Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.15.1 (ACE)
 18,300 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In Eisenmenger's syndrome a central left-to-right shunt in the heart, a congenital anomaly, leads to pulmonary hypertension which subsequently causes the shunt to be reversed. The hypoxaemia resulting from a right-to-left shunt is compensated by an increase of the haemoglobin concentration due to a rise of the haematocrit. In adult patients not operated (adequately), the symptoms are the consequence of the erythrocytaemia and an increased haemorrhagic diathesis. In the long run heart failure develops. Phlebotomy is indicated for patients with haematocrits higher than 0.65 with signs of hyperviscosity and is also advised before non-cardiac surgery to improve coagulation parameters. Phlebotomy should be performed slowly (500 ml in 30-45 min) with simultaneous volume replacement. Excessive phlebotomy causes iron deficiency and spherocytosis which increase viscosity as well as the risk of CVA. Treatment consists of iron supplementation. Anticoagulation is indicated only in case of atrial fibrillation or mechanical valves. The use of acetylsalicylacid or NSAIDs is relatively contraindicated, because of abnormal haemostasis in these patients. During treatment with ACE inhibitors and other vasodilators, hypovolaemia should be avoided, because at a lower systemic blood pressure the right-to-left shunt increases and a potentially fatal cyanosis may occur.
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PMID:[Eisenmenger syndrome in adults]. 1032 Dec 57

During pregnancy cardiac output rises by 40-50%. At first stroke volume in particular increases, with the heart rate rising mainly in the second part of the pregnancy. More and more women with congenital heart disease are reaching adulthood and want to become pregnant. The haemodynamic changes during pregnancy are an additional burden for women with heart disease. Risk factors for occurrence of important cardiac problems during pregnancy are: previous cardiac events, lower functional class, cyanosis, left-sided heart obstruction or a diminished systolic function of the systemic ventricle. Pregnancy is contraindicated in pulmonary hypertension, in Marfan syndrome with an ascending aorta diameter of > 50 mm and in severe mitral or aortic valve stenosis. The recurrence risk for children varies between 3 and 50%, depending on the nature of the congenital heart disease. Foetal outcome is depending on the haemodynamic situation and medication use of the mother. ACE inhibitors are contraindicated during pregnancy.
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PMID:[Congenital heart disease and pregnancy]. 1583 34