Cardiomyopathy in newborns and infants: a broad spectrum of aetiologies and poor prognosis.
OBJECTIVE
This study set out to describe the initial clinical findings, morbidity, mortality and aetiology of infant cardiomyopathy focusing on potential risk factors for an adverse outcome.


METHODS
We retrospectively analysed clinical and laboratory findings of all patients diagnosed at our institution from 1995 to 2004 with cardiomyopathy within their first year of life.


RESULTS
Of the 35 patients, cardiomyopathy was classified as dilated in 18, hypertrophic in 14 and unclassified in 3. The aetiologies were genetic syndromes (8), metabolic diseases (5), familial isolated cardiomyopathy (3) and myopathy (1). During a median follow-up of 1.5 years (range 0-9 years), 13 patients died from progressive heart failure and two underwent heart transplants. Estimated survival and freedom from transplant was 69, 66, 58 and 50% after 0.5, 1, 2 and 6 years, respectively. Patients with severe heart failure symptoms within the first month of life had significantly worse outcomes than patients without heart failure symptoms.


CONCLUSIONS
High morbidity and poor prognosis result through progressive heart failure. Aetiology and clinical course are especially heterogeneous in infants. The most commonly identified aetiologies are genetic syndromes and metabolic diseases. A multidisciplinary approach is recommended for defining the aetiology and developing individual treatment strategies.
Badertscher
Andrea
A
Division of Cardiology, University Children's Hospital, Zurich, Switzerland.
Bauersfeld
Urs
U
Arbenz
Urs
U
Baumgartner
Matthias R
MR
Schinzel
Albert
A
Balmer
Christian
C
D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013557:Switzerland / Q000453:epidemiology