Heart and large vessel interaction in congenital heart disease, assessed by magnetic resonance imaging

The main objective of the current thesis is to assess aortic wall elasticity and aortic dimensions and their impact on aortic valve competence and LV function in patients with a bicuspid aortic valve, transposition of the great arteries, tetralogy of Fallot and patients after the Ross procedure, with the use of MRI. In addition, MRI was also used to test whether a similar interaction is present between pulmonary artery dynamics and the right ventricle in the above mentioned entities. Chapter 2 reviews the 5 most common entities of inherited connective tissue disorders and classical congenital heart disease with intrinsic aortic wall abnormalities, with description of the potential role of MRI in their evaluation and management. Chapter 3 describes in vivo validation, as well as testing of reproducibility of aortic pulse wave velocity as assessed with MRI, as indicator of aortic elasticity. Chapter 4 studies aortic elasticity, aortic valve competence and LV function in non-stenotic bicuspid aortic valve patients. Chapters 5 and 6 describe aortic elasticity, aortic valve competence and LV function, and the results of pulmonary flow dynamics in relationship with right ventricular function in patients after the arterial switch operation, respectively. Chapter 7 addresses aortic dimensions and elasticity in conjunction with aortic valve and LV function in patients with repaired tetralogy of Fallot after pulmonary valve replacement. Chapter 8 describes the outcome of patients who have previously undergone the Ross procedure in our institution for a dysfunctioning aortic valve. Chapter 9 studies aortic elasticity, aortic valve competence and LV function in patients after the Ross procedure. Finally, Chapter 10 describes the results of pulmonary flow dynamics in relationship with right ventricular function in patients after the Ross procedure. In Chapter 11 all studies presented in this thesis are summarized and future directions are discussed.Nederlandse Hartstichting, Astra Zeneca B.V., Bayer B.V., Philips Medical Systems, Boehringer-Ingelheim B.V., J.E. Jurriaanse Stichting, Guerbet Nederland B.V., Laboratorium voor Klinische en Experimentele Beeldverwerking (LKEB)UBL - phd migration 201

Abnormal vortex formation in the right pulmonary artery after the arterial switch operation

Cardiovascular Aspects of Radiolog

Abnormal Myocardial Contractility After Pediatric Heart Transplantation by Cardiac MRI

Cardiolog

4D flow cardiovascular magnetic resonance derived energetics in the Fontan circulation correlate with exercise capacity and CMR-derived liver fibrosis/congestion

Aim This study explores the relationship between in vivo 4D flow cardiovascular magnetic resonance (CMR) derived blood flow energetics in the total cavopulmonary connection (TCPC), exercise capacity and CMR-derived liver fibrosis/congestion. Background The Fontan circulation, in which both caval veins are directly connected with the pulmonary arteries (i.e. the TCPC) is the palliative approach for single ventricle patients. Blood flow efficiency in the TCPC has been associated with exercise capacity and liver fibrosis using computational fluid dynamic modelling. 4D flow CMR allows for assessment of in vivo blood flow energetics, including kinetic energy (KE) and viscous energy loss rate (EL). Methods Fontan patients were prospectively evaluated between 2018 and 2021 using a comprehensive cardiovascular and liver CMR protocol, including 4D flow imaging of the TCPC. Peak oxygen consumption (VO2) was determined using cardiopulmonary exercise testing (CPET). Iron-corrected whole liver T1 (cT1) mapping was performed as a marker of liver fibrosis/congestion. KE and EL in the TCPC were computed from 4D flow CMR and normalized for inflow. Furthermore, blood flow energetics were compared between standardized segments of the TCPC. Results Sixty-two Fontan patients were included (53% male, 17.3 +/- 5.1 years). Maximal effort CPET was obtained in 50 patients (peak VO2 27.1 +/- 6.2 ml/kg/min, 56 +/- 12% of predicted). Both KE and EL in the entire TCPC (n = 28) were significantly correlated with cT1 (r = 0.50, p = 0.006 and r = 0.39, p = 0.04, respectively), peak VO2 (r = - 0.61, p = 0.003 and r = - 0.54, p = 0.009, respectively) and % predicted peak VO2 (r = - 0.44, p = 0.04 and r = - 0.46, p = 0.03, respectively). Segmental analysis indicated that the most adverse flow energetics were found in the Fontan tunnel and left pulmonary artery. Conclusions Adverse 4D flow CMR derived KE and EL in the TCPC correlate with decreased exercise capacity and increased levels of liver fibrosis/congestion. 4D flow CMR is promising as a non-invasive screening tool for identification of patients with adverse TCPC flow efficiency.Cardiovascular Aspects of Radiolog

Reduced scan time and superior image quality with 3D flow MRI compared to 4D flow MRI for hemodynamic evaluation of the Fontan pathway

Long scan times prohibit a widespread clinical applicability of 4D flow MRI in Fontan patients. As pulsatility in the Fontan pathway is minimal during the cardiac cycle, acquiring non-ECG gated 3D flow MRI may result in a reduction of scan time while accurately obtaining time-averaged clinical parameters in comparison with 2D and 4D flow MRI. Thirty-two Fontan patients prospectively underwent 2D (reference), 3D and 4D flow MRI of the Fontan pathway. Multiple clinical parameters were assessed from time-averaged flow rates, including the right-to-left pulmonary flow distribution (main endpoint) and systemic-to-pulmonary collateral flow (SPCF). A ten-fold reduction in scan time was achieved [4D flow 15.9 min (SD 2.7 min) and 3D flow 1.6 min (SD 7.8 s), p<0.001] with a superior signal-to-noise ratio [mean ratio of SNRs 1.7 (0.8), p<0.001] and vessel sharpness [mean ratio 1.2 (0.4), p=0.01] with 3D flow. Compared to 2D flow, good-excellent agreement was shown for mean flow rates (ICC 0.82-0.96) and right-to-left pulmonary flow distribution (ICC 0.97). SPCF derived from 3D flow showed good agreement with that from 4D flow (ICC 0.86). 3D flow MRI allows for obtaining time-averaged flow rates and derived clinical parameters in the Fontan pathway with good-excellent agreement with 2D and 4D flow, but with a tenfold reduction in scan time and significantly improved image quality compared to 4D flow.Developmen

Preserved Myocardial Deformation after Successful Coarctation Repair: A CMR Feature-Tracking Study

Contains fulltext : 190059.pdf (publisher's version ) (Open Access

Structure and function of the aorta in inherited and congenital heart disease and the role of MRI

Cardiovascular Aspects of Radiolog

Resection of a cardiac aneurysm in an infant with anomalous origin of the left coronary artery from the pulmonary trunk.

Contains fulltext : 57512.pdf (publisher's version ) (Closed access)We describe an infant with an anomalous left coronary artery arising from the pulmonary trunk leading to myocardial ischaemia and development of an apical aneurysm of the left ventricle. Clinical presentation in general is based on myocardial hypoperfusion resulting in ischaemia and infarction. When presenting in infancy, however, then the features, as in our patient, may be tachypnea, dyspnea, failure to thrive and irritability, especially during feeding. Then, again as in our patient, it is possible to miss the definitive diagnosis, which is made by echocardiography and cardiac catheterization. Reimplantation of the anomalous left coronary artery into the aorta usually results in improvement of left ventricular function over time. In our patient, the myocardial infarction was complicated by formation of an aneurysm, and reimplantation alone was not sufficient to restore cardiac function. Resection of the aneurysm greatly improved the hemodynamics