Past and current cause-specific mortality in Eisenmenger syndrome

Cristel M Sørensen Hjortshøj; Aleksander Kempny; Annette Schophuus Jensen; Keld Sørensen; Edit Nagy; Mikael Dellborg; Bengt Johansson; Virginija Rudiene; Gu Hong; Alexander R Opotowsky; Werner Budts; Barbara J Mulder; Lidia Tomkiewicz-Pajak; Michele D'Alto; Katja Prokšelj; Gerhard-Paul Diller; Konstantinos Dimopoulos; Mette-Elise Estensen; Henrik Holmstrøm; Maila Turanlahti; Ulf Thilén; Michael A Gatzoulis; Lars Søndergaard

doi:10.1093/eurheartj/ehx201

Past and current cause-specific mortality in Eisenmenger syndrome

Cristel M Sørensen Hjortshøj, Aleksander Kempny, Annette Schophuus Jensen, Keld Sørensen, Edit Nagy, Mikael Dellborg, Bengt Johansson, Virginija Rudiene, Gu Hong, Alexander R Opotowsky, Werner Budts, Barbara J Mulder, Lidia Tomkiewicz-Pajak, Michele D'Alto, Katja Prokšelj, Gerhard-Paul Diller, Konstantinos Dimopoulos, Mette-Elise Estensen, Henrik Holmstrøm, Maila TuranlahtiUlf Thilén, Michael A Gatzoulis, Lars Søndergaard

Department of Clinical Medicine

34 Citations (Scopus)

Abstract

Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

Original language	English
Journal	European Heart Journal
Volume	38
Issue number	26
Pages (from-to)	2060-2067
ISSN	0195-668X
DOIs	https://doi.org/10.1093/eurheartj/ehx201
Publication status	Published - 7 Jul 2017

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Hjortshøj, C. M. S., Kempny, A., Jensen, A. S., Sørensen, K., Nagy, E., Dellborg, M., Johansson, B., Rudiene, V., Hong, G., Opotowsky, A. R., Budts, W., Mulder, B. J., Tomkiewicz-Pajak, L., D'Alto, M., Prokšelj, K., Diller, G-P., Dimopoulos, K., Estensen, M-E., Holmstrøm, H., ... Søndergaard, L. (2017). Past and current cause-specific mortality in Eisenmenger syndrome. European Heart Journal, 38(26), 2060-2067. https://doi.org/10.1093/eurheartj/ehx201

Hjortshøj, CMS, Kempny, A, Jensen, AS, Sørensen, K, Nagy, E, Dellborg, M, Johansson, B, Rudiene, V, Hong, G, Opotowsky, AR, Budts, W, Mulder, BJ, Tomkiewicz-Pajak, L, D'Alto, M, Prokšelj, K, Diller, G-P, Dimopoulos, K, Estensen, M-E, Holmstrøm, H, Turanlahti, M, Thilén, U, Gatzoulis, MA & Søndergaard, L 2017, 'Past and current cause-specific mortality in Eisenmenger syndrome', European Heart Journal, vol. 38, no. 26, pp. 2060-2067. https://doi.org/10.1093/eurheartj/ehx201

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abstract = "Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.",

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doi = "10.1093/eurheartj/ehx201",

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T1 - Past and current cause-specific mortality in Eisenmenger syndrome

AU - Hjortshøj, Cristel M Sørensen

AU - Kempny, Aleksander

AU - Jensen, Annette Schophuus

AU - Sørensen, Keld

AU - Nagy, Edit

AU - Dellborg, Mikael

AU - Johansson, Bengt

AU - Rudiene, Virginija

AU - Hong, Gu

AU - Opotowsky, Alexander R

AU - Budts, Werner

AU - Mulder, Barbara J

AU - Tomkiewicz-Pajak, Lidia

AU - D'Alto, Michele

AU - Prokšelj, Katja

AU - Diller, Gerhard-Paul

AU - Dimopoulos, Konstantinos

AU - Estensen, Mette-Elise

AU - Holmstrøm, Henrik

AU - Turanlahti, Maila

AU - Thilén, Ulf

AU - Gatzoulis, Michael A

AU - Søndergaard, Lars

PY - 2017/7/7

Y1 - 2017/7/7

N2 - Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

AB - Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

U2 - 10.1093/eurheartj/ehx201

DO - 10.1093/eurheartj/ehx201

M3 - Journal article

C2 - 28430906

SN - 0195-668X

VL - 38

SP - 2060

EP - 2067

JO - European Heart Journal

JF - European Heart Journal

IS - 26

ER -

Past and current cause-specific mortality in Eisenmenger syndrome

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