Showing papers by "Stefan Martinoff published in 2020"

Congenital Lymphatic Malformation and Aortic Aneurysm in a Patient with TSC2 Mutation.

Abstract: We present a 7-year-old boy with tuberous sclerosis and congenital segmental lymphedema (CSL) of the left leg, as well as two aortic aneurysms. He was treated with everolimus (EVE) since the age of 14 months. His CSL regressed under treatment with EVE. His first aneurysms required operative intervention at age of 17 months. Four months afterward a new aortic aneurysm had been detected above the Dracon graft, but this one remained stable since that time. The patient didn't experience severe side effects. EVE has been well tolerated without disturbance of somatic growth or serious adverse effect.

Different CMR Imaging Modalities for Native and Patch-Repaired Right Ventricular Outflow Tract Sizing: Impact on Percutaneous Pulmonary Valve Replacement Planning

Abstract: Percutaneous pulmonary valve replacement (PPVI) in native or patched right ventricular outflow tract (RVOT) has proven to be feasible. The procedure is highly dependent on the size of the RVOT. Several methods exist to evaluate the size of the RVOT by cardiovascular magnetic resonance (CMR). We evaluated different CMR modalities for measuring RVOT diameters. Thirty-one consecutive patients with native or patched RVOT were retrospectively evaluated. CMR was part of follow-up of patients with corrected Tetralogy of Fallot or pulmonary stenosis with significant pulmonary regurgitation (PR). CMR included 3D-SSFP whole-heart in systole, diastole, and contrast-enhanced MR angiography (ceMRA). Diameters of the RVOT were assessed by the three sequences. Additionally, in patients who underwent cardiac catheterization (n = 11) for PPVI, vessel diameters assessed by cine-angiography were compared to CMR. Systolic diameters of RVOT were significantly larger compared to the diameters taken in diastole and ceMRA (median difference 5.0 mm and 3.8 mm). Diastolic and ceMRA diameters did not differ significantly. CMR diameters taken in systole showed no statistical difference to systolic diameters taken by cine-angiography, while diastolic and ceMRA diameters were significantly smaller. PPVI was feasible to a maximal CMR diameter of 31 mm measured by SSFP whole-heart sequence in systole. Absolute diameters of native RVOT differ depending on the CMR sequence and timing of acquisition (systolic vs diastolic gating). Diameters taken during heart catheterization by cine-angiography best correlate to systolic CMR values. Data may help to select RVOTs suitable for PPVI.

Magnetic Resonance Imaging Risk Factors for Ventricular Arrhythmias in Tetralogy of Fallot.

Abstract: Sudden cardiac death (SCD) is the most common cause of late mortality in tetralogy of Fallot (TOF). Pulmonary regurgitation (PR) was previously found to be the most common hemodynamic abnormality associated with ventricular arrhythmias (VA), but cardiovascular magnetic resonance (CMR)-based studies did not show this association. The aim of this study is to investigate the risk factors for VA in TOF using CMR. Electronic records of TOF patients and their CMR studies between July 2006 and October 2018 in one center were retrospectively reviewed. Demographic, clinical and CMR data of patients were collected. Outcome was defined as sustained ventricular tachycardia (VT), aborted SCD and SCD. From a total of 434 TOF patients with complete CMR studies, 19 (4.4%) patients developed a positive outcome (12 sustained VT, 4 aborted SCD, 3 SCD) at a median age of 24 years. The number of surgical interventions was significantly greater in patients who developed VA. Right ventricular volumes were significantly larger in patients who suffered a positive outcome. Odds ratio for developing VA was 6.905 for RVEDVI ≥ 160 ml/m2 and 6.141 for RVESVI ≥ 80 ml/m2 (P = 0.0014 and 0.0012, respectively). Event-free survival was longer in patients with smaller right ventricular volumes. In conclusion, right ventricular dimensions are the most significant factors associated with the development of VA in TOF. The number of surgical interventions is also related to an increased risk.

Intraindividual validation of 4D flow measurement against 2D flow measurements in complex flow patterns in routine clinical cardiovascular magnetic resonance of bicuspid aortic valve

Abstract: Comparing four-dimensional flow against two-dimensional flow measurements in patients with complex flow pattern is still lacking. This study aimed to compare four-dimensional against the two-dimensional flow measurement in patients with bicuspid aortic valve and to test potentials of four-dimensional operator-dependent sources of error. The two- and four-dimensional flow data sets of sixteen patients with bicuspid aortic valve and eighteen healthy subjects were studied. Flow analyses were performed by two observers blindly. Patients with bicuspid aortic valve mean differences between the two- and four-dimensional measurements in both observers were − 8 and − 4 ml, respectively. Four-dimensional measurements resulted in systematically higher flow values than the two-dimensional flow in bicuspid aortic valve patients. The upper and lower limits of agreement between the two- and four-dimensional measurements by both observers were + 12/− 28 ml and + 14/− 21 ml, respectively. In the healthy volunteers, mean differences between the two- and four-dimensional measurements in both observers were ± 0 and + 1 ml, respectively. The upper and lower limits of agreement between the two- and four-dimensional measurements by both observers were + 21/− 18 ml and + 12/− 13 ml, respectively. Inter-observer variability in four-dimensional flow measurement was 4% mean net forward flow in bicuspid aortic valve patients and 8% in healthy volunteers. Inter-observer variability in four-dimensional flow assessment is 8% or less which is acceptable for clinical cardiac MRI routine. There is close agreement of two- and four-dimensional flow tools in normal and complex flow pattern. In complex flow pattern, however, four-dimensional flow measurement picks up 4–9% higher flow values. It seems, therefore, that four-dimensional flow is closer to real flow values than two-dimensional flow, which is however to be proven by further studies.