https://doi.org/10.1007/s00392-025-02625-4
1Medizinische Hochschule Hannover Kardiologie und Angiologie Hannover, Deutschland; 2Philipps-Universität Marburg Fachbereich Medizin - Die Dekanin Marburg, Deutschland
Background:
Peripartum cardiomyopathy (PPCM) is a heart disease that develops peripartum in previously heart healthy women.
In the past decades, clinical and experimental studies helped to improve the understanding of the pathophysiology and the clinical course of the disease, resulting in new therapeutic approaches and an improved outcome. However, data regarding long-term follow-up of PPCM patients are sparse. In this study, we analyze the 10-year follow-up of PPCM patients, using the German PPCM registry.
Methods and results:
Overall, 67 patients with confirmed diagnosis of PPCM met the inclusion criteria. Median follow-up time was 122 months. At diagnosis, left ventricular (LV) function was markedly reduced with a mean LVEF of 27±9%, and most patients presented with severe symptoms of heart failure (80% with NYHA III or IV).
Within 6 months, mean LVEF increased to 48±11%. Full cardiac recovery (LVEF >50%) at 6-month follow-up was observed in 46%, while partial recovery occurred in 40% and no recovery in 14% of cases. At 5-year follow-up the mean LVEF was 54±7% with full cardiac recovery present in 68%, partial recovery in 28% and no recovery in 3%.
At 10-year follow-up, the mean LVEF was 53±9%. Full cardiac recovery was observed in 72%, while partial recovery was present in 22% and no recovery in 6% of the cases.
Two patients needed a left ventricular assist device (LVAD) for mechanical cardiac support two weeks and two months after diagnosis. In one patient the LVAD was explanted eight months after implantation, but had to be re-implanted after 5 days due to a recurrent deterioration of LV-function. After eight years this patient received a heart transplant (HTX). The other patient with implanted LVAD died three years after implantation due to multiple strokes after LVAD thrombosis. Remarkably, this is the only patient that died in this cohort, resulting in an overall mortality rate of 1%.
Three patients showed delayed deterioration 176, 171 and 163 days after diagnosis of PPCM. In all three cases deterioration was associated with COVID-19 infection or vaccination. Two of these patients are currently stable under intensified heart failure medication and one patient is listed for HTX.
Two patients received an ICD for secondary prevention and 5 patients received an ICD for primary prevention due to a persistently severely reduced LV-function. Among them 4 patients received a CRT-D due to LBBB. At 10-year follow-up, full cardiac recovery could be observed in all 4 patients with implanted CRT-D.
At 10-year follow-up 74% of the patients still received any kind of heart failure medication and 17% of the patients were treated with “triple heart failure medication” consisting of beta blockers, angiotensin-converting enzyme inhibitors or angiotensin receptor blockers and mineralocorticoid receptor antagonists.
16 patients from our 10-year follow-up presented with a subsequent pregnancy (SSP) and 15 patients completed the pregnancy and gave live birth. No significant deterioration of LV-function could be observed, comparing LV-function before SSP and after delivery (mean LVEF: 54±7% vs 52±9%).
Conclusion:
In our collective, after 10 years, mortality was low and recovery rate was high and stable, underlining the rather good prognosis of this disease when treated in experienced teams. However, despite full cardiac recovery, most patients still used heart failure medication, questioning optimal tapering strategies.