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Structured abstract: Objectives: To describe the workflow and value of three-dimensional rotational angiography (3DRA) in percutaneous pulmonary valve implantation (PPVI). Background: 3DRA offers visualization of the entire topography in the chest and may enhance safety and reduce the risk for complications in PPVI through improved pre-procedural planning and per-procedural guidance. Methods: All PPVI procedures with the use of 3DRA performed between August 2011 and December 2022 were reviewed. Success rate, complications and radiation dose were assessed. Radiation dose of the latest 3DRA protocol was compared to historical 3DRA data. Results: PPVI was successful in 95 of 102 procedures. Seven procedures were aborted due to coronary compression after balloon testing (n = 3), main pulmonary artery (MPA) oversize (n = 3) and not passing of a Melody valve through a calcified Melody valve in situ (n = 1). PPVI was attempted in 61 homografts, 19 native right ventricular outflow tracts (including transannular patch), 4 previously implanted Melody valves, 2 in previously implanted Sapien valves and 16 in other bioprosthetic valves. A Melody valve was implanted in 43, a Sapien valve in 49 and a Pulsta valve in 1 patient. In 2 patients a Melody as well as a Sapien valve were subsequently implanted. Mean total dose area product (DAP) was 11813 mGycm2 and 179 mGycm2/kg for all attempted PPVI's. For successful PPVI 9835 mGycm2 and 174 mGycm2/kg. After optimizing the 3DRA protocols the mean dose reduced from 12677 mGycm2 to 8551 mGycm2 (200 mGycm2/kg to 163 mGycm2/kg). Four patients experienced one or more complications. There were no deaths peri-procedural or during follow-up. Complications were; need for cardiopulmonary resuscitation (n = 2), MPA paravasation (n = 1), valve dysfunction (n = 2). Conclusions: The use of rotational angiography for the guidance of PPVI results in a high success rate, low number of complications with the use of a low amount of radiation.

Abstract 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.

Abstract 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

Background. Improvement of immunosuppressive therapies and surgical techniques has increased the survival rate after heart transplantation. Nevertheless, a large number of patients still experience complications, such as allograft rejection, vasculopathy, kidney dysfunction, and diabetes in response to immunosuppressive therapy. Variants in HLA genes have been extensively studied for their role in clinical outcomes after transplantation, whereas the knowledge about non-HLA genetic variants in this setting is still limited. Non-HLA polymorphisms are involved in the metabolism of major immunosuppressive therapeutics and may play a role in clinical outcomes after cardiac transplantation. This systematic review summarizes the existing knowledge of associations between non-HLA genetic variation and heart transplant outcomes. Methods. The current evidence available on genetic polymorphisms associated with outcomes after heart transplantation was identified by a systematic search in PubMed and Embase. Studies reporting on polymorphisms significantly associated with clinical outcomes after cardiac transplantation were included. Results. A total of 56 studies were included, all were candidate gene studies. These studies identified 58 polymorphisms in 36 genes that were associated with outcomes after cardiac transplantation. Variants in TGFB1, CYP3A5, and ABCB1 are consistently replicated across multiple studies for various transplant outcomes. Conclusions. The research currently available supports the hypothesis that non-HLA polymorphisms are associated with clinical outcomes after heart transplantation. However, many genetic variants were only identified in a single study, questioning their true effect on the clinical outcomes tested. Further research in larger cohorts with well-defined phenotypes is warranted.