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Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fon...

Right ventricular (RV) volume assessment by three-dimensional (3D) transthoracic echocardiography is recommended but one of the limitations is its poor acoustic window. We attempted 3D RV analysis using transesophageal echocardiography (TEE), which could maintain a wide region of interest for the RV

There is a wide range from imaging using whole heart coronary sequences to imaging using 2D steady state free precession (SSFP). In recent years, there has also been a report on blood vessel imaging by non-contrast radial scan using gradient echo. Siemens MAGNETOM Sola was introduced from April

Makoto Satoh1)*, Kanki Inoue2), Taku Ishii1), Kanako Kishiki1), Akio Inage1), Yuki Nakamoto1), Yuichi Ishikawa3), Tomomi Ueda1), Tadahiro Yoshikawa1), and In-Sam Park1) 1) Department of Pediatric Cardiology, Sakakibara Heart Institute, Japan Research Promotion Society for Cardiovascular Diseases, Tokyo, Japan 2) Department of Cardiology, Sakakibara Heart Institute, Japan Research Promotion Society for Cardiovascular Diseases, Tokyo, Japan 3) Cardiovascular Imaging Clinic, Iidabashi, Tokyo, Japan * Current a liation: Department of Pediatrics, Yamagata University Faculty of Medicine, Yamagata, Japan

The objective of this study was to investigate into correlation between right ventricular (RV) myocardial deformation and tricuspid valve (TV) displacement, and functional parameters in repaired tetralogy of Fallot (TOF) compared to normal subjects using new magnetic resonance tracking program. We

Echocardiographic estimation of right atrial pressure (RAP) using inferior vena cava (IVC) dimensions and its collapsibility in children remains controversial. To access IVC measurements without the influence of translation of IVC into another plane during respiration is essential but challenging.

Central hypercapnic chemosensitivity (Chemo) influences the enhanced ventilatory and sympathetic responses in heart failure patients; however, its influence on these responses in Fontan patients is unknown.To measure Chemo and compare the results with rest and exercise ventilatory characteristics in Fontan patients.We measured Chemo (l/min/mmHg), hemodynamics, pulmonary function, cardiac autonomic nervous and neurohumoral activities and compared the results with the ventilatory response during exercise in 42 Fontan patients and 12 referents. Chemo did not differ significantly between the Fontan patients (1.5 +/- 0.9) and referents (1.3 +/- 0.4). However, a higher Chemo in addition to lower resting arterial oxygen saturation (SaO(2)) and higher dead space ventilation (Vd/Vt) independently determined a higher resting minute ventilation (VE) and, except for the Chemo, these factors also independently determined the higher resting ventilatory equivalent for carbon dioxide output (VE/VCO(2)) (p0.05-0.001). At peak exercise, the higher Chemo as well as the higher peak Vd/Vt and aerobic exercise capacity independently determined the higher peak VE and VE/VCO(2) (p0.01-0.001). Among cardiac autonomic and neurohumoral activities, only the higher plasma norepinephrine concentration was associated with higher Chemo in Fontan patients (r=0.40, p0.01) and age was correlated positively with Chemo in the high Chemo (or =2.1) Fontan patients (n=10).In addition to lower SaO(2) and higher Vd/Vt, an increased Chemo associated with sympathetic activation has a significant impact on accelerated rest and exercise ventilation in some Fontan patients, especially in adult patients.

Abnormal exercise-related heart rate (HR) dynamics, that is, blunted exercise HR response, lower peak HR, and delayed HR recovery after exercise, are associated with high morbidity and mortality in adults with acquired and congenital heart disease (CHD). However, the precise mechanisms underlying the abnormal HR dynamics remain unclear.The purpose of this study is to evaluate the precise contribution of cardiac autonomic nervous activity (CANA) and sinus node function on exercise-related HR dynamics in postoperative patients with CHD.We analyzed our previous data in 53 postoperative patients with CHD who had undergone pharmacologic evaluation, including intrinsic HR, and compared the results with HR dynamics.Intrinsic HR (84% +/- 11%) was lower than the expected value and independently correlated with resting HR (P.05). Univariate analysis demonstrated that all CANA indices significantly correlated with most HR dynamic parameters. On multivariate analysis, basal parasympathetic nervous activity significantly influenced all HR dynamics (P.05-.0001), except for peak HR, whereas postsynaptic beta sensitivity of the sinus node significantly influenced all HR dynamics (P.05-.001), except for early HR recovery. Resting plasma norepinephrine significantly correlated with all HR dynamics (P.05-.001), except for resting HR.Lower resting and peak HRs are independently associated with intrinsic sinus node dysfunction and abnormal sympathetic CANA, respectively. A blunted HR increase and delayed early HR recovery are independently associated with impaired sympathetic and parasympathetic CANAs with the greater influence on a blunted exercise HR increase.

Severe pulmonary arterial hypertension (PAH) has high mortality, often secondary to right ventricular failure. It is difficult to predict when PAH patients may decompensate. We hypothesized that inter-chamber relationships using simultaneous measurement of longitudinal strain (LS) in all 4 cardiac

Background— Inherited cardiac arrhythmia susceptibility contributes to sudden death during infancy and may contribute to perinatal and neonatal mortality, but the molecular basis of this risk and the relationship to genetic disorders presenting later in life is unclear. We studied the functional and pharmacological properties of a novel de novo cardiac sodium channel gene ( SCN5A ) mutation associated with an extremely severe perinatal presentation of long-QT syndrome in unrelated probands of different ethnicity. Methods and Results— Two subjects exhibiting severe fetal and perinatal ventricular arrhythmias were screened for SCN5A mutations, and the functional properties of a novel missense mutation (G1631D) were determined by whole-cell patch clamp recording. In vitro electrophysiological studies revealed a profound defect in sodium channel function characterized by ≈10-fold slowing of inactivation, increased persistent current, slowing of recovery from inactivation, and depolarized voltage dependence of activation and inactivation. Single-channel recordings demonstrated increased frequency of late openings, prolonged mean open time, and increased latency to first opening for the mutant. Subjects carrying this mutation responded clinically to the combination of mexiletine with propranolol and survived. Pharmacologically, the mutant exhibited 2-fold greater tonic and use-dependent mexiletine block than wild-type channels. The mutant also exhibited enhanced tonic (2.4-fold) and use-dependent block (≈5-fold) by propranolol, and we observed additive effects of the 2 drugs on the mutant. Conclusions— Our study demonstrates the molecular basis for a malignant perinatal presentation of long-QT syndrome, illustrates novel functional and pharmacological properties of SCN5A -G1631D, which caused the disorder, and reveals therapeutic benefits of propranolol block of mutant sodium channels in this setting.