Significance of End-Diastolic Forward Flow in Patients With Repaired Tetralogy of Fallot - Its Interaction With the Left Ventricular Property and End Organ Damage.

Background: Although right ventricular (RV) enlargement may affect RV diastolic dysfunction assessed by end-diastolic forward flow (EDFF) in patients with repaired tetralogy of Fallot (TOF), EDFF may also be modified by left ventricular (LV) hemodynamics. We hypothesized that EDFF is affected by LV hemodynamics, not limited to RV diastolic stiffening.

Methods and results: Among 145 consecutive patients with repaired TOF who underwent catheterization, hemodynamic properties in 47 with consistent EDFF and 75 without EDFF were analyzed.

Chronological T-wave alternation before and after the onset of arrhythmogenic right ventricular cardiomyopathy.

Identification of arrhythmogenic right ventricular cardiomyopathy (ARVC) during childhood is challenging due to the lack of specific ECG manifestation. We report chronological ECG alteration before several years of the ARVC onset in two affected children. Their ECG at the age of 6 years was almost normal for their age, and their chronological ECGs exhibited inversion of T wave in inferior leads, which are typical for ARVC, developed at younger age than that in precordial leads.

Venous Properties in a Fontan Patient with Successful Remission of Protein-Losing Enteropathy.

We present the case of a 1-year-old boy who developed protein-losing enteropathy (PLE) within 2 months of a fenestrated Fontan procedure. His fenestration rapidly closed despite bilateral pulmonary stenosis (BPS). Subsequent to PLE onset, both fenestration and the bilateral pulmonary artery were reconstructed, and the patient's PLE had been in remission, with additive use of medications, for more than 2 years.

Echocardiogram Unmasked Hemodynamic Advantage of Atrial Pacing in Securing Ventricular Preload in a Fontan Patient with Junctional Rhythm.

While the advancement of perioperative management has expanded Fontan candidacy, not all patients have a successful postoperative course. Our case was a right isomerism patient who could not leave the ICU due to high central venous pressure and low output syndrome. Initial observation of the monitor ECG showed his rhythm to be supraventricular, however, an echocardiogram indicated simultaneous contraction of the atrium and ventricle, implying a junctional rhythm.

Prevalence of Short Stature and Growth Hormone Deficiency and Factors Associated With Short Stature After Fontan Surgery.

Fontan circulation is characterized by many features commonly observed in heart failure that may affect physical growth regardless of pituitary gland dysfunction status. The aims of the present study were to investigate the prevalence of short stature and growth hormone deficiency (GHD) and determine the factors associated with short stature after Fontan surgery. On retrospective evaluation of 47 patients after Fontan surgery, a very high prevalence of short stature was observed (38.

Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery.

Isolated unilateral absence of the pulmonary artery (UAPA) is a congenital anomaly where involution of the extrapulmonary PA is insufficient and the intrapulmonary PA is only fed by the ductus arteriosus. Affected lung disorder causes complications years after ductus closure; thus, early diagnosis is of importance to avoid these complications. Here, we present the case of a male infant who was admitted to the neonatal intensive care unit because of transient tachypnea of the newborn and absence of the left PA (LPA) was indicated.

Blood reservoir function in patients with Fontan circulation and asplenia syndrome.

Splanchnic circulation constitutes a major portion of the vasculature capacitance and plays an important role in maintaining blood perfusion. Because patients with asplenia syndrome lack this vascular bed as a blood reservoir, they may have a unique blood volume and distribution, which may be related to their vulnerability to the haemodynamic changes often observed in clinical practice. During cardiac catheterisation, the mean circulatory filling pressure was calculated with the Valsalva manoeuvre in 19 patients with Fontan circulation, including 5 patients with asplenia syndrome.

Fenestration in the Fontan circulation as a strategy for chronic cardioprotection.

Background: Fenestration in the Fontan circulation potentially liberates patients from factors leading to cardiovascular remodelling, through stable haemodynamics with attenuated venous congestion. We hypothesised that a fenestrated Fontan procedure would possess chronic haemodynamic advantages beyond the preload preservation.

Methods: We enrolled 35 patients with fenestrated Fontan with a constructed pressure-volume relationship under dobutamine (DOB) infusion and/or transient fenestration occlusion (TFO).

Thyroid Function in Patients With a Fontan Circulation.

In this study, we tested our hypothesis that thyroid function is impaired and contributes to perturbed hemodynamics in patients after Fontan operation. Cardiac catheterization and blood tests for thyroid function were performed in 37 patients who underwent a Fontan operation. Among them, 12 patients (33%) had subclinical thyroid dysfunction with an elevated thyroid-stimulating hormone level despite normal thyroxine levels.

Importance of dynamic central venous pressure in Fontan circulation.

We tested our hypotheses that central venous pressure (CVP) shows an excessive increase in response to volume overload in Fontan circulation according to the extent of the reduction in venous capacitance (Cv), and that the maximum CVP after volume loading is associated with hepatic congestion. Changes in CVP after angiography (volume loading) were examined in 40 patients with Fontan circulation and 29 controls with biventricular circulation. CVP significantly increased with angiography in both groups, but the changes were much more evident in the Fontan group than in controls (3.

Assessment of central venous physiology of Fontan circulation using peripheral venous pressure.

Objective: Elevated central venous pressure is a major cause of morbidity and mortality after the Fontan operation. The difference between mean circulatory filling pressure and central venous pressure, a driving force of venous return, is important in determining dynamic changes in central venous pressure in response to changes in ventricular properties or loading conditions. Thus, noninvasive central venous pressure and mean circulatory filling pressure estimation may contribute to optimal management in patients undergoing the Fontan operation.

Ventricular-vascular dynamics in pediatric patients with heart failure and preserved ejection fraction.

Objective: The details of the ventricular-vascular dynamics of heart failure with preserved ejection fraction (HFpEF) in children remain poorly understood. We tested the hypothesis that pediatric HFpEF patients have ventricular systolic, diastolic, and arterial stiffening at rest as well as impaired reserve function associated with coronary supply/demand imbalance.

Methods: We studied the ventricular pressure-area relationship in 22 pediatric HFpEF patients and 22 control subjects before and after dobutamine infusion and during abdominal compression.

Non-invasive assessment of liver fibrosis by magnetic resonance elastography in patients with congenital heart disease undergoing the Fontan procedure and intracardiac repair.

Background: The incidence of late liver complications such as fibrosis or cirrhosis has increased among patients who have undergone the Fontan procedure. Magnetic resonance elastography (MRE) recently emerged as a technique to clinically evaluate liver fibrosis. However, few reports have described its use in evaluating liver fibrosis in children with congenital heart disease (CHD).

Vulnerability of Coronary Circulation After Norwood Operation.

Background: We hypothesized that the myocardial oxygen supply-demand balance is impaired in patients after a Norwood procedure and that an abnormal oxygen supply-demand balance is associated with pronounced activation of the renin-angiotensin-aldosterone system and worse clinical outcome after this procedure.

Methods: To investigate the myocardial oxygen supply-demand balance, the subendocardial viability ratio (SEVR) was measured in 29 hypoplastic left heart syndrome patients after the Norwood procedure, in 27 patients with pulmonary atresia whose pulmonary blood flow was supplied from the aortopulmonary (AP) shunt, and in 30 control patients who were considered to have normal biventricular circulation. The SEVR in Norwood (0.

Novel mechanisms for cerebral blood flow regulation in patients with congenital heart disease.

Background: The mechanisms that regulate cerebral flow in patients after surgery for congenital heart diseases (CHDs) remain poorly understood. We tested our hypothesis that postoperative patients with CHD have disease- or hemodynamic-specific compensatory mechanisms for maintaining cerebral perfusion.

Methods: A total of 89 children with specific hemodynamics including Glenn (n = 14), Fontan (n = 19), repaired tetralogy of Fallot (n = 24), and control patients (n = 32) were enrolled.

Clinical Evaluation of the Hemodynamic Effects of the High-Flow Nasal Cannula Therapy on the Fontan Circulation.

High-flow nasal cannula (HFNC) therapy supports respiratory effort with a minimal elevation in airway pressure. We examined hemodynamic effects of HFNC therapy in a 10-year-old girl with Fontan circulation, in which positive airway pressure has deleterious hemodynamic effects. The HFNC therapy at 30 L/min improved oxygenation without an increase in central venous pressure.

Influence of Cardiac Function and Loading Conditions on the Myocardial Performance Index - Theoretical Analysis Based on a Mathematical Model.

Background: The myocardial performance index (MPI) has emerged as a Doppler-derived index for global ventricular function capable of estimating combined systolic and diastolic performance. While several studies have reported its load-dependency, responses of the MPI to various hemodynamic changes have not been fully characterized.

Methods And Results: The response characteristics of the MPI were examined and compared with ejection fractions (EF) by changing hemodynamic parameters within the physiological range in a lumped parameter model of the cardiovascular system.

Cardiac biomarkers in children with congenital heart disease.

Background: Most congenital heart diseases (CHDs) have specific hemodynamics, including volume and pressure overload, as well as cyanosis and pulmonary hypertension, associated with anatomical abnormalities. Such hemodynamic abnormalities can cause activation of neurohormones, inflammatory cytokines, fibroblasts, and vascular endothelial cells, which in turn contribute to the development of pathologic conditions such as cardiac hypertrophy, fibrosis, and cardiac cell damages and death. Measuring biomarker levels facilitates the prediction of these pathological changes, and provides information about the stress placed on the myocardial cells, the severity of the damage, the responses of neurohumoral factors, and the remodeling of the ventricle.

Prevalence, implication, and determinants of worsening renal function after surgery for congenital heart disease.

Accumulating data in adults indicate the prognostic importance of worsening renal function (WRF) during treatment of acute heart failure. Venous congestion appears to play a dominant role in WRF; however, data regarding WRF in children with congenital heart disease (CHD) are limited. The present study was conducted to elucidate the prevalence and characteristics of WRF after surgery for CHD in children.

Influence of Left Ventricular Stiffness on Hemodynamics in Patients With Untreated Atrial Septal Defects.

Background: Although left ventricular (LV) stiffening with age is believed to increase left-to-right shunting in patients with atrial septal defects (ASD), clinical data have not confirmed this. We sought determinants of the pulmonary-to-systemic flow ratio (Qp/Qs) in patients with untreated ASD.

Methods And Results: We retrospectively studied 180 patients with ASD who underwent percutaneous ASD closure between 2007 and 2011.

Cystatin C and body surface area are major determinants of the ratio of N-terminal pro-brain natriuretic peptide to brain natriuretic peptide levels in children.

Background: Production of N-terminal pro-brain natriuretic peptide (NT) and BNP is equimolar. Although NT clearance occurs only in the kidneys, BNP clearance occurs in the kidneys and other organs. This study tested the hypothesis that NT/BNP ratio in children may be independently related to cystatin C (CysC), a glomerular filtration rate marker, when diastolic function and age/body size are taken into consideration.

Arterial stiffness in patients after Kawasaki disease without coronary artery involvement: Assessment by performing brachial ankle pulse wave velocity and cardio-ankle vascular index.

Background: It remains unclear whether systemic arterial beds other than the coronary arteries are truly healthy in patients without coronary artery lesions (CAL) after Kawasaki disease (KD). We tested the hypothesis that patients with KD without echocardiographic evidence of CAL during the acute phase of the disease have abnormal mechanical properties in systemic arteries later.

Methods And Results: We studied 201 consecutive patients with KD (age 2-23 years, mean 10±4 years; 109 male, 92 female) without CAL during the acute phase.