A5005750229- Congenital Heart Disease Studies
- Cardiovascular Function and Risk Factors
- Congenital Diaphragmatic Hernia Studies
- Cardiovascular Issues in Pregnancy
- Cardiac Valve Diseases and Treatments
- Cardiac Arrhythmias and Treatments
- Tracheal and airway disorders
- Pulmonary Hypertension Research and Treatments
- Hemodynamic Monitoring and Therapy
- Cardiac Structural Anomalies and Repair
- Vascular anomalies and interventions
- Aortic Disease and Treatment Approaches
- Congenital Anomalies and Fetal Surgery
- Cardiovascular and Diving-Related Complications
- Coronary Artery Anomalies
- Advanced MRI Techniques and Applications
- Cardiovascular Conditions and Treatments
- Prenatal Screening and Diagnostics
- Mechanical Circulatory Support Devices
- Cardiac Imaging and Diagnostics
- Fetal and Pediatric Neurological Disorders
- Cardiac pacing and defibrillation studies
- Congenital heart defects research
- Neonatal Respiratory Health Research
- Cardiac electrophysiology and arrhythmias
Universitätsklinikum Aachen
2023-2025
University Hospital Bonn
2015-2024
RWTH Aachen University
2023-2024
Philips (Germany)
2023
University of Bonn
2013-2022
University of California, San Francisco
2022
University of Cologne
2021
Zentrum für Kinderheilkunde
2006-2020
Universitätskinderklinik
2006-2020
Baylor College of Medicine
2019
Background: The ratios of tricuspid annular plane systolic excursion (TAPSE)/echocardiographically measured pulmonary artery pressure (PASP), fractional area change/invasively mean pressure, right ventricular (RV) change/end-systolic area, TAPSE/pulmonary acceleration time, and stroke volume/end-systolic have been proposed as surrogates RV-arterial coupling. relationship these with the gold standard measure coupling (invasive pressure-volume loop-derived end-systolic/arterial elastance...
<h3>Background</h3> Next generation sequencing has become the core technology for gene discovery in rare inherited disorders. However, interpretation of numerous sequence variants identified remains challenging. We assessed application exome diagnostics complex I deficiency, a disease with vast genetic heterogeneity. <h3>Methods</h3> Ten unrelated individuals deficiency were selected and sequential bioinformatic filtering. Cellular rescue experiments performed to verify pathogenicity novel...
Fetal aortic valvuloplasty (FV) aims to prevent fetal valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history disease life remains poorly defined. The primary aim this study was describe stenosis, and a secondary test previously published criteria designed identify cases emerging HLHS with potential for biventricular (BV) outcome after FV.From...
Mitochondrial acyl-CoA dehydrogenase family member 9 (ACAD9) is essential for the assembly of mitochondrial respiratory chain complex I. Disease causing biallelic variants in ACAD9 have been reported individuals presenting with lactic acidosis and cardiomyopathy. We describe genetic, clinical biochemical findings a cohort 70 patients, whom 29 previously unpublished. found 34 known 18 unreported ACAD9. No patients harbored loss function mutations, indicating that this combination unlikely to...
The EACTS/AEPC Guidelines represent the views of EACTS and AEPC were produced after careful consideration scientific medical knowledge evidence available at time their dating.The are not responsible in event any contradiction, discrepancy and/or ambiguity between other official recommendations or guidelines issued by relevant public health authorities, particular relation to good use healthcare therapeutic strategies.Health professionals encouraged take fully into account when exercising...
To apply Doppler US (DUS)-gated fetal cardiac cine MRI in clinical routine and investigate diagnostic performance complex congenital heart disease (CHD) compared with that of echocardiography.In this prospective study (May 2021 to March 2022), women fetuses CHD underwent echocardiography DUS-gated on the same day. For MRI, balanced steady-state free precession images were acquired axial optional sagittal and/or coronal orientations. Overall image quality was assessed a four-point Likert...
To assess long term changes in cardiac morphology and function survivors of severe twin to transfusion syndrome (TTTS) after intrauterine laser coagulation placental anastomoses.Prospective follow up fetuses with TTTS treated by anastomoses. Fetal echocardiography Doppler studies feto-placental haemodynamic were performed at the time (median gestational age 21.7 weeks). Postnatal included a detailed echocardiographic study systolic diastolic median 21.1 months.Paediatric cardiology unit.89...
Abstract Objective To evaluate the prenatal distribution, associated conditions and outcome of different types right aortic arch (RAA) detected in fetal life. Methods This was a retrospective review all cases RAA prenatally between 1998 2005 two tertiary referral centers. Results In study period 71 were detected; 26 (37%) had with aberrant left subclavian artery, 23 (32%) mirror‐image branching, 20 (28%) unknown type (3%) double arch. While 20/26 artery isolated findings, branching cardiac...
Fetal aortic stenosis may progress to hypoplastic left heart syndrome. valvuloplasty (FV) has been proposed improve hemodynamics and maintain biventricular (BV) circulation. The aim of this study was assess FV efficacy by comparing survival postnatal circulation between fetuses that underwent those did not.This a retrospective multicenter with 2005 2012, compared contemporaneously enrolled natural history (NH) cases sharing similar characteristics at presentation but not undergoing FV. Main...
We sought to assess the feasibility of constructing right ventricular (RV) pressure-volume (PV) loops solely by echocardiography.We performed RV conductance and pressure wire (PW) catheterization with simultaneous echocardiography in 35 patients pulmonary hypertension. To generate echocardiographic PV loops, a reference curve was constructed using pooled PW data from first 20 (initial cohort). Individual curves were then generated adjusting according isovolumic ejection phase duration...
ABSTRACT Objective To assess the incidence and impact of extracardiac anomalies on prognosis fetuses with heterotaxy syndrome. Methods All diagnosed syndrome by three experienced examiners over a period 14 years (1999–2013) were reviewed retrospectively. Results In total, 165 in study period. One hundred fifty (90.9%) had cardiac defects; that did not involve spleen present 26/165 (15.8%) cases. Of total cohort, termination pregnancy was performed 49 (29.7%) cases, intrauterine death...
Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially isolated SS who asymptomatic and might be diagnosed accidentally. We queried a large multi-institutional registry of to evaluate the natural history this condition determine efficacy surgical treatment terms survival clinical status. collected data on 485 from 51 institutions; 279 (57%) were treated surgically (STPs) 206 (43%) clinically monitored (CMPs). Median age at last follow-up was 11.6 years...
In primary hyperoxaluria Type 1 (PH1), endogenous oxalate overproduction significantly elevates urinary excretion, resulting in recurrent urolithiasis and/or progressive nephrocalcinosis and often early end-stage renal disease (ESRD). ESRD, dialysis cannot sufficiently remove oxalate; plasma (Pox) increases markedly, inducing systemic deposition (oxalosis) death. Interventions to reduce Pox PH1 subjects with ESRD could have significant clinical impact. This ongoing Phase II, open-label trial...
Abstract Objectives We aimed to evaluate the effect of technical aspects fetal aortic valvuloplasty (FAV) on procedural risks and pregnancy outcomes. Background FAV is performed in cases severe mid‐gestation stenosis with goal preventing hypoplastic left heart syndrome (HLHS). Methods The International Fetal Cardiac Intervention Registry was queried for fetuses who underwent from 2002 2018, excluding one high‐volume center. Results 108 had an attempted cardiac puncture (mean gestational age...
<b><i>Introduction:</i></b> Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical stenosis (PS) has been performed small single-institution series reporting technical physiological success. We present the first multicenter experience. <b><i>Objectives:</i></b> Describe maternal characteristics of those being evaluated FCI, including pregnancy/neonatal outcome data using International...
In medicine, reference curves serve as an important tool for everyday clinical practice. Pediatricians assess the growth process of children with help percentile curves. The statistical methods construction these are complex and their application often requires programming skills. An easy-to-use approach physicians life scientists is missing. aim this project was to develop RefCurv, intuitive user-friendly software clinicians that simplifies medical parameters.
Frauen in der Kardiologie und Kinderkardiologie sind besonderen gesundheitlichen Risiken ausgesetzt, die eine bedenkenlose Weiterbeschäftigung während Schwangerschaft Stillzeit infrage stellen mitunter zu betrieblichen Beschäftigungsverboten führen können. Schwangere, stillende jüngst entbundene unterliegen einem gesetzlichen Schutz, um sie (und ihre Kinder) vor unverantwortbarer Gefährdung beruflicher Benachteiligung bewahren. In diesem Konsensuspapier Deutschen Gesellschaft für...
Abstract Objective To evaluate the intrauterine course and outcome of tricuspid atresia detected in fetus. Methods This was a retrospective review all confirmed cases prenatally between 1998 2006 three tertiary referral centers Germany. Results Fifty‐four were during study period postnatally: 28 (51.9%) had concordant ventriculoarterial connection which 14 also pulmonary outflow obstruction; 25 (46.3%) discordant aortic obstruction, six tract obstruction two other associated intracardiac...