Marfan's Syndrome
In women with Mafan's Syndrome, pregnancy poses two problems:
(1) cardiovascular complications, and (2) high risk of child inheriting this condition.The cardiovascular complications that may occur are: (a) dilatation of ascending aorta which may result in aostic regurgitation and in heart failure, (b) proximal and distal dissection of aorta with possible extension to coronaries or iliac arteries. Dilated aorta is a risk for aortic dissection. Minor involvement of cardiovascular system and aortic size <40min in diameter are favorable signs in pregnancy and' they tolerate the pregnancy well. Colnplications usually occur in later stage of pregn'mcy. Obstetric complications like cervical incompetence, abnormal placental site and post partuni hemorrhage can occur in patients with Marfan's syndrome.
During pregnancy in most cases of associated HCM, there is a favorable outcome, but potentially associated with increased morbidity and mortality. In about 10-20 per cent of patients, worsening of symptoms is reported particularly in women who had symptoms prior to pregnancy. Arrhythmias, chest pain, palpitations, dizziness can occur. Persistent supraventricular tachycardias associated with fetal distress has been reported. Development of atrial fibrillation causes hemodynamic deterioration and may require electiic cardioversion. Ventricular arrhythmias may be responsible for maternal mortality. Fetal prematurity can occur in women wit11 symptoms before pregnancy. The risk of inheriting the disease may be as high as 10 per cent in familial cases and less in sporadic cases.
The approach in the management of these patients depends on presence of symptoms and LV out flow obstruction.During labor and delivery, blood loss is to be avoided in symptomatic patients with LV out flow obstruction and also drugs that cause vasodilatation or sympathetic stimulation are to be avoided. The medications during geslation period include anti arrhythmic drugs and drugs for the treatment of heart failure. Beta-adrenergic blocking agents and diuretics are to be used and calcium ar~lagonisls may be added if beta-blockers alone are not sufficient. In patients with syncope cardioverter defibrillators (automatic) and life threatening a r r h y t h ~ a s implantable may be considered before pregnancy i n patie~lts with histoly of syncope. Vaginal delivery is safe in patients with HCM and pregnancy bul lhe second stage of labor may be shortened by use of forceps. Use of prostaglandins to induce uterine contractions may be risky in obstructive cardiomyopathy because of their vasodilatory effects. Spinal and epidural anesthetics should be used wilh caution because of the same reason.
Antibiotic prophylaxis is considered in patients with obstructive cardiomyopathy because of risk of endocarditis during labor and delivery. Data on surgical treatment or percutaneous transcatheter septa1 ablation duiing pregnancy is not available.
In women with Mafan's Syndrome, pregnancy poses two problems:
(1) cardiovascular complications, and (2) high risk of child inheriting this condition.The cardiovascular complications that may occur are: (a) dilatation of ascending aorta which may result in aostic regurgitation and in heart failure, (b) proximal and distal dissection of aorta with possible extension to coronaries or iliac arteries. Dilated aorta is a risk for aortic dissection. Minor involvement of cardiovascular system and aortic size <40min in diameter are favorable signs in pregnancy and' they tolerate the pregnancy well. Colnplications usually occur in later stage of pregn'mcy. Obstetric complications like cervical incompetence, abnormal placental site and post partuni hemorrhage can occur in patients with Marfan's syndrome.
During pregnancy in most cases of associated HCM, there is a favorable outcome, but potentially associated with increased morbidity and mortality. In about 10-20 per cent of patients, worsening of symptoms is reported particularly in women who had symptoms prior to pregnancy. Arrhythmias, chest pain, palpitations, dizziness can occur. Persistent supraventricular tachycardias associated with fetal distress has been reported. Development of atrial fibrillation causes hemodynamic deterioration and may require electiic cardioversion. Ventricular arrhythmias may be responsible for maternal mortality. Fetal prematurity can occur in women wit11 symptoms before pregnancy. The risk of inheriting the disease may be as high as 10 per cent in familial cases and less in sporadic cases.
The approach in the management of these patients depends on presence of symptoms and LV out flow obstruction.During labor and delivery, blood loss is to be avoided in symptomatic patients with LV out flow obstruction and also drugs that cause vasodilatation or sympathetic stimulation are to be avoided. The medications during geslation period include anti arrhythmic drugs and drugs for the treatment of heart failure. Beta-adrenergic blocking agents and diuretics are to be used and calcium ar~lagonisls may be added if beta-blockers alone are not sufficient. In patients with syncope cardioverter defibrillators (automatic) and life threatening a r r h y t h ~ a s implantable may be considered before pregnancy i n patie~lts with histoly of syncope. Vaginal delivery is safe in patients with HCM and pregnancy bul lhe second stage of labor may be shortened by use of forceps. Use of prostaglandins to induce uterine contractions may be risky in obstructive cardiomyopathy because of their vasodilatory effects. Spinal and epidural anesthetics should be used wilh caution because of the same reason.
Antibiotic prophylaxis is considered in patients with obstructive cardiomyopathy because of risk of endocarditis during labor and delivery. Data on surgical treatment or percutaneous transcatheter septa1 ablation duiing pregnancy is not available.
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