raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:563

Peripartum cardiomyopathy

Synonyms: Postpartum cardiomyopathy

Type
Disease
Group
Disorder
Inheritance
Unknown
Age of onset
Adult
Status
active

Peripartum cardiomyopathy (PPCM) is an idiopathic, potentially fatal form of dilated cardiomyopathy that develops during the final month of pregnancy or within five months after delivery.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPeripartum cardiomyopathyPostpartum cardiomyopathy
frMyocardiopathie gravidique primitiveCardiomyopathie du péri-partum · Cardiomyopathie gravidique · Syndrome de Meadows
deKardiomyopathie, peripartaleKardiomyopathie, postpartale · Meadows-Syndrom · PPCM · PPCMP · Peripartumkardiomyopathie
esMiocardiopatía peripartoCardiomiopatía periparto · Cardiomiopatía postparto · Síndrome de Meadow
itCardiomiopatia peripartoCardiomiopatia postparto · Sindrome di Meadow
nlPeripartumcardiomyopathiePostpartumcardiomyopathie
ptMiocardiopatia peri-partoMiocardiopatia pós-parto
plKardiomiopatia okołoporodowaKardiomiopatia poporodowa
csPeripartum kardiomyopatiePostpartum kardiomyopatie
ukПеринатальна кардіоміопатіяПісляпологова кардіоміопатія
zh围产期心肌病产后心肌病
trPeripartum kardiyomiyopatisiDoğum sonrası kardiyomiyopati

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10O90.3Especificvalidated
ICD-11JB44.3Especificvalidated
MONDO0018920Evalidated
UMLSC0877208Evalidated
GARD220Evalidated
MedDRA10049430Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (3):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

7 records
TypeClassValueAreaQualificationValidationSource
Prevalence at birth1-5 / 10 00030 / 100 000WorldwideValue and classNot yet validated10703781[PMID]
Prevalence at birth1-5 / 10 00036 / 100 000United StatesValue and classNot yet validated23090517[PMID]_10703781[PMID]_16765131[PMID]
Prevalence at birth6-9 / 10 00089 / 100 000SingaporeValue and classValidated23338912[PMID]
Prevalence at birth1-9 / 100 0005 / 100 000JapanValue and classValidated21617320[PMID]
Prevalence at birth>1 / 1000334 / 100 000HaitiValue and classValidated16342653[PMID]
Prevalence at birth>1 / 1000100 / 100 000South AfricaValue and classValidated16920474[PMID]
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

45 annotations · validated 2019-05-28
HPOTerm (as annotated by Orphanet)Diagnostic
Frequent (79-30%)
HP:0005135Abnormal T-wave
HP:0001635Congestive heart failure
HP:0001644Dilated cardiomyopathy
HP:0002094Dyspnea
HP:0030848Elevated jugular venous pressure
HP:0002875Exertional dyspnea
HP:0012378Fatigue
HP:0000822Hypertension
HP:0001712Left ventricular hypertrophy
HP:0025169Left ventricular systolic dysfunction
HP:0012764Orthopnea
HP:0001962Palpitations
HP:0010741Pedal edema
HP:0012664Reduced left ventricular ejection fraction
HP:0011703Sinus tachycardia
HP:0001649Tachycardia
Occasional (29-5%)
HP:0006705Abnormal atrioventricular valve morphology
HP:0005120Abnormal cardiac atrium morphology
HP:0100749Chest pain
HP:0030830Crackles
HP:0030148Heart murmur
HP:0030356Increased serum interferon-gamma level
HP:0031295Left atrial enlargement
HP:0001653Mitral regurgitation
HP:0012819Myocarditis
HP:0012763Paroxysmal dyspnea
HP:0012398Peripheral edema
HP:0100602Preeclampsia
HP:0002092Pulmonary arterial hypertension
HP:0005133Right ventricular dilatation
HP:0001708Right ventricular failure
HP:0001907Thromboembolism
HP:0100603Toxemia of pregnancy
HP:0004756Ventricular tachycardia
Very rare (<4-1%)
HP:0002027Abdominal pain
HP:0002926Abnormality of thyroid physiology
HP:0001903Anemia
HP:0002099Asthma
HP:0002960Autoimmunity
HP:0030149Cardiogenic shock
HP:0000819Diabetes mellitus
HP:0011713Left bundle branch block
HP:0001513Obesity
HP:0002878Respiratory failure
HP:0002401Stroke-like episode

Annotation source: 27045128[PMID]_28765406[PMID]_25636745[PMID]

7

Sources for this card