Abdominal lymphatic pathway in Fontan circulation using non-invasive magnetic resonance lymphangiography.

Lymphatic congestion is known to play an important role in the development of late Fontan complications. This study aimed to (1) develop a gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA) contrast three-dimensional heavily T2-weighed MR technique that can detect abnormal lymphatic pathway in the abdomen while simultaneously evaluating hepatocellular carcinoma (HCC) and to (2) propose a new classification of abnormal abdominal lymphatic pathway using a non-invasive method in adults with Fontan circulation. Twenty-seven adults with Fontan circulation who underwent Gd-EOB-DTPA abdominal MR imaging were prospectively enrolled in this study. We proposed MR lymphangiography that suppresses the vascular signal on heavily T2-weighted imaging after EOB contrast. The patients were classified as follows: grade 1 with almost no lymphatic pathway, grade 2 with a lymphatic pathway mainly around the bile duct and liver surface, and grade 3 with a lymphatic pathway mainly around the vertebral body and inferior vena cava. The grade 3 group showed the lowest oxygen saturation level, highest central venous pressure, highest incidence of massive ascites, HCC, and focal nodular hyperplasia. This group also tended to have patients with the oldest age and highest cardiac index; however, the difference was not statistically significant. As for the blood test, the grade 3 group showed the lowest platelet count and serum albumin level and the highest fibrosis-4 index. A novel technique, Gd-EOB-DTPA MR lymphangiography, can detect abnormal abdominal lymphatic pathways in Fontan circulation, which can reflect the severity of failing Fontan.

Aortic Vorticity, Helicity, and Aortopathy in Adult Patients with Tetralogy of Fallot: Pilot Study Using Four-Dimensional Flow Magnetic Resonance Images.

To employ quantitative analysis in the vorticity and helicity of the aortic root and the ascending aorta (AAo) in adults with tetralogy of Fallot (TOF), and to evaluate aortopathy and the relevant factors. Prospectively, 51 consecutive adults with TOF underwent 4 dimensional flow magnetic resonance imaging study for the assessment of vorticity and helicity of the aortic root and AAo, wall shear stress (WSS), viscous energy loss (EL), and the left ventricular outflow tract - aortic root (LVOT-Ao) angle. Patients were divided into the two groups: dilated aortic root and/or AAo (indexed diameter > 25 mm/cm2), Group A (15 patients); non-dilated aortic patients, Group B (36 patients). Ten age-matched controls were also enrolled. Group A showed more acute LVOT-Ao angle, higher incidence of aortic regurgitation, and initial anatomy of pulmonary atresia, compared to Group B (P < 0.0001, 0.02, 0.043). Group A showed greater clockwise vorticity at the level of Valsalva, AAo, and proximal arch, sagittal vorticity, AAo helicity, WSS, and EL than in Group B (P < 0.001, < 0.001, < 0.001, 0.045, 0.049, 0.02, 0.026). More acute LVOT-Ao angle correlated with the diameter of the aortic root and AAo, AAo vorticity, helicity, WSS, and EL (P = 0.004, 0.023, 0.045, 0.004, 0.0004, 0.017). On a univariate logistic analysis, more acute LVOT-Ao angle, AAo vorticity, AAo helicity, and maximum WSS were relevant factors of AAo dilatation (P = 0.02, 0.02, 0.045, 0.03, 0.046). On a multivariate logistic analysis, more acute LVOT-Ao angle was the most important factor of AAo dilatation (odds ratio 0.66, 95% CI 0.46-0.95, P < 0.024). TOF adults presenting dilated AAo have greater vorticity, helicity, and acute LVOT-Ao angle. Flow eccentricity and these flow hemodynamic parameters may be adjunctive predictions of aortopathy in this population.

Liver Strain Using Feature Tracking of Cine Cardiac Magnetic Resonance Imaging: Assessment of Liver Dysfunction in Patients with Fontan Circulation and Tetralogy of Fallot.

We propose a novel method to quantify pulsatile liver deformation using the feature tracking method of cardiac cine magnetic resonance imaging (MRI) and investigate its association with liver dysfunction in long-term postoperative patients after Fontan and intracardiac repair for the tetralogy of Fallot (TOF). Standard cine MRI which was previously performed for cardiac evaluation of 85 patients who underwent Fontan operation (mean age, 22.9 years), 43 patients with TOF (mean age, 34.6 years), and 32 healthy controls (mean age, 42.3 years) were retrospectively analyzed. Pulsatile liver deformation in the craniocaudal direction was calculated using the feature tracking method of cardiac cine imaging derived from cine-balanced turbo field-echo sequences performed on a 1.5 Tesla MR scanner, and was defined as liver strain. The liver strain was compared across the three patient groups using one-way analysis of variance. Liver dysfunction by a liver strain were compared using the Mann-Whitney U test. Liver strain for patients who underwent Fontan operation and TOF patients was significantly lower than controls (Fontan, 13.3 ± 6.5%; TOF, 15.0 ± 11.2%; controls, 23.1 ± 10.2%, p < 0.0001). In Fontan and TOF patients, MELD score was significantly greater for patients with a liver strain < 15% than those with values > 15% (5.9 ± 5.8 vs. 2.9 ± 2.9, p < 0.001). Lower liver strain values were found in adolescent and adult patients after Fontan operation and TOF, and correlates with the severity of liver injury, expressed as MELD score. Our method can evaluate hepatic function in adult congenital heart disease, together with the assessment of cardiac function.

Potential Value of Native T1 Mapping in Symptomatic Adults with Congenital Heart Disease: A Preliminary Study of 3.0 Tesla Cardiac Magnetic Resonance Imaging.

The native T1 value at 3.0 Tesla is a sensitive marker of diffuse myocardial damage. We evaluated the clinical usefulness of native T1 mapping in symptomatic adults with congenital heart disease (CHD), particularly in the systemic right ventricle (RV). Prospectively, 45 consecutive symptomatic adults with CHD were enrolled: 20 with systemic RV and 25 with tetralogy of Fallot underwent cardiac magnetic resonance (CMR) imaging at 3.0 Tesla. The Modified Look-Locker Inversion recovery sequence was used for T1 mapping. Cardiovascular events in the systemic RV were defined as heart failure and tachyarrhythmia. Brain natriuretic peptide (BNP) and indexed systemic ventricular end-diastolic volume were significantly higher in the systemic RV group. The native T1 value and extracellular volume (ECV) of the septal and lateral walls were higher in the systemic RV group, suggesting high impairment of the myocardium in the systemic RV group. There was a strong correlation between the native T1 value and ECV of the septum (r = 0.58, P = 0.03) and lateral wall (r = 0.56, P = 0.046) in the systemic RV group. Seven patients with systemic RV had cardiovascular events. In univariate logistic regression analysis, BNP and native T1 values of the insertion point were important for predicting cardiovascular events. The native T1 value at 3.0 Tesla may be a sensitive, contrast-free, and non-invasive adjunct marker of myocardial damage in CHD and predictive of cardiovascular events in the systemic RV.

Stroke volume ratio derived from magnetic resonance imaging as an indicator of interventricular dyssynchrony predicts future cardiac event in patients with biventricular Fontan circulation.

The prognostic factors in patients with biventricular heart who underwent Fontan surgery remain unclear. This study wanted to assess the hypothesis that interventricular dyssynchrony evaluated by cardiac magnetic resonance imaging (MRI) can predict future cardiac events in patients with biventricular heart who have undergone Fontan surgery. We prospectively enrolled consecutive patients with biventricular Fontan circulation from 2003 to 2016, and performed protocolized cardiac MRI. We determined the stroke volume ratio (SVr) using the following formula to assess interventricular dyssynchrony: (stroke volume (SV) of the two whole ventricles)/(SV of the right ventricle + SV of the left ventricle), by tracing cine MRI data. If interventricular dyssynchrony existed, blood flowed and returned to each ventricle through the ventricular septal defect; therefore, the SVr in this instance should be less than 1.0. We enrolled 40 patients. SVr ranged from 0.81 to 1.0 (median 0.95). Low SVr (< 0.95) was associated with worse New York Heart Association functional class, longer QRS duration, right bundle branch block, low biventricular indexed stroke volume, and low biventricular ejection fraction. During the follow-up period (median 53.5 months), 10 cardiac events occurred (six cases of acute exacerbation of heart failure, three cases of supraventricular tachycardia, and one case of exacerbation of protein-losing enteropathy). Univariate analysis showed four clinical predictors: SVr < 0.95 [hazard ratio (HR) 9.3, 95% confidential interval (CI) 1.7-171.5]; biventricular ejection fraction < 0.45 (HR 9.4, 95% CI 2.2-65.3); left ventricular indexed end-diastolic volume > 73 mL/m2 (HR 4.5, 95% CI 1.1-15.7); and the presence of the aorta directly arising from the right ventricular conus (HR 5.8, 95% CI 1.1-106). SVr derived from MRI can predict future cardiac events in Fontan patients with biventricular hearts.

Prediction of Fontan-Associated Liver Disease Using a Novel Cine Magnetic Resonance Imaging "Vortex Flow Map" in the Right Atrium.

BACKGROUND: Long-term hepatic dysfunction is an increasingly recognized complication of the Fontan operation for univentricular hearts. The purpose of this study was to determine whether Fontan-associated liver disease (FALD) could be predicted by flow dynamics in the right atrium (RA) of Fontan circulation.Methods and Results:Cardiac MRI and the serum levels of total bilirubin (TBil) and hyaluronic acid (HA) were analyzed in 36 patients who underwent an atriopulmonary connection type of Fontan operation. The mean follow-up period was 53 months. Three views (axial, coronal, and sagittal) of the cine images were scanned for the maximum cross-section of the RA obtained with1.5-Tesla scanner. We developed a "vortex flow map" to demonstrate the ratio of the circumferential voxel movement in each phase to the total movement throughout a cardiac cycle towards the center of the RA. The maximum ratio was used as the magnitude of vortex flow (MVF%) in the 3 views of the RA cine imaging. Patients with coronal MVF ≥13.6% had significantly lower free rates of TBil ≥1.8 mg/dL than those with coronal MVF <13.6% (log-rank value=4.50; P<0.05; hazard ratio=4.54). Patients with sagittal MVF ≥14.0% had significantly lower free rates of HA ≥50 ng/mL than those with coronal MVF <14.0% (log-rank value=4.40; P<0.05; hazard ratio=4.12). CONCLUSIONS: A reduced vortex flow in the RA during the late phase of the Fontan operation was associated with the development of FALD. MVF can be used as an imaging biomarker to predict FALD.

Inter- and intra-ventricular dyssynchrony in the systemic right ventricle is a surrogate marker of major cardiac events in mildly symptomatic patients.

The aim of the study was to evaluate systemic right ventricular (RV) dyssynchrony in patients with congenitally corrected transposition of the great arteries (CCTGA) and transposition of the great arteries (TGA) with New York Heart Association functional class (NYHA FC) < III. We used cardiac magnetic resonance (CMR) to evaluate the dyssynchrony and assessed whether RV dyssynchrony can be predictive of major cardiac events in their early stages in these patients. We enrolled 71 consecutive, NYHA FC < III patients with systemic RV who underwent CMR between April 1995 and December 2016. We measured intra- and inter-ventricular dyssynchrony using a feature-tracking method of cine magnetic resonance imaging. The predictors of major cardiac events were analyzed using the Cox hazard analysis. The data from 36 patients with CCTGA and 35 patients with TGA after an atrial switch were analyzed. Seven (19.4%) patients with CCTGA and 6 (17.1%) patients with TGA showed a QRS duration of ≥ 130 ms. There were significant intra- and inter-dyssynchrony in the systemic RV groups, compared to healthy controls. The average follow-up period was 5.1 ± 3.9 years. From among patients with CCTGA, 9 (25.0%) had major cardiac events. The parameters including NYHA FC, indexed RV volume, longitudinal early diastolic strain rate, and intra- and inter-ventricular dyssynchrony were predictive of major cardiac events. From among patients with TGA, 12 (34.3%) had major cardiac events. Age, NYHA FC, QRS duration, RV volume, RV mass index, LV volume, global longitudinal/circumferential strain and intraventricular dyssynchrony, were all predictive of major cardiac events. Systemic RV in NYHA FC < III patients with CCTGA and TGA, have obvious intra- and inter-dyssynchrony, suggesting ineffective wall motion and potential RV dysfunction. Intraventricular dyssynchrony can be an adjunct predictor of major cardiac events in mildly symptomatic patients with both CCTGA and TGA.

Vortex Flow in the Right Atrium Surrogates Supraventricular Arrhythmia and Thrombus After Atriopulmonary Connection-Type Fontan Operation: Vortex Flow Analysis Using Conventional Cine Magnetic Resonance Imaging.

We developed a novel imaging technique, designated as vortex flow (VF) mapping, which presents a vortex flow visually on conventional two-dimensional (2D) cine MRI. Using it, we assessed circumferential VF patterns and influences on RA thrombus and supraventricular tachycardia (SVT) in AP connection-type Fontan circulation. Retrospectively, we enrolled 27 consecutive patients (25.1 ± 9.2 years) and 7 age-matched controls who underwent cardiac MRI. Conventional cine images acquired using a 1.5-Tesla scanner were scanned for axial and coronal cross section of the RA. We developed "vortex flow mapping" to demonstrate the ratio of the circumferential voxel movement at each phase to the total movement throughout a cardiac cycle towards the RA center. The maximum ratio was used as a magnitude of vortex flow (MVF%) in RA cine imaging. We also measured percentages of strong and weak VF areas (VFA%). Furthermore, in 10 out of 27, we compared VF between previous CMR (3.8 ± 1.5 years ago) and latest CMR. Of the patients, 15 had cardiovascular complications (Group A); 12 did not (Group B). A transaxial image showed that strong VFA% in Group A was significantly smaller than that in Group B or controls. A coronal view revealed that strong VFA% was also smaller, and weak VFA% was larger in Group A than in Group B or controls (P < 0.05, and P < 0.05). Maximum MVF% in Group A was significantly smaller than in other groups (P < 0.001). Univariate logistic analyses revealed weak VFA% on a coronal image, and serum total bilirubin level as factors affecting cardiovascular complications (Odds ratio 1.14 and 66.1, 95% CI 1.004-1.30 and 1.59-2755.6, P values < 0.05 and < 0.05, respectively). Compared to the previous CMR, smaller maximum VMF%, smaller strong VFA%, and larger weak VFA% were identified in the latest CMR. Circumferentially weak VFA% on a coronal image can be one surrogate marker of SVT and thrombus in AP connection-type Fontan circulation. This simple VF assessment is clinically useful to detect blood stagnation.

Cardio-Ankle Vascular Index (CAVI) and Plasma Transforming Growth Factor-ß1 (TGF-ß1) Level Correlate with Aortopathy in Adults with Repaired Tetralogy of Fallot.

It is challenging to evaluate aortopathy in congenital heart disease using conventional investigations such as brachial-ankle pulse wave velocity (baPWV). Therefore, we evaluated the cardio-ankle vascular index (CAVI), a dimension of the ascending aorta and plasma transforming growth factor-ß1 (TGF-ß1) level, in order to find novel noninvasive parameters of aortopathy in adults with repaired tetralogy of Fallot (TOF). Prospectively, we enrolled 42 consecutive adults with TOF (28 patients with repaired TOF were not on ARB, 8 patients with repaired TOF on ARB, 6 patients with no repair or only palliative repair) and 20 age-matched healthy controls. We measured CAVI, baPWV, plasma TGF-ß1 level and a diameter of the ascending aorta using echocardiography. The mean age of repaired TOF without ARB were 31.9 ± 9.1 years. An aortic diameter of the ascending aorta, CAVI, and plasma TGF-ß1 level were significantly higher in repaired TOF without ARB than those in controls, whereas baPWV did not differ. On a univariate analysis, CAVI, plasma TGF-ß1 level and Rastelli procedure were important factors for an aortic diameter of the ascending aorta (r = 0.56, P < 0.01, r = 0.59, P < 0.01, r = 0.39, P < 0.05, respectively) in this population. There was no significant correlation with age, baPWV, NT-pro brain natriuretic peptide (BNP), repair age, shunt duration, aortic regurgitation or right aortic arch. On the other hand, patients with unrepaired or palliative TOF, who had obvious volume overload, showed no significant correlation with CAVI, baPWV or plasma TGF-ß1 level. CAVI and plasma TGF-ß1 level, not baPWV, correlate to aortopathy in adults with repaired TOF.

4D flow MRI-derived energy loss and RV workload in adults with tetralogy of Fallot.

PURPOSE: To assess flow energy loss (EL) pattern inside the pulmonary circulation in adult patients with repaired tetralogy of Fallot (TOF), particularly in TOF with pulmonary stenosis (PS) and pulmonary regurgitation (PR), as a cardiac workload parameter and its relationship to symptoms and major adverse cardiovascular events (MACE). METHODS: Prospectively, 51 consecutive TOF adults after intracardiac repair, who underwent four-dimensional flow magnetic resonance imaging, were enrolled. All of them had significant PR (PR regurgitant fraction >25 %). TOF patients who had already reached the conventional criteria were excluded. We defined MACE as the following: 1) fatal arrhythmias, 2) sudden cardiac death, 3) surgical pulmonary valvular repair (PVR), 4) right heart failure (HF) needing diuretics and/or hospitalization within 2 years. RESULTS: A total of 15 patients had MACE; 1) 10 patients underwent PVR within 2 years, 2) 2 patients had ventricular tachycardia, and 3) 6 patients developed right HF (overlapped). Right ventricular (RV) end diastolic volume index (EDVI), RV end systolic volume index (ESVI), average EL/cardiac output (CO), and diastolic EL/CO in patients with MACE were greater than ones without MACE. On a multivariate logistic analysis, the diastolic EL/CO ratio and RVEDVI had the highest odds with MACE in all TOF (odds ratio, 40.7 and 1.15. 95%CI, 1.83-905 and 1.02-13.0; p-value, 0.02 and 0.03). In sub-analysis within 29 patients with moderate PS with PR, and 10 patients with MACE showed higher diastolic EL/CO. Average and diastolic EL/CO negatively correlated with RV ejection fraction (EF) in this sub-analysis. CONCLUSIONS: High EL, particularly, high diastolic EL/CO, were the important factors for MACE in adult TOF. Higher diastolic EL/CO was also related to lower RV EF and deteriorated RV function in adult TOF with PS and PR. Right-sided EL can be a sensitive marker of excessive cardiac workload which integrates both afterload and preload in adult patients with TOF, beyond the RV size.

Mitral complex geometric changes aggravate mitral stenosis after transcatheter aortic valve replacement.

Severe aortic valve stenosis (AS) often coexists with mitral valve stenosis (MS). MS aggravation after transcatheter aortic valve replacement (TAVR) is common, and its etiology is multifactorial. We hypothesized that geometric changes in the mitral complex (mitral valvular and annular deformities) are adjunctive factors aggravating MS after TAVR, particularly in older adults with a smaller left ventricle (LV). This study aimed to evaluate the mitral complex geometric changes before and after TAVR and to assess the important predictors of MS aggravation after TAVR. This retrospective study enrolled consecutive adult patients who underwent TAVR and surgical AVR (SAVR) for severe AS. The mitral valve area (MVA), the angle between the anterior mitral valve leaflet (AMVL) and left ventricular outflow tract (LVOT), AMVL length, mitral annular diameter, presence of mitral annular calcification, and LV size were evaluated using transthoracic echocardiography. This study included 258 patients who underwent TAVR and SAVR. MVA index decreased from 2.3 ± 0.6 cm² to 1.9 ± 0.5 cm² in the TAVR group. The angle between the AMVL and LVOT was 56.3 ± 9.7° preoperatively and increased to 67.3 ± 11.5° after TAVR. In multivariate analysis, the most important predictive factors of MS aggravation after TAVR were a smaller mitral annular diameter, restricted AMVL mobility, and implantation depth (odds ratio: 4.5, 5.3,3.0; 95% confidence interval: 1.6-14, 1.9-17, 1.0-8.9; and p = 0.005, p = 0.001, p = 0.042, respectively). The reduction in MVA after TAVR was related to the restriction of AMVL opening, depth of implantation and narrowing of the mitral annulus.

Quantitative flow ratio versus fractional flow reserve for Heart Team decision-making in multivessel disease: the randomised, multicentre DECISION QFR trial.

BACKGROUND: Vessel-level physiological data derived from pressure wire measurements are one of the important determinant factors in the optimal revascularisation strategy for patients with multivessel disease (MVD). However, these may result in complications and a prolonged procedure time. AIMS: The feasibility of using the quantitative flow ratio (QFR), an angiography-derived fractional flow reserve (FFR), in Heart Team discussions to determine the optimal revascularisation strategy for patients with MVD was investigated. METHODS: Two Heart Teams were randomly assigned either QFR- or FFR-based data of the included patients. They then discussed the optimal revascularisation mode (percutaneous coronary intervention [PCI] or coronary artery bypass grafting [CABG]) for each patient and made treatment recommendations. The primary endpoint of the trial was the level of agreement between the treatment recommendations of both teams as assessed using Cohen's kappa. RESULTS: The trial included 248 patients with MVD from 10 study sites. Cohen's kappa in the recommended revascularisation modes between the QFR and FFR approaches was 0.73 [95% confidence interval {CI} : 0.62-0.83]. As for the revascularisation planning, agreements in the target vessels for PCI and CABG were substantial for both revascularisation modes (Cohen's kappa=0.72 [95% CI: 0.66-0.78] and 0.72 [95% CI: 0.66-0.78], respectively). The team assigned to the QFR approach provided consistent recommended revascularisation modes even after being made aware of the FFR data (Cohen's kappa=0.95 [95% CI:0.90-1.00]). CONCLUSIONS: QFR provided feasible physiological data in Heart Team discussions to determine the optimal revascularisation strategy for MVD. The QFR and FFR approaches agreed substantially in terms of treatment recommendations.

Percutaneous aspiration embolectomy is beneficial for pulmonary thromboembolism in adult patients with Fontan circulation.

In patients with acute pulmonary thromboembolism (PTE), increased pulmonary vascular resistance (PVR) due to thrombus sometimes causes hemodynamic instability, requiring reperfusion therapy with drugs, surgery, or catheterization. In particular, patients with Fontan circulation, which is strongly affected by PVR, are prone to hemodynamic instability. Moreover, these patients sometimes have bleeding complications such as hemoptysis and intrathoracic adhesions, following multiple prior thoracotomies, making it difficult to choose pharmacotherapy. Percutaneous aspiration embolectomy (PAE) is a useful treatment option because it minimizes bleeding complications, is less invasive, and more rapid than surgery, and is easy to perform. Herein, we report two cases of Fontan patients having PTE treated with PAE. In Case 1, a 21-year-old man with a history of a Fontan procedure suddenly developed acute PTE, for which anticoagulants were administered immediately. However, his condition was refractory to treatment and he underwent PAE on the third day of illness. In Case 2, a 28-year-old woman with a history of Fontan procedure who had been on anticoagulants, developed acute PTE on the ninth postpartum day, and underwent PAE on the day of onset. In both cases, the respiratory condition improved, and re-treatment for PTE was not required. Learning objective: Fontan patients with acute pulmonary thromboembolism often require reperfusion therapy because they can easily become hemodynamically unstable due to increased pulmonary vascular resistance. For them, who often have adhesions following multiple prior thoracotomies and bleeding complications, percutaneous aspiration embolectomy may be effective as it has minimal bleeding risk and is minimally invasive, rapid, and straightforward.

Characteristics of the aortic root morphology in conotruncal anomaly of the congenital heart disease.

BACKGROUND: Aortopathy is a well-known feature of conotruncal anomalies, but it remains unknown whether valve-sparing aortic root replacement, such as the David procedure, is feasible in young patients with severe aortic regurgitation. We assessed the aortic valve complex and aortic root morphology in patients with conotruncal anomalies using echocardiography. Furthermore, we evaluated the relevant factors associated with aortopathy in this population. METHODS: A total of 172 adult patients with conotruncal anomalies were enrolled in this study. Dimensions of the aortic valve complex were measured at the level of the sinus of Valsalva (SV) and sinotubular junction (STJ). The geometric height (GH), effective height (EH), and coaptation length (CL) were also assessed to analyze the aortic valve complex in detail. RESULTS: Sixteen of 172 patients were excluded due to poor imaging; 105 patients with tetralogy of Fallot, 24 with double outlet right ventricle, and 27 with transposition of the great arteries totaling 156 patients (32+/-11 years old) were included in the analysis. The patients were divided into four groups: Group 1 (98 patients) had no dilatation of SV or STJ; Group 2 (32 patients) had dilated SV and STJ; Group 3 (14 patients) had dilated SV; and Group 4 (12 patients) had dilated STJ. GH and EH in Group 2 were also highest among the four, whereas CL was not significantly shortened. Multivariate analysis revealed that male sex, age, and conduit repair were risk factors for aortopathy in this population. CONCLUSIONS: Patients with dilated SV and STJ (Group 2) were the most common among the patients with aortopathy (Groups 2, 3, and 4). The aortic valve leaflets themselves were enlarged, and the poor coaptation of the valve tips was compensated in spite of aortic root dilatation, which plays an important role in preventing severe aortic regurgitation in this population. Overall, valve-sparing aortic valve replacement is more feasible in the young populations than we expected.

Heart Team risk assessment with angiography-derived fractional flow reserve determining the optimal revascularization strategy in patients with multivessel disease: Trial design and rationale for the DECISION QFR randomized trial.

In patients with multivessel disease (MVD), functional information on lesions improves the prognostic capability of the SYNTAX score. Quantitative flow ratio (QFR®) is an angiography-derived fractional flow reserve (FFR) that does not require a pressure wire or pharmacological hyperemia. We aimed to investigate the feasibility of QFR-based patient information in Heart Teams' discussions to determine the optimal revascularization strategy for patients with MVD. We hypothesized that there is an acceptable agreement between treatment recommendations based on the QFR approach and recommendation based on the FFR approach. The DECISION QFR study is a prospective, multicenter, randomized controlled trial that will include patients with MVD who require revascularization. Two Heart Teams comprising cardiologists and cardiac surgeons will be randomized to select a revascularization strategy (percutaneous coronary intervention or coronary artery bypass graft) according to patient information either based on QFR or on FFR. All 260 patients will be assessed by both teams with reference to the anatomical and functional SYNTAX score/SYNTAX score II 2020 derived from the allocated physiological index (QFR or FFR). The primary endpoint of the trial is the level of agreement between the treatment recommendations of both teams, assessed using Cohen's κ. As of March 2022, the patient enrollment has been completed and 230 patients have been discussed in both Heart Teams. The current trial will indicate the usefulness of QFR, which enables a wireless multivessel physiological interrogation, in the discussions of Heart Teams to determine the optimal revascularization strategy for MVD.

Secondary sarcopenia assessed by computed tomography can predict hospitalization for heart failure in adults with Fontan circulation.

BACKGROUND: Sarcopenia is one of the important predictors of heart failure (HF) in patients with cardiac problems. Skeletal muscle pump is important to maintain Fontan circulation. We aimed to quantify the volume of the psoas major muscle (PMM) and investigate whether it is relevant to HF hospitalization in adults with Fontan circulation. METHODS: A total of 154 adults with Fontan circulation, and 30 age-matched adults were studied retrospectively. We identified secondary sarcopenia by measuring the PMM volume, using non-contrast abdominal computed tomography. RESULTS: PMM indexed volume in Fontan patients was lower than controls (206.4+/-18.0 cc/m2 vs 288.0+/-61.1 cc/m2, p < 0.0001). In Fontan men, PMM indexed volume correlated with New York Heart Association functional class (NYHA FC). PMM indexed volume in Fontan women correlated with age, NYHA FC, and serum creatinine. Using a multivariate Cox hazards analysis, NYHA FC ≧3 was an important predictor of hospitalization due to HF in Fontan men. In Fontan women, NYHA FC ≧3, brain natriuretic peptide, PMM indexed volume, and protein losing enteropathy were important predictions of hospitalization due to HF. Among all Fontan patients, those with low PMM volumes had a poorer prognosis in HF [log rank p = 0.012 (men) and 0.0009 (women)]. CONCLUSIONS: PMM volume reduction has an adjunctive prediction of HF hospitalization in adults with Fontan circulation, particularly in Fontan females. Secondary sarcopenia may have a negative impact on the prognosis of HF in this population. The assessment of skeletal muscle mass also may be a comprehensive screening tool for multi-organ dysfunction in Fontan circulation.

Potential of Liver T1 Mapping for the Detection of Fontan-associated Liver Disease in Adults.

PURPOSE: The native T1 value at 3T MRI is a sensitive marker for diffuse fibrosis or damage in various organs including the heart, liver, and pancreas. Despite the fact that Fontan-associated liver disease (FALD) is a crucial issue in adults with Fontan circulation, there are only a few studies with liver T1 mapping in children and adolescents. We investigated the potential of the liver native T1 mapping in detecting FALD in adult patients. METHODS: We prospectively enrolled 16 consecutive adults with Fontan circulation (age 31.3 ± 8.5 years), who were in New York Heart Association Functional class II-IV. Twenty with tetralogy of Fallot (TOF), and 20 age-matched controls also underwent cardiac magnetic resonance (CMR) imaging at 3T. Myocardial T1 mapping with a Modified Look-Locker Inversion recovery sequence was applied to liver T1 mapping. Patients in the Fontan group underwent the right heart catheter and liver function tests, including those for fibrotic markers. RESULTS: Liver native T1 values in the Fontan group were significantly higher than that in TOF and controls (P < 0.001). In the Fontan group, the liver native T1 value was significantly correlated with age, γ-glutamyltransferase, model for end-stage liver disease XI score, and albumin-bilirubin score (P = 0.01, 0.01, 0.044, 0.001). However, it demonstrated no correlation with central venous pressure, pulmonary vessel resistance, or fibrotic markers. CONCLUSION: Liver native T1 value derived from CMR may be a non-invasive adjunctive and/or screening marker to detect FALD.