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Cardiomyopathy in newborns and infants: a broad spectrum of aetiologies and poor prognosis


Badertscher, A; Bauersfeld, U; Arbenz, U; Baumgartner, M R; Schinzel, A; Balmer, C (2008). Cardiomyopathy in newborns and infants: a broad spectrum of aetiologies and poor prognosis. Acta Paediatrica, 97(11):1523-1528.

Abstract

AIM: 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. CONCLUSION: 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.

Abstract

AIM: 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. CONCLUSION: 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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Institute of Medical Genetics
04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:2008
Deposited On:02 Dec 2008 08:23
Last Modified:05 Apr 2016 12:37
Publisher:Wiley-Blackwell
ISSN:0803-5253
Publisher DOI:https://doi.org/10.1111/j.1651-2227.2008.00957.x
PubMed ID:18652581

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