Top

Published in:

01-04-2019 | Original Article

Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience

Authors: Samuel G. Wittekind, Thomas D. Ryan, Zhiqian Gao, Farhan Zafar, Richard J. Czosek, Clifford W. Chin, John L. Jefferies

Published in: Pediatric Cardiology | Issue 4/2019

Abstract

Background

Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx.

Methods

All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated. Outcomes of those listed for HTx (N = 18) were compared to a benchmark of contemporaneous pediatric RCM patients in the UNOS database (N = 377). Proportional hazards models were used to determine predictors of adverse outcomes.

Results

The mean age was 11 ± 9 years and 49% were male. 14 of 18 patients listed received HTx. Overall mortality (12%) was identical between the phenotypes; however, RCM patients were more likely to be listed (P = 0.001) and receive HTx (P = 0.02) compared to RCM/HCM. Prior to HTx, 60% had documented arrhythmia, 16% had cardiac arrest, and 7% required mechanical circulatory support. 4 of 17 patients with an ICD/PM received device therapies (four of five shocks appropriate for VT/VF, and two effective anti-tachycardia pacing interventions). Outcomes of those listed for HTx at our center were similar to the UNOS benchmark. In multivariate analysis, markers of congestive heart failure were associated with adverse outcomes.

Conclusion

Heart failure and arrhythmia treatments can delay or possibly prevent the need for HTx in some cases of pediatric RCM. Survival post-HTx is not compromised using this approach.

Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, Moss AJ, Seidman CE, Young JB (2006) 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 Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 113(14):1807–1816. https://doi.org/10.1161/circulationaha.106.174287 CrossRefPubMed

Lipshultz SE, Sleeper LA, Towbin JA, Lowe AM, Orav EJ, Cox GF, Lurie PR, McCoy KL, McDonald MA, Messere JE, Colan SD (2003) The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 348(17):1647–1655. https://doi.org/10.1056/NEJMoa021715 CrossRefPubMed

Nugent AW, Daubeney PEF, Chondros P, Carlin JB, Cheung M, Wilkinson LC, Davis AM, Kahler SG, Chow CW, Wilkinson JL, Weintraub RG (2003) The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 348(17):1639–1646. https://doi.org/10.1056/NEJMoa021737 CrossRefPubMed

Weller RJ, Weintraub R, Addonizio LJ, Chrisant MRK, Gersony WM, Hsu DT (2002) Outcome of idiopathic restrictive cardiomyopathy in children. Am J Cardiol 90(5):501–506. https://doi.org/10.1016/S0002-9149(02)02522-5 CrossRefPubMed

Chen S-C, Balfour IC, Jureidini S (2001) Clinical spectrum of restrictive cardiomyopathy in children. J Heart Lung Transplant 20(1):90–92. https://doi.org/10.1016/S1053-2498(00)00162-5 CrossRefPubMed

Lewis AB (1992) Clinical profile and outcome of restrictive cardiomyopathy in children. Am Heart J 123(6):1589–1593. https://doi.org/10.1016/0002-8703(92)90814-C CrossRefPubMed

Cetta F, O’Leary PW, Seward JB, Driscoll DJ (1995) Idiopathic restrictive cardiomyopathy in childhood: diagnostic features and clinical course. Mayo Clin Proc 70(7):634–640. https://doi.org/10.4065/70.7.634 CrossRefPubMed

Denfield SW, Rosenthal G, Gajarski RJ, Bricker JT, Schowengerdt KO, Price JK, Towbin JA (1997) Restrictive cardiomyopathies in childhood. Etiologies and natural history. Tex Heart Inst J 24(1):38–44PubMedPubMedCentral

Russo LM, Webber SA (2005) Idiopathic restrictive cardiomyopathy in children. Heart 91(9):1199–1202. https://doi.org/10.1136/hrt.2004.043869 CrossRefPubMedPubMedCentral

10.

Rivenes SM, Kearney DL, Smith EOB, Towbin JA, Denfield SW (2000) Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy. Circulation 102(8):876–882. https://doi.org/10.1161/01.cir.102.8.876 CrossRefPubMed

11.

Webber SA, Lipshultz SE, Sleeper LA, Lu M, Wilkinson JD, Addonizio LJ, Canter CE, Colan SD, Everitt MD, Jefferies JL, Kantor P, Lamour JM, Margossian R, Pahl E, Rusconi P, Towbin JA (2012) Outcomes of restrictive cardiomyopathy in childhood and the influence of phenotype: a report from the pediatric cardiomyopathy registry. Circulation. https://doi.org/10.1161/circulationaha.112.104638 CrossRefPubMed

12.

Dipchand AI, Naftel DC, Feingold B, Spicer R, Yung D, Kaufman B, Kirklin JK, Allain-Rooney T, Hsu D (2009) Outcomes of children with cardiomyopathy listed for transplant: a multi-institutional study. J Heart Lung Transplant 28(12):1312–1321. https://doi.org/10.1016/j.healun.2009.05.019 CrossRefPubMed

13.

Zangwill SD, Naftel D, L’Ecuyer T, Rosenthal D, Robinson B, Kirklin JK, Stendahl G, Dipchand AI (2009) Outcomes of children with restrictive cardiomyopathy listed for heart transplant: a multi-institutional study. J Heart Lung Transplant 28(12):1335–1340. https://doi.org/10.1016/j.healun.2009.06.028 CrossRefPubMed

14.

Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK, Lai WW, Geva T (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23(5):465–495. https://doi.org/10.1016/j.echo.2010.03.019 CrossRefPubMed

15.

Mogensen J, Kubo T, Duque M, Uribe W, Shaw A, Murphy R, Gimeno JR, Elliott P, McKenna WJ (2003) Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations. J Clin Investig 111(2):209–216. https://doi.org/10.1172/JCI16336 CrossRefPubMed

16.

Sluysmans T, Colan SD (2005) Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol 99(2):445–457. https://doi.org/10.1152/japplphysiol.01144.2004 CrossRefPubMed

17.

Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, Flachskampf FA, Foster E, Goldstein SA, Kuznetsova T, Lancellotti P, Muraru D, Picard MH, Rietzschel ER, Rudski L, Spencer KT, Tsang W, Voigt J-U (2015) Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 28(1):1–39.e14. https://doi.org/10.1016/j.echo.2014.10.003 CrossRefPubMed

18.

Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H (2011) 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines developed in collaboration with the American Association for Thoracic Surgery, American Society of echocardiography, American Society of nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 58(25):e212–e260CrossRefPubMed

19.

Pauli C, Fakler U, Genz T, Hennig M, Lorenz H-P, Hess J (2002) Cardiac output determination in children: equivalence of the transpulmonary thermodilution method to the direct Fick principle. Intensive Care Med 28(7):947–952. https://doi.org/10.1007/s00134-002-1334-2 CrossRefPubMed

20.

Su JA, Menteer J (2017) Outcomes of Berlin Heart EXCOR® pediatric ventricular assist device support in patients with restrictive and hypertrophic cardiomyopathy. Pediatr Transplant 21(8):e13048. https://doi.org/10.1111/petr.13048 CrossRef

21.

Villa C, Broderick J, Rizwan R, Lorts A (2018) Utilization of VADs in children with restrictive and hypertrophic cardiomyopathy: are we there yet? Prog Pediatr Cardiol. https://doi.org/10.1016/j.ppedcard.2018.03.003 CrossRef

22.

Walsh MA, Grenier MA, Jefferies JL, Towbin JA, Lorts A, Czosek RJ (2012) Conduction abnormalities in pediatric patients with restrictive cardiomyopathy. Circulation: Heart Fail. https://doi.org/10.1161/circheartfailure.111.964395 CrossRef

Title: Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience
Authors: Samuel G. Wittekind
Thomas D. Ryan
Zhiqian Gao
Farhan Zafar
Richard J. Czosek
Clifford W. Chin
John L. Jefferies
Publication date: 01-04-2019
Publisher: Springer US
Published in: Pediatric Cardiology / Issue 4/2019
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-018-2043-0

ACC 2024 Congress Coverage

Heart Failure Video

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience

Abstract

Background

Methods

Results

Conclusion

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 4/2019

Usefulness of Electroanatomical Mapping with Contact Force Monitoring for Accessory Pathways Ablation in Pediatric Population

Computational Identification of Ventricular Arrhythmia Risk in Pediatric Myocarditis

Catheterization Performed in the Early Postoperative Period After Congenital Heart Surgery in Children

Impact of Time Interval Between Glenn and Fontan Procedures on Fontan Operative and Long-Term Follow-up Results

Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein’s Anomaly

Transseptal Puncture for Catheter Ablation in Children

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page