https://doi.org/10.4081/monaldi.2024.2860
First-degree atrioventricular block in hypertrophic cardiomyopathy patients: an easy and worthy prognostic marker?
Submitted: December 1, 2023
Accepted: February 15, 2024
Published: March 6, 2024
Accepted: February 15, 2024
Abstract Views: 235
PDF_EARLY VIEW: 123
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. Recently, a connection has been observed between the presence of first-degree atrioventricular block (FDAVB) and cardiovascular outcomes, although the pathophysiology of this association remains poorly understood. Considering the period 2000-2023, we retrospectively included HCM patients at sinus rhythm at the first appointment and sought possible interactions of FDAVB (defined as PR interval >200 ms) with different clinical and imaging variables and with the occurrence of cardiovascular events, including atrial fibrillation (AF). A total of 97 patients were included, of whom 57 (58.8%) were men, with a mean age of 51±19 years, and 14 (14.4%) had FDAVB. During a median of 4.29 (P25 1.92, P75 7.67) years of follow-up, 35 cardiovascular events occurred, including 13 de novo diagnoses of AF, 8 hospitalizations due to heart failure, 8 new-onset strokes, 4 myocardial infarctions, and 2 implantations of cardio defibrillators in secondary prevention; no HCM-related death occurred. We did not find any association between outcomes and the presence of FDAVB. The role of FDAVB as a prognostic marker in HCM patients requires further investigation. We found that FDAVB patients were older, more frequently reported dyspnea, had a larger QRS duration, a higher E/e' ratio, and lower maximal left ventricle wall thickness by magnetic resonance (p<0.05). After multivariable analysis, FDAVB was independently associated with a higher echocardiographic E/e' ratio (p=0.039) (odds ratio=1.588). This is the first paper to document an independent association between FGAVB and a higher E/e' ratio in HCM patients.
Maron BJ, Desai MY, Nishimura RA, et al. Diagnosis and evaluation of hypertrophic cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol 2022;79:372-89.
DOI: https://doi.org/10.1016/j.jacc.2021.12.002
Medical Masterclass contributors, Firth J. Cardiology: hypertrophic cardiomyopathy. Clin Med (Lond) 2019;19:61-3.
DOI: https://doi.org/10.7861/clinmedicine.19-1-61
Popa-Fotea NM, Micheu MM, Bataila V, et al. Exploring the continuum of hypertrophic cardiomyopathy-from DNA to clinical expression. Medicina (Kaunas) 2019;55:299.
DOI: https://doi.org/10.3390/medicina55060299
Maron BJ, Desai MY, Nishimura RA, et al. Management of hypertrophic cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol 2022;79:390-414.
DOI: https://doi.org/10.1016/j.jacc.2021.11.021
Zampieri M, Berteotti M, Ferrantini C, et al. Pathophysiology and treatment of hypertrophic cardiomyopathy: new perspectives. Curr Heart Fail Rep 2021;18:169-79.
DOI: https://doi.org/10.1007/s11897-021-00523-0
Dragasis S, Vlachos K, Kariki O, et al. Atrial fibrillation in hypertrophic cardiomyopathy - a contemporary mini-review. Hellenic J Cardiol 2022;67:66-72.
DOI: https://doi.org/10.1016/j.hjc.2022.05.002
Alphonse P, Virk S, Collins J, et al. Prognostic impact of atrial fibrillation in hypertrophic cardiomyopathy: a systematic review. Clin Res Cardiol 2021;110:544-54.
DOI: https://doi.org/10.1007/s00392-020-01730-w
Arunachalam K, Maan A, Chu A. Atrial fibrillation in hypertrophic cardiomyopathy: evidence-based review about mechanism, complications and management. Crit Pathw Cardiol 2020;19:87-9.
DOI: https://doi.org/10.1097/HPC.0000000000000210
Masri A, Kanj M, Thamilarasan M, et al. Outcomes in hypertrophic cardiomyopathy patients with and without atrial fibrillation: a survival meta-analysis. Cardiovasc Diagn Ther 2017;7:36-44.
DOI: https://doi.org/10.21037/cdt.2016.11.23
Authors/Task Force members, Elliott PM, Anastasakis A, et al. 2014 ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy: the task force for the diagnosis and management of hypertrophic cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014;35:2733-79.
DOI: https://doi.org/10.1093/eurheartj/ehu284
Ommen SR, Mital S, Burke MA, et al. 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 2020;142:558-631.
DOI: https://doi.org/10.1161/CIR.0000000000000945
Cheng S, Keyes MJ, Larson MG, et al. Long-term outcomes in individuals with prolonged PR interval or first-degree atrioventricular block. JAMA 2009;301:2571-7.
DOI: https://doi.org/10.1001/jama.2009.888
Crisel RK, Farzaneh-Far R, Na B, Whooley MA. First-degree atrioventricular block is associated with heart failure and death in persons with stable coronary artery disease: data from the Heart and Soul Study. Eur Heart J 2011;32:1875-80.
DOI: https://doi.org/10.1093/eurheartj/ehr139
Schumacher K, Buttner P, Dagres N, et al. Association between PR interval prolongation and electro-anatomical substrate in patients with atrial fibrillation. PLoS One 2018;13:e0206933.
DOI: https://doi.org/10.1371/journal.pone.0206933
Chan YH, Hai JJ, Lau KK, et al. PR interval prolongation in coronary patients or risk equivalent: excess risk of ischemic stroke and vascular pathophysiological insights. BMC Cardiovasc Disord 2017;17:233.
DOI: https://doi.org/10.1186/s12872-017-0667-2
Liu M, Du Z, Sun Y. Prognostic significance of first-degree atrioventricular block in a large Asian population: a prospective cohort study. BMJ Open 2022;12:e062005.
DOI: https://doi.org/10.1136/bmjopen-2022-062005
Aro AL, Anttonen O, Kerola T, et al. Prognostic significance of prolonged PR interval in the general population. Eur Heart J 2014;35:123-9.
DOI: https://doi.org/10.1093/eurheartj/eht176
Kimura Y, Noda T, Matsuyama TA, et al. Heart failure in patients with arrhythmogenic right ventricular cardiomyopathy: what are the risk factors?. Int J Cardiol 2017;241:288-94.
DOI: https://doi.org/10.1016/j.ijcard.2017.04.061
Kimura Y, Fukuda K, Nakano M, et al. Prognostic significance of PR interval prolongation in adult patients with total correction of tetralogy of fallot. Circ Arrhythm Electrophysiol 2018;11:e006234.
DOI: https://doi.org/10.1161/CIRCEP.118.006234
Kwok CS, Rashid M, Beynon R, et al. Prolonged PR interval, first-degree heart block and adverse cardiovascular outcomes: a systematic review and meta-analysis. Heart 2016;102:672-80.
DOI: https://doi.org/10.1136/heartjnl-2015-308956
Nielsen JB, Pietersen A, Graff C, et al. Risk of atrial fibrillation as a function of the electrocardiographic PR interval: results from the Copenhagen ECG Study. Heart Rhythm 2013;10:1249-56.
DOI: https://doi.org/10.1016/j.hrthm.2013.04.012
Higuchi S, Minami Y, Shoda M, et al. Prognostic implication of first-degree atrioventricular block in patients with hypertrophic cardiomyopathy. J Am Heart Assoc 2020;9:e015064.
DOI: https://doi.org/10.1161/JAHA.119.015064
Nistri S, Olivotto I, Betocchi S, et al. Prognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian Registry for hypertrophic cardiomyopathy). Am J Cardiol 2006;98:960-5
DOI: https://doi.org/10.1016/j.amjcard.2006.05.013
O’Mahony C, Jichi F, Pavlou M, et al; Hypertrophic cardiomyopathy outcomes investigators. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J 2014;35:2010-20.
DOI: https://doi.org/10.1093/eurheartj/eht439
Minami Y, Haruki S, Yashiro B, et al. Enlarged left atrium and sudden death risk in hypertrophic cardiomyopathy patients with or without atrial fibrillation. J Cardiol 2016;68:478-84.
DOI: https://doi.org/10.1016/j.jjcc.2016.01.006
Holmqvist F, Thomas KL, Broderick S, et al. Clinical outcome as a function of the PR-interval-there is virtue in moderation: data from the Duke Databank for cardiovascular disease. Europace 2015;17:978-85.
DOI: https://doi.org/10.1093/europace/euu211
Hisamatsu T, Miura K, Fujiyoshi A, el at. NIPPON DATA80 research group. Long-term outcomes associated with prolonged PR interval in the general Japanese population. Int J Cardiol 2015;184:291-3.
DOI: https://doi.org/10.1016/j.ijcard.2015.02.028
Patel SI, Ackerman MJ, Shamoun FE, et al. QT prolongation and sudden cardiac death risk in hypertrophic cardiomyopathy. Acta Cardiol 2019;74:53-8.
DOI: https://doi.org/10.1080/00015385.2018.1440905
Delcrè SD, Di Donna P, Leuzzi S, et al. Relationship of ECG findings to phenotypic expression in patients with hypertrophic cardiomyopathy: a cardiac magnetic resonance study. Int J Cardiol 2013;167:1038-45.
DOI: https://doi.org/10.1016/j.ijcard.2012.03.074
Lopes LR, MS Rahman, and P.M. Elliott, A systematic review and meta-analysis of genotype-phenotype associations in patients with hypertrophic cardiomyopathy caused by sarcomeric protein mutations. Heart 2013;99:1800-11.
DOI: https://doi.org/10.1136/heartjnl-2013-303939
Gossios T, Savvatis K, Zegkos T, et al. Deciphering hypertrophic cardiomyopathy with electrocardiography. Heart Fail Rev 2022;27:1313-23.
DOI: https://doi.org/10.1007/s10741-021-10147-0
Mewton N, Liu CY, Croisille P, et al. Assessment of myocardial fibrosis with cardiovascular magnetic resonance. J Am Coll Cardiol 2011;57:891-903.
DOI: https://doi.org/10.1016/j.jacc.2010.11.013
Nagueh SF, Smiseth OA, Appleton CP, et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 2016;29:277-314.
DOI: https://doi.org/10.1016/j.echo.2016.01.011
Haland TF, Edvardsen T. The role of echocardiography in management of hypertrophic cardiomyopathy. J Echocardiogr 2020;18:77-85.
DOI: https://doi.org/10.1007/s12574-019-00454-9
Badran HM, Soltan G, Almeleigi R, et al. Prognostic significance of left ventricular end diastolic pressure using E/E' in patients with hypertrophic cardiomyopathy. Echocardiography 2019;36:2167-75.
DOI: https://doi.org/10.1111/echo.14539
Alis D, Guler A, Asmakutlu O, et al. The association between the extent of late gadolinium enhancement and diastolic dysfunction in hypertrophic cardiomyopathy. Indian J Radiol Imaging 2021;31:284-90.
DOI: https://doi.org/10.1055/s-0041-1734333
Laurent G, Eicher JC, Wolf JE. First-degree atrioventricular block and pseudopacemaker syndrome. Arch Cardiovasc Dis 2013;106:690-3.
DOI: https://doi.org/10.1016/j.acvd.2012.02.014
Bonow RO, Frederick TM, Bacharach SL, et al. Atrial systole and left ventricular filling in hypertrophic cardiomyopathy: effect of verapamil. Am J Cardiol 1983;51:1386-91.
DOI: https://doi.org/10.1016/0002-9149(83)90317-X
Sunderji I, Singh V, Fraser AG. When does the E/e' index not work? The pitfalls of oversimplifying diastolic function. Echocardiography 2020;37:1897-907.
DOI: https://doi.org/10.1111/echo.14697
Ethics Approval
The research protocol was approved by the Hospital do São João Ethics Committee (reference number: CE-275-22) and patients’ consent was acquired. The study was performed according to the recommendations of the Helsinki Declaration.How to Cite
Amador, Ana Filipa, Catarina Martins da Costa, João da Silva Santos, Cláudia Camila Dias, and Elisabete Martins. 2024. “First-Degree Atrioventricular Block in Hypertrophic Cardiomyopathy Patients: An Easy and Worthy Prognostic Marker?”. Monaldi Archives for Chest Disease, March. https://doi.org/10.4081/monaldi.2024.2860.
Copyright (c) 2024 The Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
