Eisenmenger Syndrome

(Eisenmenger syndrome: diseases with increased pulmonary vascular resistance)

The Eisenmenger syndrome is a complication of untreated, large intracardiac left-right shunt. An increased pulmonary resistance can develop over time, which leads in the end to a bidirectional shunting and right-left shunt. Nichtoxygeniertes blood flows into the systemic circulation and causes hypoxia. The heart sounds and heart sounds depend on the underlying abnormalities. The diagnosis is made by echocardiography or cardiac catheterization. The treatment is usually only supportive, but a heart-lung transplant may be an option in severe cases. Endocarditis prophylaxis is recommended.

Among the congenital heart abnormalities that can lead to Eisenmenger syndrome untreated, include the following:

The Eisenmenger syndrome is a complication of untreated, large intracardiac left-right shunt. An increased pulmonary resistance can develop over time, which leads in the end to a bidirectional shunting and right-left shunt. Nichtoxygeniertes blood flows into the systemic circulation and causes hypoxia. The heart sounds and heart sounds depend on the underlying abnormalities. The diagnosis is made by echocardiography or cardiac catheterization. The treatment is usually only supportive, but a heart-lung transplant may be an option in severe cases. Endocarditis prophylaxis is recommended. Among the congenital heart abnormalities that can lead to Eisenmenger syndrome untreated, include the following: ventricular septal defect atrioventricular canal atrial septal defect Patent ductus arteriosus truncus arteriosus transposition of the great vessels in the United States, the incidence significantly diminished since the diagnosis is made early and a final correction causing the abnormality is performed. A right-left shunt through a Eisenmenger syndrome causes cyanosis and its complications. The low systemic oxygen saturation leads to drumstick fingers and toes, secondary to polycythemia, hyperviscosity, hemoptysis, CNS findings (z. B. brain abscess or stroke), to an increased turnover of red blood cells, with all its consequences (eg. As hyperuricemia, gout caused and hyperbilirubinemia, which leads to gallstones and an iron deficiency with or without anemia). Symptoms and signs The symptoms do not occur before 20 to 40 years of age and include cyanosis, syncope, exertional dyspnea, fatigue, chest pain, atrial – ventricular arrhythmias and, rarely, a right heart failure (hepatomegaly, peripheral edema, dilated jugular veins) one. Hemoptysis is a late symptom. Sign cerebral embolism, a brain abscess or endocarditis may develop. The secondary polycythemia occurs frequently causes the following symptoms: temporary ischemic attacks of slurred speech, or other neurological symptoms, blurred vision, headache, increased fatigue or sign of a thromboembolic disorder. The abdominal pain can be caused by gallstones. On clinical examination, one finds a central cyanosis and clubbing. Rarely signs of right heart failure can be seen (heart failure). A holosystolic sound of tricuspid regurgitation may be present in the lower left sternal border. A frühdiastolisches decrescendo a Pulmonalklappeninsuffizienz can be heard at the left sternal border may. A loud, single second heart sound (S2) is always present, a Auswurfton is frequent. One-third of patients have scoliosis. Diagnostic chest X-ray and ECG Diagnosis is by echocardiography or cardiac catheterization. The diagnosis is suspected in a non-corrected heart defects through the history, supported by an X-ray and ECG and confirmed by a two-dimensional Farbechokardiographie and Doppler sonography. A cardiac catheterization is often performed to measure the pulmonary artery pressure, pulmonary vascular resistance and response to pulmonary vasodilators. Laboratory research polycythemia with a hematocrit> 55%. The increased turnover of red blood cells leads to iron deficiency (eg. As Mikrozythämie), hyperuricemia and hyperbilirubinemia. Radiographs show prominent central pulmonary arteries, peripheral, plum-like shading and a right ventricular enlargement. The ECG can be found a right ventricular hypertrophy, a right deviation of the axis of the heart and, occasionally, an enlargement of the right atrium. Treatment medications to reduce the pressure of the pulmonary arteries Supportive care Heart and Lung Transplantation Ideally, should be the surgical correction of the heart defect in time to prevent Eisenmenger syndrome (z. B. prostacyclin antagonists, endothelin antagonists, nitric oxide enhancer). If the syndrome has developed, there is no specific treatment other than the heart and lung transplantation. Drugs that lower the pressure in the pulmonary artery, are explored. These include Prostacyclinantagonisten (Treprostinol, epoprostenol) Endothelantagonisten (bosentan) and nitric oxide liberators (Sildenafil). Supportive therapy includes all measures to prevent an exacerbation of the syndrome (z. B. Pregnancy, volume depletion, isometric exercises great heights), and an additional dose of O2. The symptomatic polycythemia can be carefully treated with phlebotomy to reduce the hematocrit to 55-65% while volume expansion with 0.9% saline. However, a compensated and asymptomatic polycythemia requires no bleeding, regardless of Hct; Bloodletting eventually leads to iron deficiency and does not alter the natural course. The hyperuricemia is p.o. with allopurinol 300 mg once daily. An anticoagulation with warfarin is potentially harmful and their use should be dosed individually, but aspirin 81 mg p.o. is displayed once a day to prevent thrombotic complications. Life expectancy depends on the type and severity of the underlying congenital malformation and is between 20 and 50 years, the median age of death is 37 years. However, the low resilience and sequelae limit the quality of life considerably. The heart-lung transplantation is an option, but can be considered only for severe cases of intolerable quality of life. The long-term prognosis after transplantation is not promising. All patients should be before dental procedures or surgery that can cause bacteremia, endocarditis prophylaxis (see Table: Recommended endocarditis during dental procedures or surgery of the airways *) were obtained. Important points heart defects include the large intracardiac left-right shunts often cause increased pulmonary resistance in the end, the first to bidirectional shunting and finally right-left shunt (shunt reversal) leads. In the shunt reversal deoxygenated blood passes into the systemic circulation, resulting in hypoxia and its complications (e.g., clumping of the fingers and toes, secondary polycythemia.); polycythemia can cause hyperviscosity, stroke or other thromboembolic diseases and / or hyperuricemia. The symptoms do not occur before 20 to 40 years of age and include cyanosis, syncope, exertional dyspnea, fatigue, chest pain, atrial – ventricular arrhythmias and, rarely, a right heart failure. Conducting a surgical correction of cardiac anomaly underlying the appropriate age can prevent Eisenmenger syndrome. If the syndrome has developed, there is no specific treatment other than the heart and lung transplantation. Drugs that lower the pressure in the pulmonary artery (z. B. prostacyclin antagonists, endothelin antagonists, nitric oxide enhancer) will be explored.

Health Life Media Team

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