Idiopathic Restrictive Cardiomyopathy in Children and Young Adults

Heather N. Anderson, Frank Cetta, David J. Driscoll, Timothy Mark Olson, Michael John Ackerman, Jonathan N. Johnson

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Idiopathic restrictive cardiomyopathy (IRC) is a rare condition characterized by reduced ventricular compliance. Children with IRC have poor outcomes with most patients proceeding to cardiac transplantation. We sought to analyze our institutional experience and assess contemporary outcomes for children with IRC. We reviewed the medical record for patients (<21 years old) evaluated for a primary diagnosis of IRC between 1975 and 2013 at our institution. Demographic, clinical, echocardiographic, and catheterization data were abstracted. The patients were divided into 2 groups comprising a historical cohort (HC) (diagnosis: 1975 to 1993, n = 8) and a contemporary cohort (CC) (diagnosis: 1994 to 2013, n = 12). Twenty children were identified with IRC (mean age at presentation 9.7 ± 6.5 years, 55% female). Mean length of follow-up was 6.5 ± 8.4 years (range 0.1 to 35.6 years). In the CC, 7 of 12 patients (58%) progressed to cardiac transplantation (mean age of 9 ± 4 years at transplant, mean interval from diagnosis of IRC: 1.5 ± 0.9 years). Overall survival was improved significantly in the CC compared with the HC (80% vs. 38%, p = 0.02), but transplantation free survival was no different between the CC and HC over 5 years (38% vs 38%, p = 0.65). In the CC, elevation of mitral valve Doppler E/e' ratio on echocardiography was associated with increased mortality (p = 0.01). In conclusion, IRC continues has a poor prognosis. Early referral for transplantation was associated with improved overall survival in the modern era. Patients with markedly elevated E/e' ratio may have increased risk of death.

Original languageEnglish (US)
JournalAmerican Journal of Cardiology
DOIs
StateAccepted/In press - Jan 1 2018

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Restrictive Cardiomyopathy
Young Adult
Heart Transplantation
Survival
Transplantation
Mitral Valve
Catheterization
Compliance
Medical Records
Echocardiography
Referral and Consultation
Demography
Transplants
Mortality

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Idiopathic Restrictive Cardiomyopathy in Children and Young Adults. / Anderson, Heather N.; Cetta, Frank; Driscoll, David J.; Olson, Timothy Mark; Ackerman, Michael John; Johnson, Jonathan N.

In: American Journal of Cardiology, 01.01.2018.

Research output: Contribution to journalArticle

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abstract = "Idiopathic restrictive cardiomyopathy (IRC) is a rare condition characterized by reduced ventricular compliance. Children with IRC have poor outcomes with most patients proceeding to cardiac transplantation. We sought to analyze our institutional experience and assess contemporary outcomes for children with IRC. We reviewed the medical record for patients (<21 years old) evaluated for a primary diagnosis of IRC between 1975 and 2013 at our institution. Demographic, clinical, echocardiographic, and catheterization data were abstracted. The patients were divided into 2 groups comprising a historical cohort (HC) (diagnosis: 1975 to 1993, n = 8) and a contemporary cohort (CC) (diagnosis: 1994 to 2013, n = 12). Twenty children were identified with IRC (mean age at presentation 9.7 ± 6.5 years, 55{\%} female). Mean length of follow-up was 6.5 ± 8.4 years (range 0.1 to 35.6 years). In the CC, 7 of 12 patients (58{\%}) progressed to cardiac transplantation (mean age of 9 ± 4 years at transplant, mean interval from diagnosis of IRC: 1.5 ± 0.9 years). Overall survival was improved significantly in the CC compared with the HC (80{\%} vs. 38{\%}, p = 0.02), but transplantation free survival was no different between the CC and HC over 5 years (38{\%} vs 38{\%}, p = 0.65). In the CC, elevation of mitral valve Doppler E/e' ratio on echocardiography was associated with increased mortality (p = 0.01). In conclusion, IRC continues has a poor prognosis. Early referral for transplantation was associated with improved overall survival in the modern era. Patients with markedly elevated E/e' ratio may have increased risk of death.",
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