Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly

Marco Canepa, Iraklis Pozios, Pier Filippo Vianello, Pietro Ameri, Claudio Brunelli, Luigi Ferrucci, Theodore P. Abraham

Research output: Contribution to journalArticle

Abstract

The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family history of HCM and/or sudden cardiac death and the presence of exertional symptoms usually support HCM, though they are less likely in older patients with HCM, and poorly investigated in individuals with VSB. A more severe hypertrophy and the presence of left ventricular outflow obstruction are considered diagnostic of HCM, though stress echocardiography has not been consistently used in VSB. Mitral annulus calcification is very prevalent in both conditions, whereas a restrictive filling pattern is found in a minority of older patients with HCM. Genetic testing has low applicability in this differential diagnosis at the current time, given that a causative mutation is found in less than 10% of elderly patients with suspected HCM. Emerging imaging modalities that allow non-invasive detection of myocardial fibrosis and disarray may help, but have not been fully investigated. Nonetheless, there remains a considerable morphological overlap between the two conditions. Comprehensive studies, particularly imaging based, are warranted to offer a more evidence-based approach to elderly patients with focal septal thickening.

Original languageEnglish (US)
JournalHeart
DOIs
StateAccepted/In press - Apr 27 2016

Fingerprint

Hypertrophic Cardiomyopathy
Hypertrophy
Ventricular Outflow Obstruction
Stress Echocardiography
Sudden Cardiac Death
Genetic Testing
Differential Diagnosis
Fibrosis
Hypertension
Mutation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Canepa, M., Pozios, I., Vianello, P. F., Ameri, P., Brunelli, C., Ferrucci, L., & Abraham, T. P. (Accepted/In press). Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly. Heart. https://doi.org/10.1136/heartjnl-2015-308764

Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly. / Canepa, Marco; Pozios, Iraklis; Vianello, Pier Filippo; Ameri, Pietro; Brunelli, Claudio; Ferrucci, Luigi; Abraham, Theodore P.

In: Heart, 27.04.2016.

Research output: Contribution to journalArticle

Canepa, M, Pozios, I, Vianello, PF, Ameri, P, Brunelli, C, Ferrucci, L & Abraham, TP 2016, 'Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly', Heart. https://doi.org/10.1136/heartjnl-2015-308764
Canepa, Marco ; Pozios, Iraklis ; Vianello, Pier Filippo ; Ameri, Pietro ; Brunelli, Claudio ; Ferrucci, Luigi ; Abraham, Theodore P. / Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly. In: Heart. 2016.
@article{5eecc90fab074e198ae9bce12db3bc69,
title = "Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly",
abstract = "The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family history of HCM and/or sudden cardiac death and the presence of exertional symptoms usually support HCM, though they are less likely in older patients with HCM, and poorly investigated in individuals with VSB. A more severe hypertrophy and the presence of left ventricular outflow obstruction are considered diagnostic of HCM, though stress echocardiography has not been consistently used in VSB. Mitral annulus calcification is very prevalent in both conditions, whereas a restrictive filling pattern is found in a minority of older patients with HCM. Genetic testing has low applicability in this differential diagnosis at the current time, given that a causative mutation is found in less than 10{\%} of elderly patients with suspected HCM. Emerging imaging modalities that allow non-invasive detection of myocardial fibrosis and disarray may help, but have not been fully investigated. Nonetheless, there remains a considerable morphological overlap between the two conditions. Comprehensive studies, particularly imaging based, are warranted to offer a more evidence-based approach to elderly patients with focal septal thickening.",
author = "Marco Canepa and Iraklis Pozios and Vianello, {Pier Filippo} and Pietro Ameri and Claudio Brunelli and Luigi Ferrucci and Abraham, {Theodore P.}",
year = "2016",
month = "4",
day = "27",
doi = "10.1136/heartjnl-2015-308764",
language = "English (US)",
journal = "Heart",
issn = "1355-6037",
publisher = "BMJ Publishing Group",

}

TY - JOUR

T1 - Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly

AU - Canepa, Marco

AU - Pozios, Iraklis

AU - Vianello, Pier Filippo

AU - Ameri, Pietro

AU - Brunelli, Claudio

AU - Ferrucci, Luigi

AU - Abraham, Theodore P.

PY - 2016/4/27

Y1 - 2016/4/27

N2 - The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family history of HCM and/or sudden cardiac death and the presence of exertional symptoms usually support HCM, though they are less likely in older patients with HCM, and poorly investigated in individuals with VSB. A more severe hypertrophy and the presence of left ventricular outflow obstruction are considered diagnostic of HCM, though stress echocardiography has not been consistently used in VSB. Mitral annulus calcification is very prevalent in both conditions, whereas a restrictive filling pattern is found in a minority of older patients with HCM. Genetic testing has low applicability in this differential diagnosis at the current time, given that a causative mutation is found in less than 10% of elderly patients with suspected HCM. Emerging imaging modalities that allow non-invasive detection of myocardial fibrosis and disarray may help, but have not been fully investigated. Nonetheless, there remains a considerable morphological overlap between the two conditions. Comprehensive studies, particularly imaging based, are warranted to offer a more evidence-based approach to elderly patients with focal septal thickening.

AB - The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family history of HCM and/or sudden cardiac death and the presence of exertional symptoms usually support HCM, though they are less likely in older patients with HCM, and poorly investigated in individuals with VSB. A more severe hypertrophy and the presence of left ventricular outflow obstruction are considered diagnostic of HCM, though stress echocardiography has not been consistently used in VSB. Mitral annulus calcification is very prevalent in both conditions, whereas a restrictive filling pattern is found in a minority of older patients with HCM. Genetic testing has low applicability in this differential diagnosis at the current time, given that a causative mutation is found in less than 10% of elderly patients with suspected HCM. Emerging imaging modalities that allow non-invasive detection of myocardial fibrosis and disarray may help, but have not been fully investigated. Nonetheless, there remains a considerable morphological overlap between the two conditions. Comprehensive studies, particularly imaging based, are warranted to offer a more evidence-based approach to elderly patients with focal septal thickening.

UR - http://www.scopus.com/inward/record.url?scp=84966286476&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84966286476&partnerID=8YFLogxK

U2 - 10.1136/heartjnl-2015-308764

DO - 10.1136/heartjnl-2015-308764

M3 - Article

JO - Heart

JF - Heart

SN - 1355-6037

ER -