Eisenmenger reaction
| Classification according to ICD-10 | |
|---|---|
| I27.8 | Other specified pulmonary heart diseases |
| ICD-10 online (WHO version 2019) | |
The Eisenmenger reaction is a reaction of the lungs to increased pressure in their blood vessels. It is caused by a congenital heart defect in which a so-called shunt creates an unphysiological connection between the right and left heart. If the primary left-right shunt is so pronounced that an increase in pulmonary resistance with consecutive shunt reversal and clear cyanosis develops or can develop, it is referred to as Eisenmenger syndrome .
description
The left heart supplies the whole body with oxygen-rich blood and builds up a relatively high blood pressure (see below). The left half of the heart and the arteries it supplies are called the high-pressure system. If there is a connection between the right and left halves of the heart, this pressure can continue into the right heart and the lungs, which physiologically have a much lower pressure (low-pressure system). The vessels of the pulmonary circulation are not designed for such a high pressure and react with media hypertrophy , i.e. a thickening of the muscles in the wall of the arteries. As a result, the lumen of the pulmonary vessels decreases and the resistance to the inflowing blood increases. This process continues until the blood pressure in the lungs exceeds that of the circulatory system. As a result, the blood flows in the opposite direction through the circulatory defect (now a right-left shunt). By reversing the direction of flow, less blood flows through the lungs and it is therefore less enriched with oxygen, the affected people become cyanotic and develop, among other things. a. a heart failure .
The disease is progressive , that is, worsens over time, and if left untreated is associated with a shortening of life. About four in five people survive the first ten years after the onset of the Eisenmenger reaction. Depending on when the shunt is reversed, the average age is around 20 to 50 years. Drug therapy can only delay the consequences; as a curative treatment is ultimately only a heart-lung transplant is available.
terminology
The terms Eisenmenger syndrome and Eisenmenger complex are occasionally used synonymously for the term Eisenmenger reaction . However, this is not correct. See: Eisenmenger complex or Eisenmenger syndrome . An Eisenmenger reaction can arise in the presence of Eisenmenger syndrome, in which the primary left-right shunt is so pronounced that an increase in pulmonary resistance with subsequent shunt reversal and marked cyanosis develops or can develop.
history
The Eisenmenger reaction was named after the Austrian doctor Viktor Eisenmenger (1864–1932), who first described the peculiarity in 1897 based on a then 32-year-old man who had pulmonary hypertension (pulmonary high pressure), a ventricular septal defect (VSD) and a rider Aorta .
Anatomical explanations for understanding
The left and right halves of the heart are usually anatomically separated so that the heart as a whole can be divided into a right heart and a left heart.
A shunt ( English , German: "Weiche, Nebenschluss", in medicine " Blood transfer between blood vessels or heart chambers") occurs when there are malformations in the anatomical separation of the two halves of the heart, e.g. B. a hole between the heart chambers ( ventricular septal defect , VSD), a hole between atria ( atrial septal defect , ASD) or a hole between the heart chamber and atrium with malformed heart valves ( atrio-ventricular septal defect , AV canal, AVSD).
The task of the left ventricle is to pump the oxygen-rich ( red ) blood through the human body via the system arteries ( large circulation / body circulation). It is stronger than the right ventricle, so it generates about six times higher blood pressure. The pumping volume of the left ventricle is called cardiac output .
The task of the right ventricle is to pump the oxygen-poor ( blue ) blood through the pulmonary arteries through the lungs ( small circulation / pulmonary circulation). There the blood is supplied with oxygen. The pressure in it is lower than in the left ventricle because the blood can get through the lungs with less resistance. The pumping volume of the right ventricle is called the lung time volume .
In people without septal defects, the blood volume of the circuits is balanced. Cardiac output and lung output are identical.
If, due to an anatomical peculiarity, there is a left-right shunt between the two halves of the heart, oxygen-rich ( red ) blood flows from the left half 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. If, on the other hand, there is a right-left shunt between the two halves of the heart due to an anatomical peculiarity, oxygen-poor blood flows from the right half of the heart into the left half of the heart (and from there into the circulatory system ), as the pressure in the right half of the heart is greater than in the left . If both defects are present at the same time, this is called a bidirectional shunt because the blood flows in both directions. The shunt volume is the blood flow per unit of time. In general, the equation cardiac output + left-right shunt volume = lung time volume + right-left shunt volume applies.
If the malformations between the two halves of the heart are minor, this is usually not a major problem. However, if the malformations are significant, so that there is a large shunt volume (e.g. in the case of a large VSD, the complete AV canal / AVSD or the ductus arteriosus apertus), a large amount of oxygen-rich blood is additionally and very high left ventricular pressure in the Pulmonary circulation pumped. This leads to improper stress on the right heart and the pulmonary vessels.
This is usually the point in time at which an operative correction of the corresponding heart defect is necessary at the latest, because the lungs are anatomically not designed to deal with the excessive blood supply and in particular with the excessively high pressure of the blood.
If the heart malformation is not corrected or if no correction can be made, sooner or later there will be an increase in pulmonary vascular resistance (increased pulmonary resistance ). The vascular resistance of the lungs initially increases, and finally the arterioles irreversibly change their wall structure. The lungs harden, so to speak, they become inelastic, vessels constrict and become smaller ( vasoconstriction ). This is a defense reaction of the lungs to the extra blood. The high pressure of the blood in the lungs is called pulmonary hypertension ( high lung pressure ). The extent of the vascular damage depends on how long you are exposed to pulmonary hypertension and how high the pressure you have to withstand.
If this high blood pressure persists in the pulmonary vessels for a longer period of time, the blood circulation in the heart is reversed: the pulmonary high pressure causes the blood to build up in the right half of the heart, which nevertheless tries to pump the blood into the lungs. This leads to an enlargement of the right ventricle and an increase in pressure.
Sooner or later the pressure in the right half of the heart becomes as great as the pressure in the left and when it finally becomes greater than this, the blood flows following the pressure gradient from the right half of the heart to the left half of the heart. The vascular resistance in the lungs has exceeded the vascular resistance in the body's circulation. This is called shunt reversal : The previously existing left-right shunt without cyanosis ( Li-Re shunt / blood flows from the left to the right half of the heart) is reversed after a phase of crossed shunt lasting differently long ( pendulum shunt / biventricular shunt ) into a right-left shunt with cyanosis ( Re-Li shunt / blood flows from the right to the left half of the heart).
In this way, the oxygen -poor ( blue ) blood from the right half of the heart mixes with the oxygen-rich ( red ) blood in the left half of the heart and is pumped from there into the body's circulation.
As a result, the body is more or less under-supplied with oxygen due to an insufficient oxygen content in the arteries (see also hypoxemia ), which causes cyanosis ( Greek : κυάνεος " blue "). This manifests itself in a purple to bluish tint of the skin (mostly on the face) and the mucous membranes as well as a bluish discoloration of the lips and the capillary vessels under the fingernails. The cyanosis is called late cyanosis because it has not been a symptom to begin with . Cyanosis can occur both in stressful situations and (usually in an advanced stage) in a resting state. The latter, in particular, signals an already well advanced destruction of the pulmonary vascular bed and is therefore a signum mali ominis ( Latin : bad sign, bad premonition ) for the prognosis.
If, due to pulmonary hypertension in the left-right shunt, the pulmonary arterial pressure of the blood or the pulmonary vascular resistance permanently exceeds the usual system arterial resistance, the shunt reversal manifests itself and the affected person becomes cyanotic.
The occurrence of cyanosis caused by fixed pulmonary hypertension as a result of shunt reversal in congenital heart defects that were not initially associated with cyanosis is known as the Eisenmenger reaction .
Depending on the severity of the underlying malformations in the heart, the Eisenmenger reaction occurs as early as the first year of life, but sometimes later and sometimes even before birth .
diagnosis
In the Eisenmenger reaction, a strongly accentuated closing tone of the pulmonary valve (P2) without cleavage can be heard during the examination with the stethoscope and often the so-called Graham Steell noise , a high-frequency early diastolic pulmonary insufficiency noise after the second heart tone , audible in the second to third intercostal space to the left of the breastbone. The heart murmur , named after the American Graham Steell, was first described in 1868 by the Polish coroner and internist Karol Gilewski (1832–1871). reflects the significant increase in resistance in the pulmonary circulation. The chest X-ray shows, on the one hand, a slight enlargement of the heart ( cardiomegaly ) with a clear expansion of the main pulmonary artery trunk (strong pulmonary segment , wide central hilar pulmonary vessels) and, on the other hand, a vascularized, transparent and reduced blood flow lung periphery with the caliber jump phenomenon. The echocardiography and an examination using a cardiac catheter are part of the diagnostic examinations. An increasing right heart hypertrophy can be seen.
Effects, treatment, prognosis
Since the shunt reversal means that there are no longer any symptoms of pulmonary flooding such as rapid breathing, prolonged pulse acceleration, sweating, restlessness, acrocyanosis , poor drinking, insufficient weight gain, peripheral edema or enlarged liver ( hepatomegaly ), the affected person feels better, but the point has now been reached , from which the heart defect can no longer be corrected surgically, as the anatomical changes (enlargement of the right half of the heart, hardening of the lungs due to the excessive pressure to which it was exposed and had to withstand) cannot be compensated, and the long-term changes in pressure conditions continue to exist would.
Despite the surgical closure of the hole between the two halves of the heart, the increased pressure in the right ventricle would remain. After the operation, the normal circulatory conditions would be restored, but the right ventricle would still work incorrectly and finally the right heart would decompensate .
Because of this, despite intensive research in the field of drug intervention, the only possible treatment after the onset of the Eisenmenger reaction is a combined heart-lung transplant , which is associated with high risks. Without this measure, the life expectancy of the affected person can be assumed to be significantly limited. The administration of medication currently only has a delaying effect with regard to the consequences. Palliative attempts can be made to reduce the pulmonary hypertension by administering tolazoline or prostacyclin .
While people with Eisenmenger's reaction in childhood are often normal at rest and often only show signs such as shortness of breath and unusually rapid exhaustion during physical exertion, the severity and intensity of the symptoms usually increase in the second to third decades of life at the latest:
From the age of 24 in particular, there is comparatively frequent lung bleeding , which often leads to death . Therefore, despite the increased likelihood of is thromboembolism taking aspirin is not recommended. However, this leaves the blood unusually viscous, which greatly increases the risk of developing embolisms in the brain (usually microembolisms) and brain abscesses . Occasionally this leads to cramps. In addition, uric acid levels can increase, which can lead to gout attacks and kidney damage ( nephropathy ). Malignant cardiac arrhythmias are common with age . People with Eisenmenger syndrome and congenital heart defects develop central cyanosis with polyglobulia , accompanied by the secondary signs of chronic hypoxemia with excessive growth of small blood vessels ( angiogenesis / symptom including watch glass nails ).
Many affected people have recurring fainting spells ( syncope ), angina pectoris and sometimes they get a cough that is accompanied by bloody sputum ( hemoptysis ). Hypoxic seizures can occur repeatedly, which require immediate administration of oxygen. This can be viewed as a sign of a significant deterioration in the overall condition, which usually means an unfavorable prognosis with regard to the service life. In the case of frequent symptoms of oxygen deficiency and significantly reduced oxygen saturation values , the (nightly) administration of oxygen can help to slow down the progression of a strong increase in the number of red blood cells in the peripheral blood. Blood vessel dilators ( vasodilators ) are not recommended for long-term therapy. Only a few examples show an improvement in the resilience of people with Eisenmenger reaction under nifedipine therapy or with the use of an oral prostacyclin analogue .
People with Eisenmenger's syndrome develop a steadily increasing right heart failure ( right heart failure ) and their lung function is disturbed, there is audible respiratory disabilities. As a result, you become less and less physically resilient, stress dyspnea occurs, which means that the use of a wheelchair is sometimes indicated for walks or the like . After all, almost every form of special physical activity means the greatest effort and the people affected are increasingly dependent on the help of other people. Dying from sudden cardiac death is possible.
The susceptibility to bronchopulmonary infections is sometimes greatly increased and corresponding diseases are usually very poorly tolerated . Activities that involve an increased risk of heart and lung diseases should therefore be avoided. In particular, the active and passive consumption of substances that inhibit oxygen uptake and utilization (e.g. tobacco smoke ) should be avoided .
The life expectancy of affected people is clearly limited and depends, among other things. a. from the time the Eisenmenger reaction manifested itself. This usually happens up to the 12th month after the birth. Four out of five people survive the first ten years after manifestation. Death from chronic right heart failure or from severe pneumonia occurs in patients with Eisenmenger's reaction between the ages of 20 and 50.
pregnancy
The presence of an Eisenmenger reaction harbors very high risks for the woman during pregnancy 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, that women’s Eisenmenger syndrome is an indication for the earliest possible termination of pregnancy . However, when deciding to have an abortion, the risks of the anesthesia required for it must also be considered.
If the decision to carry the child to term is not made, the pregnant woman must take great care and present herself for check-ups at short intervals. During the labor phase , delivery and in the puerperium , particular care must be taken to ensure that there is no reduction in the amount of blood in the bloodstream ( hypovolemia ) or a drop in blood pressure and, as a result, a drop in cardiac output . The result would be fainting spells or coughing up blood. Pregnant women with Eisenmenger's reaction often die of z. B. sudden cardiac death , hypovolemia, shock due to bleeding during or after delivery, rupture of the pulmonary arteries or brain abscesses. Three to five out of ten women do not survive pregnancy, the death rate of children is just under 30% and around half of babies are born prematurely .
In pregnant women with Eisenmenger's reaction, a spontaneous delivery under careful epidural anesthesia should be sought; A vacuum extraction ( suction cup delivery) can shorten the birth process if necessary. The delivery should take place in a hospital prepared for high-risk births .
Regarding drug treatment, it must be said that it is essential to ensure that certain medications can strengthen the right-left shunt. To prevent thrombosis and embolism, it is advisable to administer a drug to prevent blood clotting ( anticoagulation ) in the last eight to ten weeks of pregnancy and in the first four weeks after delivery .
In view of the high risks that pregnancy entails for women with Eisenmenger's reaction, the question of definitive contraception is also in the room: Side effects of contraceptives are sometimes to be viewed as critical with regard to the coagulation balance. In order to permanently avoid pregnancies, the interruption of the fallopian tubes ( tube ligation ) during a laparoscopic procedure could be considered as a low-risk method of contraception.
Web links
- Graphic representations of the blood flow in the Eisenmenger reaction with description in English: 1
- 11/2007: Finally a therapy for Eisenmenger in sight
- Congenital heart diseases and pulmonary hypertension in adults (PDF file; 881 kB)
swell
- ^ Eisenmenger's syndrome - www.whonamedit.com
- ^ Klaus Holldack, Klaus Gahl: Auscultation and percussion. Inspection and palpation. Thieme, Stuttgart 1955; 10th, revised edition, ibid 1986, ISBN 3-13-352410-0 , p. 153.
- ↑ T. Borth-Bruhns, A. Eichler: Pediatrische Kardiologie. Springer, Berlin et al. 2004, ISBN 3-540-40616-6 , p. 131.
- ^ AA Schmaltz: Pregnancy with congenital heart defects. Universitätsklinikum Essen Medical facility of the university - GH Essen Center for Pediatric and Adolescent Medicine Department for Pediatric Cardiology, 2004.
