
Depending on how far Eisenmenger’s syndrome has progressed, a heart-lung transplant may still be possible. In most cases, however, treatment is limited to palliative measures. These must be used in any case, because otherwise the already limited lifetime will be shortened even further.
Therefore, if you suspect a heart disease, you must speak to a doctor immediately. Parents who notice unusual symptoms in their child that cannot be attributed to any other cause should consult their pediatrician. The doctor can diagnose Eisenmenger syndrome and promptly initiate cardiological treatment.
Treatment & Therapy
Treatment of the patient is usually limited to palliative treatment. A curative, healing, therapeutic measure is only possible through a heart-lung transplant. Repairing the defect in the presence of PAH is pointless, as the increased pressure caused by the Eisenmenger reaction has already caused irreversible damage to the lungs.
Surgical correction can only take place before this event. Such an operation is contraindicated in adults, as there is a high rate of complications and mortality. Diuretics, antiarrhythmics and anticoagulations, as well as digitalis, can slightly prevent the progression of the disease.
Diuretics lower the volume in our body by excreting more water through the kidney. Less volume means less pressure in the buttocks. The heart is relieved. Antiarrhythmics prevent atrial or ventricular fibrillation. The fibrillation promotes the development of an embolism in the lungs and brain. The anticoagulations, also known as blood thinners, also reduce the risk of embolism.
Modern approaches are drug interventions in the hemodynamics. This is attempted using endothelin receptor antagonists, PDE-5 inhibitors and prostacyclin analogs. These drugs cause blood vessels to dilate. The resistance in the vessels decreases. Less resistance requires less pressure. The cardiac output is improved. The oxygen saturation does not.
Outlook & forecast
The age at which the diagnosis is made is decisive for the outlook for Eisenmenger syndrome. If this is done at an early age, before the blood vessels in the lungs are irreversibly damaged and pulmonary hypertension has occurred, the malformation can be corrected with an operation that promises to be successful. The chances of survival are lower if the abnormality is more extensive or if another disease occurs in addition to Eisenmenger syndrome, such as: B. Down syndrome.
Pregnancy is also associated with mortal danger for those affected. The death rate is over 50% in pregnant women with Eisenmenger syndrome. The general life expectancy of patients with this disease is between 20 and 50 years, depending on the type and extent of the anomaly; those affected who have fewer symptoms due to a minor malformation can even live to be around 60 years. The average age at death is around 37 years.
The quality of life of those affected is significantly reduced due to the restricted resilience and the occurrence of secondary diseases. However, due to the poor long-term prospects after the transplant, a heart-lung transplant is usually only considered if the quality of life is severely restricted.
The patient’s education and training is the most decisive factor for the best possible outcome. Patients should not smoke to avoid further lung damage. Alcohol consumption should be kept moderate. Exercise and sport only take place where possible. Pregnancy should be avoided as 50 percent of patients die during it. Particularly good dental hygiene is important as infections in the oral cavity can spread to the heart.
In most cases, the patient with Eisenmenger syndrome does not have any special or direct measures and options for follow-up care. The disease must first and foremost be recognized and treated at an early stage so that other complaints or complications no longer arise. In most cases, Eisenmenger syndrome significantly reduces the life expectancy of those affected.
Since this is also a congenital disease, genetic counseling can also be carried out if you want to have children. This may prevent Eisenmenger syndrome from being inherited. In most cases, those affected with this disease are dependent on surgery and medication. However, the procedure can only take place in childhood.
In general, the person concerned should not strain their heart unnecessarily and lead a healthy lifestyle. You should also refrain from alcohol or tobacco. When taking the medication, always pay attention to the correct dosage and to the instructions of the doctor in order to alleviate the symptoms of Eisenmenger syndrome. In many cases, those affected are also dependent on the help of their families in their everyday lives.
You can do that yourself
In Eisenmenger syndrome, treatment is limited to palliative measures. Depending on how far the disease has progressed, those affected can take some measures themselves to alleviate the symptoms.
First of all, however, bed warmth and protection apply, as the disease puts an enormous strain on the entire body. At the same time, the patients should speak to the responsible doctor on a regular basis. Unusual symptoms can be clarified and further treatment options discussed with the doctor.
As a rule, the doctor will also recommend therapeutic advice to the patient or establish contact with other affected persons. Attending a self-help group in particular can help the seriously ill to better understand and accept the illness and the symptoms it brings with it.
The individual symptoms can at least be alleviated by conservative measures. Headaches, fatigue, and dizziness are best countered with a long walk or nap. In the event of breathlessness, the person affected should lie down and consult a doctor as soon as possible. In severe cases with breathing difficulties and cardiovascular complaints of all kinds, the emergency services should be called directly.