Eisenmenger's syndrome

Eisenmenger, the reaction is a reaction of the lung to an elevated pressure in its blood vessels. It is caused by a congenital heart defect in which a so-called shunt creates a non-physiological connection between right and left heart. When a pronounced primary left- to-right shunt is produced showing that pulmonary resistance increase can with consecutive Shuntumkehr and clearly cyanosis developed or developing, this is called Eisenmenger's syndrome.

Description

The left heart supplies the body with oxygen-rich blood and builds up to a relatively high blood pressure (see below). It denotes the left half of the heart and the arteries supplied from a high-pressure system. When a connection between right and left side of the heart is, this pressure may continue in the right heart and the lungs in which physiological, a much lower pressure prevails (low pressure system ). The vessels of the pulmonary circulation are not designed for such a high pressure, and react with a media hypertrophy, that is a thickening of the muscle in the wall of the arteries. Thereby decreases the lumen of the pulmonary vessels, and the resistance against the incoming blood is increased. This process continues until the blood pressure in the lungs exceeds that of the systemic circulation. As a result, the blood flows in the opposite direction through the circuit defect (now a right-left shunt). Due to the flow direction reversal less blood flows through the lungs and it is thus less oxygenated, the affected people are cyanotic and develop, inter alia, of heart failure.

The disease progresses in stages aggravating ( technical term: progressive) and is untreated connected to a shortening of the life span. About four out of five people survive the first ten years after the onset of Eisenmenger reaction. Depending on the time of shunt reversal of an age of about 20 to 50 years is achieved on average. Drug therapy can only delay the consequences; as a curative treatment is ultimately only a heart- lung transplant is available.

Terminology

Synonymous with the concept of Eisenmenger reaction are occasionally used the terms Eisenmenger syndrome and Eisenmenger's complex. However, this is incorrect. See: Eisenmenger Complex and Eisenmenger's syndrome. A Eisenmenger reaction can occur in the presence of Eisenmenger's syndrome, in which a pronounced primary left- to-right shunt is present, can that pulmonary resistance increase with consecutive Shuntumkehr and significant cyanosis developed or developing.

History

The Eisenmenger reaction was named after the Austrian physician Victor Eisenmenger (1864-1932), of the special first described in 1897 based on a then 32 - year-old man, of a pulmonary hypertension ( pulmonary hypertension ), a ventricular septal defect (VSD ) and a horse aorta had.

Anatomic Explanations of

Usually, the left and right side of the heart are anatomically separated, so that the heart can be divided as a whole in the right heart and left heart.

A shunt (English, German: " Soft, shunt " in medicine " blood transfer between blood vessels or chambers of the heart " ) arises when there are malformations of the anatomical separation of the two halves of the heart, such as a hole between the ventricles ( ventricular septal defect, VSD), a hole between the atria ( atrial septal ASD ) or a hole between ventricle and atrium with malformed heart valve ( atrial ventricular septal defect, AV canal, AVSD ).

The task of the left ventricle is the oxygen-rich (red ) blood to pump through the system arteries through the body of people (large circulation / systemic circulation ). It is stronger than the right ventricle, thus it generates an approximately six times higher blood pressure.

The object of the right ventricle is deoxygenated (blue ) blood from the lungs through the pulmonary arteries to pump (small circulation / pulmonary circulation ). Where the blood is oxygenated. The pressure in it is lower than in the left ventricle, since the blood with less resistance to pass through the lungs. In people without Eisenmenger reaction, the blood volume of the cycles is balanced.

Is due to an anatomical feature a left-right shunt between the two halves of the heart, oxygen-rich (red ) blood to flow from the left side of the heart into the right half of the heart ( and from there into the pulmonary circulation ), since the pressure in the left half of the heart is greater than in the right.

Are the malformations between the halves of the heart low, this is usually not a major problem. Are the malformations but significantly, so that a large shunt volume is (as with a large VSD, the complete AV canal / AVSD or the patent ductus arteriosus ), oxygen-rich blood is pumped in addition and with a very high left ventricular pressure in the pulmonary circulation very much. This leads to an incorrect loading of the right heart and pulmonary vessels.

This is usually the point at which surgical correction of the corresponding heart defect is necessary at the latest, because the lung is anatomically designed to deal with the excessive blood supply and in particular the excessive pressure of the blood.

If no correction of the cardiac malformation made ​​or may be made no correction, it comes sooner or later to an increase in pulmonary vascular resistance (pulmonary resistance increase ). The vascular resistance of the lung increases initially, eventually change the arterioles irreversibly different wall structure. The lung hardened, so to speak, it is inelastic, vessels constrict and become smaller ( vasoconstriction ). This is a defensive reaction of the lung to the extra blood. The high pressure of the blood in the lungs is known as pulmonary hypertension ( pulmonary hypertension ). Accept what extent the vascular damage depends on what period of time they are exposed to pulmonary hypertension and how high is the pressure to which they have to withstand.

Is this high blood pressure in the pulmonary vessels over a long period, there is a reversal of the blood circulation in the heart: by pulmonary hypertension the blood accumulates in the right half of the heart, but still continues to try to pump the blood into the lungs. This leads to an enlargement of the right ventricle and a pressure increase.

Sooner or later, the pressure in the right half of the heart is as great as the pressure in the left and when it finally becomes higher than the latter, the blood flows following the pressure gradient from the right heart to the left heart half. The vascular resistance in the pulmonary vascular resistance exceeded in the systemic circulation. This is called a shunt reversal: The previously existing left-right shunt without cyanosis (Li -Re - shunt / blood flows from the left to the right side of the heart ) returns after a different long-lasting stage of a crossed shunts (pendulum shunt / biventricular shunt) in a right-left shunt with cyanosis to (Re- Li- shunt / blood flows from the right to the left side of the heart ).

The oxygen-poor (blue ) blood from the right heart mingles in this manner with the oxygen-rich ( red ) blood in the left half of the heart and is pumped from there into the systemic circulation.

As a result, there is a more or less extensive oxygen supply of the body by insufficient oxygen levels in the arteries (see also hypoxemia ), which cyanosis (Greek: κυάνεος "blue" ) causes. This is reflected in a violet to bluish tint of the skin ( usually on the face ) and the mucous membranes, and a bluish discoloration of the lips and the capillaries under the nails. Cyanosis is called Spätzyanose because it has not passed from the start as a symptom. Cyanosis may as well (mostly in the advanced stage ) occur both in stressful situations at rest. The latter in particular signaled an already far advanced destruction of the pulmonary vascular bed and is therefore considered Signum bad omen (Latin: bad sign, evil omen ) for the forecast.

If, because of pulmonary hypertension in left- to-right shunt pulmonary artery pressure of the blood, or pulmonary vascular resistance exceeds the normal systemic arterial resistance permanently, the shunt reversal manifests, and the affected person is cyanotic.

The conditional by a fixed pulmonary hypertension occurrence of cyanosis as a result of shunt reversal with congenital heart disease who were initially not associated with cyanosis is called Eisenmenger reaction.

Depending on the nature of the causative malformations in the heart of the Eisenmenger reaction occurs already during the first year of life, but also in part until later and sometimes even before birth one.

Diagnosis

In the Eisenmenger reaction during examination with a stethoscope can be heard without cleavage and often called Graham - Steell noise, a high-frequency frühdiastolisches pulmonary insufficiency noise after the second heart sound, heard in the second or third intercostal space, a strongly accented Pulmonalklappenschlußton (P2 ) left of the sternum. It reflects the significant increase in resistance against the pulmonary circulation. In the radiograph of the chest shows on the one hand a slight enlargement of the heart ( cardiomegaly ) with a significant expansion of the Pulmonalarterienhauptstammes ( strong Pulmonalissegment, wide central hilar pulmonary vessels ) and on the other a vessel- poor, transparent and reduces perfused lung periphery with the caliber jump phenomenon. Echocardiography as well as an investigation by means of a cardiac catheter are part of the diagnostic tests. Evident is an increasing right heart.

Effects, treatment, prognosis

Since the shunt reversal no lung flooding symptoms such as rapid breathing, persistent rapid pulse, sweating, restlessness, acrocyanosis, poor feeding, poor weight gain, peripheral edema or liver enlargement ( hepatomegaly ) are more, the affected person feels indeed better, but the point has now been reached from which the heart defect is no longer operational to correct, as the anatomical changes (enlargement of the right heart, hardening of the lungs by the above high pressure to which it was exposed and had to withstand ) are not corrected, and the resulting long-term altered pressures persist would.

Despite an operative closure of the hole between the halves of the heart, the increased pressure in the right ventricle would remain. After the operation, although the usual circulatory condition would again be present, the right ventricle but still not work properly and finally the right heart would decompensate.

As a result, only a combined heart -lung transplantation is despite intensive research in the field of drug intervention as a treatment after the onset of Eisenmenger reaction is currently still in question, which is associated with high risks. Without this measure is to start from a clearly limited life expectancy of the affected people. Medication currently has only a delaying effect in terms of the consequences. Palliative care can be tried to reduce pulmonary hypertension by administration of tolazoline or prostacyclin.

While people are often inconspicuous with Eisenmenger reaction in children at rest and often show only on exertion symptoms such as breathlessness and abnormally rapid fatigue, the symptoms intensified in severity and intensity usually no later than the second to third decade of life:

In particular, from the age of 24 it relatively frequently comes to pulmonary hemorrhage, which often leads to death. Therefore, despite the increased likelihood of thromboembolism, the use of aspirin is not recommended. However, the blood thus remains unusually viscous, which greatly increases the risk of emboli in the brain to develop (usually microemboli ) and brain abscesses. Occasionally, as a result, to convulsions. In addition, it may lead to increase in uric acid, which can lead to gout and kidney damage ( nephropathy). With increasing age, malignant cardiac arrhythmias are common. People with Eisenmenger's syndrome and congenital heart defects develop a central cyanosis with polycythemia, associated with the secondary signs of chronic hypoxemia with überschießendem growth of small blood vessels (angiogenesis / symptom among others Watchglass ).

Many affected people have recurring fainting spells ( syncope ), angina pectoris and in part will experience a cough that is accompanied with bloody sputum (haemoptysis ). Repeated hypoxic seizures can occur in the immediate administration of oxygen is necessary. This can be viewed as a sign of a significant deterioration in the overall condition, which usually means a poor prognosis in terms of durability. In heaping oxygen deficiency symptoms and significantly decreased oxygen saturation values, the ( nocturnal) administration of oxygen may help to slow the progression of a strong increase in the number of red blood cells in peripheral blood. Vasodilating agents ( vasodilators ) are not recommended for long-term therapy. Only a few examples show an improvement in the capacity of humans with Eisenmenger reaction Nifedipintherapie or applying an oral Prostazyklinanalogons.

People with Eisenmenger reaction develop an ever increasing right heart failure ( right heart failure ) and their lung function is disturbed, there is audible respiratory disabilities. They are thus always less physically fit, exertional dyspnea occurs, whereby the use of a wheelchair or the like is displayed in part for walks. Finally, means almost any form of special physical activity greatest effort and the affected people are increasingly relying on the help of other persons dependent. A decease of sudden cardiac arrest is possible.

The susceptibility to bronchopulmonary infections is sometimes greatly increased and corresponding diseases are often very poorly tolerated. Therefore should be avoided activities that are associated with an increased risk of heart and lung diseases. In particular, should be avoided to the active and passive consumption of such substances that inhibit the oxygen uptake and utilization (eg tobacco smoke).

The life expectancy of affected patients is significantly limited and depends, inter alia, from the date on which the Eisenmenger reaction has manifested. This is usually done until 12 months after birth. Four out of five people survive the first ten years after the manifestation. The death on chronic right heart failure or severe pneumonia occurs in patients with Eisenmenger's a reaction between 20 and 50 years.

Pregnancy

The presence of Eisenmenger reaction involves in pregnancy very high risks for the woman and leads to the classification of gestation as a high- risk pregnancy. An individual assessment of the hemodynamic situation is required; Experience has shown, however, the Eisenmenger syndrome of women an indication for the earliest possible termination of pregnancy. When deciding to have an abortion but also the risks of anesthesia required for this must be observed.

Turns out the decision to carry the child, the pregnant woman must be very beautiful and imagine at short intervals to check-ups. Particular attention must be paid during the stage of labor, childbirth and the puerperium, that it is not to reduce the amount of blood in the circulation ( hypovolemia ) or drop in blood pressure and along with this comes in a decrease in cardiac output. This would result in fainting or coughing up blood. Pregnant with Eisenmenger reaction often die of sudden cardiac death, eg, hypovolemia, in shock states due to bleeding during or after delivery, rupture of the pulmonary arteries or brain abscesses. Three to five out of ten women survive pregnancy does not, the death rate of children is close to 30 % and about half of the babies comes in a premature birth to the world.

In pregnant women with Eisenmenger reaction, a spontaneous delivery should be pursued with careful epidural anesthesia; vacuum extraction ( suction cup childbirth ) may optionally shorten the course of labor. The delivery should take place in a hospital setting on high-risk births.

For drug treatment is to say that it is essential to ensure that certain medications may increase the right-left shunt. For prevention of thrombosis and embolism administration of a medicament for inhibiting blood clotting ( anticoagulation ) is advised in the last eight to ten weeks of pregnancy and during the first four weeks after delivery.

In view of the high risks associated with pregnancy for women with Eisenmenger reaction with itself, the question of a definitive contraception is in the air: Side effects of contraceptives are sometimes regarded as critical to the clotting budget. To permanently prevent the interruption of tubal pregnancies ( tubal ligation ) came during a laparoscopic surgery as compared with the lowest risk prevention ability in question.