Skip to main content
Erschienen in: European Radiology 2/2024

28.08.2023 | Cardiac

Association between left atrial myopathy and sarcomere mutation in patients with hypertrophic cardiomyopathy: insights into left atrial strain by MRI feature tracking

verfasst von: Jiaxin Wang, Xuan Ma, Kankan Zhao, Shujuan Yang, Kai Yang, Shiqin Yu, Gang Yin, Zhixiang Dong, Yanyan Song, Chen Cui, Jinghui Li, Shihua Zhao, Xiuyu Chen

Erschienen in: European Radiology | Ausgabe 2/2024

Einloggen, um Zugang zu erhalten

Abstract

Objectives

Left atrial (LA) myopathy, characterized by LA enlargement and mechanical dysfunction, is associated with worse prognosis in hypertrophic cardiomyopathy (HCM) while the impact of sarcomere mutation on LA myopathy remains unclear. We aimed to assess the association between LA myopathy and sarcomere mutation and to explore the incremental utility of LA strain in mutation prediction.

Methods

A total of 105 consecutive HCM patients (mean age 47.8 ± 11.9 years, 71% male) who underwent HCM-related gene screening and cardiac MRI were retrospectively enrolled. LA volume, ejection fraction and strain indices in reservoir, conduit, and booster-pump phases were investigated respectively.

Results

Fifty mutation-positive patients showed higher LA maximal volume index (59.4 ± 28.2 vs 43.8 ± 18.1 mL/m2, p = 0.001), lower reservoir (21.3 ± 7.9 vs 26.2 ± 6.6%, p < 0.001), and booster-pump strain (12.1 ± 5.4 vs 17.1 ± 5.0%, p < 0.001) but similar conduit strain (9.2 ± 4.5 vs 9.1 ± 4.5%, p = 0.909) compared with mutation-negative patients. In multivariate logistic regression, LA booster-pump strain was associated with sarcomere mutation (odds ratio = 0.86, 95% confidence interval: 0.77–0.96, p = 0.010) independent of maximal wall thickness, late gadolinium enhancement, and LA volume. Furthermore, LA booster-pump strain showed incremental value for mutation prediction added to Mayo II score (AUC 0.798 vs 0.709, p = 0.024).

Conclusions

In HCM, mutation-positive patients suffered worse LA enlargement and worse reservoir and booster-pump functions. LA booster-pump strain was a strong factor for sarcomere mutation prediction added to Mayo II score.

Clinical relevance statement

The independent association between sarcomere mutation and left atrial mechanical dysfunction provide new insights into the pathogenesis of atrial myopathy and is helpful to understand the adverse prognosis regarding atrial fibrillation and stroke in mutation-positive patients.

Key Points

• In patients with hypertrophic cardiomyopathy, left atrial (LA) reservoir and booster-pump function, but not conduit function, were significantly impaired in mutation-positive patients compared with mutation-negative patients.
• LA booster-pump strain measured by MRI-derived feature tracking is feasible to predict sarcomere mutation with high incremental value added to Mayo II score.

Graphical abstract

Literatur
1.
Zurück zum Zitat Authors/Task Force m, Elliott PM, Anastasakis A et al (2014) N Engl J Med 7:655–668 Authors/Task Force m, Elliott PM, Anastasakis A et al (2014) N Engl J Med 7:655–668
2.
Zurück zum Zitat Maron BJ (2018) Clinical course and management of hypertrophic cardiomyopathy. N Engl J Med 7:655–668CrossRef Maron BJ (2018) Clinical course and management of hypertrophic cardiomyopathy. N Engl J Med 7:655–668CrossRef
3.
Zurück zum Zitat Richard P, Charron P, Carrier L et al (2003) Hypertrophic cardiomyopathy. Circulation 17:2227–2232CrossRef Richard P, Charron P, Carrier L et al (2003) Hypertrophic cardiomyopathy. Circulation 17:2227–2232CrossRef
4.
Zurück zum Zitat Neubauer S, Kolm P, Ho CY et al (2019) Distinct subgroups in hypertrophic cardiomyopathy in the NHLBI HCM registry. J Am Coll Cardiol 19:2333–2345CrossRef Neubauer S, Kolm P, Ho CY et al (2019) Distinct subgroups in hypertrophic cardiomyopathy in the NHLBI HCM registry. J Am Coll Cardiol 19:2333–2345CrossRef
5.
Zurück zum Zitat Ommen SR, Mital S, Burke MA et al (2020) 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 25:e558–e631 Ommen SR, Mital S, Burke MA et al (2020) 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 25:e558–e631
6.
Zurück zum Zitat Ho CY, Day SM, Ashley EA et al (2018) Genotype and lifetime burden of disease in hypertrophic cardiomyopathy: insights from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). Circulation 14:1387–1398CrossRef Ho CY, Day SM, Ashley EA et al (2018) Genotype and lifetime burden of disease in hypertrophic cardiomyopathy: insights from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). Circulation 14:1387–1398CrossRef
7.
Zurück zum Zitat Lee SP, Ashley EA, Homburger J et al (2018) Incident atrial fibrillation is associated with MYH7 sarcomeric gene variation in hypertrophic cardiomyopathy. Circ Heart Fail 9:e005191CrossRef Lee SP, Ashley EA, Homburger J et al (2018) Incident atrial fibrillation is associated with MYH7 sarcomeric gene variation in hypertrophic cardiomyopathy. Circ Heart Fail 9:e005191CrossRef
8.
Zurück zum Zitat Lim WW, Neo M, Thanigaimani S et al (2021) Electrophysiological and structural remodeling of the atria in a mouse model of troponin-I mutation linked hypertrophic cardiomyopathy: implications for atrial fibrillation. Int J Mol Sci 13:6941CrossRef Lim WW, Neo M, Thanigaimani S et al (2021) Electrophysiological and structural remodeling of the atria in a mouse model of troponin-I mutation linked hypertrophic cardiomyopathy: implications for atrial fibrillation. Int J Mol Sci 13:6941CrossRef
9.
Zurück zum Zitat Farhad H, Seidelmann SB, Vigneault D et al (2017) Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. J Cardiovasc Magn Reson 1:107CrossRef Farhad H, Seidelmann SB, Vigneault D et al (2017) Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. J Cardiovasc Magn Reson 1:107CrossRef
10.
Zurück zum Zitat Huurman R, Bowen DJ, Mutluer FO et al (2022) Prognostic significance of left atrial strain in sarcomere gene variant carriers without hypertrophic cardiomyopathy. Echocardiography 9:1209–1218CrossRef Huurman R, Bowen DJ, Mutluer FO et al (2022) Prognostic significance of left atrial strain in sarcomere gene variant carriers without hypertrophic cardiomyopathy. Echocardiography 9:1209–1218CrossRef
11.
Zurück zum Zitat Chen CJ, Su MM, Liao YC et al (2020) Long-term outcomes and left ventricular diastolic function of sarcomere mutation-positive and mutation-negative patients with hypertrophic cardiomyopathy: a prospective cohort study. Eur Heart J Cardiovasc Imaging 12:1397–1404 Chen CJ, Su MM, Liao YC et al (2020) Long-term outcomes and left ventricular diastolic function of sarcomere mutation-positive and mutation-negative patients with hypertrophic cardiomyopathy: a prospective cohort study. Eur Heart J Cardiovasc Imaging 12:1397–1404
12.
Zurück zum Zitat Chung H, Kim Y, Park CH et al (2021) Contribution of sarcomere gene mutations to left atrial function in patients with hypertrophic cardiomyopathy. Cardiovasc Ultrasound 1:4CrossRef Chung H, Kim Y, Park CH et al (2021) Contribution of sarcomere gene mutations to left atrial function in patients with hypertrophic cardiomyopathy. Cardiovasc Ultrasound 1:4CrossRef
13.
Zurück zum Zitat Raman B, Smillie RW, Mahmod M et al (2021) Incremental value of left atrial booster and reservoir strain in predicting atrial fibrillation in patients with hypertrophic cardiomyopathy: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 1:109CrossRef Raman B, Smillie RW, Mahmod M et al (2021) Incremental value of left atrial booster and reservoir strain in predicting atrial fibrillation in patients with hypertrophic cardiomyopathy: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 1:109CrossRef
14.
Zurück zum Zitat Yang F, Wang L, Wang J et al (2021) Prognostic value of fast semi-automated left atrial long-axis strain analysis in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson 1:36CrossRef Yang F, Wang L, Wang J et al (2021) Prognostic value of fast semi-automated left atrial long-axis strain analysis in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson 1:36CrossRef
15.
Zurück zum Zitat Raafs AG, Vos JL, Henkens M et al (2022) Left atrial strain has superior prognostic value to ventricular function and delayed-enhancement in dilated cardiomyopathy. JACC Cardiovasc Imaging 6:1015–1026CrossRef Raafs AG, Vos JL, Henkens M et al (2022) Left atrial strain has superior prognostic value to ventricular function and delayed-enhancement in dilated cardiomyopathy. JACC Cardiovasc Imaging 6:1015–1026CrossRef
16.
Zurück zum Zitat Shi R, Shi K, Huang S et al (2022) Association between heart failure with preserved left ventricular ejection fraction and impaired left atrial phasic function in hypertrophic cardiomyopathy: evaluation by cardiac MRI feature tracking. J Magn Reson Imaging 1:248–259CrossRef Shi R, Shi K, Huang S et al (2022) Association between heart failure with preserved left ventricular ejection fraction and impaired left atrial phasic function in hypertrophic cardiomyopathy: evaluation by cardiac MRI feature tracking. J Magn Reson Imaging 1:248–259CrossRef
17.
Zurück zum Zitat Maheshwari A, Norby FL, Inciardi RM et al (2023) Left atrial mechanical dysfunction and the risk for ischemic stroke in people without prevalent atrial fibrillation or stroke : a prospective cohort study. Ann Intern Med 1:39–48CrossRef Maheshwari A, Norby FL, Inciardi RM et al (2023) Left atrial mechanical dysfunction and the risk for ischemic stroke in people without prevalent atrial fibrillation or stroke : a prospective cohort study. Ann Intern Med 1:39–48CrossRef
18.
Zurück zum Zitat Zhou H, Li L, Liu Z et al (2021) Deep learning algorithm to improve hypertrophic cardiomyopathy mutation prediction using cardiac cine images. Eur Radiol 6:3931–3940CrossRef Zhou H, Li L, Liu Z et al (2021) Deep learning algorithm to improve hypertrophic cardiomyopathy mutation prediction using cardiac cine images. Eur Radiol 6:3931–3940CrossRef
19.
Zurück zum Zitat Schulz-Menger J, Bluemke DA, Bremerich J et al (2020) Standardized image interpretation and post-processing in cardiovascular magnetic resonance - 2020 update : Society for Cardiovascular Magnetic Resonance (SCMR): Board of Trustees Task Force on Standardized Post-Processing. J Cardiovasc Magn Reson 1:19CrossRef Schulz-Menger J, Bluemke DA, Bremerich J et al (2020) Standardized image interpretation and post-processing in cardiovascular magnetic resonance - 2020 update : Society for Cardiovascular Magnetic Resonance (SCMR): Board of Trustees Task Force on Standardized Post-Processing. J Cardiovasc Magn Reson 1:19CrossRef
20.
Zurück zum Zitat Kowallick JT, Kutty S, Edelmann F et al (2014) Quantification of left atrial strain and strain rate using cardiovascular magnetic resonance myocardial feature tracking: a feasibility study. J Cardiovasc Magn Reson 1:60CrossRef Kowallick JT, Kutty S, Edelmann F et al (2014) Quantification of left atrial strain and strain rate using cardiovascular magnetic resonance myocardial feature tracking: a feasibility study. J Cardiovasc Magn Reson 1:60CrossRef
21.
Zurück zum Zitat Li L, Chen X, Yin G et al (2020) Early detection of left atrial dysfunction assessed by CMR feature tracking in hypertensive patients. Eur Radiol 2:702–711CrossRef Li L, Chen X, Yin G et al (2020) Early detection of left atrial dysfunction assessed by CMR feature tracking in hypertensive patients. Eur Radiol 2:702–711CrossRef
22.
Zurück zum Zitat Williams LK, Chan RH, Carasso S et al (2015) Effect of left ventricular outflow tract obstruction on left atrial mechanics in hypertrophic cardiomyopathy. Biomed Res Int 2015:481245 Williams LK, Chan RH, Carasso S et al (2015) Effect of left ventricular outflow tract obstruction on left atrial mechanics in hypertrophic cardiomyopathy. Biomed Res Int 2015:481245
23.
Zurück zum Zitat Kowallick JT, Silva Vieira M, Kutty S et al (2017) Left atrial performance in the course of hypertrophic cardiomyopathy: relation to left ventricular hypertrophy and fibrosis. Invest Radiol 3:177–185CrossRef Kowallick JT, Silva Vieira M, Kutty S et al (2017) Left atrial performance in the course of hypertrophic cardiomyopathy: relation to left ventricular hypertrophy and fibrosis. Invest Radiol 3:177–185CrossRef
24.
Zurück zum Zitat Essayagh B, Resseguier N, Michel N et al (2021) Left atrial dysfunction as marker of poor outcome in patients with hypertrophic cardiomyopathy. Arch Cardiovasc Dis 2:96–104CrossRef Essayagh B, Resseguier N, Michel N et al (2021) Left atrial dysfunction as marker of poor outcome in patients with hypertrophic cardiomyopathy. Arch Cardiovasc Dis 2:96–104CrossRef
25.
Zurück zum Zitat O’Mahony C, Jichi F, Pavlou M et al (2014) A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J 30:2010–2020CrossRef O’Mahony C, Jichi F, Pavlou M et al (2014) A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J 30:2010–2020CrossRef
26.
Zurück zum Zitat Cau R, Bassareo P, Suri JS, Pontone G, Saba L (2022) The emerging role of atrial strain assessed by cardiac MRI in different cardiovascular settings: an up-to-date review. Eur Radiol 7:4384–4394CrossRef Cau R, Bassareo P, Suri JS, Pontone G, Saba L (2022) The emerging role of atrial strain assessed by cardiac MRI in different cardiovascular settings: an up-to-date review. Eur Radiol 7:4384–4394CrossRef
27.
Zurück zum Zitat Lee HJ, Kim HK, Rhee TM et al (2022) Left atrial reservoir strain-based left ventricular diastolic function grading and incident heart failure in hypertrophic cardiomyopathy. Circ Cardiovasc Imaging 4:e013556 Lee HJ, Kim HK, Rhee TM et al (2022) Left atrial reservoir strain-based left ventricular diastolic function grading and incident heart failure in hypertrophic cardiomyopathy. Circ Cardiovasc Imaging 4:e013556
28.
Zurück zum Zitat Malaescu GG, Mirea O, Capota R, Petrescu AM, Duchenne J, Voigt JU (2022) Left atrial strain determinants during the cardiac phases. JACC Cardiovasc Imaging 3:381–391CrossRef Malaescu GG, Mirea O, Capota R, Petrescu AM, Duchenne J, Voigt JU (2022) Left atrial strain determinants during the cardiac phases. JACC Cardiovasc Imaging 3:381–391CrossRef
29.
Zurück zum Zitat Huber AT, Lamy J, Rahhal A et al (2018) Cardiac MR strain: a noninvasive biomarker of fibrofatty remodeling of the left atrial myocardium. Radiology 1:83–92CrossRef Huber AT, Lamy J, Rahhal A et al (2018) Cardiac MR strain: a noninvasive biomarker of fibrofatty remodeling of the left atrial myocardium. Radiology 1:83–92CrossRef
30.
Zurück zum Zitat Ohtani K, Yutani C, Nagata S, Koretsune Y, Hori M, Kamada T (1995) High prevalence of atrial fibrosis in patients with dilated cardiomyopathy. J Am Coll Cardiol 5:1162–1169CrossRef Ohtani K, Yutani C, Nagata S, Koretsune Y, Hori M, Kamada T (1995) High prevalence of atrial fibrosis in patients with dilated cardiomyopathy. J Am Coll Cardiol 5:1162–1169CrossRef
31.
Zurück zum Zitat Gruner C, Ivanov J, Care M et al (2013) Toronto hypertrophic cardiomyopathy genotype score for prediction of a positive genotype in hypertrophic cardiomyopathy. Circ Cardiovasc Genet 1:19–26CrossRef Gruner C, Ivanov J, Care M et al (2013) Toronto hypertrophic cardiomyopathy genotype score for prediction of a positive genotype in hypertrophic cardiomyopathy. Circ Cardiovasc Genet 1:19–26CrossRef
32.
Zurück zum Zitat Bos JM, Will ML, Gersh BJ, Kruisselbrink TM, Ommen SR, Ackerman MJ (2014) Characterization of a phenotype-based genetic test prediction score for unrelated patients with hypertrophic cardiomyopathy. Mayo Clin Proc 6:727–737CrossRef Bos JM, Will ML, Gersh BJ, Kruisselbrink TM, Ommen SR, Ackerman MJ (2014) Characterization of a phenotype-based genetic test prediction score for unrelated patients with hypertrophic cardiomyopathy. Mayo Clin Proc 6:727–737CrossRef
33.
Zurück zum Zitat Newman R, Jefferies JL, Chin C et al (2018) Hypertrophic cardiomyopathy genotype prediction models in a pediatric population. Pediatr Cardiol 4:709–717CrossRef Newman R, Jefferies JL, Chin C et al (2018) Hypertrophic cardiomyopathy genotype prediction models in a pediatric population. Pediatr Cardiol 4:709–717CrossRef
34.
Zurück zum Zitat Aziz A, Musiol SK, Moody WE, Pickup L, Cooper R, Lip GYH (2021) Clinical prediction of genotypes in hypertrophic cardiomyopathy: a systematic review. Eur J Clin Invest 8:e13593CrossRef Aziz A, Musiol SK, Moody WE, Pickup L, Cooper R, Lip GYH (2021) Clinical prediction of genotypes in hypertrophic cardiomyopathy: a systematic review. Eur J Clin Invest 8:e13593CrossRef
35.
Zurück zum Zitat de la Rosa A, Shah M, Shiota T, Siegel R, Rader F (2022) Comparing echocardiographic characteristics in genotype positive-phenotype positive hypertrophic cardiomyopathy and hypertensive left ventricular hypertrophy. Eur Heart J Cardiovasc Imaging 3:340–348CrossRef de la Rosa A, Shah M, Shiota T, Siegel R, Rader F (2022) Comparing echocardiographic characteristics in genotype positive-phenotype positive hypertrophic cardiomyopathy and hypertensive left ventricular hypertrophy. Eur Heart J Cardiovasc Imaging 3:340–348CrossRef
Metadaten
Titel
Association between left atrial myopathy and sarcomere mutation in patients with hypertrophic cardiomyopathy: insights into left atrial strain by MRI feature tracking
verfasst von
Jiaxin Wang
Xuan Ma
Kankan Zhao
Shujuan Yang
Kai Yang
Shiqin Yu
Gang Yin
Zhixiang Dong
Yanyan Song
Chen Cui
Jinghui Li
Shihua Zhao
Xiuyu Chen
Publikationsdatum
28.08.2023
Verlag
Springer Berlin Heidelberg
Erschienen in
European Radiology / Ausgabe 2/2024
Print ISSN: 0938-7994
Elektronische ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-023-10128-x

Weitere Artikel der Ausgabe 2/2024

European Radiology 2/2024 Zur Ausgabe

Darf man die Behandlung eines Neonazis ablehnen?

08.05.2024 Gesellschaft Nachrichten

In einer Leseranfrage in der Zeitschrift Journal of the American Academy of Dermatology möchte ein anonymer Dermatologe bzw. eine anonyme Dermatologin wissen, ob er oder sie einen Patienten behandeln muss, der eine rassistische Tätowierung trägt.

Ein Drittel der jungen Ärztinnen und Ärzte erwägt abzuwandern

07.05.2024 Klinik aktuell Nachrichten

Extreme Arbeitsverdichtung und kaum Supervision: Dr. Andrea Martini, Sprecherin des Bündnisses Junge Ärztinnen und Ärzte (BJÄ) über den Frust des ärztlichen Nachwuchses und die Vorteile des Rucksack-Modells.

Endlich: Zi zeigt, mit welchen PVS Praxen zufrieden sind

IT für Ärzte Nachrichten

Darauf haben viele Praxen gewartet: Das Zi hat eine Liste von Praxisverwaltungssystemen veröffentlicht, die von Nutzern positiv bewertet werden. Eine gute Grundlage für wechselwillige Ärztinnen und Psychotherapeuten.

Akuter Schwindel: Wann lohnt sich eine MRT?

28.04.2024 Schwindel Nachrichten

Akuter Schwindel stellt oft eine diagnostische Herausforderung dar. Wie nützlich dabei eine MRT ist, hat eine Studie aus Finnland untersucht. Immerhin einer von sechs Patienten wurde mit akutem ischämischem Schlaganfall diagnostiziert.

Update Radiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.