Aleksander Kempny1, Cristel Sørensen Hjortshøj2, Hong Gu2, Wei Li2, Alexander R Opotowsky2, Michael J Landzberg2, Annette Schophuus Jensen2, Lars Søndergaard2, Mette-Elise Estensen2, Ulf Thilén2, Werner Budts2, Barbara J Mulder2, Ilja Blok2, Lidia Tomkiewicz-Pająk2, Kamil Szostek2, Michele D'Alto2, Giancarlo Scognamiglio2, Katja Prokšelj2, Gerhard-Paul Diller2, Konstantinos Dimopoulos2, Stephen J Wort2, Michael A Gatzoulis2. 1. From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.). kempny@gmail.com. 2. From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
Abstract
BACKGROUND: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. METHODS: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. RESULTS: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. CONCLUSIONS: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
BACKGROUND:Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndromepatients. METHODS: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. RESULTS: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. CONCLUSIONS: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
Authors: Christian Opitz; Ekkehard Grünig; Stephan Rosenkranz; Ann-Sophie Kaemmerer; Matthias Gorenflo; Dörte Huscher; David Pittrow; Peter Ewert; Christine Pausch; Marion Delcroix; Hossein A Ghofrani; Marius M Hoeper; Rainer Kozlik-Feldmann; Andris Skride; Gerd Stähler; Carmine Dario Vizza; Elena Jureviciene; Dovile Jancauskaite; Lina Gumbiene; Ralf Ewert; Ingo Dähnert; Matthias Held; Michael Halank; Dirk Skowasch; Hans Klose; Heinrike Wilkens; Katrin Milger; Christian Jux; Martin Koestenberger; Laura Scelsi; Eva Brunnemer; Michael Hofbeck; Silvia Ulrich; Anton Vonk Noordegraaf; Tobias J Lange; Leonhard Bruch; Stavros Konstantinides; Martin Claussen; Judith Löffler-Ragg; Hubert Wirtz; Christian Apitz; Rhoia Neidenbach; Sebastian Freilinger; Attila Nemes Journal: Cardiovasc Diagn Ther Date: 2021-12
Authors: Anca Chiriac; David C Riley; Matthew Russell; Jeremy P Moore; Deepak Padmanabhan; David O Hodge; Matthew R Spiegel; Emily R Vargas; Sabrina D Phillips; Naser M Ammash; Malini Madhavan; Samuel J Asirvatham; Christopher J McLeod Journal: J Am Heart Assoc Date: 2020-03-15 Impact factor: 5.501