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Tachycardias in children originating in the right ventricular outflow tract: lack of clinical features predicting the presence and severity of the histopathological substrate

Published online by Cambridge University Press:  19 August 2008

Fabrizio Drago ,
Andrea Mazza ,
Maria Giulia Gagliardi ,
Maurizio Bevilacqua ,
Paolo Di Renzi ,
Armando Calzolari ,
Paola Francalanci ,
Renata Boldrini ,
Cesare Bosman  and
Gaetano Di Liso
...Show all authors
Fabrizio Drago*
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Andrea Mazza
Affiliation:
Department of Cardiology, Santo Spirito Hospital, Rome, Italy
Maria Giulia Gagliardi
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Maurizio Bevilacqua
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Paolo Di Renzi
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Armando Calzolari
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Paola Francalanci
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Renata Boldrini
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Cesare Bosman
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Gaetano Di Liso
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
Pietro Ragonese
Affiliation:
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Ges– Children's Hospital; Rome, Italy
*
Dr Fabrizio Drago, Dipartimento Medico-Chirurgico di Cardiologia Pediacrica, Ospedale Bambino Ges–, Piazza S. Onofrio 4, 1-00165, Rome, Italy. Tel: 39 6 68591; Fax: 39 6 8801931
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Abstract

The aim was to determine whether the clinical features of tachycardias originating from the right ventricular outflow tract in children with an apparently normal heart could predict the presence and the severity of the histopathological substrate. Thirteen children (median age 6 years; range 6 months–12 years) with tachycardia originating from the right ventricular outflow tract of apparently normal hearts, were assessed by echocardiography, heart catheterization with angiography, endomyocardial biopsy (13 patients) and magnetic resonance imaging (MRJ) (nine patients). Tachycardia was symptomatic in six and sustained in nine. Endomyocardial biopsy and MRI revealed acute myocarditis in five patients (38%), fatty infiltration of the right ventricle in two (15%), and minor histologic abnormalities in three (23%). Myocarditis was diagnosed in three of nine patients with sustained ventricular tachycardia, as opposed to two of four with non-sustained tachycardia (p = NS); in three of six symptomatic versus two of seven asymptomatic patients (p = NS); and in two of eight patients in whom ventricular tachycardia was induced during exercise testing as opposed to one of three in which it was not inducible (p = NS). A histopathological substrate was found in six of nine patients with sustained ventricular tachycardia, and in all four with non-sustained tachycardia (p = NS); in five of six patients with symptoms versus five of seven asymptomatic patients (p = NS); and in five of eight with inducible ventricular tachycardia during exercise testing versus all three in whom it was not inducible (p = NS). The mean rate of tachycardia was 184 ± 39 beats min−1 in patients with myocarditis, as opposed to 171 ± 48 in patients without myocarditis (p = NS); and 163 ± 33 in patients with a histopathological substrate compared with 210 ± 65 in patients without a histopathological substrate (p = NS). It is concluded that a histopathological substrate is present in the greater majority of children affected by the so-called right ventricular outflow tract tachycardia, but that the clinical features of the tachycardia do not predict the presence and the severity of this histopathological substrate.

Type
Original Articles
Information
Cardiology in the Young , Volume 9 , Issue 3 , May 1999 , pp. 273 - 279
Copyright
Copyright © Cambridge University Press 1999

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