Left Ventricular Physiology and Ventricular-Vascular Interactions in Young Patients After Heart Transplantation.

Background In patients after heart transplantation, systemic arterial hypertension and enhanced central aortic stiffness contribute to increased ventricular afterload, which might lead to graft dysfunction. The aim of our study was to characterize systemic arterial elastance and its impact on left ventricular function and ventriculo-arterial coupling in a cohort of children, adolescents, and young adults after heart transplantation using invasive conductance catheter technique. Methods and Results Thirty patients who had heart transplants (age, 20.0±6.5 years, 7 female) underwent invasive cardiac catheterization including pressure-volume loop analysis. Load-independent parameters of systolic (ventricular elastance [Ees]) and diastolic (ventricular compliance) function as well as systemic arterial elastance (Ea, end-systolic pressure/stroke volume) and ventriculo-arterial coupling (Ea/Ees) were assessed at baseline level and during dobutamine infusion (10 µg/kg/min). Ees showed an appropriate increase under inotropic stimulation from 0.43 (0.11-2.52) to 1.00 (0.20-5.10) mm Hg/mL/m2 (P<0.0001), whereas ventricular compliance remained rather unchanged (0.16±0.10 mm Hg/mL/m2 to 0.12±0.07 mm Hg/mL/m2; P=0.10). Ventriculo-arterial coupling Ea/Ees was abnormal at rest and did not improve significantly under dobutamine (1.7 [0.6-6.7] to 1.3 [0.5-4.9], P=0.70) due to a simultaneous rise in Ea from 0.71 (0.37-2.82) to 1.10 (0.52-4.03) mm Hg/mL/m2 (P<0.0001). Both Ees and ventricular compliance were significantly associated with Ea at baseline and under dobutamine infusion. Conclusions Patients who underwent heart transplantation show impaired ventriculo-arterial coupling at rest and under inotropic stimulation despite preserved left ventricular contractile reserve. An abnormal response in vascular function resulting in increased afterload seems to represent an important factor that may play a role for the development of late graft failure.

Aortic wall shear stress in bicuspid aortic valve disease-10-year follow-up.

Background: Bicuspid aortic valve (BAV) disease leads to deviant helical flow patterns especially in the mid-ascending aorta (AAo), potentially causing wall alterations such as aortic dilation and dissection. Among others, wall shear stress (WSS) could contribute to the prediction of long-term outcome of patients with BAV. 4D flow in cardiovascular magnetic resonance (CMR) has been established as a valid method for flow visualization and WSS estimation. The aim of this study is to reevaluate flow patterns and WSS in patients with BAV 10 years after the initial evaluation. Methods: Fifteen patients (median age 34.0 years) with BAV were re-evaluated 10 years after the initial study from 2008/2009 using 4D flow by CMR. Our particular patient cohort met the same inclusion criteria as in 2008/2009, all without enlargement of the aorta or valvular impairment at that time. Flow patterns, aortic diameters, WSS and distensibility were calculated in different aortic regions of interest (ROI) with dedicated software tools. Results: Indexed aortic diameters in the descending aorta (DAo), but especially in the AAo did not change in the 10-year period. Median difference 0.05 cm/m2 (95% CI: 0.01 to 0.22; P=0.06) for AAo and median difference -0.08 cm/m2 (95% CI: -0.12 to 0.01; P=0.07) for DAo. WSS values were lower in 2018/2019 at all measured levels. Aortic distensibility decreased by median 25.6% in the AAo, while stiffness increased concordantly (median +23.6%). Conclusions: After a ten years' follow-up of patients with isolated BAV disease, indexed aortic diameters did not change in this patient cohort. WSS was lower compared to values generated 10 years earlier. Possibly a drop of WSS in BAV could serve as a marker for a benign long-term course and implementation of more conservative treatment strategies.

Family screening of hypertrophic cardiomyopathy in children: a case report.

Background: Paediatric hypertrophic cardiomyopathy (HCM) caused by sarcomere protein gene mutations is more common than previously thought. We present the case of a 9-year-old boy that was diagnosed with HCM during family screening. Case summary: We present a case of a 9-year-old boy with a family history of sarcomeric HCM who was diagnosed with hypertrophic obstructive cardiomyopathy (HOCM) during clinical screening. Echocardiography and cardiovascular magnetic resonance imaging revealed asymmetric left ventricular hypertrophy with a maximum wall thickness of 18-19 mm. Cardiovascular magnetic resonance late gadolinium enhancement imaging showed patchy fibrosis within the area of maximum wall thickness. Genetic testing confirmed the presence of the familial mutation in the MYL2 gene. The patient was started on bisoprolol. Furthermore, risk stratification was performed and a recommendation for implantable cardioverter-defibrillator implantation was made. Discussion: This case demonstrates that significant HCM can already start in childhood and discusses the recommendations for family screening on the basis of recently published studies and the present European Society of Cardiology guideline.

Le Cœur en Sabot: shape associations with adverse events in repaired tetralogy of Fallot.

BACKGROUND: Maladaptive remodelling mechanisms occur in patients with repaired tetralogy of Fallot (rToF) resulting in a cycle of metabolic and structural changes. Biventricular shape analysis may indicate mechanisms associated with adverse events independent of pulmonary regurgitant volume index (PRVI). We aimed to determine novel remodelling patterns associated with adverse events in patients with rToF using shape and function analysis. METHODS: Biventricular shape and function were studied in 192 patients with rToF (median time from TOF repair to baseline evaluation 13.5 years). Linear discriminant analysis (LDA) and principal component analysis (PCA) were used to identify shape differences between patients with and without adverse events. Adverse events included death, arrhythmias, and cardiac arrest with median follow-up of 10 years. RESULTS: LDA and PCA showed that shape characteristics pertaining to adverse events included a more circular left ventricle (LV) (decreased eccentricity), dilated (increased sphericity) LV base, increased right ventricular (RV) apical sphericity, and decreased RV basal sphericity. Multivariate LDA showed that the optimal discriminative model included only RV apical ejection fraction and one PCA mode associated with a more circular and dilated LV base (AUC = 0.77). PRVI did not add value, and shape changes associated with increased PRVI were not predictive of adverse outcomes. CONCLUSION: Pathological remodelling patterns in patients with rToF are significantly associated with adverse events, independent of PRVI. Mechanisms related to incident events include LV basal dilation with a reduced RV apical ejection fraction.

Impact of Right Ventricular Pressure Load After Repair of Tetralogy of Fallot.

Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow-up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow-up of 10.1 (0.1-12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00-1.06; P=0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47-9.27; P=0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15-30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

Exercise-dependent changes in ventricular-arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study.

AIMS: Inefficient ventricular-arterial (V-A) coupling has been described in Fontan patients and may result in adverse haemodynamics. A varying amount of aortopulmonary collateral (APC) flow is also frequently present that increases volume load of the single ventricle. The aim of the study was to assess changes in V-A coupling and APC flow during exercise CMR. METHODS AND RESULTS: Eighteen Fontan patients (age 24 ± 3 years) and 14 controls (age 23 ± 4 years) underwent exercise CMR using a cycle ergometer. Ventricular volumetry and flow measurements in the ascending aorta (AAO), inferior (IVC), and superior (SVC) vena cava were assessed using real-time sequences during stepwise increases in work load. Measures of systemic arterial elastance Ea, ventricular elastance Ees, and V-A coupling (Ea/Ees) were assessed. APC flow was quantified as AAO - (SVC + IVC). Ea remained unchanged during all levels of exercise in both groups (P = 0.39 and P = 0.11). Ees increased in both groups (P = 0.001 and P < 0.001) with exercise but was lower in the Fontan group (P = 0.04). V-A coupling was impaired in Fontan patients at baseline (P = 0.04). Despite improvement during exercise (P = 0.002) V-A coupling remained impaired compared with controls (P = 0.001). Absolute APC flow in Fontan patients did not change during exercise even at maximum work load (P = 0.98). CONCLUSIONS: Inefficient V-A coupling was already present at rest in Fontan patients and aggravated during exercise due to a limited increase in ventricular contractility which demonstrates the importance of a limited functional reserve of the single ventricle. APC flow remained unchanged suggesting no further increase in volume load during exercise.

Favorable Atrial Remodeling After Percutaneous Pulmonary Valve Implantation and Its Association With Changes in Exercise Capacity and Right Ventricular Function.

Background Right atrial (RA) dilatation and impaired right ventricular (RV) filling are common in patients with RV outflow tract dysfunction. We aimed to study potential correlations between atrial function with clinically relevant hemodynamic parameters and to assess the predictive impact of atrial performance on the recovery of exercise capacity and RV pump function after percutaneous pulmonary valve implantation (PPVI). Methods and Results Altogether, 105 patients with right ventricular outflow tract dysfunction (median age at PPVI, 19.2 years; range, 6.2-53.4 years) who underwent cardiac magnetic resonance imaging before and 6 months after PPVI were included. RA and left atrial maximal and minimal volumes as well as atrial passive and active emptying function were assessed from axial cine slices. RA emptying function was inversely related to invasive RV end-diastolic pressure, and RA passive emptying correlated significantly with peak oxygen uptake. After PPVI, a significant decrease in RA minimum volume was observed, whereas RA passive emptying function improved, and RA active emptying function decreased significantly. Patients with predominant right ventricular outflow tract stenosis showed more favorable changes in RA active and left atrial passive emptying than those with primary volume overload. None of the RA and left atrial emptying parameters was predictive for recovery of peak oxygen uptake or RV ejection fraction. Conclusions In patients with right ventricular outflow tract dysfunction, impaired RA emptying assessed by cardiac magnetic resonance imaging was associated with increased RV filling pressures and lower exercise capacity. PPVI leads to a reduction in RA size and improved passive RA emptying function. However, RA function was not associated with improved exercise performance and RV pump function.

Hemodynamic and prognostic impact of the diastolic pulmonary arterial pressure in children with pulmonary arterial hypertension-a registry-based analysis.

BACKGROUND: Diastolic pulmonary arterial pressure (dPAP) is regarded to be less sensitive to flow metrics as compared to mean PAP (mPAP), and was therefore proposed for the assessment of a precapillary component in patients with postcapillary pulmonary hypertension (PH). To analyze the diagnostic and prognostic impact of dPAP in patients with pure precapillary PH, we purposed to compare the correlation between dPAP and mPAP, as well as hemodynamically-derived calculations [ratio of PAP to systemic arterial pressure (PAP/SAP), pulmonary vascular resistance index (PVRI), transpulmonary gradient (TPG)], using both dPAP and mPAP, at rest and during acute vasoreactivity testing (AVT) in children with idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH). Furthermore, we aimed to assess the association of these metrics (at baseline and changes after AVT) with transplant-free survival. METHODS: We conducted a retrospective analysis of the TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) registry including 246 IPAH/HPAH patients. Of these, 45 children (18.3%) died, and 13 (5.3%) received lung transplantation during the observation period. RESULTS: dPAP and mPAP-derived variables showed almost linear relationship. Higher mPAP/mSAP, and dPAP-/mPAP-derived PVRI at rest was associated with time to death/transplantation. At maximum AVT-response, the decrease of dPAP and mPAP, diastolic pulmonary gradient (DPG) and TPG, as well as dPAP/dSAP and mPAP/mSAP was associated with time to death/transplantation, showing higher significance than corresponding baseline values. Remarkably, no predictive value was found for PVRI-reduction during AVT, neither dPAP- nor mPAP-derived. CONCLUSIONS: There is a strong relationship between dPAP and mPAP-derived variables. According to our results, hemodynamics during AVT (irrespectively of dPAP- or mPAP-derived) may have more prognostic implications than resting hemodynamics in children with IPAH/HPAH, except for PVRI.

Role of cardiovascular magnetic resonance in pediatric pulmonary hypertension-novel concepts and imaging biomarkers.

Pulmonary hypertension (PH) in children is a heterogenous disease of the small pulmonary arteries characterized by a progressive increase in pulmonary vascular resistance. Despite adequate medical therapy, long-term pressure overload is frequently associated with a progressive course leading to right ventricular failure and ultimately death. Invasive hemodynamic assessment by cardiac catheterization is crucial for initial diagnosis, risk stratification and therapeutic strategy. Although echocardiography remains the most important imaging modality for the assessment of right ventricular function and pulmonary hemodynamics, cardiovascular magnetic resonance (CMR) has emerged as a valuable non-invasive imaging technique that enables comprehensive evaluation of biventricular performance, blood flow, morphology and the myocardial tissue. In this review, we summarize the principles and applications of CMR in the evaluation of pediatric PH patients and present an update about novel CMR based concepts and imaging biomarkers that may provide further diagnostic, therapeutic and prognostic information.

Heart failure therapy based on interventricular mechanics and cardio-vascular communications.

The heart should not be divided in right and left, whether in health nor in disease. However, the morphological and functional differences between the right and left ventricle should be known and the impact of the ventricle's position considered. Further, the parameters beyond heart rate, contractility, pre- and afterload guaranteeing a sufficient systemic cardiac output have to be integrated in therapeutic measures; preferentially the influence of interventricular mechanics. Despite of recent developments of specific drug therapies, heart failure is associated with a high rate of morbidity and mortality in children. During the progression of heart failure, pulmonary vascular disease is the consequence or the reason for further failing. Clinical symptoms are associated with congestion and low cardiac output at rest or exercise. Improved understanding of the pathophysiological mechanisms particularly of ventricular failure has resulted in the development of innovative therapies that target atrial/ventricular/arterial interactions. Recent advances in interventional and surgical approaches provide promising new strategies to deal with right and left ventricular deterioration. These techniques may delay listing for heart and (heart-) lung transplantation or even make redundant in individual cases. The beneficial effects of these ventricular interaction strategies are mainly based on the mechanics of the interventricular septum and improvement of systolic and diastolic ventricular performance.

A Comprehensive Functional Analysis in Patients after Atrial Switch Surgery.

BACKGROUND: Long-term course after atrial switch operation is determined by increasing right ventricular (RV) insufficiency. The aim of our study was to investigate subtle functional parameters by invasive measurements with conductance technique and noninvasive examinations with cardiac magnetic resonance imaging (CMR). METHODS: We used invasive (pressure-volume loops under baseline conditions and dobutamine) and noninvasive techniques (CMR with feature tracking [FT] method) to evaluate RV function. All patients had cardiopulmonary exercise testing (CPET). RESULTS: From 2011 to 2013, 16 patients aged 28.2 ± 7.3 (22-50) years after atrial switch surgery (87.5% Senning and 12.5% Mustard) were enrolled in this prospective study. All patients were in New York Heart Association (NYHA) class I to II and presented mean peak oxygen consumption of 30.1 ± 5.7 (22.7-45.5) mL/kg/min. CMR-derived end-diastolic volume was 110 ± 22 (78-156) mL/m2 and RV ejection fraction 41 ± 8% (25-52%). CMR-FT revealed lower global systolic longitudinal, radial, and circumferential strain for the systemic RV compared with the subpulmonary left ventricle. End-systolic elastance (Ees) was overall reduced (compared with data from the literature) and showed significant increase under dobutamine (0.80 ± 0.44 to 1.89 ± 0.72 mm Hg/mL, p ≤ 0.001), whereas end-diastolic elastance (Eed) was not significantly influenced (0.11 ± 0.70 to 0.13 ± 0.15 mm Hg/mL, p = 0.454). We found no relevant relationship between load-independent conductance indices and strain or CPET parameters. Conductance analysis revealed significant mechanical dyssynchrony, higher during diastole (mean 30 ± 4% baseline, 24 ± 6% dobutamine) than during systole (mean 17 ± 6% baseline, 19 ± 7% dobutamine). CONCLUSIONS: Functional assessment of a deteriorating systemic RV remains demanding. Conductance indices as well as the CMR-derived strain parameters showed overall reduced values, but a significant relationship was not present (including CPET). Our conductance analysis revealed intraventricular and predominantly diastolic RV dyssynchrony.

Unsupervised Domain Adaptation From Axial to Short-Axis Multi-Slice Cardiac MR Images by Incorporating Pretrained Task Networks.

Anisotropic multi-slice Cardiac Magnetic Resonance (CMR) Images are conventionally acquired in patient-specific short-axis (SAX) orientation. In specific cardiovascular diseases that affect right ventricular (RV) morphology, acquisitions in standard axial (AX) orientation are preferred by some investigators, due to potential superiority in RV volume measurement for treatment planning. Unfortunately, due to the rare occurrence of these diseases, data in this domain is scarce. Recent research in deep learning-based methods mainly focused on SAX CMR images and they had proven to be very successful. In this work, we show that there is a considerable domain shift between AX and SAX images, and therefore, direct application of existing models yield sub-optimal results on AX samples. We propose a novel unsupervised domain adaptation approach, which uses task-related probabilities in an attention mechanism. Beyond that, cycle consistency is imposed on the learned patient-individual 3D rigid transformation to improve stability when automatically re-sampling the AX images to SAX orientations. The network was trained on 122 registered 3D AX-SAX CMR volume pairs from a multi-centric patient cohort. A mean 3D Dice of 0.86 ± 0.06 for the left ventricle, 0.65 ± 0.08 for the myocardium, and 0.77 ± 0.10 for the right ventricle could be achieved. This is an improvement of 25% in Dice for RV in comparison to direct application on axial slices. To conclude, our pre-trained task module has neither seen CMR images nor labels from the target domain, but is able to segment them after the domain gap is reduced. Code: https://github.com/Cardio-AI/3d-mri-domain-adaptation.

Real-time CMR guidance for intracardiac and great vessel pressure mapping in patients with congenital heart disease using an MR conditional guidewire-results of 25 patients.

BACKGROUND: The aim of this study was to test a CE-certified MR-conditional guidewire to facilitate blood pressure measurement in cardiovascular magnetic resonance (CMR) using fluid-filled catheters in patients with congenital heart disease (CHD). The main purpose was to determine procedural success in a post market clinical follow-up (PMCF) for routine procedure in a diagnostic and interventional workflow. Real-time CMR provides high quality imaging without the risk of exposing the patient to X-rays, especially for patients with irregular heart anatomy and patients who are susceptible to radiation and iodinated contrast media. To date, the assessment of blood pressure gradients is not a common feature of CMR, as these gradients cannot be accurately evaluated in routine CMR. METHODS: Twenty-five CHD patients who were planned for combined clinical CMR and diagnostic and/or interventional catheterization were enrolled in the trial. Prior to inclusion, a specific procedure for catheterization in CMR was defined, encompassing the assessment of pressure and pressure gradients in the heart and great vessels. RESULTS: By the use of an MR-conditional guidewire we successfully measured specific pressure and pressure gradients in up to 92% of cases with liquid-filled catheters which were guided exclusively under CMR guidance. There were no guidewire-related adverse events, and guidewire guidance and manipulation of catheters were successful. CONCLUSIONS: Using a MR-conditional guidewire assists in easily reaching targets in the heart and great vessels and makes the catheter itself visible, so that invasive blood pressure assessment by CMR guidance with liquid-filled catheters can be improved. KEYWORDS: Cardiovascular magnetic resonance (CMR); congenital heart disease (CHD); cardiac catheterization; magnetic resonance; pressure; guidewire.

Aortic diameter assessment by cardiovascular magnetic resonance: do we really need contrast enhanced images?

BACKGROUND: Cardiovascular magnetic resonance (CMR) is widely used for aortic diameter assessment but there is no consensus on the sequence or cardiac cycle phase in which the measures should be taken. The most used sequence is contrast-enhanced-magnetic-resonance-angiography (angiography), usually non-ECG-triggered. An alternative is a navigated 3D-whole-heart-steady-state-free-precession sequence which is contrast-free and breath- and ECG-gated (mostly diastolic gating), producing very sharp anatomical rendering. Nonetheless, its routine use has not yet spread. Our aim was evaluating aortic diameters by a systolic-gated 3D and put additional effort in the validation of diastolic-gated 3D as alternative to angiography. METHODS: We retrospectively analysed 30 patients scheduled for routine Angiography. We measured the aorta at 9 standard positions by three different sequences (angiography, 3D-diastole and 3D-systole) and compared the diameters obtained by calculating the differences and by paired t-test analysis. RESULTS: Diameters by 3D-systole were larger than by 3D-diastole and angiography (P<0.01). In the ascending aorta we found the maximal differences between systole and diastole and between systole and angiography which were 1.7± SD 1.02 mm and 1.5± SD 1.07 mm respectively. There was no significant difference between diastolic and angiography measurements (mean difference 0.2± SD 0.16 mm, P not significant). CONCLUSIONS: Our results support the use of navigated 3D-whole-heart CMR to evaluate aortic diameters. Systolic-gated 3D produces larger diameter, especially in the ascending aorta.

Impact of aortopulmonary collateral flow and single ventricle morphology on longitudinal hemodynamics in Fontan patients: A serial CMR study.

BACKGROUND: Single ventricle (SV) patients with a Fontan circulation are at risk for functional deterioration. The aim of this study was to assess longitudinal Fontan hemodynamics using serial cardiovascular magnetic resonance (CMR) data and to study the impact of aortopulmonary collateral (APC) flow and type of SV morphology. METHODS: Forty-one Fontan patients (age at first CMR 13.4 ± 6.0 years) with two CMR examinations within a time interval of at least 4 years (follow-up 5.3 ± 0.9 years) were included. The protocol consisted of short-axis cine volumetry and 2-dimensional blood flow measurements in the inferior vena cava (IVC), superior vena cava (SVC) and ascending aorta (Ao). APC flow was calculated as Ao - (SVC + IVC). Myocardial strain/strain rate was assessed using feature tracking technique. RESULTS: SV end-diastolic volume (p = 0.14) and ejection fraction (p = 0.70) remained constant. No significant changes in CMR derived myocardial strain/strain rate values were observed. Ao flow decreased (p = 0.01), IVC flow remained unchanged (p = 0.52), while SVC flow (p = 0.003) and APC flow (p = 0.006) decreased significantly. Patients with a systemic right ventricle (RV) showed unchanged APC flow and a further increase in SV size over time compared to patients with a systemic left ventricle. CONCLUSIONS: Longitudinal CMR data in a cohort of clinically stable Fontan patients revealed no significant changes in SV dimensions and myocardial performance while APC flow decreased spontaneously. Patients with a systemic RV seem to be at risk for permanent SV volume overload through APCs and might therefore benefit from consequent interventional APC embolization.

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

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.

Aortic elasticity after aortic coarctation relief: comparison of surgical and interventional therapy by cardiovascular magnetic resonance imaging.

BACKGROUND: Patients after aortic coarctation (CoA) repair show impaired aortic bioelasticity and altered left ventricular (LV) mechanics, predisposing diastolic dysfunction. Our purpose was to assess aortic bioelasticity and LV properties in CoA patients who underwent endovascular stenting or surgery using cardiovascular magnetic resonance (CMR) imaging. METHODS: Fifty CoA patients (20.5 ± 9.5 years) were examined by 3-Tesla CMR. Eighteen patients had previous stent implantation and 32 had surgical repair. We performed volumetric analysis of both ventricles (LV, RV) and left atrium (LA) to measure biventricular volumes, ejection fractions, left atrial (LA) volumes, and functional parameters (LAEFPassive, LAEFContractile, LAEFReservoir). Aortic distensibility and pulse wave velocity (PWV) were assessed. Native T1 mapping was applied to examine LV tissue properties. In twelve patients post-contrast T1 mapping was performed. RESULTS: LV, RV and LA parameters did not differ between the surgical and stent group. There was also no significant difference for aortic distensibility, PWV and T1 relaxation times. Aortic root distensibility correlated negatively with age, BMI, BSA and weight (p < 0.001). Native T1 values correlated negatively with age, weight, BSA and BMI (p < 0.001). Lower post-contrast T1 values were associated with lower aortic arch distensibility and higher aortic arch PWV (p < 0.001). CONCLUSIONS: CoA patients after surgery or stent implantation did not show significant difference of aortic elasticity. Thus, presumably other factors like intrinsic aortic abnormalities might have a greater impact on aortic elasticity than the approach of repair. Interestingly, our data suggest that native T1 values are influenced by demographic characteristics.

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.