Peripartum cardiomyopathy: a rare cause of acute heart failure
- 1 Department of Internal Medicine, Kantonsspital Graubunden, Chur, Switzerland
- 2 Department of Internal Medicine, Cardiology, Kantonsspital Graubunden, Chur, Switzerland
- Correspondence to Professor Thomas Fehr; Thomas.fehr@ksgr.ch
Abstract
A woman in her early 30s presented herself with acute dyspnoea and elevated D-dimers 5 weeks after delivery of her second child. Echocardiographic findings showed signs of acute left ventricular failure, and an MRI confirmed a non-ischaemic dilated left heart failure compatible with peripartum cardiomyopathy. The antihormonal therapy with bromocriptine during 6 weeks and an intensive heart failure therapy led to an amelioration of the heart function within 3 years, but full recovery was not yet observed.
Background
Peripartum cardiomyopathy (PPCM) is a non-ischaemic dilated heart disease presenting with left ventricular systolic dysfunction that occurs within 1 month before and 5 months after delivery.1 There is a wide range of incidence varying between 1 and 100 births in Nigeria, 1 and 5700–10 000 in Europe and 1 and 20 000 births in Japan. This may be related to ethnicity, but likely also due to underreporting in certain areas, as Mielniczuk et al suspected when observing a duplication of PPCM cases within 12 years in the USA.2–4 Published data are scarce particularly in Europe with only two studies from Denmark and Sweden reporting the incidence of PPCM. This suggests a lack of awareness and may affect adequate management and optimal outcome, leaving aside the high mortality of up to 50% if the condition is diagnosed.5
Case presentation
A woman in her early 30s was referred to the emergency department by her general practitioner with suspicion of pulmonary embolism, because she presented herself with acute dyspnoea and elevated D-dimers 5 weeks after giving birth to her second child. Family history revealed a sister with pulmonary embolism around the age of 20, but according to the patient's information, further evaluation did not show evidence for a hereditary thrombophilia.
On arrival at the emergency department the vital parameters showed an afebrile, slightly hypertensive woman with tachycardia and tachypnoea (pulse 136/min, respiratory rate 40 /min, blood pressure 149/90 mm Hg). The clinical examination showed discrete bibasal rales.
Investigations
Further investigations showed increased D-dimers (0.83 mg/L), only slightly elevated inflammation serum markers with a C reactive protein of 7.5 mg/L, a slight leucocytosis and cardiac volume overload with NT-pro-BNP at >4400 pg/mL. High sensitive troponin T was slightly elevated with 18 ng/L without further increase, and the creatin kinase value was normal. The ECG showed no pathological alterations apart from sinus tachycardia. With suspicion of pulmonary embolism a CT scan was performed. No pulmonary embolism was detected, but contrary to expectation signs of severe left heart failure such as prominent vascular markings and ground glass opacities in the lung, an enlarged left ventricle with a diameter of 6.8 cm and a convex shift of the septum interventriculare towards the right ventricle, and washback of contrast agent into the vena cava inferior were observed. The transthoracic echocardiogram showed a dilated left ventricle with diffuse hypokinesia, reduced systolic ejection fraction of 25% and a severe functional mitral valve regurgitation. Furthermore, an increased pulmonary pressure, a transtricuspid pressure gradient of 40 mm Hg, and a dilated and functionally impaired right ventricle was evident.
To further evaluate the still unclear aetiology of this unusual heart failure in this young woman, a cardiac MR tomography was performed (figure 1A–D) indicating a non-ischaemic dilated cardiomyopathy compatible with PPCM. To estimate the risk of arrhythmia a 24-hour-ECG was recorded showing a moderate number of ventricular extrasystoles, no blockages or pauses, but three non-continuous ventricular tachycardia runs.
(A–D) Findings in MR tomography; dilatation of all four chambers particularly of left ventricle. Severe restricted right and left ventricle function (left ventricle ejection fraction 33%). Significant functional mitral regurgitation. Unrestricted perfusion at rest (disclaim of stress perfusion testing), no late gadolinium enhancement. Legend yellow letters: A, anterior; P, posterior; L, left; R, right; H, head; F, feet.
Treatment
An intensive heart failure therapy with valsartan/sacubitril, metoprolol, spironolactone and torasemide was established. Furthermore an ablactating therapy with 5 mg bromocriptine for 6 weeks was introduced.
To prevent the potential risk of sudden cardiac death due to malignant arrhythmias, a ‘life vest’ west was customised and subcutaneous anticoagulation with a low molecular heparin was installed. After an in-hospital stay of 18 days the patient was discharged.
Outcome and follow-up
There was an intense medical follow-up from initial presentation up to now. Through its course the clinical symptoms recorded with NYHA (New York Heart Association) functional classification ameliorated from initially grade IV to I within 1 year. Correspondingly the levels of NT-pro-BNP decreased over time to normal values. Repeated transthoracic echocardiographs revealed a gradual improvement of the left ventricular ejection fraction, a decreasing end-diastolic volume, and also a decrease of the severity of mitral valve regurgitation (table 1). Furthermore, the established heart failure therapy could be gradually reduced without clinical relapse (figure 2). However, full recovery from heart failure has not occurred with respect to the echocardiographic findings, thus further follow-up is required.
Development of echocardiographic markers, the time specification refers to the initial presentation
| Date | LVEDVI mL/m2 | LVEDD mm | LVEF% | LAVI mL/m2 | RVEF | Mitral regurgitation | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| LAVI, Left Atrial Volume Index; LVEDD, left ventricular end-diastolic diameter; LVEDVI, Left Ventricular End-Diastolic Volume Index; LVEF, left ventricular ejection fraction; RVEF, right ventricular ejection fraction. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Initial presentation | 92 | 69 | 25 | 44 | Impaired | Severe | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 1 month | 102 | 67 | 30 | 50 | Impaired | Severe | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 2 months | 102 | 70 | 30 | 51 | Impaired | Severe | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 5 months | 60 | 65 | 40 | 22 | Impaired | Moderate | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 9 months | 67 | 63 | 50 | 34 | Preserved | Moderate | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 13 months | 73 | 62 | 50 | 21 | Preserved | Moderate | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 18 months | 80 | 62 | 50 | 33 | Preserved | Mild-moderate | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 2 years | 70 | 60 | 50 | 28 | Preserved | Mild-moderate | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 3 years | 79 | 62 | 55 | 35 | Preserved | Mild-moderate | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Discussion
PPCM is a rare and especially in Europe not broadly reported cause of pregnancy-related acute heart failure, but it represents one of the three main aetiologies of pregnancy-associated heart failure as are pre-eclampsia and amniotic fluid embolism (table 2). The incidence of PPCM broadly differs between countries implying racial differences, but also incomplete registration/reporting.2 3 6 Because of the lack of clinical studies, the specific treatment strategy depends on expert opinions and preclinical study results and is therefore challenging for clinicians.7 8
Causes of pregnancy-related acute heart failure
| Aetiology of heart failure | Incidence/births or pregnancies | Pathophysiology | Echocardiography | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| PPCM, peripartum cardiomyopathy. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| PPCM1–3 14 | 1: 300–20 000 births | Endothelial cell apoptosis, disruption of capillary structures, myocyte dysfunction and apoptosis, oxidative stress | Dilated left ventricle with diffuse hypokinesia and systolic dysfunction | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Amniotic fluid embolism15 | 1: 8000–800 000 births |
Trespassing of amniotic fluid into maternal circulation |
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| Spontaneous coronary artery dissection16 | 1: 64 000 pregnancies |
Oestrogen/progesterone induced structural changes cause weakness of tunica media |
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| Pre-eclampsia and eclampsia,14,17 | 1: 22 and 1:71 pregnancies |
Disruption of maternal endothelial function, systemic inflammatory response, increased systemic vascular resistance, increased filling pressure left atrium, diastolic dysfunction | Diastolic dysfunction of left ventricle | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Ethics statements
Patient consent for publication
Footnotes
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Contributors TF: idea for the article, attending physician during inpatient care, theoretical supervision, text corrections. SS: specialist assessment during inpatient care, clinical follow-up and echocardiographic recording, text corrections. TCEV: attending physician during inpatient care, theoretical research, article writing, patient correspondance.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
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