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3 results on '"Susanne J. Maurer"'

1. Congenitally Corrected Transposition of the Great Arteries in Adults—A Contemporary Single Center Experience

Author

Josef Auer, Claudia Pujol, Susanne J. Maurer, Nicole Nagdyman, Peter Ewert, and Oktay Tutarel

Subjects
adult congenital heart disease, transposition of the great arteries, mortality, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect (CHD). Contemporary data regarding its outcome in adults are scarce. Methods: Retrospective, single-center study of all ccTGA patients over the age of 16 years treated at our center during the time period 2006–2018. Only patients with a biventricular circulation were included. The primary endpoint was all-cause mortality. Results: Altogether, 96 patients (mean age 32.8 ± 16.0 years, female 50%) with ccTGA and a systemic right ventricle (SRV) were included in the study. An additional CHD was present in 81 patients (84.4%); most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction. Out of the whole cohort, 45 (46.9%) had already undergone cardiac surgery at baseline. During a median follow-up of 6.5 (IQR 2.8–12.7) years, the primary endpoint occurred in 10 patients (10.8%). Cause of death was cardiac in nine patients and suicide in one. Hospitalizations due to heart failure occurred in 48 patients (51.6%). Upon univariate Cox analysis, an NYHA class ≥III, severe tricuspid regurgitation, severe SRV systolic impairment, as well as a reduced left ventricular systolic function were predictors of the primary endpoint. Upon multivariable analysis, only NYHA class ≥ III (HR: 18.66, CI 95%: 3.01–115.80, p = 0.0017) and a reduced left ventricular systolic function (HR: 7.36, CI 95%: 1.18–45.99, p = 0.038) remained as independent predictors. Conclusions: Adults with ccTGA and an SRV are burdened with significant morbidity and mortality. Predictors for mortality are NYHA class and subpulmonary left ventricular function.

Published
2021
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2. Patients with Single-Ventricle Physiology over the Age of 40 Years

Author

Peter Ewert, Oktay Tutarel, Sandra Schiele, Alfred Hager, Julia Hock, Celina Fritz, Susanne J. Maurer, and Claudia Pujol

Subjects
medicine.medical_specialty, Cirrhosis, Multivariate analysis, lcsh:Medicine, Disease, 030204 cardiovascular system & hematology, Article, 03 medical and health sciences, 0302 clinical medicine, renal disease, Interquartile range, Internal medicine, single-ventricle physiology, adult congenital heart disease, Medicine, 030212 general & internal medicine, Univariate analysis, business.industry, Hazard ratio, lcsh:R, General Medicine, medicine.disease, mortality, Confidence interval, Cardiac surgery, ddc, business, Fontan
Abstract

Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce. Methods: In this study, SVP patients under active follow-up at our center who were &ge, 40 years of age at any point between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history were retrieved from hospital records. The primary end-point was all-cause mortality. Results: Altogether, 49 patients (19 female (38.8%), mean age 49.2 &plusmn, 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8&ndash, 8.5). Of these patients, 40 (81.6%) had undergone at least one cardiac surgery. The most common extracardiac comorbidities were thyroid dysfunction (n = 27, 55.1%) and renal disease (n = 15, 30.6%). During follow-up, 10 patients (20.4%) died. On univariate analysis, renal disease and liver cirrhosis were predictors of all-cause mortality. On multivariate analysis, only renal disease (hazard ratio (HR): 12.5, 95% confidence interval (CI): 1.5&ndash, 106.3, p = 0.021) remained as an independent predictor. Conclusions: SVP patients &ge, 40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality.

Published
2019

3. Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years

Author

Claudia Pujol, Jürgen Hörer, Oktay Tutarel, Katharina Stöckemann, Peter Ewert, and Susanne J. Maurer

Subjects
Down syndrome, medicine.medical_specialty, Multivariate analysis, Heart disease, lcsh:Medicine, 030204 cardiovascular system & hematology, Article, Hospital records, Lesion, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Internal medicine, pulmonary hypertension, medicine, adult congenital heart disease, 030212 general & internal medicine, Univariate analysis, Creatinine, business.industry, lcsh:R, General Medicine, medicine.disease, Pulmonary hypertension, ddc, chemistry, outcome, medicine.symptom, business
Abstract

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients &ge, 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality. Results: In total, 65 patients (67.7% female, mean age 45.19 &plusmn, 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2&ndash, 7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2&ndash, 14.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2&ndash, 118.7, p = 0.006) remained as independent predictors of all-cause mortality. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.

Published
2019

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