Systemic Artery Vasoconstrictor Therapy in Fontan Patients with High Cardiac Output-Heart Failure: A Single-Center Experience.

Failed Fontan Patients with high cardiac output (CO) heart failure (HF) might have vasodilatory syndrome and markedly high mortality rates. The aim of this study was to review the clinical effects of vasoconstrictor therapy (VCT) for failed Fontan hemodynamics. We retrospectively reviewed 10 consecutive patients with Fontan failure (median age, 33 years) and high CO-HF who had received VCT.

Electrocardiographic changes and long-term prognosis of children diagnosed with hypertrophic cardiomyopathy by the school screening program for heart disease in Japan.

Background: In Japan, the school screening program for heart disease (SS) has been performed since 1973. However, little has been reported on the electrocardiogram (ECG) changes and long-term prognosis in patients with hypertrophic cardiomyopathy (HCM) detected by the SS.

Methods: All 44 consecutive pediatric HCM patients (10.

Abnormal glucose metabolism in patients with Fontan circulation: Unique characteristics and associations with Fontan pathophysiology.

Background: Fontan patients exhibit a high prevalence of abnormal glucose metabolism (AGM). We aimed to characterize AGM and clarify its association with Fontan pathophysiology.

Methods: We prospectively evaluated AGM with plasma glucose dynamics [mg/dL; fasting glucose (FPG), and maximum glucose increase (PG-spike)] during oral glucose tolerance test and hemoglobin A1c (HbA1c) in 276 consecutive Fontan patients (aged 19 ± 7 years).

Evaluating the response to cardiac resynchronization therapy performed with a new ventricular morphology-based strategy for congenital heart disease.

In cardiac resynchronization therapy (CRT) for patients with congenital heart disease (CHD) and a ventricular morphology other than a systemic left ventricle (LV), we previously proposed pacing sites that are different from those used for a systemic LV. The leads should be placed laterally on opposite sides of both ventricles in patients with short-axis dyssynchrony and a single ventricular physiology with two ventricles, whereas they should be placed at the farthest sites along the longitudinal direction in the right ventricle (RV) in patients with long-axis dyssynchrony of the RV. Moreover, in patients with interventricular dyssynchrony and a biventricular physiology with a systemic RV, they should be placed at sites that both ventricles can contract simultaneously.

Cardiac Events and the Maximum Diameter of Coronary Artery Aneurysms in Kawasaki Disease.

Objectives: To clarify the occurrence of cardiac events based on the maximal diameter of the maximal coronary artery aneurysm (CAA) in Kawasaki disease (KD).

Study Design: Two hundred fourteen patients (160 male and 54 female) who had had at least 1 CAA in the selective coronary angiogram less than 100 days after the onset of KD were studied. We measured the maximal CAA diameters in the major branches of the initial coronary angiograms.

Hemodynamic determinants of mortality after Fontan operation.

Background: Elevated central venous pressure (CVP), low cardiac output, and mild hypoxia are common early and late after Fontan operations. However, the association of these characteristics with late mortality is unclear. We aimed to elucidate the hemodynamic determinants of mortality after Fontan operation.

Mid-Term Follow-up of School-Aged Children With Borderline Long QT Interval.

Background: There are no definitive diagnostic criteria or follow-up strategies for long QT syndrome (LQTS) in children with a borderline long QT interval (b-LQT).

Methods and results: We retrospectively evaluated the clinical course, genetic testing results, corrected QT interval (QTc), and LQTS score of 59 school-aged children (5-18 years old) with a b-LQT (400≤QTc<500 ms). Syncope, but neither aborted cardiac arrest nor sudden cardiac death, occurred in 2 patients during the follow-up (6±3.

Determinants of Aortic Size and Stiffness and the Impact on Exercise Physiology in Patients After the Fontan Operation.

The pathophysiology of congenital heart disease includes aortic dilation and increased stiffness. However, the clinical determinants and significance remain unclear in patients after the Fontan operation.Size and stiffness index (SI) of the ascending and descending aorta (aAO and dAO, respectively) were assessed using angiography in 130 consecutive Fontan patients and 30 age-matched controls.

Interventricular dyssynchrony in a patient with a biventricular physiology and a systemic right ventricle.

We describe of a unique pattern of cardiac dyssynchrony in a patient with a biventricular physiology and systemic right ventricle (RV): the interventricular dyssynchrony due to a contraction delay between the left ventricle and RV. In the present case, the cineangiography and intracardiac electrocardiography of the RV did not reveal intraventricular dyssynchrony of the RV, but revealed interventricular dyssynchrony. In addition, the pressure curves of the ventricles exhibited time phase differences between the two ventricles.

Comorbid Epilepsy and Developmental Disorders in Congenital Long QT Syndrome With Life-Threatening Perinatal Arrhythmias.

Objectives: Given the association of long QT syndrome (LQTS) and neurological disorders, we speculated that the more severe LQTS phenotype, perinatal LQTS, would exhibit more frequent comorbid neurodevelopmental anomalies than LQTS without perinatal arrhythmias (nonperinatal LQTS).

Background: Congenital LQTS with life-threatening perinatal arrhythmias (perinatal LQTS) has a poor life prognosis.

Methods: Twenty-one consecutive LQTS patients diagnosed before 1 year of age at our institution and 3 previously reported perinatal LQTS patients with neurological seizures were enrolled.

Heart failure with preserved right ventricular ejection fraction in postoperative adults with congenital heart disease: A subtype of severe right ventricular pathophysiology.

Background: Right ventricle (RV) may determine heart failure (HF) severity in adults with congenital heart disease (ACHD). However, the association of RV properties with clinical profiles remains unclear.

Purpose: To clarify the associations of RV properties with biomarkers, exercise capacity, and unscheduled hospitalization (USH) in postoperative ACHD patients.

Hyperuricemia reflects global Fontan pathophysiology and associates with morbidity and mortality in patients after the Fontan operation.

Background: Serum uric acid (UA, mg/dl) levels associate with the pathophysiology and prognosis in patients with chronic heart failure.

Purpose: To clarify the clinical significance of hyperuricemia (HUA, UA>7.0) in Fontan patients.

Prognostic value of exercise variables in 335 patients after the Fontan operation: a 23-year single-center experience of cardiopulmonary exercise testing.

Background: The prognostic value of cardiopulmonary exercise testing (CPX) for mortality risk remains controversial in Fontan patients.

Purpose: Our goal of the present study was to clarify the prognostic value of major CPX variables and the factors determining exercise capacity in a large cohort of Fontan patients.

Methods: Since 1990, heart rate (HR), oxygen uptake (VO2 ), and ventilatory equivalent for carbon dioxide production (VE/VCO2 ) at peak exercise were determined in 335 Fontan patients (18 ± 5 years old), and the CPX variables were compared with the clinical profile and events.

Low fasting plasma glucose level predicts morbidity and mortality in symptomatic adults with congenital heart disease.

Background: Adults with complex congenital heart disease (ACHD) have a high prevalence of abnormal glucose regulation (AGR: impaired glucose tolerance and diabetes mellitus). However, the impact of AGR on the prognosis remains unclear.

Purpose: Our purpose was to clarify the prognostic value of AGR in ACHD.

Prevalence and predictors of haemostatic complications in 412 Fontan patients: their relation to anticoagulation and haemodynamics.

Objectives: Our aim in the present study was to determine the prevalence of haemostatic events in our Fontan patients, to identify predictive factors and to determine their association with haemodynamics and anticoagulant therapy.

Methods: We retrospectively evaluated 424 Fontan patients and examined correlations between postoperative haemodynamics and anticoagulant regimens with haemostatic events.

Results: After exclusion of 12 patients with a mechanical valve at the time of Fontan operation, our 412 patients were sub-divided into 21 groups based on the therapeutic duration of warfarin and antiplatelet agent therapy.