Utility of urinary liver-type fatty acid-binding protein as a prognostic marker in adult congenital heart patients hospitalized for acute heart failure.

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.

Platelet volume indices correlate to severity of heart failure and have prognostic value for both cardiac and thrombotic events in patients with congenital heart disease.

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% males] hospitalized between 2014 and 2017. We assessed their clinical data, correlation between platelet indices and severity of HF, and prognosis of HF-related hospitalization and thrombus formation. In multivariate analysis, a significant correlation was found between PDW and logBNP (log-transformed brain natriuretic peptide; r = 0.30, p < 0.001), as well as between MPV and logBNP (r = 0.24, p < 0.001). After treatment for heart failure, a significant reduction was found in PDW (average value before treatment: 14.2; after: 13.2, p = 0.017). In multivariate logistic regression analysis, PDW [hazard ratio (HR) 1.365; 95% confidence interval (CI) 1.005-1.768] and MPV (HR 1.472; 95% CI 1.055-2.052) were predictors of HF-related hospitalization. Similarly, PDW (HR 1.998; 95% CI 1.461-2.630) and MPV (HR 1.792; 95% CI 1.155-2.781) were predictors of thrombus formation. Platelet volume indices correlate to severity of heart failure and have prognostic value for both cardiac and thrombotic events in patients with CHD.

Detachable coil embolisation for iatrogenic femoral arteriovenous fistula in patients with CHDs.

We present two cases of patients with iatrogenic femoral arteriovenous fistula who underwent successful embolisation using three-dimensional shape detachable coils. A 49-year-old male with Tetralogy of Fallot had arteriovenous fistula which developed from the common femoral artery to the femoral vein with an aneurysm and a 17-year-old female with single ventricle after total-cavo-pulmonary-connection had two arteriovenous fistulas which developed from the internal iliac artery to the femoral vein. A total of six and seven pieces of detachable coils were necessary for complete occlusion, respectively. No complications were recorded. The advantage of the detachable coil is a wide variation and repositioning until the coil achieves good stabilisation and an ideal configuration.

A novel procedure of puncture to access the pulmonary venous atrium in complex congenital heart disease with surgical correction.

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.

Predictors of neo-aortic valve regurgitation in the long term after arterial switch operation.

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.

Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease.

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<20ml/kg/min was 0.386 [area under the curve (AUC), 0.77; sensitivity, 0.67; specificity 0.76], and the SMI cut-off was 7.6kg/m2 (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 peakVO2 (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.

Clinical Significance of Central Venous Pressure During Exercise After Fontan Procedure.

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.

Successful staged tricuspid valve replacement following cardiac resynchronization therapy in a congenitally corrected transposition of the great arteries.

Simple tricuspid valve surgery for complex heart disease with systemic right ventricular dysfunction is a high-risk procedure; however, staged tricuspid valve surgery following cardiac resynchronization therapy seems to be a beneficial method to expect reverse systemic ventricular remodeling.

The prognostic value of high sensitivity cardiac troponin T in patients with congenital heart disease.

BACKGROUND: Cardiac troponin T (cTnT) is a specific marker of myocardial injury that is elevated in patients with coronary artery disease or heart failure; it has been investigated as a prognostic marker. A highly sensitive, commercially available assay has been developed to detect cardiac troponin T (hs-cTnT). This study aimed to evaluate the clinical implications and prognostic value of hs-cTnT in patients with congenital heart disease (CHD). METHODS: We evaluated 122 consecutive patients hospitalized at our institution because of heart failure or scheduled cardiac catheterization. We measured the serum concentration of hs-cTnT at the time of hospitalization, and we prospectively followed-up all patients for 3 years and monitored rates of cardiovascular events (e.g. cardiac death, readmission owing to worsening of heart failure or arrhythmia, and reintervention) as endpoints. RESULTS: We classified the patients according to their hs-cTnT level into non-detectable (ND group, hs-cTnT <0.003ng/mL), detectable normal (DN group, 0.003ng/mL ≤hs-cTnT <0.014ng/mL), or elevated (EL group, 0.014ng/mL ≤hs-cTnT) group; 20 of 122 (16.4%) patients were in the EL group, in which 17 cardiovascular events occurred during follow-up. In the multivariate Cox proportional hazard analyses, the EL group [p=0.024, hazard ratio (HR) 2.7, 95% confidence interval (CI) 1.1-5.8] was an independent significant predictor of cardiovascular events. A Kaplan-Meier curve revealed a high incidence of cardiovascular events in the EL group (EL vs ND log rank p<0.0001, HR 7.6, 95% CI 3.2-20.0, EL vs DN log rank p<0.0001, HR 4.1, 95% CI 2.1-7.8). CONCLUSIONS: Because the EL group is more likely to have an adverse outcome, elevated hs-cTnT level can be a prognostic marker in patients with CHD.

Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years.

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.

Successful Fontan conversion combined with cardiac resynchronization therapy for a case of failing Fontan circulation with ventricular dysfunction.

Although Fontan conversion combined with cardiac resynchronization therapy appears to be an effective surgical solution for the management of failing Fontan circulation with refractory atrial arrhythmia and cardiac dysfunction due to dyssynchronous ventricular wall motion, limited data are available on the mid- to long-term results of this treatment. We report our successful experience with Fontan conversion combined with cardiac resynchronization therapy in a male patient with failing Fontan circulation who showed favourable outcomes 5 years after the operation.

[A 14-year-old healthy boy with splenic abscess due to Salmonella enterica serovar Senftenberg].

Salmonella enterica serovar Senftenberg may very rarely cause splenic abscess, which can be diagnosed using gallium scintigraphy and drained. A 14-year-old boy admitted for stomachache, diarrhea and fever and diagnosed from his symptoms as having enteritis did not respond when treated with fosfomycin, meropenem, and clindamycin. A low-density splenic area seen in abdominal computed tomography on admission did not show contrast medium enhancement. Gallium scintigraphy on hospital day 10, however, showed abnormal splenic accumulation confirming the splenic abscess diagnosis, after which we punctured and drained the abscessout. S. Senftenberg was isolated from pus aspirated pus from the abscess, after which responded well to ceftriaxone and levofloxacin. Follow-up gallium scintigraphy on hospital day 24 showed that the abnormal splenic accumulation had disappeared, after which he has been followed up with abdominal ultrasonography and blood tests as an outpatient. He has experienced no relapse of splenic abscess.