Peiipartum cardiomyopathy is a form of dilated cardiomyopathy of uncertain etiology with (i) development of cardiac failure in the last month of pregnancy or within 5 months after delivery, (ii) absence of demonstrable cause for cardiac failure,(iii) absence of demonstrable heart disease before pregnancy, and (iv) documented systolic dysfunction. The symptoms appear during last month of pregnancy but usually the diagnosis is made in the early peripartum period.
CEnieal Features and Prognosis
Peripartum Cardiomyopatt~y is more common in multiparous women, twin pregnancies, in those with pse-eclampsia and in women older than 30 years. The exact etiology is unknown though myocarditis as a cause has been suggested by some investigators documei~ted by myocardial biopsy, but other investigators did not t?nd this association. Low selenium levels and autoantibodies have also been iillplicated in the pathogenesis of peripartum cardiomyopathy. The comnlon symptoms are, breathlessness, fatigue, chest pain, palpitations, weight gain, peripheral edema,peripheral or prxlmonary embolisation and arrhythmias. Other sympt~rns include cough, orthopnea paroxysmal nocturnal dyspneri, hemophysis, and abdominal pain.Physical examination reveals enlargements of heart, presence of third heart sound and murmurs of mitral and tricuspid regurgitation.
Investigations
The electrocardium may show sinus tachycardia, ST-T changes, conduction abnormalities and arrhythmias. Chest X-ray should be perlo~rned with abdomin;il shielding t o avoid fetal radiation during pregnancy, but it can be done safely in peripartum period. Cliest X-ray usually shows cardiomegaly, pulmonary venous congestion with interstitial o r alveolar edema and sometimes pleural effusion.Echocardiography needs to be performed in all patients, which cornmonly reveal enlargement of all cardiac chambers with reduction in left velltriclrlar systolic function. Mild to moderate pericardial effusion may be seen. Doppler study may show evidence of mitral, tricuspid and puln~onary regurgitation. The clinical presentation and hemodynanlic changes are indistinguishable from other forms of dilated cardiomyopathy. Role of cndoinyocardial biopsy is controversial. He~nodynamic monitoring may be done in critically iIl patients.
Thc clinical course of peripartium cardiomyopathy is variable with 50 to 60 per cent of patients showirig complete or near complete recovery, usually within first six months post parturn, Mortality varies from 7-50 per cent from small series. The usual causes are progressive heart failure, mhythrnia and thromboembolism. Acute maternd hypoxia can cause fetal distress. Women with p e r i p ~ i u m cardiornyopathy often develop relapse with subsequent pregnancies. leading to left ventricuIar dysfunction, symptomatic deterioration and even to death. Relapse is more common in patients with persistent abnormal cardiac function, but can occur in women in whom, left ventricular function is restored after the first episode. The reported mortality is 0 to 2 per cent in patients with norma1 left ventricular ejection fraction before the subsequent pregnancy and 8 to 17 per cent in patients with depressed ventricular ejectidn fraction. Hence subsequent pregnancies should be discouraged inpatients with petipartum cardiomyopathy with persistent cardiac dysfunction.However, womeil with recovered cardiac function also can get relapse but the risk of mortality appears to be less and hence in them also subseclueilt pregnancies are to be discouraged.
Diagnosis
There is no specific test available for the diagnosis of pei-ipartum casdioinyopathy and hence it is diagnosed by exclusion of other causes of left ventricular dilatation and systolic dysfunction. Some of these conditions are valvular lesions,cardiomyopathy due to specific causes like alcoholic, diabetic, and others. The development of cardiac failure in last of month of pregnancy or within five months after delivery, absesce of demonstrable cause of cardiac failure and documentation of left ventricular systolic dysf~iilnction, absence of heart disease before pregnancy, help in the diagnosis.
CEnieal Features and Prognosis
Peripartum Cardiomyopatt~y is more common in multiparous women, twin pregnancies, in those with pse-eclampsia and in women older than 30 years. The exact etiology is unknown though myocarditis as a cause has been suggested by some investigators documei~ted by myocardial biopsy, but other investigators did not t?nd this association. Low selenium levels and autoantibodies have also been iillplicated in the pathogenesis of peripartum cardiomyopathy. The comnlon symptoms are, breathlessness, fatigue, chest pain, palpitations, weight gain, peripheral edema,peripheral or prxlmonary embolisation and arrhythmias. Other sympt~rns include cough, orthopnea paroxysmal nocturnal dyspneri, hemophysis, and abdominal pain.Physical examination reveals enlargements of heart, presence of third heart sound and murmurs of mitral and tricuspid regurgitation.
Investigations
The electrocardium may show sinus tachycardia, ST-T changes, conduction abnormalities and arrhythmias. Chest X-ray should be perlo~rned with abdomin;il shielding t o avoid fetal radiation during pregnancy, but it can be done safely in peripartum period. Cliest X-ray usually shows cardiomegaly, pulmonary venous congestion with interstitial o r alveolar edema and sometimes pleural effusion.Echocardiography needs to be performed in all patients, which cornmonly reveal enlargement of all cardiac chambers with reduction in left velltriclrlar systolic function. Mild to moderate pericardial effusion may be seen. Doppler study may show evidence of mitral, tricuspid and puln~onary regurgitation. The clinical presentation and hemodynanlic changes are indistinguishable from other forms of dilated cardiomyopathy. Role of cndoinyocardial biopsy is controversial. He~nodynamic monitoring may be done in critically iIl patients.
Thc clinical course of peripartium cardiomyopathy is variable with 50 to 60 per cent of patients showirig complete or near complete recovery, usually within first six months post parturn, Mortality varies from 7-50 per cent from small series. The usual causes are progressive heart failure, mhythrnia and thromboembolism. Acute maternd hypoxia can cause fetal distress. Women with p e r i p ~ i u m cardiornyopathy often develop relapse with subsequent pregnancies. leading to left ventricuIar dysfunction, symptomatic deterioration and even to death. Relapse is more common in patients with persistent abnormal cardiac function, but can occur in women in whom, left ventricular function is restored after the first episode. The reported mortality is 0 to 2 per cent in patients with norma1 left ventricular ejection fraction before the subsequent pregnancy and 8 to 17 per cent in patients with depressed ventricular ejectidn fraction. Hence subsequent pregnancies should be discouraged inpatients with petipartum cardiomyopathy with persistent cardiac dysfunction.However, womeil with recovered cardiac function also can get relapse but the risk of mortality appears to be less and hence in them also subseclueilt pregnancies are to be discouraged.
Diagnosis
There is no specific test available for the diagnosis of pei-ipartum casdioinyopathy and hence it is diagnosed by exclusion of other causes of left ventricular dilatation and systolic dysfunction. Some of these conditions are valvular lesions,cardiomyopathy due to specific causes like alcoholic, diabetic, and others. The development of cardiac failure in last of month of pregnancy or within five months after delivery, absesce of demonstrable cause of cardiac failure and documentation of left ventricular systolic dysf~iilnction, absence of heart disease before pregnancy, help in the diagnosis.
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| Perlpartum Cardiomyopathy |
Management
The management of heart failure is similar to that of dilated cardiomyopathy. General treatment includes rest, low salt diet. Acute heart failure needs to be treated vigorously with oxygen, digitalis, diuretics and vasodilators.
i)Dig~xirt: Clearance of this drug is markedly increased during pregnancy and may need higher dosage. However, this drug crosses placenta and has been used to treat fetal arrhythmias, hence fetal heai-t rate monitoring is needed. It also crosses in to breast nlilk. Digoxin is particularly useful in patients with atrial fibiillation to control ventricular rate. Loading dose of 0.25mg tablets, six hourly for four doses may not be required in majority of patients and maintenance dosage is 0.125mg to 0.25mg per day may be given.
ii) Diuretics: Furoseinide is the drug of choice during pregnancy. It can cross the placenta and may affect the electrolytes and hence lowest effective dose need to be used. Furoseinide 10-20mg intravenously or 20-40mg oral tablets may be given.
iii) Vasodilators: The use of hydralazine as an after load reducing agent appears to be safe in pregnancy. Hydralazine and nitrates may be combined with advantage.Angiotensin converting enzyme inhibitors are contraindicated in pregnancy as they may cause fetal renal dysfunction and fetal death.
iv) Iitotropic agents: Dopamine, dobutamine and milrenoile have been used in severely ill patients and these patients are best managed in tertiary care centers.Temporary use of intraaortic ball2011 pump and left ventiicular assist devices inay help to stabilize the patient's condition.
V)Other medications: Intravenous immunoglobulins have shown improvenlent in left ventricular function. Patients with severe heat failure who do not recover early should be considered for cardiac transplantation.Most of the mild forms of peripartum cardiomyopathy call be managed conservatively, but if response to usual medication is not satisfactoly and severely ill patients need to be referred for centers where specialists and fecilities are available. If inother is stabilized medically, spontaneous delivery can be anticipated. Vaginal deliveries are associated with lower rates of complicatioil than cesarean deliveiy.
vi) Anticoagulants: Ai-terial or venous thrombosis has been reported to occur in as many as 50 per cent of women with peripartum cardiomyopathy, particularly in presence of atrial fibrillation. Hence anticoagulants, unfructionated heparin and in the post pa-turn period warfarin is recommended. (Details of anticoagulants therapy is discussed under prosthetic valve and pregnancy).
The management of heart failure is similar to that of dilated cardiomyopathy. General treatment includes rest, low salt diet. Acute heart failure needs to be treated vigorously with oxygen, digitalis, diuretics and vasodilators.
i)Dig~xirt: Clearance of this drug is markedly increased during pregnancy and may need higher dosage. However, this drug crosses placenta and has been used to treat fetal arrhythmias, hence fetal heai-t rate monitoring is needed. It also crosses in to breast nlilk. Digoxin is particularly useful in patients with atrial fibiillation to control ventricular rate. Loading dose of 0.25mg tablets, six hourly for four doses may not be required in majority of patients and maintenance dosage is 0.125mg to 0.25mg per day may be given.
ii) Diuretics: Furoseinide is the drug of choice during pregnancy. It can cross the placenta and may affect the electrolytes and hence lowest effective dose need to be used. Furoseinide 10-20mg intravenously or 20-40mg oral tablets may be given.
iii) Vasodilators: The use of hydralazine as an after load reducing agent appears to be safe in pregnancy. Hydralazine and nitrates may be combined with advantage.Angiotensin converting enzyme inhibitors are contraindicated in pregnancy as they may cause fetal renal dysfunction and fetal death.
iv) Iitotropic agents: Dopamine, dobutamine and milrenoile have been used in severely ill patients and these patients are best managed in tertiary care centers.Temporary use of intraaortic ball2011 pump and left ventiicular assist devices inay help to stabilize the patient's condition.
V)Other medications: Intravenous immunoglobulins have shown improvenlent in left ventricular function. Patients with severe heat failure who do not recover early should be considered for cardiac transplantation.Most of the mild forms of peripartum cardiomyopathy call be managed conservatively, but if response to usual medication is not satisfactoly and severely ill patients need to be referred for centers where specialists and fecilities are available. If inother is stabilized medically, spontaneous delivery can be anticipated. Vaginal deliveries are associated with lower rates of complicatioil than cesarean deliveiy.
vi) Anticoagulants: Ai-terial or venous thrombosis has been reported to occur in as many as 50 per cent of women with peripartum cardiomyopathy, particularly in presence of atrial fibrillation. Hence anticoagulants, unfructionated heparin and in the post pa-turn period warfarin is recommended. (Details of anticoagulants therapy is discussed under prosthetic valve and pregnancy).
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