Past and current cause-specific mortality in Eisenmenger syndrome

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt

Standard

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

I: European Heart Journal, Bind 38, Nr. 26, 2017, s. 2060-2067.

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt

Harvard

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, bind 38, nr. 26, s. 2060-2067. https://doi.org/10.1093/eurheartj/ehx201

APA

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

Vancouver

Hjortshøj CMS, Kempny A, Jensen AS, Sørensen K, Nagy E, Dellborg M o.a. Past and current cause-specific mortality in Eisenmenger syndrome. European Heart Journal. 2017;38(26):2060-2067. https://doi.org/10.1093/eurheartj/ehx201

Author

Hjortshøj, Cristel M Sørensen ; Kempny, Aleksander ; Jensen, Annette Schophuus ; Sørensen, Keld ; Nagy, Edit ; Dellborg, Mikael ; Johansson, Bengt ; Rudiene, Virginija ; Hong, Gu ; Opotowsky, Alexander R ; Budts, Werner ; Mulder, Barbara J ; Tomkiewicz-Pajak, Lidia ; D'Alto, Michele ; Prokšelj, Katja ; Diller, Gerhard-Paul ; Dimopoulos, Konstantinos ; Estensen, Mette-Elise ; Holmstrøm, Henrik ; Turanlahti, Maila ; Thilén, Ulf ; Gatzoulis, Michael A ; Søndergaard, Lars. / Past and current cause-specific mortality in Eisenmenger syndrome. I: European Heart Journal. 2017 ; Bind 38, Nr. 26. s. 2060-2067.

Bibtex

@article{379fd60b692a45a28c5fcb84cab796c6,

title = "Past and current cause-specific mortality in Eisenmenger syndrome",

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.",

author = "Hjortsh{\o}j, {Cristel M S{\o}rensen} and Aleksander Kempny and Jensen, {Annette Schophuus} and Keld S{\o}rensen and Edit Nagy and Mikael Dellborg and Bengt Johansson and Virginija Rudiene and Gu Hong and Opotowsky, {Alexander R} and Werner Budts and Mulder, {Barbara J} and Lidia Tomkiewicz-Pajak and Michele D'Alto and Katja Prok{\v s}elj and Gerhard-Paul Diller and Konstantinos Dimopoulos and Mette-Elise Estensen and Henrik Holmstr{\o}m and Maila Turanlahti and Ulf Thil{\'e}n and Gatzoulis, {Michael A} and Lars S{\o}ndergaard",

year = "2017",

doi = "10.1093/eurheartj/ehx201",

language = "English",

volume = "38",

pages = "2060--2067",

journal = "European Heart Journal",

issn = "0195-668X",

publisher = "Oxford University Press",

number = "26",

}

RIS

TY - JOUR

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

Y1 - 2017

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

VL - 38

SP - 2060

EP - 2067

JO - European Heart Journal

JF - European Heart Journal

SN - 0195-668X

IS - 26

ER -

ID: 193319788