Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016 Jun 30;5(7):e003450.
doi: 10.1161/JAHA.116.003450.

Natural History of Dilated Cardiomyopathy in Children

Ilaria Puggia  1 , Marco Merlo  2 , Giulia Barbati  3 , Teisha J Rowland  4 , Davide Stolfo  1 , Marta Gigli  1 , Federica Ramani  1 , Andrea Di Lenarda  5 , Luisa Mestroni  4 , Gianfranco Sinagra  1
Affiliations

Natural History of Dilated Cardiomyopathy in Children

Ilaria Puggia et al. J Am Heart Assoc. .

Abstract

Background: The long-term progression of idiopathic dilated cardiomyopathy (DCM) in pediatric patients compared with adult patients has not been previously characterized. In this study, we compared outcome and long-term progression of pediatric and adult DCM populations.

Methods and results: Between 1988 and 2014, 927 DCM patients were consecutively enrolled. The pediatric population (aged <18 years at enrollment) included 47 participants (5.1%). At presentation, the pediatric population compared with adult patients had a significantly increased occurrence of familial forms (P=0.03), shorter duration of heart failure (P=0.04), lower systolic blood pressure (P=0.01), decreased presence of left bundle-branch block (P=0.001), and increased left ventricular ejection fraction (P=0.03). Despite these baseline differences, long-term longitudinal trends of New York Heart Association class III to IV, left ventricular dimensions, left ventricular ejection fraction, and restrictive filling pattern were similar between the 2 populations. Regarding survival analysis, because of the size difference between the 2 populations, we compared the pediatric population with a sample of adult patients randomly matched using the above-mentioned baseline differences in a 3:1 ratio (141 adult versus 47 pediatric patients). During a median follow-up of 110 months, survival free from heart transplantation was significantly lower among pediatric patients compared with adults (P<0.001). Furthermore, pediatric age (ie, <18 years) was found to be associated with an increasing risk of both death from pump failure and life-threatening arrhythmias.

Conclusions: Despite the pediatric DCM population having higher baseline left ventricular ejection fraction and similar long-term echocardiographic progression compared with the adult DCM population, the pediatric DCM patients had worse cardiovascular prognosis.

Keywords: cardiomyopathy; death; dilated; echocardiography; heart failure; pediatrics; sudden.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Long‐term longitudinal trends of clinical and echocardiographic parameters (NYHA classes III–IV class, LVEDD_I, LVEDV‐I, LVEF, RFP) in pediatric (solid line) and adult (dotted line) populations. LVEDD_I indicates indexed left ventricular end‐diastolic diameter; LVEDV_I, indexed left ventricular end‐diastolic volume; LVEF, left ventricular ejection fraction; MR, mitral regurgitation; NYHA, New York Heart association; RFP, restrictive filling pattern.
Figure 2
Figure 2
Rates of long‐term survival free from D/HTx (A), DHF/HTx (B), and from SD/MVA (C) in 47 pediatric (solid line) vs 141 adult patients (dotted line) matched in a 1:3 ratio after adjustment for baseline differences between the 2 subgroups. D/HTx indicates death or heart transplantation; DHF/HTx, heart‐failure death or heart transplantation; SD/MVA, sudden death or major ventricular arrhythmias.
Figure 3
Figure 3
Effect of age on outcome measurements. Pediatric age (ie, <18 years) was associated with increasing risk of all major events: D/HTx (A), DHF/HTx (B), SD/MVA (C). D/HTx indicates death or heart transplantation; DHF/HTx, heart‐failure death or heart transplantation; SD/MVA, sudden death or major ventricular arrhythmias.
See this image and copyright information in PMC

References

    1. Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, Moss AJ, Seidman CE, Young JB. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Function. Circulation. 2006;113:1807–1816. - PubMed
    1. Merlo M, Pivetta A, Pinamonti B, Stolfo D, Zecchin M, Barbati G, Di Lenarda A, Sinagra G. Long‐term prognostic impact of therapeutic strategies in patients with idiopathic dilated cardiomyopathy: changing mortality over the last 30 years. Eur J Heart Fail. 2014;16:317–324. - PubMed
    1. Jefferies JL, Towbin JA. Dilated cardiomyopathy. Lancet. 2010;375:752–762. - PubMed
    1. Towbin JA, Lowe AM, Colan SD, Sleeper LA, Orav EJ, Clunie S, Messere J, Cox GF, Lurie PR, Hsu D, Canter C, Wilkinson JD, Lipshultz SE. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA. 2006;296:1867–1876. - PubMed
    1. Lipshultz SE, Sleeper LA, Towbin JA, Lowe AM, Orav EJ, Cox GF, Lurie PR, McCoy KL, McDonald MA, Messere JE, Colan SD. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med. 2003;348:1647–1655. - PubMed

MeSH terms

Supplementary concepts

LinkOut - more resources