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The prognosis of congenital heart disease (CHD) has improved, and most patients now reach adulthood. Owing to residual disease and comorbidities, it is recommended that adult CHD (ACHD) patients transition to adult care for lifelong monitoring and treatment. However, this transition period can be challenging for CHD patients owing to obstacles such as independence from their parents and establishing self-management. To achieve a seamless shift from pediatric to adult care and ensure continuity, it is important to educate and motivate patients appropriately, and an established transition system is needed that involves collaboration between CHD specialists and healthcare providers across medical specialties. The present review describes the epidemiology of ACHD and notable points in patient care as the background of transition. The concepts and an overview of transition systems, educational systems, and potential lapses in the care of their relevant causes are then provided with supporting evidence.

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BACKGROUND: Granulocyte colony stimulating factor (G-CSF) preparations are used for patients with granulocytopenia, especially to prevent febrile neutropenia. Arteritis has been recognized as a side effect of G-CSF treatment; however, there are no clear diagnostic criteria or treatment guidelines because not enough cases have been reported. Present case showed one of the diagnostic and treatment selection methods via multiple imaging modality including vascular echography. CASE SUMMARY: A 52-year-old woman underwent chemotherapy for ovarian cancer and received G-CSF because of myelosuppression. The patient experienced high and remittent fever that persisted during treatment using antibiotics and acetaminophen. Enhanced computed tomography revealed thickening of the tissue around the aortic arch and abdominal aorta. Echography of the abdominal aorta revealed thickening of the wall and a hypoechoic region around the aorta. Gadolinium-enhanced magnetic resonance imaging and 18F-fludeoxyglucose positron emission tomography also revealed that the inflammation was localized to the lesion. A suspicion of G-CSF-associated aortitis was based on the patient's history and the exclusion of other diseases that might have caused the aortitis. Her condition rapidly improved after starting corticosteroid treatment. DISCUSSION: The differential diagnosis in similar cases should consider immune diseases that cause large-vessel arteritis (Takayasu arteritis, giant cell arteritis, and another vasculitis), infection, drug-induced disease, and immunoglobulin G4-related disease. The use of different imaging modalities, including vascular echography, helped guide the diagnosis and follow-up. It is necessary to evaluate the patient's general condition before the selection of treatments.

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A 42-year-old woman presented with fever, dyspnea, lower-leg edema, significant pulmonary congestion, pleural effusion, and severely reduced left ventricular contractions. She was resistant to treatment for heart failure, including catecholamines, furosemide, phosphodiesterase III inhibitors, and human atrial natriuretic peptide, and antibiotics failed to reduce her inflammation. She had renal dysfunction and hypocomplementemia and was positive for anti-nuclear and anti-ds-DNA antibodies. The patient was diagnosed with myocarditis and pleurisy associated with systemic lupus erythematosus (SLE). Prednisolone administration improved her general condition, reducing inflammation and improving left ventricular function. On day 1, an electrocardiography (ECG) revealed a T-wave inversion similar to a T-U complex configuration in leads II, aVF, and V3-6. By day 8, however, ECG showed prolonged corrected QT (QTc) and T-wave alternans (alternating beat-to-beat T-wave patterns) in lead V3-6. Careful ECG monitoring should be used to identify potentially fatal ventricular arrhythmias during the recovery phase of SLE-related myocarditis. .

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BACKGROUND: The geriatric nutritional risk index (GNRI) is a simple and objective nutritional assessment tool for elderly patients. Lower GNRI values are associated with a worse prognosis in patients with heart failure (HF). However, few data are available regarding the prognostic effect of the GNRI value for risk stratification in patients at risk for HF.Methods and Results:We retrospectively investigated 1,823 consecutive patients at risk for HF (Stage A/B) enrolled in the IMPACT-ABI Study. GNRI on admission was calculated as follows: 14.89×serum albumin (g/dL)+41.7×body mass index/22. Patients were divided into 2 groups according to the median GNRI value (107.1). The study endpoint was a composite of cardiovascular (CV) events, including CV death and hospitalization for worsening HF. Over a 4.7-year median follow-up, CV events occurred in 130 patients. In the Kaplan-Meier analysis, patients with low GNRI (<107.1, n=904) showed worse prognoses than those with high GNRI (≥107.1, n=919) (20.2% vs. 12.4%, P<0.001). In the multivariable Cox proportional hazards analysis, low GNRI was significantly associated with the incidence of CV events (hazard ratio: 1.48, 95% confidence interval: 1.02-2.14; P=0.040). CONCLUSIONS: The simple and practical assessment of GNRI may be useful for predicting CV events in patients with Stage A/B HF.

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Objective A low ankle-brachial index (ABI) is a known predictor for future cardiovascular events and mortality in patients with chronic kidney disease (CKD). While most prior studies have defined CKD as an estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2, recent reports have suggested that the cardiovascular risk may be increased even in early stages of renal insufficiency. We hypothesized that a low ABI may predict future cardiovascular morbidity and mortality in patients with mild impairment of the renal function. Methods The IMPACT-ABI study was a retrospective, single-center, cohort study that enrolled and obtained ABI measurements for 3,131 patients hospitalized for cardiovascular disease between January 2005 and December 2012. From this cohort, we identified 1,500 patients with mild renal insufficiency (eGFR =60-89 mL/min/1.73 m2), and stratified them into 2 groups: ABI ≤0.9 (low ABI group; 9.2%) and ABI >0.9 (90.8%). The primary outcome measured was the cumulative incidence of major adverse cardiovascular events (MACE; cardiovascular death, myocardial infarction, and stroke). Results Over a mean follow-up of 5.0 years, 101 MACE occurred. The incidence of MACE was significantly higher in patients with low ABI than in those with ABI >0.9 (30.2% vs. 14.4%, log rank p<0.001). A low ABI was associated with MACE in a univariate Cox proportional hazard analysis. A low ABI remained an independent predictor of MACE in a multivariate analysis adjusted for cardiovascular risk factors (hazard ratio (HR): 2.27; 95% confidence interval (CI): 1.33-3.86; p=0.002). Conclusion Low ABI was an independent predictor for MACE in patients with mild renal insufficiency.

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BACKGROUND: The ankle-brachial index (ABI) is a marker of generalized atherosclerosis and is predictive of future cardiovascular events. However, few studies have assessed its relation to long-term future cardiovascular events, especially in patients with borderline ABI. We therefore evaluated the relationship between long-term future cardiovascular events and ABI. METHODS: In the IMPACT-ABI study, a single-center, retrospective cohort study, we enrolled 3131 consecutive patients (67 ± 13 years; 82% male) hospitalized for cardiovascular disease and measured ABI between January 2005 and December 2012. After excluding patients with an ABI > 1.4, the remaining 3056 patients were categorized as having low ABI (≤ 0.9), borderline ABI (0.91-0.99), or normal ABI (1.00-1.40). The primary endpoint was MACE (cardiovascular death, myocardial infarction [MI] and stroke). The secondary endpoints were cardiovascular death, MI, stroke, admission due to heart failure, and major bleeding. RESULTS: During a 4.8-year mean follow-up period, the incidences of MACE (low vs. borderline vs. normal: 32.9% vs. 25.0% vs. 14.6%, P<0.0001) and cardiovascular death (26.2% vs. 18.7% vs. 8.9%, P<0.0001) differed significantly across ABIs. The incidences of stroke (9.1% vs. 8.6% vs. 4.8%, P<0.0001) and heart failure (25.7% vs. 20.8% vs. 8.9%, P<0.0001) were significantly higher in the low and borderline ABI groups than in the normal ABI group. But the incidences of MI and major bleeding were similar in the borderline and normal ABI groups. The hazard ratios for MACE adjusted for traditional atherosclerosis risk factors were significantly higher in patients with low and borderline ABI than those with normal ABI (HR, 1.93; 95%CI: 1.44-2.59, P < 0.0001, HR, 1.54; 95% CI: 1.03-2.29, P = 0.035). CONCLUSIONS: The incidence of long-term adverse events was markedly higher among patients with low or borderline ABI than among those with normal ABI. This suggests that more attention should be paid to patients with borderline ABIs, especially with regard to cardiovascular death, stroke, and heart failure.

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Diastolic wall strain (DWS) is based on the linear elastic theory, according to which decreased wall thinning during diastole reflects reduced left ventricular compliance and thus increased diastolic stiffness. Increased diastolic stiffness as assessed by DWS is associated with a worse prognosis in patients who have heart failure (HF) with preserved ejection fraction. However, there are no data about the prognostic value of DWS derived by M-mode echocardiography in patients at risk for HF. We retrospectively enrolled 1829 consecutive patients without prior HF who were hospitalized for cardiovascular (CV) diseases in our hospital between 2005 and 2012. Patients were divided into two groups stratified by DWS (median value 0.34). The study endpoint was the composite of major adverse cardiovascular events (MACE), including all-cause death, myocardial infarction, stroke, and hospitalization for HF. Over a 4.2-year median follow-up, adverse events were observed in 322 patients (17.6%). In Kaplan-Meier analysis, patients with low DWS (≤ 0.34, n = 915) showed worse prognoses than those with high DWS (> 0.34, n = 914) (MACE incidence 39.4% versus 31.9%, P = 0.011). In multivariate Cox proportional hazards analysis after the adjustment for age, sex, and echocardiographic parameters, low DWS (≤ 0.34) was significantly associated with the incidence of MACE (hazard ratio: 1.26, 95% confidence interval: 1.01-1.59; P = 0 .045). In patients without prior HF, DWS is an independent predictor of MACE. Simple assessment of DWS might improve risk stratification for CV events in those patients.

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BACKGROUND: Prior reports have revealed that complete revascularization (CR) by percutaneous coronary intervention (PCI) decreased ischemic events. However, little is known about the efficacy of CR using PCI in elderly patients with multi-vessel coronary artery disease (CAD). We evaluated the 1-year effectiveness of CR-PCI in elderly patients (≥75years old) with multi-vessel CAD. METHODS: The SHINANO Registry, a prospective, observational, multi-center, all-comer cohort study, has enrolled 1923 patients. From this registry, we recruited 322 elderly patients with multi-vessel CAD. The primary endpoint was major adverse cardiovascular events ([MACE]: all-cause mortality, myocardial infarction, and stroke). RESULTS: Of the 322 elderly patients with multi-vessel CAD, 165 (51.2%) received CR and 157 (48.8%) received incomplete revascularization (ICR). MACE occurred in 44 (13.7%) patients. The incidence of MACE by survival analysis was significantly lower in the CR group than in the ICR group (7.4% vs. 21.1%, p<0.001). On multivariable Cox proportional hazards analysis of age, sex, and acute coronary syndrome (ACS), ACS and CR were independent predictors of MACE (hazard ratio [HR], 2.49; 95% confidence interval [CI], 1.29-4.80; p=0.007, HR, 0.40; 95% CI, 0.20-0.77; p=0.007, respectively). In propensity score matching of age, sex, previous heart failure, previous intracranial bleeding, ACS, and body mass index, the MACE rate was significantly lower in the CR group than in the ICR group (7.2% vs. 18.4%, p=0.015). CONCLUSIONS: Even in elderly patients over 75years old with multi-vessel CAD, CR-PCI appears to suppress mid-term ischemic events.

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The ankle brachial index (ABI) is regarded as a predictor of future cardiovascular events. However, the relationship between ABI and incident heart failure (HF) in patients without previous HF is poorly understood. This study aimed to assess the prognostic value of ABI for incident HF in patients without previous HF. The IMPACT-ABI study was a retrospective, single-center, cohort study that enrolled and measured ABI in 3131 patients hospitalized for cardiovascular disease between January 2005 and December 2012. From this cohort, 307 patients were excluded because of previous HF and high (>1.4) ABI. The remaining 2824 patients were stratified into three groups: low ABI (≤0.9), borderline ABI (0.91-0.99), and normal ABI (1.0-1.4). The primary endpoint was hospitalization for HF. Over a mean 4.8-year follow-up, 105 cases of HF occurred. The cumulative incidence of HF was significantly higher in patients with low and borderline ABIs than in those with normal ABI (19.3 vs. 21.0 vs. 10.4 %, log rank P <0.001). In multivariate Cox proportional hazard analysis, low ABI and borderline ABI were independent predictors of incident HF [hazard ratio (HR) 3.00; 95 % confidence interval (CI) 1.70-5.28; P < 0.001 and HR 2.68; 95 % CI 1.35-5.34; P = 0.005, respectively]. In conclusion, low and borderline ABI were strong predictors for future incident HF in patients without previous HF.

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BACKGROUND: Serum cystatin C (CysC), a novel marker of renal function, is associated with the components of metabolic syndrome in adults. Little is known about the utility of CysC and its association with cardiometabolic risks in young subjects. METHODS AND RESULTS: In a cohort of 454 Japanese junior high school students, the distribution of serum CysC levels and associated variables were analyzed. CysC levels were significantly higher in boys than in girls (0.92±0.10mg/L vs. 0.77±0.08mg/L, p<0.001). CysC was significantly correlated with serum creatinine (r=0.473, p<0.001), and serum uric acid (SUA) (r=0.546, p<0.001). Multivariable regression analysis revealed significant associations between CysC and SUA in all subjects (ß=0.241, p<0.001), and in boys and girls separately (ß=0.264 and 0.240, respectively, both p<0.001). Importantly, subjects with elevation of both serum CysC and SUA levels had the highest ratio of triglyceride to high-density lipoprotein cholesterol. CONCLUSIONS: CysC had significant associations with both creatinine and SUA in Japanese junior high school students. The concomitant elevation of serum CysC and SUA levels was associated with subclinical lipid metabolism dysregulation, and suggested the presence of cardiometabolic risk accumulation.

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A 71-year-old man diagnosed with cardiac sarcoidosis (CS) 11 years previously underwent implantation of an implantable cardioverter defibrillator due to sustained ventricular tachycardia. Over past decade, his condition of CS did not progress on the maintenance steroid dose of 7.5 mg per day. We attempted to taper and discontinue steroids according to the results of fluorine-18-fluorodeoxyglucose positron emission tomography (18F-FDG PET). On the basis of the results, we reduced the oral steroid dose slowly. In spite of no abnormal 18F-FDG uptake in the myocardium, advanced atrioventricular conduction block and deterioration of the ventricular pacing threshold occurred during the course of steroid withdrawal. Plasma brain natriuretic peptide (BNP) increased from 94 to 842 pg/ml. It was necessary to add new ventricular and atrial leads to synchronize atrial and ventricular contractions, and the pacing mode for bradycardia was changed to dual-chamber DDD-60 ppm. Fatigue disappeared, and BNP levels decreased to 147 pg/ml. 18F-FDG PET might have a limit to detect small scattered inflammatory foci. This case highlights the need for caution when reducing steroid doses in CS patients, guided by 18F-FDG PET only. .

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Brachial-ankle pulse wave velocity (baPWV) is known as a significant predictor of cardiovascular events. However, the previous studies have not considered age, which can affect the baPWV value. We evaluated the predictive value of baPWV for cardiovascular events in various age groups. From January 2005 to December 2012, all patients admitted to our department with any cardiovascular disease and underwent ankle-brachial index (ABI) measurement were enrolled in the IMPACT-ABI registry. The primary endpoints included major adverse cardiovascular events (MACE; cardiovascular death, myocardial infarction, and stroke). Of the 3131 patients enrolled, 2554 were included in the analysis, whereas 577 were excluded due to missing baPWV data, ABI ≤0.9 and/or >1.4, and the previous endovascular therapy and/or surgical treatment for peripheral artery disease. Patients were divided according to age 30-59 years (n = 580), 60-69 years (n = 730), 70-79 years (n = 862), and ≥80 years (n = 330). The cumulative incidence of MACE through 5 year was significantly higher in the high baPWV group (>1644 cm/s) than in the low baPWV group (≤1644 cm/s; 8.7 vs. 4.6%; log-rank: p < 0.001). However, among the age groups, only the 30-59-year group showed a significant difference in MACE incidence between those with high and low baPWV (7.0 vs. 0.9%; log-rank: p = 0.001). In conclusion, the baPWV could serve as a useful marker to predict cardiovascular events, particularly among younger patients.

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BACKGROUND: Reduced ankle-brachial index (ABI) is a predictor of cardiovascular events. However, the significance of high ABI remains poorly understood. This study aimed to assess the characteristics and outcomes of patients with high ABI. METHODS: The IMPACT-ABI study was a retrospective cohort study that enrolled and examined ABI in 3,131 patients hospitalized for cardiovascular disease between January 2005 and December 2012. From this cohort, 2,419 patients were identified and stratified into two groups: high ABI (> 1.4; 2.6%) and normal ABI (1.0-1.4; 97.3%). The primary endpoint was the cumulative incidence of major adverse cardiovascular events (MACE), including cardiovascular-associated death, myocardial infarction, and stroke. RESULTS: Compared with the normal ABI group, patients in the high ABI group showed significantly lower body mass index (BMI) and hemoglobin level, but had higher incidence of chronic kidney disease and hemodialysis. Multivariate logistic regression analysis revealed that hemodialysis was the strongest predictor of high ABI (odds ratio, 6.18; 95% confidence interval (CI), 3.05-12.52; P < 0.001). During the follow-up (median, 4.7 years), 172 cases of MACE occurred. Cumulative MACE incidence in patients with high ABI was significantly increased compared to that in those with normal ABI (32.5% vs. 14.5%; P = 0.005). In traditional cardiovascular risk factors-adjusted multivariate Cox proportional hazard analysis, high ABI was an independent predictor of MACE (hazard ratio, 2.07; 95% CI, 1.02-4.20; P = 0.044). CONCLUSION: Lower BMI, chronic kidney disease, and hemodialysis are more frequent in patients with high ABI. Hemodialysis is the strongest predictor of high ABI. High ABI is a parameter that independently predicts MACE.

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