Complex cardiomyopathy with combined left ventricle outflow tract obstruction and calcified stiff left atrium: the pathway to heart-lung transplantation
Keywords:
hypertrophic cardiomyopathy, subaortic fibromuscular membrane, stiff left atrial syndrome, left atrial calcification, atrial fibrillation, transcatheter modification, pulmonary hypertension, heart-lung transplantationSynopsis
Reviewers:
Todurov Borys Mychailovych, Corresponding Member of the Academy of Medical Sciences of Ukraine, Doctor of Medical Sciences, Professor, Honored Doctor of Ukraine; Heart Institute of the Ministry of Health of Ukraine, Kyiv, Ukraine
Nesukay Olena Gennadiivna, MD, Doctor of Medical Sciences, Professor; National Scientific Center «M.D. Strazhesko Institute of Cardiology, Clinical and Regenerative Medicine» of NAMS of Ukraine, Kyiv, Ukraine
Vasilyeva L.I., Kalashnykova O.S.
Complex cardiomyopathy with combined left ventricle outflow tract obstruction and calcified stiff left atrium: the pathway to heart-lung transplantation: Monograph /
L. Vasilyeva, O. Kalashnykova. – Karlsruhe: ScientificWorld-NetAkhatAV,
2026. – 133 p.
Від складної кардіоміопатії з поєднаною обструкцією виносного тракту лівого шлуночна та кальцинованого жорсткого лівого передсердя – до трансплантації ‘серце-легені’: монографія / Л. Васильєва, О. Калашникова - Карлсруе, 2026. – 133 с.
The monograph presents the clinical course of a female patient with an exceptionally complex form of cardiomyopathy—hypertrophic cardiomyopathy (HCM) accompanied by a subaortic muscular membrane and a mixed (fixed and dynamic) obstruction of the left ventricular outflow tract (LVOT).
Her medical history includes multiple intricate interventions for atrial fibrillation and flutter, which ultimately resulted in the development of stiff left atrial (Stiff LA) syndrome, characterized by irreversible calcification of the left atrial wall. The clinical condition is further complicated by severe, likely mixed-type pulmonary hypertension, associated with aneurysmal dilatation of the pulmonary artery.
Despite a preserved left ventricular ejection fraction and maintained sinus rhythm, subtotal calcification of the left atrium with morphologically driven restriction of venous outflow excludes the option of isolated lung transplantation. The extent of structural damage involving both the heart and pulmonary vasculature supports combined heart–lung transplantation as the only viable therapeutic approach, in accordance with current international guidelines (ISHLT, ESC/ERS, WSPH). However, such procedures currently exceed the available expertise and capacity within the Ukrainian healthcare system.
This monograph is multidisciplinary in scope and offers practical value for cardiologists, cardiac surgeons, pulmonologists, and transplant specialists.
ISBN 978-3-98924-123-7
DOI: 10.30890/978-3-98924-123-7.2026
Chapters
-
PART 1. STRUCTURED CLINICAL ANALYSIS WITH CONCLUSIONS ON MANAGEMENT STRATEGY
-
PART 2. ANALYTICAL REVIEW OF EVIDENCE, STRATEGIES, AND EDUCATIONAL ASPECTS
-
PART 3. ILLUSTRATIONS FOR THE CLINICAL CASE
-
PART 4. QUESTIONNAIRES FOR PARTICIPANTS IN THE EDUCATIONAL PROCESS
References
Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503 3626. doi:10.1093/eurheartj/ehad194.
Humbert M., Kovacs G., Hoeper MM., et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618 3731. doi:10.1093/eurheartj/ehac237.
Chin KM, Gaine SP, Gerges C., et al. Treatment algorithm for pulmonary arterial hypertension: Proceedings of the 7th World Symposium on Pulmonary Hypertension (WSPH) 2024. Eur Respir J. 2024;64(4):2401325. doi:10.1183/13993003.01222 2024.
Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart & Lung Transplantation. J Heart Lung Transplant. 2021;40(11):1349 1379. doi:10.1016/j.healun.2021.07.005.
Kurt M, Wang J, Torre-Amione G et al. Left atrium functions in diastolic heart failure. Circ Cardiovascular Imaging. 2009;2:10–15.
Bytyci I, D`Agostini A, Bajraktari G et al. Left atrial stiffness predicts cardiac events in patients with heart failure and reduced ejection fraction: The impact of diabetes. Clin Physiol Funct Imaging. 2021 Mar;41(2):208-216.doi: 10.1111/cpf.12688.
Nyberg et al. Reference Ranges for Strain From the HUNT4Echo Study. JACC: Cardiovascular imaging. 2023;16(12):1516-1531.
Ommen S.R., Ho C.Y., Asif I.M., et al. 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy. J Am Coll Cardiol. 2024;83(23):2324 2405. doi:10.1016/j.jacc.2024.02.014.
Lee JH, Kwon OS, Yu HT, et al. Risk Factors for Stiff Left Atrial Physiology 1 Year After Atrial Fibrillation Catheter Ablation. Front Physiol. 2021.Sep20;12:740600. doi:10.3389/fphys.2021.740600
Gibson D. N., Di Biase L., Mohanty P., et al.Stiff left atrial syndrome after catheter ablation for atrial fibrillation: clinical characterization, prevalence, and predictors. Heart Rhythm 2011.8:1364–1371. doi:10.1016/j.hrthm.2011.02.026
Zei PC. Left Atrial Calcification After Catheter Ablation for Atrial Fibrillation. JACC Clin Electrophysiol. 2023.9;7:1118-1120 doi:10.1016/j.jacep.2023.01.003
Banthiya S., Chowdhury M., Govil D., et al. Exploring the causes of stiff left atrial syndrome: a case series. Eur Heart J Case Rep. 2025.9,ytae702. doi:10.1093/ehjcr/ytae702
Chamberlin J., Smith C.D., Gowani Z., et al. Left atrial calcification on chest CT: atrial ablation replaces rheumatic heart disease as the most identified etiology. Pol J Radiol. 2023.88:e423-e429. doi:10.5114/pjr.2023.131214
Hindricks G., Potpara T., Dagres N., et al. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio Thoracic Surgery (EACTS). Eur Heart J.2021;42(5):373–498.
Van Gelder IC., Rienstra M., Karina V. Buntin KV., et al. 2024 ESC Guidelines for the management of atrial fibrillation developed in collaboration with the European Association for Cardio Thoracic Surgery (EACTS). Eur Heart J.2024;45(36):3314 3414. doi:10.1093/eurheartj/ehae176
Yakabe D., Ohtani K., Fukuyama Y., et al. Prognostic Value of Left Atrial Calcification After Catheter Ablation for Atrial Fibrillation. JACC: Clinical Electrophysiology 2023;9(6):671-681. doi:10.1016/j.jacep.2022.11.016.
Hayashi Y., Shimeno K., Matsumoto N., et al. A case of severely progressive left atrial calcification triggered by inflammation due to radiofrequency catheter ablation. J Cardiol Cases. 2024 Jun; 30(3): 87–89. doi: 10.1016/j.jccase.2024.05.009.
Kitai T., Kasai Y., Kasai J., et al. Iatrogenic left atrial calcification after catheter ablation for atrial fibrillation confirmed by histological assessment. Eur Heart J Case Rep. 2023 Aug 17;7(8):ytad407. doi:10.1093/ehjcr/ytad407.
2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy. Circulation. 2024;150(8):e198 doi: 10.1161/CIR.0000000000001277.
Olivotto I, Oreziak A, Barriales Villa R, et al. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER HCM): a randomised, double blind, placebo controlled phase 3 trial. Lancet. 2020;396(10253):759 769. doi: 10.1016/S0140 6736(20)31792 X.
JAMA Cardiology. Effects of Mavacamten on Measures of Cardiopulmonary Exercise Capacity in the EXPLORER HCM Trial. JAMA Cardiol. 2023;8(3):240 247. doi: 10.1001/jamacardio.2022.5099
Veselka J., Tesar D., Topalo R., et al. Follow Up of Up to 25 Years After Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy. J Am Coll Cardiol Cardiovasc Interv. 2023;16(12):1556 157. doi:10.1016/j.jcin.2023.03.049.
Ferrazzi P., Spirito P., Iacovoni A., et al. Transaortic Chordal Cutting: Mitral Valve Repair for Obstructive Hypertrophic Cardiomyopathy With Mild Septal Hypertrophy. J Am Coll Cardiol. 2015;66(15):1687 1696. doi: 10.1016/j.jacc.2015.07.069
Binaco I., Spirito P., Poggio D., Casati V., Grillo M., Ferrazzi P. Transaortic mitral valve secondary chordal cutting in patients with obstructive hypertrophic cardiomyopathy and mild septal hypertrophy. Ann Cardiothorac Surg. 2017;6(4):426 428. doi:10.21037/acs.2017.04.05.
Dorobantu LF, Utzschneider M, Ticulescu R, et al. Transaortic shallow septal myectomy and cutting of secondary fibrotic mitral valve chordae – a 5 year single center experience. J Clin Med. 2022;11(11):3083. doi:10.3390/jcm11113083.
Schaff HV, Ommen SR. Contemporary surgical management of hypertrophic cardiomyopathy. Ann Thorac Surg. 2024;117(3):561 571. doi:10.1016/j.athoracsur.2023.01134 7.
Hindricks G, Potpara T, Dagres N., et al. Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology (ESC) developed in collaboration with the European Association for Cardio Thoracic Surgery (EACTS) and the European Heart Rhythm Association (EHRA) of the ESC. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio Thoracic Surgery (EACTS). Eur Heart J. 2020;42(5):373 498. doi:10.1093/eurheartj/ehaa612.
Joglar JA, Chung MK, Armbruster AL, et al. 2023 ACC/AHA/ACCP/HRS Guideline for the Diagnosis and Management of Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2023;149(5):e109-e279. doi:10.1161/CIR.0000000000001193.
McDonagh T., Metra M., Adamo M., et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2021;42(36):3599 3726. doi:10.1093/eurheartj/ehab368.
Fang J., Liu Y., Zhu Y., et al. First in Human Transapical Beating Heart Septal Myectomy in Patients With Hypertrophic Obstructive Cardiomyopathy. J Am Coll Cardiol. 2023;82(7):575 586. doi:10.1016/j.jacc.2023.05.052.
Liu L, Li J. Beating Heart Septal Myectomy: An Innovative Approach for Obstructive Hypertrophic Cardiomyopathy. J Am Coll Cardiol: Asia. 2024; (Epub) doi:10.1016/j.jacasi.2023.11.013.
Kronenberger R, Tanaka K, de Asmundis C, Meir M L. Stiff left atrial syndrome with pulmonary veins occlusion after percutaneous radiofrequency ablation. J Cardiothorac Surg. 2023;18(1):193. doi:10.1186/s13019 023 02193 6.
Offen S., Sivasubramaniam V., Granger E., Macdonald P. Stiff left atrial syndrome and heart transplantation. J Heart Lung Transplant. 2019 Jun;38(6):675 676. doi:10.1016/j.healun.2018.12.017.
Huddleston CB. Commentary: Heart–lung transplantation—is it worth it? J Thorac Cardiovasc Surg. 2024;168(4):595-596. doi:10.1016/j.jtcvs.2024.00 181
