Peripartum cardiomyopathy: A puzzle closer to solution

Table 1 Summary of current state of knowledge about peripartum cardiomyopathy

What do we know about PPCM?	What remains unknown about PPCM?
Awareness is important for making an earlier diagnosis with less dysfunction	Actual “triggers” that initiate the process
Hypertension in pregnancy increases risk for development of PPCM	Role of virus in pathogenesis
Most serious complications can be decreased or avoided	Why higher incidence and more severe disease in those with African heritage
Full recovery occurs more frequently than with any other cardiomyopathy	How important role cardiac autoantibodies play in pathogenesis
Autoimmunity (or immune system dysfunction) a part of pathogenesis	The extent and details of genetic factors
Inflammatory cardiomyopathy is common	Importance of the role of prolactin and prolactin inhibition treatment
Higher incidence and more severe disease in those of African heritage	Importance of the role of sFLT1 in pathogenesis
There can be a genetic predisposition	Why do some recovered have a relapse of heart failure with subsequent pregnancy
Effective evidence-based treatment guidelines available	Role of micronutrients and trace metals in pathogenesis
Most recovered do not have a relapse of heart failure in subsequent pregnancy
Occurs globally, but with geographic variations for incidence and unique characteristics

PPCM: Peripartum cardiomyopathy.

Table 2 Echocardiographic parameters at diagnosis as predictors of recovery (Left ventricular ejection fraction ≥ 50%) for peripartum cardiomyopathy n (%)

Study	Recovered	Non-recovered	P
Goland et al[10,11] (25% African-American)	115 (61.5)	72 (38.5)
Diagnosis mean LVEF	0.31	0.23	< 0.0001a
Diagnosis mean LVEDd (cm)	5.5	6.1	0.002a
Amos et al[12] (51% African-American)	22 (44.9)	27 (55.1)
Diagnosis mean LVEF	0.23	0.20	0.16
Mean LVEF (%) at 2 mo	43	24	< 0.001a
Diagnosis mean LVEDd (cm)	5.6	6.2	0.01a
Modi et al[13] (88.6% African-American)	14 (35)	26 (65)
Diagnosis mean LVEF	0.29	0.21	0.02a
Diagnosis mean LVEDd (cm)	5.9	6.2	0.16
Fett et al[14] (all African heritage)	32 (27.6)	84 (72.4)
Diagnosis mean LVEF	0.28	0.23	0.002a
Diagnosis mean LVEDd (cm)	5.6	5.9	0.03a
Safirstein et al[15] (3.6% African-American)	43 (78.2)	12 (21.8)
Diagnosis mean LVEF	0.29	0.24	0.13
Diagnosis mean LVEDd (cm)	5.4	5.9	0.21
Diagnostic LVEF > 0.35	25/25	0	< 0.001a
1Haghikia et al[16]	45 (47)	51 (53)
Diagnosis mean LVEF	0.28	0.17	< 0.0001a
McNamara et al[17] (30% African-American)	59 (65)	32 (35)
Diagnostic LVEF < 0.30	10/30 (33)	20/30 (67)	0.001a
Diagnostic LVEF ≥ 0.30	58/70 (82.9)2	21/70 (17.1)2	0.001a

¹For this group, recovery defined as LVEF 0.55, mean LVEF shown for improved vs non-improved;

²Pending last echo late data entry from 12 mo postpartum. LVEDd: Left ventricular end-diastolic diameter; LVEF: Left ventricular ejection fraction; Recovered: Last LVEF ≥ 0.50; Non-recovered: Last LVEF < 0.50;

^aP≤ 0.05 vs non-recovered.

Table 3 Role of viral infection in the etiology of peripartum cardiomyopathy: Pathogenesis or mere presence?

ID	PPCM patient	Virus	Type of test	Comments
1	Author case file, Norway	Parvovirus B19	IgM/IgG +	EMB = neg myocarditis
			EMB + PCR
2	Case report, Italy[65]	Coxsackievirus B	IgM + blood	EMB = lymphocytic myocarditis
			PCR + blood
3	Case report, Germany[66]	Parvovirus B19	EMB + PCR	EMB = borderline myocarditis
4	Case report, Germany[66]	Parvovirus B19	EMB + PCR	EMB = borderline myocarditis
5	Case report, Germany[66]	E-B Virus	EMB + PCR	EMB = borderline myocarditis
6	Case report, Germany[66]	Human Herpesvirus 6	EMB + PCR	EMB = borderline myocarditis
7	Case report, Germany[66]	Human Herpesvirus 6	EMB + PCR	EMB = borderline myocarditis
8	Case report, Germany[66]	Cytomegalovirus	EMB + PCR	EMB = borderline myocarditis
9	Case report, Germany[66]	Parvovirus B19	EMB + PCR	EMB = inflammatory cardiomyopathy
10	Author case file, United States	Parvovirus B19	IgM/IgG + blood	Exposure to PVB19 child during pregnancy
11	Author case file, United States	Parvovirus B19 cytomegalovirus	IgG + blood	Hydrops fetus, stillborn
12	Case report, Japan[67]	Influenza A/B	Paired sera antibody rise	EMB = neg. Treatment with IV immunoglobulin
13	Case report, Japan[67]	Influenza B	Paired sera antibody rise	EMB neg. Treatment with IV immunoglobulin
14	Author case file, United States	Parvovirus B19	IgG + blood	Exposure to PVB19 child during pregnancy
15	Author case file, United States	Cytomegalovirus	IgM + blood	LVEF 15%, IgG + blood E-B virus
16	Case report, Taiwan[68]	PCR neg for all 4 tested	EMB/PCR neg, but myocarditis	2 mo pp, RV/LV failure, patient died VF
17	Author case file, United States	H1N1 Influenza	Nasal swab, no Rx given	LVEF 40% at Dx, day 1 postpartum
18	Case report, United States[69]	Parvovirus B19	EMB + PCR	HF 27 wk, g3p2 EMB neg myocarditis
19	Case report, Belgium[70]	E-B virus		Postpartum facial palsy full recovery 6 mo

EMB: Endomyocardial biopsy; PCR: Polymerase chain reaction; LV: Left ventricular; LVEF: LV ejection fraction; PPCM: Peripartum cardiomyopathy; PVB19: Parvovirus B19; RV: Right ventricular; VF: Ventricular fibrillation; HF: Heart failure.