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Subsequent pregnancy in patients with previous peripartum cardiomyopathy
Current Obstetrics & Gynaecology (1998) 8, 56-58 © 1998 Harcourt Brace & Co. Ltd
Lessons in clinical practice
Subsequent pregnancy in patients with previous peripartum cardiomyopathy
J. M. Rutherford
She became pregnant again 2 years later. She remained symptomatically well during her pregnancy with no clinical signs of heart failure. Echocardiography was performed every 8 weeks. The left ventricle was not dilated, left ventricular function was globally good and fractional shortening was around 30-33% on each occasion. She was admitted in spontaneous labour at 41 weeks and was delivered of a healthy female infant by vacuum extraction. She remained well and was discharged on her third postpartum day. She was reviewed 2 weeks postpartum and echocardiogram showed a slightly hypertrophied left ventricle with some reduction in left ventricular function. Fractional shortening was 27%. There was also slight right ventricular dilatation. She had no signs or symptoms of heart failure and, therefore, received no treatment. The echocardiographic appearances were unchanged 2 weeks later. At 8 weeks post delivery an echocardiogram showed further deterioration with globally reduced left ventricular function and fractional shortening of 20-23%. However, as she remained well no treatment was added. An echocardiogram 6 months later showed a non-dilated left ventricle and fractional shortening of 32%.
CASE REPORT Case 1
A 29-year-old caucasian woman was admitted at 39 weeks in her first pregnancy with pre-eclampsia. Labour was induced, but because of fetal distress in labour, a lower segment Caesarean section was carried out. A healthy female infant was delivered. Following delivery, her blood pressure remained elevated. On the seventh postpartum day she was noted to have a left parasternal heave, a displaced apex beat and an ECG showing evidence of left ventricular hypertrophy. There were no other abnormalities on examination and a chest X-ray was normal. She was commenced on atenolol, and following this her blood pressure settled. An echocardiogram showed a dilated left ventricle with markedly impaired contraction, regional wall movement abnormality with poor septal contraction, and fractional shortening of 18% (normal >30%). Heart valves were reported to be normal. Atenolol was replaced with enalapril and she was commenced on warfarin. All other investigations, including thyroid function tests, anticardiolipin antibodies, lupus anticoagulant, car~ diac enzymes, VMA and viral screen, were normal. Over the subsequent few weeks she remained well. A repeat echocardiogram was performed 6 weeks after delivery. This showed significant improvement with a non-dilated left ventricle, overall improved contraction and fractional shortening of 26%. She remained on warfarin and enalapril for 10 months. An echocardiogram performed 5 months after delivery showed some improvement but with persistently reduced left ventricular function.
Case 2
A 32-year-old caucasian woman was admitted at 31 weeks in her first pregnancy complaining of palpitations, breathlessness, and feeling faint and sweaty. On examination, her pulse was 180 beats per minute and an ECG showed a narrow complex tachycardia. Following intravenous adenosine, she reverted to sinus rhythm with a rate of 90 beats per minute. On auscultation there was a loud pan-systolic murmur heard all over the precordium and radiating to the neck. There were no signs of heart failure.
Dr Jane M. Rutherford, Research Fellow in Obstetric Medicine, Department of Therapeutics, Queen's Medical Centre, Nottingham NG7 2UH
56
Pregnancy with previous peripartum cardiomyopathy 57 At 10 h after admission she remained in sinus rhythm with a rate of 102. Her apex beat was displaced to the left and there were bilateral basal crepitations in the lungs. A chest X-ray showed cardiac enlargement, enlarged pulmonary vessels and a small left pleural effusion. Echocardiogram showed dilated left atrium and ventricle, with significant mitral regurgitation but good left ventricular function. She was commenced on digoxin and frusemide. Over the subsequent 4 weeks she remained stable. She was admitted in spontaneous labour at 35 weeks and was delivered of a healthy male infant by vacuum extraction. Postnatally she remained well and was discharged home on aspirin, frusemide and digoxin. Over the subsequent 6 weeks she was weaned off all treatment. By 8 weeks postpartum she had only a very quiet systolic murmur and echocardiography showed a dilated left ventricle with good ventricular contraction. Heart valves were normal with no evidence of mitral regurgitation. She attended for a further echocardiogram 6 months postpartum and this showed slightly dilated left ventricle with mild free-wall hypokinesia but overall good function. Fractional shortening was 35%. She became pregnant again 2 years later. She was seen at 8 weeks' gestation and at this time there were no cardiac murmurs detected and an echocardiogram was reported as normal. At 16 weeks she was noted to have a moderate pan-systolic murmur. At 20 weeks she was symptomaticallywell and the pan-systolic murmur was unchanged. The echocardiogram showed some deterioration with mitral regurgitation into a slightly dilated left atrium and a dilated left ventricle, but overall function was good. The fractional shortening was 27%. Over the subsequent weeks she developed some breathlessness and mild orthopnoea but no clinical evidence of heart failure. Echocardiography showed little change in her cardiac status with fractional shortening varying between 30 and 41%. She remained otherwise well with a persistent pan-systolic murmur. Labour was induced at 36 weeks and she had a spontaneous vertex delivery of a healthy female infant. Postnatally she showed no signs of heart failure but was commenced on enalapril. At 1 week postpartum an echocardiogram showed slight deterioration with a fractional shortening of 25%. By 3 weeks postpartum the murmur was barely audible. Echocardiography 6 weeks postpartum showed normal mitral and aortic valves, with a whiff of mitral regurgitation into a nondilated left atrium. The left ventricle was slightly dilated with reasonably good function. Fractional shortening was 42%. The enalapril was stopped and she was discharged from the clinic.
DISCUSSION Peripartum cardiomyopathy (PPCM) is characterized by: (1) development of cardiac failure in the last
month of pregnancy or within 5 months of delivery; (2) absence of determinable aetiology for the cardiac failure; and (3) absence of demonstrable heart disease prior to the last month of pregnancy? It has an incidence of between 1 in 1300 and 1 in 15 000 pregnancies.2 There is a racial difference with increased incidence in black races. 3It carries a maternal mortality rate of 25-50% 3and may cause significant morbidity in those who have persistent cardiac failure. There is little information about the prognosis in subsequent pregnancies for women who have had PPCM. Early evidence suggested that women who have recovered left ventricular function 6 months following PPCM have a much better prognosis than those who have persistent cardiomegaly? However, in the early series, echocardiography was not available and the diagnosis of persistent cardiac dysfunction was made on the basis of clinical signs and X-ray evidence. Now that echocardiography is widely available it is possible that PPCM is being diagnosed in different patients. It is not yet known whether women who have 'recurrent PPCM' during subsequent pregnancies do so because of reactivation of the original underlying disease process or because of deterioration in subclinical cardiac dysfunction following their previous episode. Reported here are two cases of women who presented in their first pregnancy with PPCM, whose echocardiographic features of cardiac function returned to near normal within 6 months of delivery, and who had no residual symptoms of heart failure. In both of these cases, in subsequent pregnancies, there was demonstrable echocardiographic evidence of deteriorating left ventricular function during pregnancy or in the immediate postpartum period. In case 1 this was completely asymptomatic and in case 2 it was associated with mild symptoms of orthopnoea and dyspnoea only. During the last 5 years we know of no other women at this hospital who became pregnant after previous PPCM. Witlin et al recently reported on six patients who had pregnancies following previous PPCM) Four of these had clinical relapse and one had deterioration in echocardiographic features. These findings differ from those of Sutton et al, who reported four women who had subsequent pregnancies following PPCM and in whom there was no deterioration in either clinical state or the findings on echocardiography.4 In another report of four patients with previous PPCM who had subsequent pregnancies, none of the patients showed evidence of deterioration in cardiac status, although it is not clear whether they were followed with echocardiography throughout pregnancy2 Our data support the findings of Witlin et al2 and suggests that there is a significant risk of either subclinical or clinical deterioration in cardiac function in subsequent pregnancies. There is, however, little evidence regarding which patients will have irreversible cardiac dysfunction and long-term morbidity. In case 2 the patient was treated with enalapril post partum in
58
Current Obstetrics & Gynaecology
her second pregnancy on an empirical basis. Since it is known that angiotensin-converting enzyme inhibitors have a beneficial effect in promoting recovery of left ventricular function following myocardial infarction, we felt that it was possible that this could also be the case in P P C M : A recent experimental study measured the contractile reserve following a dobutamine challenge in nonpregnant women who had regained left ventricular function after PPCM. 7 This showed that women with 'recovered' PPCM had reduced contractile reserve compared to matched controls. The authors concluded that since the effect of the dobutamine challenge was to mimic some of the cardiovascular adaptations to pregnancy, their findings might explain recurrence of symptoms in subsequent pregnancies in patients who have apparently recovered. It is interesting to note that our two patients both had deterioration of their left ventricular dysfunction in the postpartum period. If this deterioration was due to the effects of the added cardiovascular stress of pregnancy, then it would be logical to assume that it would occur in the early part of pregnancy, when the greatest changes are taking place, rather than postnatally.
From the evidence of these two cases it can be seen that pregnancy after previous PPCM carries a risk of deteriorating left ventricular function even in those women whose cardiac function has returned to near normal after the initial episode. REFERENCES
1. Demakis JG, Rahimtoola SH, Sutton GC et al. Natural course of peripartum cardiomyopathy. Circulation 1971; 44: 1053-1061 2. Witlin AG, Mabie WC, Sibai BM. Peripartum cardiomyopathy: an ominous diagnosis. Am J Obstet Gynecol 1997; 176:182-188 3. Homans DC. Peripartum cardiomyopathy. N Engl J Med 1985; 312:1432-1437 4. Sutton MStJ, Cole R Plappert M, Saltzman D, Goldhaber S. Effects of subsequent pregnancy on left ventricular function in peripartum cardiomyopathy. Am Heart J 1991; 121: 1776-1778 5. Desai D, Moodley J, Naidoo D. Peripartum cardiomyopathy: experiences at King Edward VIII Hospital, Durban, South Africa and a review of the literature. Tropical Doctor 1995; 25:118-123 6. Simoons ML. Myocardial infarction: ACE inhibitors for all? for ever? Lancet 1994; 344:279-281 7. Lampert MB, Weinert L, Hibbard J, Korcarz C, Lindheimer M, Lang RM. Contractile reserve in patients with peripartum cardiomyopathy and recovered left ventricular function. Am J Obstet Gynecol 1997; 176:189-195
Lessons in clinical practice
Subsequent pregnancy in patients with previous peripartum cardiomyopathy
J. M. Rutherford
She became pregnant again 2 years later. She remained symptomatically well during her pregnancy with no clinical signs of heart failure. Echocardiography was performed every 8 weeks. The left ventricle was not dilated, left ventricular function was globally good and fractional shortening was around 30-33% on each occasion. She was admitted in spontaneous labour at 41 weeks and was delivered of a healthy female infant by vacuum extraction. She remained well and was discharged on her third postpartum day. She was reviewed 2 weeks postpartum and echocardiogram showed a slightly hypertrophied left ventricle with some reduction in left ventricular function. Fractional shortening was 27%. There was also slight right ventricular dilatation. She had no signs or symptoms of heart failure and, therefore, received no treatment. The echocardiographic appearances were unchanged 2 weeks later. At 8 weeks post delivery an echocardiogram showed further deterioration with globally reduced left ventricular function and fractional shortening of 20-23%. However, as she remained well no treatment was added. An echocardiogram 6 months later showed a non-dilated left ventricle and fractional shortening of 32%.
CASE REPORT Case 1
A 29-year-old caucasian woman was admitted at 39 weeks in her first pregnancy with pre-eclampsia. Labour was induced, but because of fetal distress in labour, a lower segment Caesarean section was carried out. A healthy female infant was delivered. Following delivery, her blood pressure remained elevated. On the seventh postpartum day she was noted to have a left parasternal heave, a displaced apex beat and an ECG showing evidence of left ventricular hypertrophy. There were no other abnormalities on examination and a chest X-ray was normal. She was commenced on atenolol, and following this her blood pressure settled. An echocardiogram showed a dilated left ventricle with markedly impaired contraction, regional wall movement abnormality with poor septal contraction, and fractional shortening of 18% (normal >30%). Heart valves were reported to be normal. Atenolol was replaced with enalapril and she was commenced on warfarin. All other investigations, including thyroid function tests, anticardiolipin antibodies, lupus anticoagulant, car~ diac enzymes, VMA and viral screen, were normal. Over the subsequent few weeks she remained well. A repeat echocardiogram was performed 6 weeks after delivery. This showed significant improvement with a non-dilated left ventricle, overall improved contraction and fractional shortening of 26%. She remained on warfarin and enalapril for 10 months. An echocardiogram performed 5 months after delivery showed some improvement but with persistently reduced left ventricular function.
Case 2
A 32-year-old caucasian woman was admitted at 31 weeks in her first pregnancy complaining of palpitations, breathlessness, and feeling faint and sweaty. On examination, her pulse was 180 beats per minute and an ECG showed a narrow complex tachycardia. Following intravenous adenosine, she reverted to sinus rhythm with a rate of 90 beats per minute. On auscultation there was a loud pan-systolic murmur heard all over the precordium and radiating to the neck. There were no signs of heart failure.
Dr Jane M. Rutherford, Research Fellow in Obstetric Medicine, Department of Therapeutics, Queen's Medical Centre, Nottingham NG7 2UH
56
Pregnancy with previous peripartum cardiomyopathy 57 At 10 h after admission she remained in sinus rhythm with a rate of 102. Her apex beat was displaced to the left and there were bilateral basal crepitations in the lungs. A chest X-ray showed cardiac enlargement, enlarged pulmonary vessels and a small left pleural effusion. Echocardiogram showed dilated left atrium and ventricle, with significant mitral regurgitation but good left ventricular function. She was commenced on digoxin and frusemide. Over the subsequent 4 weeks she remained stable. She was admitted in spontaneous labour at 35 weeks and was delivered of a healthy male infant by vacuum extraction. Postnatally she remained well and was discharged home on aspirin, frusemide and digoxin. Over the subsequent 6 weeks she was weaned off all treatment. By 8 weeks postpartum she had only a very quiet systolic murmur and echocardiography showed a dilated left ventricle with good ventricular contraction. Heart valves were normal with no evidence of mitral regurgitation. She attended for a further echocardiogram 6 months postpartum and this showed slightly dilated left ventricle with mild free-wall hypokinesia but overall good function. Fractional shortening was 35%. She became pregnant again 2 years later. She was seen at 8 weeks' gestation and at this time there were no cardiac murmurs detected and an echocardiogram was reported as normal. At 16 weeks she was noted to have a moderate pan-systolic murmur. At 20 weeks she was symptomaticallywell and the pan-systolic murmur was unchanged. The echocardiogram showed some deterioration with mitral regurgitation into a slightly dilated left atrium and a dilated left ventricle, but overall function was good. The fractional shortening was 27%. Over the subsequent weeks she developed some breathlessness and mild orthopnoea but no clinical evidence of heart failure. Echocardiography showed little change in her cardiac status with fractional shortening varying between 30 and 41%. She remained otherwise well with a persistent pan-systolic murmur. Labour was induced at 36 weeks and she had a spontaneous vertex delivery of a healthy female infant. Postnatally she showed no signs of heart failure but was commenced on enalapril. At 1 week postpartum an echocardiogram showed slight deterioration with a fractional shortening of 25%. By 3 weeks postpartum the murmur was barely audible. Echocardiography 6 weeks postpartum showed normal mitral and aortic valves, with a whiff of mitral regurgitation into a nondilated left atrium. The left ventricle was slightly dilated with reasonably good function. Fractional shortening was 42%. The enalapril was stopped and she was discharged from the clinic.
DISCUSSION Peripartum cardiomyopathy (PPCM) is characterized by: (1) development of cardiac failure in the last
month of pregnancy or within 5 months of delivery; (2) absence of determinable aetiology for the cardiac failure; and (3) absence of demonstrable heart disease prior to the last month of pregnancy? It has an incidence of between 1 in 1300 and 1 in 15 000 pregnancies.2 There is a racial difference with increased incidence in black races. 3It carries a maternal mortality rate of 25-50% 3and may cause significant morbidity in those who have persistent cardiac failure. There is little information about the prognosis in subsequent pregnancies for women who have had PPCM. Early evidence suggested that women who have recovered left ventricular function 6 months following PPCM have a much better prognosis than those who have persistent cardiomegaly? However, in the early series, echocardiography was not available and the diagnosis of persistent cardiac dysfunction was made on the basis of clinical signs and X-ray evidence. Now that echocardiography is widely available it is possible that PPCM is being diagnosed in different patients. It is not yet known whether women who have 'recurrent PPCM' during subsequent pregnancies do so because of reactivation of the original underlying disease process or because of deterioration in subclinical cardiac dysfunction following their previous episode. Reported here are two cases of women who presented in their first pregnancy with PPCM, whose echocardiographic features of cardiac function returned to near normal within 6 months of delivery, and who had no residual symptoms of heart failure. In both of these cases, in subsequent pregnancies, there was demonstrable echocardiographic evidence of deteriorating left ventricular function during pregnancy or in the immediate postpartum period. In case 1 this was completely asymptomatic and in case 2 it was associated with mild symptoms of orthopnoea and dyspnoea only. During the last 5 years we know of no other women at this hospital who became pregnant after previous PPCM. Witlin et al recently reported on six patients who had pregnancies following previous PPCM) Four of these had clinical relapse and one had deterioration in echocardiographic features. These findings differ from those of Sutton et al, who reported four women who had subsequent pregnancies following PPCM and in whom there was no deterioration in either clinical state or the findings on echocardiography.4 In another report of four patients with previous PPCM who had subsequent pregnancies, none of the patients showed evidence of deterioration in cardiac status, although it is not clear whether they were followed with echocardiography throughout pregnancy2 Our data support the findings of Witlin et al2 and suggests that there is a significant risk of either subclinical or clinical deterioration in cardiac function in subsequent pregnancies. There is, however, little evidence regarding which patients will have irreversible cardiac dysfunction and long-term morbidity. In case 2 the patient was treated with enalapril post partum in
58
Current Obstetrics & Gynaecology
her second pregnancy on an empirical basis. Since it is known that angiotensin-converting enzyme inhibitors have a beneficial effect in promoting recovery of left ventricular function following myocardial infarction, we felt that it was possible that this could also be the case in P P C M : A recent experimental study measured the contractile reserve following a dobutamine challenge in nonpregnant women who had regained left ventricular function after PPCM. 7 This showed that women with 'recovered' PPCM had reduced contractile reserve compared to matched controls. The authors concluded that since the effect of the dobutamine challenge was to mimic some of the cardiovascular adaptations to pregnancy, their findings might explain recurrence of symptoms in subsequent pregnancies in patients who have apparently recovered. It is interesting to note that our two patients both had deterioration of their left ventricular dysfunction in the postpartum period. If this deterioration was due to the effects of the added cardiovascular stress of pregnancy, then it would be logical to assume that it would occur in the early part of pregnancy, when the greatest changes are taking place, rather than postnatally.
From the evidence of these two cases it can be seen that pregnancy after previous PPCM carries a risk of deteriorating left ventricular function even in those women whose cardiac function has returned to near normal after the initial episode. REFERENCES
1. Demakis JG, Rahimtoola SH, Sutton GC et al. Natural course of peripartum cardiomyopathy. Circulation 1971; 44: 1053-1061 2. Witlin AG, Mabie WC, Sibai BM. Peripartum cardiomyopathy: an ominous diagnosis. Am J Obstet Gynecol 1997; 176:182-188 3. Homans DC. Peripartum cardiomyopathy. N Engl J Med 1985; 312:1432-1437 4. Sutton MStJ, Cole R Plappert M, Saltzman D, Goldhaber S. Effects of subsequent pregnancy on left ventricular function in peripartum cardiomyopathy. Am Heart J 1991; 121: 1776-1778 5. Desai D, Moodley J, Naidoo D. Peripartum cardiomyopathy: experiences at King Edward VIII Hospital, Durban, South Africa and a review of the literature. Tropical Doctor 1995; 25:118-123 6. Simoons ML. Myocardial infarction: ACE inhibitors for all? for ever? Lancet 1994; 344:279-281 7. Lampert MB, Weinert L, Hibbard J, Korcarz C, Lindheimer M, Lang RM. Contractile reserve in patients with peripartum cardiomyopathy and recovered left ventricular function. Am J Obstet Gynecol 1997; 176:189-195