Massive global right ventricular hypertrophy with both fixed and dynamic obstruction and pulmonary valve dysplasia

Ari M. Cedars; Cylen Javidan-Nejad; Manu Goyal; Joseph Billadello; Philip Ludbrook

doi:10.1111/j.1747-0803.2011.00538.x

Massive global right ventricular hypertrophy with both fixed and dynamic obstruction and pulmonary valve dysplasia

Ari M. Cedars, Cylen Javidan-Nejad, Manu Goyal, Joseph Billadello, Philip Ludbrook

Research output: Contribution to journal › Article › peer-review

Abstract

Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.

Original language	English (US)
Pages (from-to)	66-70
Number of pages	5
Journal	Congenital Heart Disease
Volume	7
Issue number	1
DOIs	https://doi.org/10.1111/j.1747-0803.2011.00538.x
State	Published - Jan 2012
Externally published	Yes

Keywords

Dynamic right ventricular outflow tract obstruction
Hypertrophic cardiomyopathy
Noonan syndrome
Pulmonary valve dysplasia
Right ventricular hypertrophy

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Surgery
Radiology Nuclear Medicine and imaging
Cardiology and Cardiovascular Medicine

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10.1111/j.1747-0803.2011.00538.x

Cite this

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abstract = "Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.",

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AU - Javidan-Nejad, Cylen

AU - Goyal, Manu

AU - Billadello, Joseph

AU - Ludbrook, Philip

PY - 2012/1

Y1 - 2012/1

N2 - Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.

AB - Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.

KW - Dynamic right ventricular outflow tract obstruction

KW - Hypertrophic cardiomyopathy

KW - Noonan syndrome

KW - Pulmonary valve dysplasia

KW - Right ventricular hypertrophy

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Massive global right ventricular hypertrophy with both fixed and dynamic obstruction and pulmonary valve dysplasia

Abstract

Keywords

ASJC Scopus subject areas

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