The Fontan procedure is a palliative surgical technique used for complex congenital heart disease resulting in a functional single ventricle. Patients with Fontan circulation often exhibit elevated central venous pressure (CVP) and reduced cardiac output, which make intermittent hemodialysis (IHD) challenging. Between dialysis sessions, increased preload due to fluid accumulation may precipitate congestive heart failure, whereas excessive ultrafiltration can reduce preload and consequently compromise cardiac output. We report the case of a 23-year-old man with a single ventricle of left ventricular morphology who underwent a Fontan procedure at 2 years of age. He developed kidney failure secondary to bilateral hypoplastic kidneys and began peritoneal dialysis (PD) at 12 years of age. PD was continued for 9 years because IHD and kidney transplantation were contraindicated due to cardiac dysfunction with arrhythmia. He presented with a refractory exit-site infection, for which laparoscopic surgery revealed early-stage encapsulating peritoneal sclerosis. Cardiac catheterization revealed a CVP of 11–12 mmHg, and ventricular function was preserved. Based on these findings, transition to IHD was considered feasible. Permanent pacemaker implantation and insertion of a tunneled hemodialysis catheter were performed concurrently. IHD was initiated at a blood flow rate of 150 mL/min, which was well tolerated, with no episodes of hemodynamic instability observed. The patient has since been maintained on outpatient IHD for 9 months without major complications. This case demonstrates that IHD can be safely performed in selected patients with Fontan circulation, provided that careful preoperative hemodynamic assessment is undertaken to confirm adequate cardiovascular stability.

BACKGROUND:Ventricular septal defect is the most common CHD and is complicated by pulmonary hypertension in about 5% of cases. Although long-term outcomes after repair are generally good, persistent pulmonary hypertension has been reported, especially before the widespread treat-and-repair strategy. METHODS:We retrospectively analysed patients aged ≥13 years who underwent repair for ventricular septal defect with pulmonary hypertension at our centre (1970-2024). Pre- and post-operative hemodynamics were assessed. Patients were grouped by preoperative pulmonary vascular resistance (≥3 vs. <3 Wood units). Residual pulmonary hypertension was evaluated by echocardiography. RESULTS:We studied 115 patients (43 male, 72 female). Follow-up reached 47 years (median 18.5). Residual pulmonary hypertension occurred in 3/115 (2.6%). Median age at intracardiac repair was 5 months. No significant differences were seen between patients with and without residual pulmonary hypertension in age at surgery, preoperative estimated right ventricular pressure, mean pulmonary artery pressure, or postoperative estimated right ventricular pressure. Mean pulmonary artery pressure was higher in the pulmonary vascular resistance ≥3 group than in the <3 group (47 ± 13 vs. 38 ± 11 mmHg, p = 0.019), while postoperative estimated right ventricular pressure did not differ. Among the 3 with residual pulmonary hypertension, 2 underwent intracardiac repair after 1 year of age. CONCLUSIONS:Most patients with ventricular septal defect and pulmonary hypertension had resolution after intracardiac repair; however, residual pulmonary hypertension occurred in about 2.6% during long-term follow-up. Preoperative hemodynamics did not predict persistence. Long-term periodic follow-up remains important after surgery.

Background: Idiopathic/heritable pulmonary arterial hypertension (I/H-PAH), a disease with poor prognosis, often faces diagnostic delays due to nonspecific initial symptoms. In Japan, school ECG screening, performed in every 3 years for all students, identifies 41% of school-aged I/H-PAH patients; these typically show elevated pulmonary artery pressure (PAP) but have minimal symptoms (WHO-FC I/II) and preserved RV function. In this disease, the duration from the appearance of pulmonary vascular lesions to PH manifestation and the subsequent onset of RV failure are unknown. Objective: To address the precise pathological trajectory leading to PH manifestation, PH-related ECG findings at diagnosis (Dx) and pre-Dx in pediatric PAH patients were investigated utilizing Japan's school ECG screening system. Methods: Observational study of I/H-PAH patients (6-18 years), newly diagnosed 2005-2020, in Japanese pediatric cardiology teaching hospitals. School ECGs at/pre Dx were evaluated for PH-related findings (R≥1.5mV in V1, RV strain, Q in V1, IRBBB, inverted T waves) according to the criteria from the Japanese Society of Pediatric Cardiology and Cardiac Surgery. Results: In the survey, 118/143 institutions (82.5%) responded; 45 (38%) of those reported cases. 88 patients (51M, 37F; mean age 10.7y) were analyzed. Mode of detection of PAH were school ECG screening (n=36), symptoms (44), family history (5), and incidental findings (3). Compared to nonscreening group, the school screening group had higher proportion of WHO-FC I/II (91% vs 57%, p<.01) and lower plasma BNP (20 vs 102 pg/mL, p<.01), with comparable mean PAP (48 vs 55 mmHg, p=.65) and PVRI (12.1 vs 15.8 WU×m 2 , p=.38). PH-related ECG findings at Dx (n=87), which were present in 93% of all patients, were largely comparable between the groups (95% vs 91, p=.68), with the exception of higher prevalence of Q waves in V1 in the nonscreening (31% vs 15, p<.05) and R≥1.5mV in V1 in the screening group (59% vs 30, p<.05). Crucially, among 35 pre-Dx ECGs, PH-related abnormalities were observed in 77% (10/13) of those taken within 2 years of Dx, versus only 14% (3/22, p<.01) of those taken >2 years prior. Conclusion: Pre-Dx ECG analysis indicates PH-related findings can appear up to 2 years before symptom manifestation, suggesting a 1-2 year window of established PH with minimal symptoms in many pediatric patients. This highlights the potential for earlier diagnosis by performing ECG screening at even shorter intervals.

BACKGROUND:Adults with congenital heart disease have a higher risk of coronary artery disease compared to the general population. However, there is limited information on coronary artery distribution in patients with a single ventricle (SV), which is important in understanding potential cardiovascular events. This study aimed to evaluate coronary artery morphology and anomalies in patients with SV based on selective coronary angiography (CAG). METHODS:We performed a retrospective single-center study including 80 patients with SV [median age, 29 years (range 13-50); 54 % males] who underwent selective CAG at our institution between 2019 and 2023. Patients were classified into either single right or left ventricular (SRV and SLV) morphologies and categorized into D-, L-, and X-loops based on the rules of cardiovascular looping. Coronary artery morphology, dominance, and the abnormality of origin were evaluated. RESULTS:Of the 80 SV patients, 56 had SRV and 24 had SLV. Patients with coronary artery looping surrounding the rudimentary chamber were more frequent in cases with D-loops. In patients with SV, right coronary artery dominance was observed in 70 %, and anomalous origins of coronary artery origins were found in 36.3 % of cases, with a higher frequency of L-loops. Overall, cardiovascular events were observed in patients with SV as follows: 46 % of patients had arrhythmias, 13 % had heart failure, and 8 % had thrombosis; there were no cardiovascular deaths. A single coronary artery was identified in 9 cases (11 %) of SV patients, exclusively in SRV, of which 2 cases (22 %) had arrhythmias and 1 case (11 %) had thrombus. CONCLUSION:Selective CAG revealed a high prevalence of coronary artery anomalies in patients with SV, emphasizing the importance of comprehensive assessment and long-term follow-up to manage cardiovascular risk in this patient population.

Abstract The persistent fifth aortic arch (PFAA) is a rare disease caused by developmental anomalies associated with aortic coaction (CoA) and an interrupted aortic arch (IAA). It has been reported that the histological findings of the fifth aortic arch differ from those of normal aortic structures. Therefore, histological findings are important in deciding whether PFAA should be resected or used during aortic arch reconstruction. In the present case, the PFAA was resected during aortic arch reconstruction. The treatment strategy for PFAA is discussed based on histological findings.

BACKGROUND:Peripheral pulmonary stenosis (PPS) is a condition characterized by the narrowing of the pulmonary arteries, which impairs blood flow to the lung. The mechanisms underlying PPS pathogenesis remain unclear. Thus, the aim of this study was to investigate the genetic background of patients with severe PPS to elucidate the pathogenesis of this condition. METHODS AND RESULTS:We performed genetic testing and functional analyses on a pediatric patient with PPS and Williams syndrome (WS), followed by genetic testing on 12 patients with WS and mild-to-severe PPS, 50 patients with WS but not PPS, and 21 patients with severe PPS but not WS. Whole-exome sequencing identified a rare PTGIS nonsense variant (p.E314X) in a patient with WS and severe PPS. Prostaglandin I2 synthase (PTGIS) expression was significantly downregulated and cell proliferation and migration rates were significantly increased in cells transfected with the PTGIS p.E314X variant-encoding construct when compared with that in cells transfected with the wild-type PTGIS-encoding construct. p.E314X reduced the tube formation ability in human pulmonary artery endothelial cells and caspase 3/7 activity in both human pulmonary artery endothelial cells and human pulmonary artery smooth muscle cells. Compared with healthy controls, patients with PPS exhibited downregulated pulmonary artery endothelial prostaglandin I2 synthase levels and urinary prostaglandin I metabolite levels. We identified another PTGIS rare splice-site variant (c.1358+2T>C) in another pediatric patient with WS and severe PPS. CONCLUSIONS:In total, 2 rare nonsense/splice-site PTGIS variants were identified in 2 pediatric patients with WS and severe PPS. PTGIS variants may be involved in PPS pathogenesis, and PTGIS represents an effective therapeutic target.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH.Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death.Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of =2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009).Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of =2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

Patients with heart failure (HF) reportedly have activated platelets with increased platelet distribution width (PDW) and mean platelet volume (MPV), which lead to thrombotic events. These platelet indices are easily measured by routine blood tests and have been proposed as potential markers of cardiac events. We performed the present study to clarify whether platelet indices correlate to the severity of HF and to the prognosis of patients with congenital heart disease (CHD). We performed a retrospective single-center study including 400 patients with CHD [median age, 34 years (range 12–76); 49

Aim This study aimed to summarize cases of successful pregnancy and delivery in patients with transposition of the great arteries (TGA) after atrial switch operation (ASO), to provide management, clinical experience, and maternal and fetal outcomes. Methods During a 16-year period (2004-2019), we experienced 30 pregnancies in 15 patients after ASO at our institution. We retrospectively reviewed the medical records of the patients. Results In 30 pregnancies, there were 21 (70%) live births, five (17%) miscarriages, and four (13%) artificial abortions. There were no maternal or neonatal deaths. Cardiac complications occurred in nine (43%) patients: deterioration of right ventricular (RV) function in one, symptomatic heart failure (HF) in three, supraventricular tachyarrhythmia requiring electrical cardioversion in two, sick sinus syndrome that required pacemaker implantation in two, and hemoptysis in one. Obstetric complications occurred in five (24%). Neonatal complications were premature births (delivery at <37 weeks of gestation) in 15 (71%), and birthweight <2500 g in 18 (86%). The mode of delivery consisted of vaginal delivery (VD) in five (24%), and cesarean section (CS) in 16 (76%). Conclusion A high incidence of preterm CS and cardiac complications including deterioration of RV function was observed in patients who had undergone ASO for TGA.

Progression to acute kidney injury (AKI) under treatment in adult congenital heart disease (ACHD) patients with heart failure is associated with poor prognosis, early detection and interventions are necessary. We aimed to explore the utility of urinary liver-type fatty acid binding protein (L-FABP) in ACHD patients hospitalized for acute decompensated heart failure (ADHF). We prospectively evaluated hemodynamic, biochemical data, and urinary biomarkers including urinary L-FABP in ACHD patients hospitalized in our institution from June 2019 to March 2022. The primary outcomes were the development of AKI and death. AKI was defined as serum creatinine level increased by 0.3 mg/dl or more within 5 days after hospitalization. A total of 104 ADHF patients aged 31 (36–51) years were enrolled. 26 cases (25% of ADHF patients) developed AKI during hospitalization and 4 died after hospital discharge. Serum creatinine (sCr), serum total bilirubin, brain natriuretic peptide (BNP), and urinary L-FABP in AKI patients were significantly higher than in non-AKI patients, whereas systemic oxygen saturation of the peripheral artery (SpO2) and estimated glomerular filtration ratio in AKI patients were lower than non-AKI patients. There was no difference in the intravenous diuretic dose on admission and during hospitalization between the two groups. In the receiver operating characteristic (ROC) analysis, the maximum area under the curve (AUC) of urinary biomarkers in AKI patients was urinary L-FABP (AUC = 0.769, p < 0.001) with a cutoff value of 4.86 µg/gCr. Urinary L-FABP level on admission was associated with a predictor for AKI development during hospitalization after adjusting for sCr, BNP and SpO2. Urinary L-FABP was a useful predictor for the development of AKI in ACHD patients hospitalized for ADHF. Monitoring of urinary L-FABP allows us to detect a high-risk patient earlier than the conventional biomarkers.

Right aortic arch in association with coarctation of the aorta and vascular ring is a rare anatomy. We present an infant with chromosome 22q11.2 deletion, who had the right aortic arch with retroesophageal aberrant left subclavian artery and left internal carotid artery. The left external carotid artery and right common carotid artery originated together from the ascending aorta as a bovine branch. The infant also had severe coarctation, which was prostaglandin dependent.

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 < grade II). In the long-term phase, the diastolic blood pressure was significantly lower and the ascending aortic diameter was significantly larger in the AR group than in the non-AR group (p = 0.004 and p = 0.006, respectively). The systolic blood pressure (SBP) and pulse pressure (PP) were similar in both groups. Meanwhile, in the early surgical phase, SBP and PP were significantly higher in the AR group than in the non-AR group (p = 0.029 and p = 0.002, respectively). The receiver operating characteristic curve for late AR showed that the area under the curve for SBP and PP in the early surgical phase were 0.746 and 0.853, respectively. Even though sensitivity analysis was performed, SBP or PP greater than the cutoff value in the early surgical phase was identified as predictors for late AR. Our results suggested that high SBP or PP in the early surgical phase could influence the development of AR in the long term after ASO.

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/height(2)), mineral index (MI, mineral mass/height(2)), percent body fat (%BF), and exercise capacity [peak oxygen uptake (peakVO(2))] via a cardiopulmonary exercise test. Results: The multivariate analysis revealed a significant correlation between peakVO(2) and EI (r = 0.55) and peakVO(2) and SMI (r = 0.49). The receiver operating characteristic curve analysis showed that the EI cut-off for peakVO(2) < 20 ml/kg/min was 0.386 [area under the curve (AUC), 0.77; sensitivity, 0.67; specificity 0.76], and the SMI cut-off was 7.6 kg/m(2) (AUC, 0.78; sensitivity, 0.76; specificity 0.75). Compared with patients who had biventricular morphology, patients with single ventricular morphology had a higher EI (mean, 0.381 vs. 0.387, respectively) and lower SMI (8.5 vs. 7.7, respectively), resulting in a lower peakVO(2) (27.1 vs. 20.8, respectively). The Kaplan-Meier analysis showed that a low SMI was associated with an increased risk of future heart failure-related admissions. Conclusions: SMI determined by BIA is a determinant of exercise capacity and can be used as a prognostic predictor in patients with CHD. (c) 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

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 <15), moderate (15 <= respiratory disturbance index <30), and severe (respiratory disturbance index >= 30) sleep apnea, respectively. A large majority of the sleep apnea cases were categorised as obstructive sleep apnea (92%, n = 58). The respiratory disturbance index >= 15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnea (respiratory disturbance index <5). Multivariable analysis showed that New York Heart Association class >= II (OR, 4.36; 95% CI, 1.09-20.87) and body mass index >= 25 (OR, 4.29; 95% CI, 1.32-15.23) were independent risk factors for a respiratory disturbance index >= 15. 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.

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 < 0.05), gamma-glutamyl transpeptidase (63 ± 33 vs. 114 ± 30 IU/l, p < 0.05), type IV collagen 7S (6.7 ± 1.3 vs. 8.1 ± 1.3 ng/ml, p < 0.05), and creatinine (0.72 ± 3.14 vs. 0.83 ± 3.16 mg/dl, p < 0.05) levels were significantly higher in the high-CVP group than in the low-CVP group. Elevated CVP during exercise may be associated with long-term complications after the Fontan procedure; hence, CVP should be measured during exercise in patients who underwent the Fontan procedure to accurately predict the risk of developing such complications.

Abstract Background Although sleep apnoea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with CHD 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 apnoea using a type 3 portable overnight polygraph in adult patients with CHD were evaluated. Results This 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 <15), moderate (15⩽ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnoea, respectively. A large majority of the sleep apnoea cases were categorised as obstructive sleep apnoea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnoea (respiratory disturbance index <5). Multi-variable analysis showed that NYHA class ≥II, whose odds ratio 4.36, 95% confidence interval 1.09–20.87, and body mass index ≥25, whose odds ratio 4.29, 95% confidence interval 1.32–15.23, were independent risk factors for a respiratory disturbance index ≥15. Conclusion Our 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.

Introduction: It is well known that, in patients with congenital heart disease, such as Tetralogy of Fallot, aortic root dilatation may gradually progress. Similar phenomenon also occur in patients with single ventricle circulation. However, the factors associated with aortic dilatation in these patients are still unknown. Hypothesis: We assessed the hypothesis that vascular function associated with the aortic root dilatation after Fontan procedure. Methods: Between July 2013 and April 2016, sixty-one patients who underwent the Fontan procedure (age, 12.8–55.8 years; median age, 23.2 years; postoperative duration, 8.2-31.3 years; median postoperative duration, 16.8 years) were enrolled. These patients were divided into 2 groups according to the presence or absence of aortic root dilatation and analyzed the relation between aortic root size and clinical profile, vascular function, hemodynamics, and neurohormonal factors. Aortic root dilatation was defined as observed/expected aortic root diameter >1.5 (aortic root dilatation, n=15, aortic root non-dilatation, n=46). Results and Conclusions: Age at Fontan procedure was significantly higher in the dilatation group than in the non-dilatation group (10.5±7.3 vs 5.3±4.0, P<0.01). FMD was significantly lower in the dilatation group than in the non-dilatation group (5.9±1.4 vs 8.3±2.3, OR 0.49, 95%CI 0.28-0.74, P<0.0001). Pulse wave velocity (PWV) was significantly higher in the dilatation group than in the non-dilatation group (1081±277 vs 943±140, P<0.05). Although hemodynamic variables and neurohormonal factors was not associated with aortic root size, pulmonary atresia was associated with aortic root dilatation (OR 5.43, 95%CI 1.55-20.18, P<0.01). In the multivariate analysis, impaired FMD had a close association with aortic dilatation (OR 0.45, 95%CI 0.19-0.82, P<0.01). FMD showed a negative correlation between age at Fontan procedure, PWV and parameter β(age at Fontan procedure r: -0.56, p<0.0001, PWV r: -0.32, p<0.05, β r: -0.27, p<0.05). We concluded that, in patients after Fontan procedure, vascular dysfunction such as aortic stiffness occurred in the patients who underwent Fontan procedure at older age and may play a role in progressive aortic dilatation.

Introduction: The prognostic factors in patients with biventricular heart who underwent Fontan operation remain unclear. Hypothesis: We assessed the hypothesis that ventricular dilatation, ventricu...