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Disopyramide use in infants and children with hypertrophic cardiomyopathy

Published online by Cambridge University Press:  07 March 2018

Matthew J. O’Connor ,
Kelley Miller ,
Robert E. Shaddy ,
Kimberly Y. Lin ,
Brian D. Hanna ,
Chitra Ravishankar  and
Joseph W. Rossano
Matthew J. O’Connor*
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
Kelley Miller
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
Robert E. Shaddy
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
Kimberly Y. Lin
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
Brian D. Hanna
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
Chitra Ravishankar
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
Joseph W. Rossano
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, United States of America
*
Corresponding to: M. J. O’Connor, Division of Cardiology, The Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, United States of America. Tel: +267 426 5700; Fax: +267 426 9800; E-mail: oconnorm@email.chop.edu
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Abstract

Hypertrophic cardiomyopathy has a range of clinical severity in children. Treatment options are limited, mainly on account of small patient size. Disopyramide is a sodium channel blocker with negative inotropic properties that effectively reduces left ventricular outflow tract gradients in adults with hypertrophic cardiomyopathy, but its efficacy in children is uncertain. A retrospective chart review of patients ⩽21 years of age with hypertrophic cardiomyopathy at our institution and treated with disopyramide was performed. Left ventricular outflow tract Doppler gradients before and after disopyramide initiation were compared as the primary outcome measure. Nine patients received disopyramide, with a median age of 5.6 years (range 6 days–12.9 years). The median left ventricular outflow tract Doppler gradient before initiation of disopyramide was 81 mmHg (range 30–132 mmHg); eight patients had post-initiation echocardiograms, in which the median lowest recorded Doppler gradient was 43 mmHg (range 15–100 mmHg), for a median % reduction of 58.2% (p=0.002). With median follow-up of 2.5 years, eight of nine patients were still alive, although disopyramide had been discontinued in six of the nine patients. Reasons for discontinuation included septal myomectomy (four patients), heart transplantation (one patient), and side effects (one patient). Disopyramide was effective for the relief of left ventricular outflow tract obstruction in children with hypertrophic cardiomyopathy, although longer-term data suggest that its efficacy is not sustained. In general, it was well tolerated. Further study in larger patient populations is warranted.

Keywords

Hypertrophic cardiomyopathypaediatricchildrendisopyramide
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 4 , April 2018 , pp. 530 - 535
Copyright
© Cambridge University Press 2018 

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