Asian Cardiovasc Thorac Ann 2000;8:155-157
© 2000 Asia Publishing EXchange Pte Ltd
Unusual Left Ventricular Function and Survival in Peripartum Cardiomyopathy
Mandeep Bhargava, DM,
Mandeep Singh, DM,
Pujar Venkateshacharya Suresh, DM,
Rajneesh Juneja, DM,
Harinder Kumar Bali, DM,
Jagmohan Varma, DM
Department of Cardiology
Postgraduate Institute of Medical Education and Research
Chandigarh, India
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For reprint information contact: Mandeep Bhargava, DM Tel: 91 172 74 7585 Fax: 91 172 74 4401 email: mandeepbhargava{at}hotmail.com Department of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
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Abstract
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A 34-year-old woman presented with congestive heart failure 3 months after her third pregnancy. She was stabilized in New York Heart Association functional class II with digoxin and diuretics for 21 years. She was readmitted with worsening symptoms and found to have a regional contraction abnormality. With the addition of angiotensin-converting enzyme inhibitors to her medication, she has been maintained in functional class III for a further 4 years.
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Introduction
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Peripartum cardiomyopathy is a disease of unknown etiology characterized by dilatation and severe global hypokinesia of the ventricles, occurring late in pregnancy or in the early postpartum period. In a large proportion of these patients, the size of the heart returns to normal within 6 to 12 months. In patients who have persistent cardiomegaly beyond 6 months, survival is poor. This is mainly due to progressive heart failure, arrhythmias, or a thromboembolic phenomenon. A 25-year follow-up is described in a patient of the latter subgroup who also had evidence of a regional contraction abnormality, both rare features being evidence of heterogeneity in the clinical pattern of this disease.
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Case Report
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A 34-year-old woman first presented at our institute with congestive heart failure 3 months after her third pregnancy. She had no history of a preceding viral or rheumatic illness. She was stabilized on digoxin and diuretics and discharged. She continued on medical treatment with irregular follow-up. She was reasonably stable for the next 20 years and remained in New York Heart Association functional class II.
Twenty-one years later, at the age of 55, she presented with worsening of her symptoms over the preceding month and was in New York Heart Association class IV on admission. Physical examination showed elevated jugular venous pressure, cardiomegaly, and a grade 2/3 left parasternal heave. Her blood pressure was 130/80 mm Hg. The pulmonic component of the second heart sound was loud, there was a left ventricular third heart sound and evidence of severe mitral regurgitation. A tender hepatomegaly was noted. The electrocardiogram revealed sinus tachycardia, first-degree atrioventricular block, and left atrial enlargement. There was no evidence of an old myocardial infarction. Chest radiography showed cardiomegaly, left atrial enlargement, and a grossly dilated main pulmonary artery (
Figures
1A
and
1B
). An echocardiogram showed dilation of the left atrium (5.7 cm) and left ventricle (M-mode dimensions in parasternal long-axis view were 7.1 cm in diastole and 5.6 cm in systole), with severe mitral regurgitation, trivial tricuspid regurgitation, and severe left ventricular dysfunction (ejection fraction, 42%).
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Figure 1.
(
A
) Chest radiograph of the patient at 34 years old, showing a cardiothoracic ratio of 0.64. (
B
) Chest radiograph of the patient at 55 years old, showing a cardiothoracic ratio of 0.75 and prominent main pulmonary artery.
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After initial stabilization on medical treatment with digoxin, angiotensin-converting enzyme inhibitors, and diuretics, the patient underwent cardiac catheterization. She had moderate pulmonary arterial hypertension (mean pressure, 33 mm Hg), the mean pulmonary artery wedge pressure was elevated to 20 mm Hg, and left ventricular end-diastolic pressure was 18 mm Hg. Her coronary arteriogram indicated normal epicardial coronary arteries with right dominant circulation. Her left ventriculogram (
Figures
2A
and
2B
) showed a dilated left ventricle with akinesia of the posterior basal and inferior segments and hypokinesia of the other regions. The indexed end-diastolic and end-systolic volumes were 221 mL and 132 mL, respectively. The left ventricular ejection fraction was 40%. An endomyocardial biopsy showed hypertrophy of the cells with no features of myocarditis. The patient has been on irregular follow-up for a further 4 years with progressive heart failure (New York Heart Association functional class III) and features of biventricular congestion. Treatment with digoxin, angiotensin-converting enzyme inhibitors, and diuretics has been continued.
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Figure 2.
(
A
) Left ventricular angiogram in diastole, showing a grossly dilated ventricle. (
B
) Systolic frame showing akinesia of the inferior and posterior basal segments and hypokinesia of the other regions.
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Discussion
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Peripartum cardiomyopathy is a rare congestive cardiomyopathy. It occurs in 1 out of 3000 to 15,000 pregnancies, with a higher incidence in Africa. In the past, diagnosis was made solely on clinical grounds. The diagnostic criteria established by Demakis and colleagues
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included development of cardiac failure in the last month of pregnancy or within 5 months after delivery, absence of a determinable cause of cardiac failure, and absence of demonstrable heart failure before the last month of pregnancy. Lampert and Lang
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suggested that echocardiographically demonstrable impairment in left ventricular systolic function be added as the fourth criterion for diagnosis.
The etiology is currently unknown. Recent studies do not support the role of nutritional deficiencies, as thought earlier.
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A relationship has been suggested with age over 30 years, multiple pregnancies, African descent, cocaine abuse, toxemia of pregnancy, postpartum hypertension, and tocolytic therapy. Currently, most evidence suggests that peripartum cardiomyopathy is actually a type of myocarditis arising from an infectious, autoimmune, or idiopathic process.
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Midei and colleagues
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showed that myocarditis was present in 78% of such patients and the presence of myocarditis indicated a likelihood of response to steroids, thus, it was a marker of better prognosis. The presence of focal infiltrates with false negative results leads to difficulty in diagnosis by endomyocardial biopsy. Some authors have questioned whether peripartum cardiomyopathy is a distinct clinical entity or merely an initial presentation of latent dilated cardiomyopathy. The hyperdynamic state of pregnancy is associated with peak increases in plasma volume and cardiac output at 28 to 32 weeks when a latent cardiac illness would be expected to present. However, the clustering of cases in the last month of pregnancy and early postpartum phase, the relationship with myocarditis, the more favorable prognosis, and higher incidence of return of heart size to normal suggest that peripartum cardiomyopathy may be a distinct clinical entity. Patients in whom cardiomegaly persists beyond 6 to 12 months after diagnosis have a course quite similar to that of dilated cardiomyopathy.
Echocardiography shows dilatation of the left ventricle and left atrium and sometimes of the right heart chambers also. There is global hypokinesia of the ventricle with decreased fractional shortening and ejection fraction. Left ventriculography demonstrates global reduction of systolic function. Regional contraction abnormality, as shown in our patient, has been reported only twice previously in peripartum cardiomyopathy.
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,
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Cepin and colleagues
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described a patient with peripartum cardiomyopathy who presented in pulmonary edema. At catheterization, she had diastolic dysfunction and left ventriculography showed systolic function at the lower limit of normal. An impressive feature was a clearly demonstrated area of segmental apical hypokinesis. Segmental wall motion abnormalities have been well described in dilated cardiomyopathy and usually involve the apical and anterior segments.
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Inferior and posterior basal segment involvement as seen in this patient, is an uncommon phenomenon.
The reported mortality in peripartum cardiomyopathy varies from 25% to 50%.
1
–
3
In approximately 50% of patients, heart size returns to normal within the first 6 months.
1
Demakis and colleagues
1
noted 14% mortality during a mean follow-up of 10.7 years. However, these patients may continue to have reduced contractile reserve. In the other subgroup of patients, cardiomegaly and congestive symptoms may persist beyond 6 months. Such patients could be identified earlier on echocardiography as they would fail to show any trend towards improvement even 4 to 6 weeks after initiation of pharmacologic therapy. In this subgroup, 85% died during a mean follow-up of 5.4 years.
1
The average survival was 4.7 years. Walsh and colleagues
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reported 75% mortality in this subgroup of patients at a mean follow-up of 29 months (range, 4 to 58 months).
Our patient displayed two unique features. She had significant regional wall motion abnormality, a phenomenon not unknown in dilated cardiomyopathy but only twice reported in peripartum cardiomyopathy. She also exemplified the markedly variable rate of progression of this disease. She had persistent cardiomegaly beyond 6 months of onset, which is usually associated with the subgroup of patients with poor prognosis. Such patients are usually older, multiparous, predominantly blacks, have onset of symptoms more than 2 weeks postpartum, and have larger cardiothoracic ratios and left ventricular end-diastolic diameters. This patient's 25-year survival is possibly the longest in this subgroup of patients with peripartum cardiomyopathy.
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References
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Demakis JG, Rahimtoola SH, Sutton GC, Meadows R, Szanto PB, Tobin JR, et al. Natural course of peripartum cardiomyopathy. Circulation
1971; 44
:1053
–61.[Abstract/Free Full Text]
-
Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J
1995; 130
:860
–70.[Medline]
-
Brown CS, Berlolet BD. Peripartum cardiomyopathy: a comprehensive review. Am J Obstet Gynecol
1998; 178
:409
–14.[Medline]
-
Midei MG, De Ment SH, Feldman AM, Hutchins GM, Baughman KL. Peripartum myocarditis and cardiomyopathy. Circulation
1990; 81
:922
–8.[Abstract/Free Full Text]
-
Cepin D, James F, Carabello BA. Left ventricular function in peripartum cardiomyopathy. Chest
1983; 83
:701
–4.[Abstract/Free Full Text]
-
Shimamoto H, Okamoto M, Sueda T, Sakura E, Yokote Y, Tsuchioka Y, et al. Transient regional wall motion abnormality and increased wall thickness of the left ventricle in acute myopericarditis occurring in the puerperium. Hiroshima J Med Sci
1986; 35
:285
–7.[Medline]
-
Yamaguchi S, Tsuiki K, Haysaka M, Yasui S. Segmental wall motion abnormalities in dilated cardiomyopathy: hemodynamic characteristics and comparison with thallium-201 myocardial scintigraphy. Am Heart J
1987; 113
:1123
–8.[Medline]
-
Walsh JJ, Burch GE, Black WC, Ferrans VJ, Hibbs RG. Idiopathic myocardiopathy of the puerperium (post-partal heart disease). Circulation
1965; 32
:19
–31.[Abstract/Free Full Text]
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Abstract
Introduction
