Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy

IntroductionApical hypertrophic cardiomyopathy (ApHCM) is a subtype of hypertrophic cardiomyopathy (HCM) that affects up to 25% of Asian patients and is not as well understood in non-Asian patients. Although ApHCM has been considered a more “benign” variant, it is associated with increased risk of a...

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Main Authors: Vidhu Anand, Megan K. Covington, Ushasi Saraswati, Christopher G. Scott, Alexander T. Lee, Robert P. Frantz, Nandan S. Anavekar, Jeffrey B. Geske, Adelaide M. Arruda-Olson, Kyle W. Klarich
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-01-01
Series:Frontiers in Cardiovascular Medicine
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Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2023.1288747/full
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author Vidhu Anand
Megan K. Covington
Ushasi Saraswati
Christopher G. Scott
Alexander T. Lee
Robert P. Frantz
Nandan S. Anavekar
Jeffrey B. Geske
Adelaide M. Arruda-Olson
Kyle W. Klarich
author_facet Vidhu Anand
Megan K. Covington
Ushasi Saraswati
Christopher G. Scott
Alexander T. Lee
Robert P. Frantz
Nandan S. Anavekar
Jeffrey B. Geske
Adelaide M. Arruda-Olson
Kyle W. Klarich
author_sort Vidhu Anand
collection DOAJ
description IntroductionApical hypertrophic cardiomyopathy (ApHCM) is a subtype of hypertrophic cardiomyopathy (HCM) that affects up to 25% of Asian patients and is not as well understood in non-Asian patients. Although ApHCM has been considered a more “benign” variant, it is associated with increased risk of atrial and ventricular arrhythmias, apical thrombi, stroke, and progressive heart failure. The occurrence of pulmonary hypertension (PH) in ApHCM, due to elevated pressures on the left side of the heart, has been documented. However, the exact prevalence of PH in ApHCM and sex differences remain uncertain.MethodsWe sought to evaluate the prevalence, risk associations, and sex differences in elevated pulmonary pressures in the largest cohort of patients with ApHCM at a single tertiary center. A total of 542 patients diagnosed with ApHCM were identified using ICD codes and clinical notes searches, confirmed by cross-referencing with cardiac MRI reports extracted through Natural Language Processing and through manual evaluation of patient charts and imaging records.ResultsIn 414 patients, echocardiogram measurements of pulmonary artery systolic pressure (PASP) were obtained at the time of diagnosis. The mean age was 59.4 ± 16.6 years, with 181 (44%) being females. The mean PASP was 38 ± 12 mmHg in females vs. 33 ± 9 mmHg in males (p < 0.0001). PH as defined by a PASP value of > 36 mmHg was present in 140/414 (34%) patients, with a predominance in females [79/181 (44%)] vs. males [61/233 (26%), p < 0.0001]. Female sex, atrial fibrillation, diagnosis of congestive heart failure, and elevated filling pressures on echocardiogram remained significantly associated with PH (PASP > 36 mmHg) in multivariable modeling. PH, when present, was independently associated with mortality [hazard ratio 1.63, 95% CI (1.05–2.53), p = 0.028] and symptoms [odds ratio 2.28 (1.40, 3.71), p < 0.001].ConclusionPH was present in 34% of patients with ApHCM at diagnosis, with female sex predominance. PH in ApHCM was associated with symptoms and increased mortality.
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spelling doaj.art-e16aff4668c5466b877c1e68faaf9cff2024-01-11T05:11:30ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2024-01-011010.3389/fcvm.2023.12887471288747Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathyVidhu Anand0Megan K. Covington1Ushasi Saraswati2Christopher G. Scott3Alexander T. Lee4Robert P. Frantz5Nandan S. Anavekar6Jeffrey B. Geske7Adelaide M. Arruda-Olson8Kyle W. Klarich9Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, United StatesDepartment of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United StatesIntroductionApical hypertrophic cardiomyopathy (ApHCM) is a subtype of hypertrophic cardiomyopathy (HCM) that affects up to 25% of Asian patients and is not as well understood in non-Asian patients. Although ApHCM has been considered a more “benign” variant, it is associated with increased risk of atrial and ventricular arrhythmias, apical thrombi, stroke, and progressive heart failure. The occurrence of pulmonary hypertension (PH) in ApHCM, due to elevated pressures on the left side of the heart, has been documented. However, the exact prevalence of PH in ApHCM and sex differences remain uncertain.MethodsWe sought to evaluate the prevalence, risk associations, and sex differences in elevated pulmonary pressures in the largest cohort of patients with ApHCM at a single tertiary center. A total of 542 patients diagnosed with ApHCM were identified using ICD codes and clinical notes searches, confirmed by cross-referencing with cardiac MRI reports extracted through Natural Language Processing and through manual evaluation of patient charts and imaging records.ResultsIn 414 patients, echocardiogram measurements of pulmonary artery systolic pressure (PASP) were obtained at the time of diagnosis. The mean age was 59.4 ± 16.6 years, with 181 (44%) being females. The mean PASP was 38 ± 12 mmHg in females vs. 33 ± 9 mmHg in males (p < 0.0001). PH as defined by a PASP value of > 36 mmHg was present in 140/414 (34%) patients, with a predominance in females [79/181 (44%)] vs. males [61/233 (26%), p < 0.0001]. Female sex, atrial fibrillation, diagnosis of congestive heart failure, and elevated filling pressures on echocardiogram remained significantly associated with PH (PASP > 36 mmHg) in multivariable modeling. PH, when present, was independently associated with mortality [hazard ratio 1.63, 95% CI (1.05–2.53), p = 0.028] and symptoms [odds ratio 2.28 (1.40, 3.71), p < 0.001].ConclusionPH was present in 34% of patients with ApHCM at diagnosis, with female sex predominance. PH in ApHCM was associated with symptoms and increased mortality.https://www.frontiersin.org/articles/10.3389/fcvm.2023.1288747/fullapical hypertrophic cardiomyopathypulmonary hypertensionpulmonary artery systolic pressuresex differencesall-cause mortality
spellingShingle Vidhu Anand
Megan K. Covington
Ushasi Saraswati
Christopher G. Scott
Alexander T. Lee
Robert P. Frantz
Nandan S. Anavekar
Jeffrey B. Geske
Adelaide M. Arruda-Olson
Kyle W. Klarich
Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
Frontiers in Cardiovascular Medicine
apical hypertrophic cardiomyopathy
pulmonary hypertension
pulmonary artery systolic pressure
sex differences
all-cause mortality
title Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
title_full Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
title_fullStr Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
title_full_unstemmed Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
title_short Prevalence, sex differences, and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
title_sort prevalence sex differences and implications of pulmonary hypertension in patients with apical hypertrophic cardiomyopathy
topic apical hypertrophic cardiomyopathy
pulmonary hypertension
pulmonary artery systolic pressure
sex differences
all-cause mortality
url https://www.frontiersin.org/articles/10.3389/fcvm.2023.1288747/full
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