Initial Right Ventricular Dysfunction Severity Identifies Severe Peripartum Cardiomyopathy Phenotype With Worse Early and Overall Outcomes: A 24‐Year Cohort Study

Abstract

BackgroundOutcomes in peripartum cardiomyopathy (PPCM) vary. We sought to determine whether severity of left or right ventricular dysfunction (RVD) at PPCM diagnosis differentially associates with adverse outcomes.Methods and ResultsWe conducted a single‐center retrospective cohort study of 53 patients with PPCM. The primary outcome was a composite of left ventricular assist device implantation, cardiac transplantation, or death. We used Kaplan‐Meier curves to examine event‐free survival and Cox proportional hazards models to examine associations of left ventricular (LV) ejection fraction <30%, LV end‐diastolic diameter ≥60 mm, and moderate‐to‐severe RVD at PPCM diagnosis with the primary outcome. Median (interquartile range) follow‐up time was 3.6 (1.4–7.3) years. Seventeen patients (32%) experienced the primary outcome, of whom 11 had moderate‐to‐severe RVD at time of PPCM diagnosis. Overall event‐free survival differed by initial RVD severity and LV ejection fraction <30%, but not by LV end‐diastolic diameter ≥60 mm. In univariable analyses, LV ejection fraction <30% and moderate‐to‐severe RVD were associated with the outcome (hazard ratios [95% confidence intervals] of 4.85 [1.11–21.3] and 4.26 [1.47–11.6], respectively). In a multivariable model with LV ejection fraction <30%, LV end‐diastolic diameter ≥60 mm, and moderate‐to‐severe RVD, only moderate‐to‐severe RVD was independently associated with the outcome (hazard ratio [95% confidence interval], 3.21 [1.13–9.10]). Although most outcomes occurred within the first year, nearly a third occurred years after PPCM diagnosis.ConclusionsInitial moderate‐to‐severe RVD is associated with a more advanced cardiomyopathy phenotype and increased risk of adverse outcomes in PPCM, within and beyond the first year of diagnosis. By identifying a worse PPCM phenotype, initial moderate‐to‐severe RVD may prompt earlier consideration of advanced heart replacement therapies.

The Role of Multimodality Cardiovascular Imaging in Peripartum Cardiomyopathy

“…The results of the Investigations of Pregnancy-Associated Cardiomyopathy (IPAC) study, a prospective multicenter study on 100 PPCM patients, highlighted that baseline LVEF <30% and LV dilatation with a left ventricular end-diastolic diameter (LVEDD) >60 mm were strongly associated with lower rates of both 12-month event-free survival and LV functional recovery (39). Similar findings have been reproduced in other studies, although the effect of racial and ethnic differences requires future research (40)(41)(42)(43).…”

“…In the large IPAC cohort (52), the presence of RV systolic dysfunction was associated with persistent LV systolic impairment and poor outcome, and among different RV functional parameters, fractional area change (FAC) was the sole independent predictor of clinical outcome. The prognostic significance of baseline RV dysfunction was confirmed in a retrospective cohort study analysis of 53 patients (43) where moderate-to-severe RV systolic dysfunction was associated with 12-month higher risk of left ventricular assist device (LVAD) implantation, heart transplantation, or death. This evidence would suggest routine RV functional evaluation with FAC calculation in order to classify PPCM patients at higher risk of hemodynamic instability that may benefit from early advanced HF therapy.…”

Pathophysiology, diagnosis and management of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy

“…Early cardiac transplantation should be reserved for patients with refractory severe heart failure where MCS is not possible or not desirable for individual reasons, mainly for cases with biventricular failure or severe initial right ventricular dysfunction . Patients with PPCM appear to have higher rates of graft failure and death after heart transplantation, which may be partly explained by higher allosensitization, higher pre‐transplant acuity, and increased rejection .…”

Association Between Right Ventricular Dysfunction and Adverse Outcomes in Peripartum Cardiomyopathy: Insights From the BRO-HF Quebec Cohort Study

“…There was a significant association between measured FAC and reported RV function (Figure 1), confirming that the estimated grades of RV dysfunction had some accuracy. Median measured FAC was 43% (34-47) in women with reported normal function, 32% (26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36) in women with reported mild dysfunction and 30% (21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36) in those with reported moderate dysfunction (p=0.04). Similar findings were observed with measured TAPSE but with less statistical significance (p=0.05).…”