A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy

A healthy 11-year-old girl presented with exercise intolerance of unclear etiology, and her physical exam was notable for a 3/6 systolic ejection murmur at the left upper sternal border with radiation to the back. Extensive noninvasive workup consisted of ECG, transthoracic echocardiogram, and cardi...

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Main Authors: Elyssa Cohen, Conor P. O’Halloran, Philip T. Thrush, T. Marsha Ma, Paul Tannous
Format: Article
Language:English
Published: Wiley 2022-01-01
Series:Case Reports in Cardiology
Online Access:http://dx.doi.org/10.1155/2022/4707309
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author Elyssa Cohen
Conor P. O’Halloran
Philip T. Thrush
T. Marsha Ma
Paul Tannous
author_facet Elyssa Cohen
Conor P. O’Halloran
Philip T. Thrush
T. Marsha Ma
Paul Tannous
author_sort Elyssa Cohen
collection DOAJ
description A healthy 11-year-old girl presented with exercise intolerance of unclear etiology, and her physical exam was notable for a 3/6 systolic ejection murmur at the left upper sternal border with radiation to the back. Extensive noninvasive workup consisted of ECG, transthoracic echocardiogram, and cardiac MRI/MRA, which were all nondiagnostic. She was ultimately referred for cardiac catheterization. Baseline invasive hemodynamics demonstrated a normal cardiac index and pulmonary vascular resistance but was notable for mildly elevated right and left end-diastolic pressures. A diagnosis remained elusive, so a 500 mL volume challenge was performed, which unmasked right and left ventricular waveform transformations to reveal the pathognomonic “square root sign” of restrictive cardiomyopathy with concordant RV/LV respirophasic variation. These findings and her clinical history allowed for the rare pediatric diagnosis of restrictive cardiomyopathy early in her clinical course, prior to the development of overt signs of pathologic myocardial remodeling, such as pulmonary hypertension and biatrial enlargement.
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spelling doaj-art-ff5431f297134726b8d0bc232027a5222025-02-03T06:11:51ZengWileyCase Reports in Cardiology2090-64122022-01-01202210.1155/2022/4707309A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive CardiomyopathyElyssa Cohen0Conor P. O’Halloran1Philip T. Thrush2T. Marsha Ma3Paul Tannous4Ann & Robert H. Lurie Children’s Hospital of ChicagoAnn & Robert H. Lurie Children’s Hospital of ChicagoAnn & Robert H. Lurie Children’s Hospital of ChicagoLoyola University Medical CenterAnn & Robert H. Lurie Children’s Hospital of ChicagoA healthy 11-year-old girl presented with exercise intolerance of unclear etiology, and her physical exam was notable for a 3/6 systolic ejection murmur at the left upper sternal border with radiation to the back. Extensive noninvasive workup consisted of ECG, transthoracic echocardiogram, and cardiac MRI/MRA, which were all nondiagnostic. She was ultimately referred for cardiac catheterization. Baseline invasive hemodynamics demonstrated a normal cardiac index and pulmonary vascular resistance but was notable for mildly elevated right and left end-diastolic pressures. A diagnosis remained elusive, so a 500 mL volume challenge was performed, which unmasked right and left ventricular waveform transformations to reveal the pathognomonic “square root sign” of restrictive cardiomyopathy with concordant RV/LV respirophasic variation. These findings and her clinical history allowed for the rare pediatric diagnosis of restrictive cardiomyopathy early in her clinical course, prior to the development of overt signs of pathologic myocardial remodeling, such as pulmonary hypertension and biatrial enlargement.http://dx.doi.org/10.1155/2022/4707309
spellingShingle Elyssa Cohen
Conor P. O’Halloran
Philip T. Thrush
T. Marsha Ma
Paul Tannous
A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy
Case Reports in Cardiology
title A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy
title_full A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy
title_fullStr A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy
title_full_unstemmed A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy
title_short A Volume Challenge Reveals the Diagnosis of Pediatric Restrictive Cardiomyopathy
title_sort volume challenge reveals the diagnosis of pediatric restrictive cardiomyopathy
url http://dx.doi.org/10.1155/2022/4707309
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