Phenotypic differences between peripartum cardiomyopathy and idiopathic or familial cardiomyopathy in young women
Authors: Viljoen C A , Cirota J , Hoevelmann J , Hahnle L , Makotoko M , Thomas K , Thomas M , Sliwa K , Ntusi N , Kraus S 
Published: November 2023

Abstract

Introduction
Peripartum cardiomyopathy (PPCM) is a form of heart failure (HF) characterised by left ventricular (LV) dysfunction, which develops in previously healthy women towards the end of pregnancy or in the months following delivery. PPCM should be distinguished from underlying familial or idiopathic dilated cardiomyopathy (DCM), as management and outcomes of these conditions differ.

Purpose
We sought to investigate the phenotypic differences in women with PPCM as compared to those with DCM, the aetiology of which was either familial or idiopathic.

Methods
Sub-analysis was conducted on data collected by the African Cardiomyopathy and Myocarditis Registry Program (IMHOTEP), including 226 women from 3 sites in South Africa. Patients with a diagnosis of PPCM were compared to DCM (idiopathic or familial cardiomyopathy), with regards to their clinical presentation, echocardiography, and electrocardiography.

Results
Women with PPCM (n=132) were significantly younger than those with DCM (n=94) (29 [23-34] vs 38 [28-45] years). Both cohorts consisted mostly of patients of Black African descent (PPCM 66.7%, DCM 60.6%). A family history of HF was more commonly reported with DCM (36.6% vs 15%, p&lt;0.001). Comorbidities, including hypertension, were uncommon in both groups. Two thirds of patients (66.4%) with PPCM had NYHA functional class III/IV at time of presentation, as compared to half of those with DCM (52.4%, p=0.14). Median systolic BP (113mmHg), diastolic BP (72mmHg) and pulse rate (91 bpm) did not differ between groups. On the ECG, there were significant differences in the intervals between patients with PPCM and DCM (PR interval of 152ms vs 168ms, p&lt;0.001; QRS duration of 87ms vs 106ms, p&lt;0.001; QTc by Bazett 429ms vs 451ms, p&lt;0.001). Atrial fibrillation/flutter (5.5% vs 0.8%, p=0.083), left axis deviation (20.2% vs 2.6%, p&lt;0.001) and left bundle branch block (22.6% vs 3.9%, p&lt;0.001) occurred more frequently DCM compared to PPCM. Patients with PPCM more often manifested T wave inversion in the precordial leads (62.1% vs 46.8%, p=0.022). While there was no difference in LV systolic function (LVEF 28% [22-37]) between the groups, women with DCM had larger ventricles (LVEDD 63mm [57-69] vs 61mm [57-65], p=0.023), and atria (left atrial area 26.8 cm2 [21.9-30.2] vs 20.9 cm2 [18.2-26.1], p=0.002). However, LV thrombus was more frequently reported in PPCM compared to DCM (9.1% vs 2.1%, 0.032).

Conclusion
In this study, we demonstrate that compared to women with idiopathic and familial DCM, patients with PPCM were younger and had a number of phenotypic differences. The 12-lead ECG may be useful in the differentiation of both conditions. Accurate phenotyping of patients presenting with cardiomyopathy in the peripartum period may help to determine which patients require further investigations for DCM and influence the immediate and long-term care of these women.Figure 1