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Background In complex congenital heart disease, supraventricular arrhythmia develops long term after surgical repair. The arrhythmia could have pharmacological tolerance and sometimes be fatal. We report our experiences with puncturing tough or calcified surgical repair material to access the pulmonary venous atrium for the purpose of the management of arrhythmia in complex congenital heart disease with surgical correction. Methods From June 2016, subsequent 9 patients underwent the procedure. Their age at the procedure ranged from 11 to 43 years old (median 26.4 years old). Surgical procedures were Mustard procedure (XenomedicaⓇ) in 6, lateral tunnel total cavo-pulmonary connection with autologous pericardium in 2, and extra-cardiac total cavo-pulmonary connection with expanded polytetrafluoroethylene (ePTFE) tube in 1. Results Multi-planar reconstruction imaging by cardiac computed tomography was done for making plans before the procedures. Under intracardiac echocardiography guidance, an 8 French steerable sheath was perpendicularly adjusted to target objects. The sharpened edge of 0.014 inch guide wire penetrated with gentle forward force. Subsequently, the puncture site was dilated from a small balloon to a large one step by step. In all patients, penetration was successfully completed. Catheter ablation was achieved in 8 patients and a pacemaker was implanted through the re-canalized superior caval vein in 1 patient. No complications were recorded. Conclusion The trans-catheter puncture of the synthetic or calcified material is safe and feasible, although careful planning is required with imaging. A steerable sheath could easily be controlled to the make appropriate angle to the target object for preventing slippage and conveying effective force.

Recently, targeted therapy has been developed for idiopathic pulmonary arterial hypertension (IPAH). Studies evaluating the prognosis of IPAH have been conducted in adults. However, there is no nationwide survey of pediatric patients with IPAH regarding the long-term prognosis in Japan. Therefore, we investigated the clinical outcomes of Japanese pediatric patients with IPAH and risk factors for a poor prognosis. This multi-center, retrospective cohort study included pediatric patients with IPAH under the age of 15 years, who were gleaned from the nationwide network of Japanese Society of Pediatric Cardiology and Cardiac Surgery (JSPCCS). The questionnaire was sent to members of JSPCCS in 2015. Patients who were diagnosed with IPAH from 1994 to 2014 were included. The primary endpoint was death or lung transplantation. Ninety-five patients were finally enrolled. Both the mean age at diagnosis and the mean follow-up duration were 7 years. Ninety-five percent of patients had received targeted therapy for IPAH during follow-up. The overall 1, 3, 5, and 10-year event free rate, estimated using Kaplan-Meier survival estimate, was 96, 91, 83, and 74%, respectively. The prognosis was significantly poorer in patients with increased right ventricular systolic pressure (RVp), mean pulmonary artery pressure (mPAP) (≥ 52 mmHg), cardiothoracic ratio (≥ 55%), and levels of B-type natriuretic peptide (BNP) during follow-up (≥ 300 pg/mL) than in those without these parameters. In conclusion, in Japanese children with IPAH, the event-free rate for death or lung transplantation was found to be good. Greater RVp, mPAP, BNP levels during follow-up, and cardiothoracic ratio may be predictive indicators for a poor prognosis.

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

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.

Although arterial switch operations (ASOs) have been performed globally to repair d-transposition of the great arteries (d-TGA) in neonates and infants, few studies have been reported regarding the influence of the hemodynamics of patients with d-TGA who have undergone ASO on the development of neo-aortic valve regurgitation (AR). We aimed to investigate the relationship between the hemodynamics and development of AR after ASO in patients with d-TGA by catheter evaluation. This observational study screened 114 consecutive patients who underwent ASO for d-TGA or Taussig–Bing anomaly and who subsequently underwent catheter evaluations in our institution. We reviewed their records for the past 20 years and collected their first catheterization data post-ASO in early childhood. Thirty-six post-ASO patients who underwent catheter evaluations in both the early surgical and long-term phases were finally analyzed. Patients were divided into the following groups according to the presence of significant AR in the long-term phase: the AR group (n = 9 with AR ≥ grade II by the Sellers classification) and the non-AR group (n = 27 with AR

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

A wide variety of pathologies are involved in heart failure in patients with congenital heart disease (CHD). Specific causes of heart failure after the Fontan procedure include not only single-ventricle circulation, but also the function of the right ventricle as the systemic ventricle, atrioventricular or semilunar valve stenosis or regurgitation, pulmonary hypertension, and left ventricular dysfunction secondary to right ventricular enlargement or dysfunction. As heart failure can occur post-Fontan, for a variety of reasons, clarification of the pathophysiology is the first step in management and treatment. At the same time, it is important to understand each patient's current condition and treatment plan to make an accurate prognosis. Because of the wide variety of pathophysiologies in post-Fontan CHD patients, however, no single biomarker is useful in all situations. Relevant biomarkers must be selected according to each patient's disease state, and combinations of multiple biomarkers should also be considered. In this review, the author describes the clinical importance of various biomarkers for patients who have undergone a Fontan procedure.

OBJECTIVES Patients who have achieved Fontan circulation may require reoperation. We reviewed the outcomes of reoperation after Fontan completion and assessed the risk factors for poor outcomes. METHODS This was a retrospective study of 106 patients undergoing open-heart reoperations after Fontan completion in 2003 at a single institution. RESULTS The mean age at reoperation was 24.6 ± 8.3 years. A history of Fontan failure or end-organ dysfunction was noted in 30 patients. The reoperations included 73 total cavopulmonary connection conversions, 29 atrioventricular or semilunar valve operations (17 with total cavopulmonary connection conversions) and 4 other operations. Eight early deaths occurred. During a median follow-up of 5.5 (0.01–16.2) years, there were 3 late deaths and 9 second cardiac operations. The 10-year survival rate after reoperation was 89.8%, and the 5-year second cardiac operation-free survival was 84.3%. The 10-year survival rates were significantly lower in patients who underwent surgery before 2011 (75.8% vs 100%), had a history of Fontan failure or end-organ dysfunction (71.7% vs 97.3%), had preoperative central venous pressure >15 mmHg (64.9% vs 96.5%) and were operated on with deep hypothermic circulatory arrest (DHCA) (60.0% vs 91.3%). A history of Fontan failure or end-organ dysfunction, preoperative central venous pressure >15 mmHg and requirement of DHCA were identified as risk factors for mortality. CONCLUSIONS Reoperation after Fontan completion resulted in excellent mid-term outcomes. A history of failed Fontan circulation and the requirement of DHCA negatively affected survival outcomes.

Background:Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.Methods:In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnea using a type-3 portable overnight polygraph in adult patients with congenital heart disease were evaluated.Results:There were 104 patients [median age: 36 (interquartile range: 28–48) years] who were admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37%, 16%, and 10% for mild (5≤ respiratory disturbance index Conclusion:Our results showed a high prevalence of sleep apnea in adult patients with congenital heart disease. Its unique haemodynamics may be associated with a high prevalence of sleep apnea. Congestive heart failure and being overweight are important risk factors for sleep apnea. Management of heart failure and general lifestyle improvements will be important for controlling sleep apnea symptoms in these patients.

Purpose Dilated aortic root and ascending aorta (AAO) with progressive aortic regurgitation is a well-known sequela after arterial switch operation (ASO) in adults with transposition of the great arteries (TGA). We aimed to quantitatively assess aortic flow profiles in adults with TGA after ASO (Jatene procedure with LeCompte maneuver) using echo planar imaging (EPI) 4D flow MRI. Methods Prospectively, 9 consecutive adults (30.2 ± 6.6 years) after ASO (Jatene operation with LeCompte technique), 13 consecutive adults (34.3 ± 7.2 years) after the atrial switch operation with Senning procedure, and 8 age-matched control patients, who underwent turbo field echo (TFE) EPI 4D flow MRI (average scan time of approximately 4 min), were enrolled. Results TGA after ASO showed a markedly dilated sinus of Valsalva, compared to TGA after atrial switch operation (26.6. ± 4.9 vs. 18.6. ± 1.5 mm/cm2). Vorticity, helicity, wall share stress (WSS), and energy loss (EL) in the aortic root and the AAO in TGA were greater than in the controls. Vorticity, helicity, WSS, and EL in the aortic root and the AAO were also greater in TGA after ASO than after atrial switch operation. More acute aortic arch angle correlated with greater vorticity of the aortic root, and the significant diameter ratio of the sinus of Valsalva and the AAO was relevant to greater vorticity, helicity, and EL in TGA after ASO. Conclusions A non-physiological blood flow pattern of the aortic root was identified in TGA adults after the ASO (Jatene procedure with LeCompte maneuver). Missing spiral looping of the great arteries and the unique structure after the Jatene procedure may play an adjunctive role in promoting aortopathy. The evaluation of aortic flow profile using EPI 4D flow MRI may be useful for risk stratification for aortopathy in this population.

We used 4D-flow MRI to investigate circulation, an area integral of vorticity, in the main pulmonary artery (MPA) as a new hemodynamic parameter for assessing patients with a repaired Tetralogy of Fallot (TOF). We evaluated the relationship between circulation, right ventricular (RV) function and the pulmonary regurgitant fraction (PRF). Twenty patients with a repaired TOF underwent cardiac MRI. Flow-sensitive 3D-gradient sequences were used to obtain 4D-flow images. Vortex formation in the MPA was visualized, with short-axis and longitudinal vorticities calculated by software specialized for 4D flow. The RV indexed end-diastolic/end-systolic volumes (RVEDVi/RVESVi) and RV ejection fraction (RVEF) were measured by cine MRI. The PR fraction (PRF) and MPA area were measured by 2D phase-contrast MRI. Spearman ρ values were determined to assess the relationships between circulation, RV function, and PRF. Vortex formation in the MPA occurred in 15 of 20 patients (75%). The longitudinal circulation (11.7 ± 5.1 m2/s) was correlated with the RVEF (ρ = − 0.85, p = 0.0002), RVEDVi (ρ = 0.62, p = 0.03), and RVESVi (ρ = 0.76, p = 0.003) after adjusting for the MPA size. The short-axis circulation (9.4 ± 3.4 m2/s) in the proximal MPA was positively correlated with the MPA area (ρ = 0.61, p = 0.004). The relationships between the PRF and circulation or RV function were not significant. Increased longitudinal circulation in the MPA, as demonstrated by circulation analysis using 4D flow MRI, was related to RV dysfunction in patients with a repaired TOF.

The heart murmur associated with atrial septal defects is often faint and can thus only be detected by chance. Although electrocardiogram examination can prompt diagnoses, identification of specific findings remains a major challenge. We demonstrate improved diagnostic accuracy realized by incorporating a proposed deep learning model, comprising a convolutional neural network (CNN) and long short-term memory (LSTM), with electrocardiograms. This retrospective observational study included 1192 electrocardiograms of 728 participants from January 1, 2000, to December 31, 2017, at Tokyo Women's Medical University Hospital. Using echocardiography, we confirmed the status of healthy subjects—no structural heart disease—and the diagnosis of atrial septal defects in patients. We used a deep learning model comprising a CNN and LTSMs. All pediatric cardiologists (n = 12) were blinded to patient groupings when analyzing them by electrocardiogram. Using electrocardiograms, the model’s diagnostic ability was compared with that of pediatric cardiologists. We assessed 1192 electrocardiograms (828 normally structured hearts and 364 atrial septal defects) pertaining to 792 participants. The deep learning model results revealed that the accuracy, sensitivity, specificity, positive predictive value, and F1 score were 0.89, 0.76, 0.96, 0.88, and 0.81, respectively. The pediatric cardiologists (n = 12) achieved means of accuracy, sensitivity, specificity, positive predictive value, and F1 score of 0.58 ± 0.06, 0.53 ± 0.04, 0.67 ± 0.10, 0.69 ± 0.18, and 0.58 ± 0.06, respectively. The proposed method is a superior alternative to accurately diagnose atrial septal defects.

Introduction: Sarcopenia is one of the important predictors of heart failure (HF) in patients with cardiac problems. Hypothesis: Skeletal muscle pump is very 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 (CT). 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 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.

Introduction and hypothesis: Flow hemodynamic parameters may relate to aortopathy in adults with tetralogy of Fallot. We aimed 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. Methods: Prospectively, 51 consecutive adults with TOF underwent 4 dimensional flow magnetic resonance imaging study for the assessment of vorticity and helicity of in 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 dimeter>25mm/cm2), Group A (15 patients); non-dilated aortic patients, Group B (36 patients). Ten age matched controls were also enrolled. Results: Group A showed acute LVOT-Ao angle, higher incidence of aortic regurgitation, and initial anatomy of pulmonary atresia, compared to Group B (P Conclusions: TOF Adults with TOF presenting dilated AAo have greater vorticity, helicity, and acute LVOT-Ao angle. Flow eccentricity correlates with vorticity and helicity, which may affect the aortopathy in adults with TOF. AAo vorticity and helicity are also relevant factors of aortic dilatation, and these flow hemodynamic parameters may be adjunctive predictions of aortopathy in this population.

Cardiomyocytes derived from induced pluripotent stem cells (iPSC) are used to evaluate the function and risk factors of genetic variations linked to congenital and adult heart diseases in human beings. Our purpose in this study was to establish an in vitro cardiac disease model from an immortalized B-cell line stock using patient-specific iPSC-derived cardiomyocytes. Long QT syndrome (LQTS) 3 is caused by a gain-of-function mutation in the SCN5A gene, resulting in a prolonged QT interval as well as the development of early after-depolarizations (EADs). We generated LQT3-iPSC lines from immortalized B-cell lines transformed from peripheral blood obtained from a 24-day-old male (patient #1) and an 11-year-old female (patient #2), both diagnosed with type-3 LQTS due to SCN5A missense mutation (patient #1: exon 28, G4868A, R1623Q; patient #2: exon 20, G3578A, R1193Q). Several control iPSC lines were also created from samples collected from healthy candidates (26–36-year-old male and female). In both the generated patient iPSC lines, mutated genes were not altered after reprogramming, and normal karyotypes were observed. The pluripotency of all these iPSC lines was proven by in vivo teratoma formation and the expression of pluripotent stem cell marker genes and proteins. To evaluate the electrophysiological properties at the multicellular level, we studied the iPSC-derived cardiac cell layers with a microelectrode array mapping system (MED 64, Alpha MED Scientific Inc.). The recorded extracellular electrograms were analyzed to measure the field-potential duration (FPD), which was normalized to account for variations in beating frequency, using Fridericia’s correction (FPDcF, analogous to the QTc interval in the electrocardiogram). FPDcF was observed to be longer in LQT3 patient-derived specimens when compared to that in healthy control specimens (patient #1: P < 0.05, N = 17 and patient #2: P < 0.0001, N = 19 when compared to healthy control N = 39). Moreover, EADs were observed in both LQT3 patient specimens after 15–30 nM of E-4031 was added exclusively to patient samples. The results indicated that LQT3 patient iPSC-derived cardiac cells expressed prolonged QT duration (a common pathophysiological feature of LQTS) and EAD development with marked arrhythmogenicity in our in vitro disease model system.

BackgroundAlthough sleep apnoea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with CHD are limited.MethodsIn this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnoea using a type 3 portable overnight polygraph in adult patients with CHD were evaluated.ResultsThis study comprised 104 patients of median age 36 years with interquartile range of 28–48 years, admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnoea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37, 16, and 10% for mild (5≤ respiratory disturbance index ConclusionOur results showed a high prevalence of sleep apnoea in adult patients with CHD. Its unique haemodynamics may be associated with a high prevalence of sleep apnoea. Congestive heart failure and being overweight are important risk factors for sleep apnoea. Management of heart failure and general lifestyle improvements are important for controlling sleep apnoea symptoms in these patients.

The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]), laboratory values, and HF-related admission prevalence.Patients with New York Heart Association (NYHA) functional classes III-IV had a higher EI than those with NYHA classes I-II (mean ± SD, 0.398 ± 0.011 vs 0.384 ± 0.017, P .001). EI was significantly correlated with brain natriuretic peptide level (r = 0.51, P .001). During the mean follow-up period of 7.1 months, Kaplan-Meier analysis showed that a discharge EI 0.386, the median value in the present study, was significantly associated with a future increased risk of HF-related admission (HR = 4.15, 95% CI = 1.70－11.58, P .001). A body weight reduction during hospitalization was also related to EI reduction.EI determined using BIA could be a useful marker for HF severity that could predict future HF-related admissions in adult patients with CHD.

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)

Aim We investigated the clinical courses before and during pregnancy and after delivery in patients with repaired anomalous origin of the left coronary artery from the pulmonary artery to determine the impact of the hemodynamic changes and cardiac function on the selection of the appropriate mode of delivery. Methods Six patients who underwent coronary artery reimplantation delivered 10 infants. We scrutinized the patients' hemodynamic changes on echocardiographs and the plasma brain natriuretic peptide levels before and during pregnancy and after delivery, the perinatal outcomes and maternal and fetal events. Results All patients were asymptomatic and categorized as having New York Heart Association functional class I before pregnancy. In 8 of 10 pregnancies, vaginal deliveries were performed; two elective cesarean sections were performed because of symptomatic heart failure. The hemodynamic parameters were stable throughout pregnancy and postdelivery, and no maternal or fetal events occurred in the patients who underwent vaginal deliveries. One cesarean section patient developed significant heart failure during the late second and third trimesters, which was accompanied by hemodynamic changes, including increased brain natriuretic peptide levels, left ventricular diastolic dysfunction and worsening arrhythmias, and thrombosis and post-partum hemorrhage occurred postdelivery. The baby had intrauterine growth retardation and small for gestational age. None of the babies had congenital anomalies. Conclusion Pregnancy was safe in most of the asymptomatic patients long after anomalous origin of the left coronary artery from the pulmonary artery was repaired. Symptomatic heart failure might occur during pregnancy in patients with persisting myocardial damage. Pregnancy and delivery should be carefully managed.

Recent studies showed that a low ratio between the levels of eicosapentaenoic acid and those of arachidonic acid (EPA/AA) is associated with higher incidence of coronary artery disease and poor prognosis of heart failure, arrhythmia, and cardiac sudden death. However, the clinical implications of EPA/AA in adult patients with congenital heart disease remain unclear. We aimed to assess the prognostic value of EPA/AA regarding cardiac events in adult patients with congenital heart disease. We measured the serum levels of eicosapentaenoic acid and arachidonic acid in 130 adult patients (median age, 31 years) stratified into two groups according to their EPA/AA (low, ≤0.22; high, >0.22). We prospectively analyzed the association between EPA/AA and incidence of cardiac events during a mean observation period of 15 months, expressed in terms of hazard ratio (HR) with 95% confidence interval (95% CI). In the subgroup of patients with biventricular circulation (2VC) (n = 76), we analyzed the same clinical endpoints. In our study population, EPA/AA was not associated with the incidence of arrhythmic events (HR, 1.52; 95% CI, 0.82-2.85; p = 0.19), but low EPA/AA was a predictor of heart failure hospitalization (HR, 2.83; 95% CI, 1.35-6.30; p

Elevated right atrial (RA) pressure and progressive RA dilation are thought to play pivotal roles in the development of late complications after atriopulmonary connection (APC) Fontan surgery. However, no clear cut-off value for RA pressure or RA volume has been determined for stratifying the risk of developing Fontan complications. We hypothesized that RA tension, which incorporates information about both RA pressure and volume, might help predict the risk of developing complications. We retrospectively studied 51 consecutive APC Fontan patients (median postoperative period 14 years). RA tension was computed from the RA pressure and RA radius, which was calculated from RA volume measured by RA angiography. The correlation between the cardiac catheterization hemodynamic data and the complications of APC Fontan was investigated. Of the 51 patients, 28 had complications, including liver fibrosis (n = 28), arrhythmia (n = 8), protein-losing enteropathy (n = 1), and RA thrombosis (n = 1). Among the hemodynamic data, RA volume and RA tension, but not RA pressure, were significantly higher in patients with complications than in those without (P = 0.004 and P = 0.001, respectively). The cut-off level for RA tension to predict Fontan complications was 26,131 dyne/cm by receiver operating characteristic curve (area under the curve 0.79, sensitivity 71.4%, and specificity 73.9%). The present study demonstrated the significance of RA tension rather than high venous pressure for the development of Fontan complications. Amid the uncertainty about clinical outcomes, the present results, subject to further validation, may contribute to the indications for Fontan conversion.

Background Coagulation abnormality is associated with a high incidence of intracardiac thrombus (ICT) and systemic thromboembolism in Fontan patients. The biomarker for detecting ICT is currently unknown. Methods We retrospectively investigated the underlying coagulation abnormality and useful biomarkers to screen for ICT in adult Fontan patients. We measured various biomarkers of blood coagulation, fibrinolysis, and platelet activity in 122 Fontan patients (Fontan group: median age [P25–P75]: 27 [20–34] years) and compared them to those in 50 patients with atrial septal defect (ASD group: 31 [24–40] years). Results Regardless of whether the patient had ICT, the Fontan group showed significantly lower levels of antithrombin III, thrombomodulin, and α2-antiplasmin; lower protein C and protein S activities; and significantly higher levels of thrombin-antithrombin complex and α2-plasmin inhibitor complex than the ASD group. Among various biomarkers, D-dimer level measured by using latex immunoassay was significantly higher in the patients with ICT (thrombus group: n =21) than in the patients without ICT (non-thrombus group: n =101). Fifteen (26%) of 57 patients on warfarin achieved prothrombin time international normalized ratios (PT-INRs) of >2. The proportion of patients with PT-INRs of >2 tended to be lower in the thrombus group than in the non-thrombus group (13% vs 31%). Persistent atrial arrhythmia and D-dimer level were significant risk factors associated with ICT formation in the multivariate analysis (persistent atrial arrhythmia: hazard ratio [HR], 6.89; 95% confidence interval [CI], 1.44–34.5; D-dimer: HR, 0.29; 95% CI, 0.13–0.50). Receiver-operating characteristic curve analysis revealed that the appropriate cutoff D-dimer level for screening for ICT was 1.8μg/mL (area under the curve, 0.94), with a negative predictive value of 95%. Conclusions In the adult Fontan patients, blood coagulation abnormalities existed regardless of the absence of ICT. D-dimer level may be a useful biomarker for screening for ICT in adult Fontan patients.

Background Patients with congenital heart disease (CHD) reportedly have reduced exercise capacity. Underlying cardiac anatomy and a sedentary lifestyle are thought to be associated with exercise impairment. However, little has been reported regarding the relationship between quantitative body composition and exercise capacity. Bioelectrical impedance analysis (BIA) is a rapid and safe assessment method that has been widely used in clinical research. We hypothesized that bioelectrical impedance parameters are determinants of exercise capacity, and the skeletal muscle index (SMI) determined by BIA is a prognostic predictor in patients with CHD. Methods We conducted a retrospective single-center study of 305 consecutive patients with CHD [median age, 26 years (range 12–60]; 48% males] admitted between 2014 and 2017. The BIA parameters were reviewed, including the edema index (EI, extracellular water to total body water ratio), SMI (skeletal muscle mass/height2), mineral index (MI, mineral mass/height2), percent body fat (%BF), and exercise capacity [peak oxygen uptake (peakVO2)] via a cardiopulmonary exercise test. Results The multivariate analysis revealed a significant correlation between peakVO2 and EI (r = 0.55) and peakVO2 and SMI (r = 0.49). The receiver operating characteristic curve analysis showed that the EI cut-off for peakVO2 Conclusions SMI determined by BIA is a determinant of exercise capacity and can be used as a prognostic predictor in patients with CHD.

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.

To investigate the relationship between the extracellular volume fraction (ECV) measured using cardiac magnetic resonance (CMR) T1 mapping and cardiac events in symptomatic adults with tetralogy of Fallot (TOF).A total of 60 consecutive symptomatic adults (35.4±13.8 years old) with repaired TOF who were in New York Heart Association functional class 2-4 were prospectively enrolled. Native T1 values and ECV of the free walls of the right ventricle (RV), the left ventricle, and the ventricular septum were obtained by CMR T1 mapping using a 3.0T scanner and the saturation recovery method. Correlations between T1 mapping measurements, conventional parameters, and predictors of adverse cardiac events were analyzed. The patients with episodes of only atrial arrhythmia were excluded.Significant correlations were observed between RV functional parameters, RV-ECV, and septum-ECV. Follow-up period was 747.1±315 days and 13 patients had cardiac events: Acute heart failure and/or sustained ventricular tachycardia occurred in 8 patients and 5 patients underwent cardiac surgery. Cox-hazard analysis revealed that septum-ECV and RV-ECV were predictors of adverse events (hazard ratio of septum-ECV and RV-ECV: 1.41 and 1.19, 95% CI: 1.05-1.89, 1.004-1.41 with p-values of 0.02, and 0.045, respectively). Adults with septum-ECV29.0% and RV ejection fraction (EF)45.0% were more likely to experience cardiac events (log rank test: p0.043).RV-ECV and septum-ECV correlate with RV functional parameters. Biventricular ECVs can be predictors of adverse cardiac events in adults with TOF. In particular, the combination of septum-ECV and RVEF was a useful predictor, compared to the use of a single CMR parameter.

Various late complications are associated with the Fontan procedure. In patients who undergo the Fontan procedure, the central venous pressure (CVP) tends to be higher than normal. However, the relationship between CVP (at rest and during exercise) and late complications associated with the Fontan procedure is unknown. Thirty-four patients who underwent the Fontan procedure were enrolled in this study. The median age was 19.3 years, and the median time after the Fontan procedure was 12.7 years. With exercise, the CVP increased from a median of 11 to 19 mmHg, and the cardiac index increased from a median of 2.1 to 4.4 l/min/m2. In 38% of the patients, CVP measured at the resting condition and during exercise differed. Laboratory results indicated no significant difference between the patients in the high-CVP group and low-CVP group at rest. In contrast, during exercise, brain natriuretic peptide (67 ± 38 vs. 147 ± 122 pg/ml, p

To assess ventricular function and dyssynchrony using three-dimensional (3D) computed tomography (CT) strain in adult congenital heart disease (ACHD). We prospectively analyzed a multiphase cardiac CT data set for 22 adult patients with CHD, including 8 patients with congenital systemic right ventricle (RV) and 14 patients with repaired Tetralogy of Fallot (TOF). Eight patients had a cardiac pacemaker. Volume of Interest was drawn on a multiplanar reconstruction of the ventricle with strain overlay using a 3D-strain algorithm. Ventricular strain, inter- and intraventricular dyssynchrony, and right ventricle outflow tract (RVOT)–apex dyssynchrony were calculated. RVOT–apex dyssynchrony by ventriculography was also compared in 15 patients. Pulmonary ventricular strain, systemic ventricular strain, and septal wall strain were lower in ACHD patients than in the controls, and lower in the ACHD with pacing group than without pacing group as well. Maximum interventricular time difference and intraventricular time difference were longer than in ACHD than in the controls, and longer in the ACHD with pacing group than without pacing group as well. RVOT–apex delay was significantly longer in patients with a pacemaker than in those without a pacemaker (118.1 ± 31.9 ms vs. 76.1 ± 36.2 ms, p = 0.03). RVOT delay determined by 3D CT strain significantly correlated with that determined by ventriculography (Pearson r = 0.55, p = 0.03). 3D CT strain can detect reduced biventricular contraction and inter- and intraventricular and RVOT–apex mechanical dyssynchrony can be assessed in patients with ACHD.

Although mutations in several genes have been reported in pulmonary arterial hypertension (PAH), most of PAH cases do not carry these mutations. This study aimed to identify a novel cause of PAH. To determine the disease-causing variants, direct sequencing and multiplex ligation-dependent probe amplification were performed to analyze 18 families with multiple affected family members with PAH. In one of the 18 families with PAH, no disease-causing variants were found in any of BMPR2, ACVRL1, ENG, SMAD1/4/8, BMPR1B, NOTCH3, CAV1, or KCNK3. In this family, a female proband and her paternal aunt developed PAH in their childhood. Whole-exome next-generation sequencing was performed in the 2 PAH patients and the proband’s healthy mother, and a BRCA1-associated protein (BRAP) gene variant, p.Arg554Leu, was identified in the 2 family members with PAH, but not in the proband’s mother without PAH. Functional analyses were performed using human pulmonary arterial smooth muscle cells (hPASMCs). Knockdown of BRAP via small interfering RNA in hPASMCs induced p53 signaling pathway activation and decreased cell proliferation. Overexpression of either wild-type BRAP or p.Arg554Leu-BRAP cDNA constructs caused cell death confounding these studies, however we observed higher levels of p53 signaling inactivation and hPASMC proliferation in cells expressing p.Arg554Leu-BRAP compared to wild-type BRAP. In addition, p.Arg554Leu-BRAP induced decreased apoptosis of hPASMCs compared with wild-type BRAP. In conclusion, we have identified a novel variant of BRAP in a Japanese family with PAH and our results suggest it could have a gain-of-function. This study sheds light on new mechanism of PAH pathogenesis.

Although some studies have attempted to find useful prognostic factors in hypertrophic cardiomyopathy (HCM), those results are not fully helpful for use in actual clinical practice. Furthermore, several genetic abnormalities associated with HCM have been identified. However, the genotype-phenotype correlation in HCM remains to be elucidated. Here, we attempted to assess patients with different types of gene mutations causing HCM and investigate the prognosis. A total of 140 patients with HCM underwent a screening test for myofilament gene mutations by direct sequencing of eight sarcomeric genes. Patients with a single mutation in cardiac troponin T, cardiac troponin I, α-tropomyosin, and regulatory and essential light chains were excluded from the study because the number of cases was too small. The clinical presentations and outcomes of the remaining 127 patients with HCM, 31 β-myosin heavy chain (MYH7) mutation carriers, 19 cardiac myosin-binding protein C (MYBPC3) mutation carriers, and 77 mutation non-carriers were analyzed retrospectively. MYBPC3 mutation carriers had a high frequency of ventricular arrhythmia and syncope. Kaplan-Meier curves revealed no significant difference in prognosis among the three groups, but a lack of family history of sudden death (SD) and a past history of syncope were significantly related to poor prognosis. An absence of family history of SD and past history of syncope are useful prognostic factors in patients with HCM. MYH7 and MYBPC3 mutations did not significantly influence prognosis compared to non-carriers. However, patients with the MYBPC3 mutation should be closely followed for the possibility of SD.

Objective It has been recently reported that vitamin D deficiency may contribute to systemic illnesses that accompany chronic heart failure. These reports also suggest the serum levels of parathormone, which activates vitamin D in the liver, can be a useful marker of heart failure. This study was designed to evaluate the clinical implications of vitamin D and parathormone levels in patients with congenital heart diseases and chronic heart failure. Design We measured 25-hydroxyvitamin D and parathormone serum levels in 103 adult patients with congenital heart diseases (age range 20–89 years). Of 103 patients, 54 were in New York Heart Association functional classes II or III. Their clinical data regarding cardiothoracic ratio, fractional shortening of the systemic ventricle, brain natriuretic peptide plasma levels, and pulse oximetry were also evaluated. Results Of 54 patients with chronic heart failure, 50 (93%) exhibited vitamin D deficiency (25-hydroxyvitamin D serum levels 65 pg/mL). These two parameters were inversely correlated. In multivariate analyses including age, gender, 25-hydroxyvitamin D, parathormone, pulse oximetry, cardiothoracic ratio, calcium, phosphorus, glomerular filtration rate, albumin, creatine kinase, end-diastolic diameter and fractional shortening of the systemic ventricle, and ratio of early diastolic transmitral flow velocity to mitral annular velosity, only the parathormone serum levels (P

Objectives This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. Methods Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1–123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). Results The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. Conclusions In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.

Long QT syndrome (LQTS) can cause syncope, ventricular fibrillation, and death. Recently, several disease-causing mutations in ion channel genes have been identified, and compound mutations have also been detected. It is unclear whether children who are carriers of compound mutations exhibit a more severe phenotype than those with single mutations. Although predicting phenotypic severity is clinically important, the availability of prediction tools for LQTS is unknown. To determine whether the severity of the LQTS phenotype can be predicted by the presence of compound mutations in children is needed. We detected 97 single mutations (Group S) and 13 compound mutations (Group C) between 1998 and 2012, age at diagnosis ranging 0-19 years old (median age is 9.0) and 18.0 years of follow-up period. The phenotypes and Kaplan-Meier event-free rates of the two groups were compared for cardiac events. This study investigated phenotypic severity in relation to the location of mutations in the protein sequence, which was analyzed using two sequence homology-based tools. In results, compound mutations in children were associated with a high incidence of syncope within the first decade (Group S: 32 % vs. Group C: 61 %), requiring an ICD in the second decade (Group S: 3 % vs. Group C: 56 %). Mortality in these patients was high within 5 years of birth (23 %). Phenotypic prediction tools correctly predicted the phenotypic severity in both Groups S and C, especially by using their coupling method. The coupling prediction method is useful in the initial evaluation of phenotypes both with single and compound mutations of LQTS patients. However, it should be noted that the compound mutation makes more severe phenotype.

Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear.We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants.We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p 0.05 for all). The incidence of cancer-related mortality increased markedly with age40 years.Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.

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

The aim of this study was to determine whether major adverse cardiac events (MACE) during the late phase of the Fontan procedure could be predicted by strain measurements of single ventricles using cardiac magnetic resonance imaging with feature tracking (CMR-FT).One hundred adolescent patients who underwent the Fontan procedure (mean age, 21 years) were examined retrospectively with CMR-FT to assess the systemic single-ventricle function. Vertical long-axis cine imaging was divided into six myocardial segments. Global longitudinal strain (GLS) was determined by averaging the peak strain values of each of the six segments. The dyssynchrony index was defined as the standard deviation of the time to peak strain for six segments. The primary outcome was MACE, defined as cardiac death and unscheduled hospitalization.MACE occurred in 18 patients during a mean follow-up of 62 months. According to the multivariate logistic regression analysis results for potential predictor variables, GLS and the dyssynchrony index are independent predictors of MACE. Patients with GLS ≥11.8% had significantly higher MACE-free rates than did those with GLS11.8% [log-rank value, 14.15; p = 0.0002; hazard ratio, 6.82; 95% confidence interval (CI), 2.51-18.56]. Patients with a dyssynchrony index63.5 ms had significantly higher MACE-free rates than did those with dyssynchrony index ≥63.5 ms (log-rank value, 28.17; p 0.0001; hazard ratio, 21.69; 95% CI, 6.96-67.56).GLS and the dyssynchrony index found using CMR-FT are independent predictors of MACE for adolescent patients after the Fontan procedure and provide information regarding risk stratification beyond clinical parameters and biomarkers.

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/m

Introduction We examined the perinatal outcomes and right ventricular function before pregnancy, during pregnancy, and after delivery in women with Ebstein's anomaly. Material and methods We retrospectively investigated the clinical course and mode of delivery and monitored hemodynamic parameters throughout pregnancy in 17 women with Ebstein's anomaly who delivered at our institution during the period of 1995-2015. Results Eight women, including nine pregnancies, underwent elective cesarean section, and nine women, including 14 pregnancies, underwent vaginal delivery. Elective cesarean section was performed in cases with significant heart failure or arrhythmias and in the presence of more than two of the following: cardiothoracic ratio ≥60%, moderate or severe tricuspid valve regurgitation, tricuspid valve regurgitation pressure gradient ≥35 mmHg during pregnancy. The cardiothoracic ratio and tricuspid valve regurgitation pressure gradient significantly increased during pregnancy compared with prepregnancy values. The New York Heart Association classification deteriorated from class I to class II or III in five cases during pregnancy. Conclusions Although pregnancy was relatively safe among women with Ebstein's anomaly, some women developed cyanosis, arrhythmia, and heart failure, leading to elective cesarean section. Monitoring clinical and hemodynamic changes throughout pregnancy is advised to minimize maternal cardiac risk and select the appropriate mode of delivery.

Red cell distribution width (RDW) is known to be associated with anemia and mortality in cardiovascular diseases, while anemia itself is related to increased mortality. RDW may also be related to cytokine activation. We investigated the potential of RDW to predict anemia-adjusted mortality in patients with adult congenital heart disease (ACHD) and we evaluated the relationships among RDW, anemia, and interleukin-6 (IL-6).This was a single-center, retrospective cohort study. Blood RDW and IL-6 levels were measured in 144 patients with ACHD (median age [interquartile range (IQR)], 28 [22-36] years), 84% in New York Heart Association class I/II. During a mean 4.8-year follow-up, 21 (15%) patients died of cardiovascular causes. Elevated RDW (15.0%) correlated significantly with mortality risk in a univariate analysis (RDW hazard ratio [HR]: 1.570; 95% confidence interval [CI]: 1.208-2.040 per 1 standard deviation increase; P=0.001). Elevated RDW levels correlated significantly with increased anemia-adjusted mortality (adjusted RDW HR: 1.912; 95% CI: 1.369-2.670; P0.001). The high RDW group had significantly elevated serum IL-6 levels (RDW15%, median [IQR], 3.7 [0.9-13.9] pg/ml vs. RDW ≤15%, 1.4 [0.8-2.5 pg/ml]; P=0.001), as did patients with anemia (anemia, 1.9 [0.9-5.2] pg/ml vs. no anemia, 1.4 [0.8-2.5 pg/ml]; P=0.021).Elevation of RDW may be related with increased IL-6 and anemia-adjusted cardiovascular mortality in patients with ACHD.

Introduction We examined the perinatal outcomes and right ventricular function before pregnancy, during pregnancy, and after delivery in women with Ebstein's anomaly. Material and methods We retrospectively investigated the clinical course and mode of delivery and monitored hemodynamic parameters throughout pregnancy in 17 women with Ebstein's anomaly who delivered at our institution during the period of 1995-2015. Results Eight women, including nine pregnancies, underwent elective cesarean section, and nine women, including 14 pregnancies, underwent vaginal delivery. Elective cesarean section was performed in cases with significant heart failure or arrhythmias and in the presence of more than two of the following: cardiothoracic ratio ≥60%, moderate or severe tricuspid valve regurgitation, tricuspid valve regurgitation pressure gradient ≥35 mmHg during pregnancy. The cardiothoracic ratio and tricuspid valve regurgitation pressure gradient significantly increased during pregnancy compared with prepregnancy values. The New York Heart Association classification deteriorated from class I to class II or III in five cases during pregnancy. Conclusions Although pregnancy was relatively safe among women with Ebstein's anomaly, some women developed cyanosis, arrhythmia, and heart failure, leading to elective cesarean section. Monitoring clinical and hemodynamic changes throughout pregnancy is advised to minimize maternal cardiac risk and select the appropriate mode of delivery.

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