Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy

Kevin C. Ong, Jeffrey B. Geske, Virginia B. Hebl, Rick A. Nishimura, Hartzell V Schaff, Michael John Ackerman, Kyle W. Klarich, Konstantinos C. Siontis, Thais Coutinho, Joseph A. Dearani, Steve R. Ommen, Bernard J. Gersh

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Aims Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM. Methods and results The study cohort consisted of 1570 (54±15 years; 53% male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) .36 mmHg] was identified in 38% of patients who were older (57±15 vs. 52±15 years, P <0.0001), more likely to be female (59 vs. 40%, P <0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45%, P <0.0001) and atrial fibrillation (29 vs. 15%, P <0.0001) vs.Those without PH. Only 12% had moderate or severe PH (PASP .50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95% CI 1.28-1.96, P <0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95% CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT. Conclusions Over one-Third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.

Original languageEnglish (US)
Pages (from-to)604-610
Number of pages7
JournalEuropean Heart Journal Cardiovascular Imaging
Volume17
Issue number6
DOIs
StatePublished - Jun 1 2016

Fingerprint

Hypertrophic Cardiomyopathy
Pulmonary Hypertension
Survival
Pulmonary Artery
Blood Pressure
Atrial Fibrillation
Mortality
Heart Diseases
Cohort Studies
Therapeutics
Referral and Consultation
Regression Analysis

Keywords

  • Echocardiography
  • Hypertrophic Cardiomyopathy
  • Pulmonary Hypertension
  • Surgery
  • Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Radiology Nuclear Medicine and imaging

Cite this

Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy. / Ong, Kevin C.; Geske, Jeffrey B.; Hebl, Virginia B.; Nishimura, Rick A.; Schaff, Hartzell V; Ackerman, Michael John; Klarich, Kyle W.; Siontis, Konstantinos C.; Coutinho, Thais; Dearani, Joseph A.; Ommen, Steve R.; Gersh, Bernard J.

In: European Heart Journal Cardiovascular Imaging, Vol. 17, No. 6, 01.06.2016, p. 604-610.

Research output: Contribution to journalArticle

Ong, KC, Geske, JB, Hebl, VB, Nishimura, RA, Schaff, HV, Ackerman, MJ, Klarich, KW, Siontis, KC, Coutinho, T, Dearani, JA, Ommen, SR & Gersh, BJ 2016, 'Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy', European Heart Journal Cardiovascular Imaging, vol. 17, no. 6, pp. 604-610. https://doi.org/10.1093/ehjci/jew024
Ong, Kevin C. ; Geske, Jeffrey B. ; Hebl, Virginia B. ; Nishimura, Rick A. ; Schaff, Hartzell V ; Ackerman, Michael John ; Klarich, Kyle W. ; Siontis, Konstantinos C. ; Coutinho, Thais ; Dearani, Joseph A. ; Ommen, Steve R. ; Gersh, Bernard J. / Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy. In: European Heart Journal Cardiovascular Imaging. 2016 ; Vol. 17, No. 6. pp. 604-610.
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abstract = "Aims Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM. Methods and results The study cohort consisted of 1570 (54±15 years; 53{\%} male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) .36 mmHg] was identified in 38{\%} of patients who were older (57±15 vs. 52±15 years, P <0.0001), more likely to be female (59 vs. 40{\%}, P <0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45{\%}, P <0.0001) and atrial fibrillation (29 vs. 15{\%}, P <0.0001) vs.Those without PH. Only 12{\%} had moderate or severe PH (PASP .50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95{\%} CI 1.28-1.96, P <0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95{\%} CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT. Conclusions Over one-Third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.",
keywords = "Echocardiography, Hypertrophic Cardiomyopathy, Pulmonary Hypertension, Surgery, Survival",
author = "Ong, {Kevin C.} and Geske, {Jeffrey B.} and Hebl, {Virginia B.} and Nishimura, {Rick A.} and Schaff, {Hartzell V} and Ackerman, {Michael John} and Klarich, {Kyle W.} and Siontis, {Konstantinos C.} and Thais Coutinho and Dearani, {Joseph A.} and Ommen, {Steve R.} and Gersh, {Bernard J.}",
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T1 - Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy

AU - Ong, Kevin C.

AU - Geske, Jeffrey B.

AU - Hebl, Virginia B.

AU - Nishimura, Rick A.

AU - Schaff, Hartzell V

AU - Ackerman, Michael John

AU - Klarich, Kyle W.

AU - Siontis, Konstantinos C.

AU - Coutinho, Thais

AU - Dearani, Joseph A.

AU - Ommen, Steve R.

AU - Gersh, Bernard J.

PY - 2016/6/1

Y1 - 2016/6/1

N2 - Aims Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM. Methods and results The study cohort consisted of 1570 (54±15 years; 53% male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) .36 mmHg] was identified in 38% of patients who were older (57±15 vs. 52±15 years, P <0.0001), more likely to be female (59 vs. 40%, P <0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45%, P <0.0001) and atrial fibrillation (29 vs. 15%, P <0.0001) vs.Those without PH. Only 12% had moderate or severe PH (PASP .50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95% CI 1.28-1.96, P <0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95% CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT. Conclusions Over one-Third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.

AB - Aims Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM. Methods and results The study cohort consisted of 1570 (54±15 years; 53% male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) .36 mmHg] was identified in 38% of patients who were older (57±15 vs. 52±15 years, P <0.0001), more likely to be female (59 vs. 40%, P <0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45%, P <0.0001) and atrial fibrillation (29 vs. 15%, P <0.0001) vs.Those without PH. Only 12% had moderate or severe PH (PASP .50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95% CI 1.28-1.96, P <0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95% CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT. Conclusions Over one-Third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.

KW - Echocardiography

KW - Hypertrophic Cardiomyopathy

KW - Pulmonary Hypertension

KW - Surgery

KW - Survival

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