Valley index as a predictor of prenatal diagnosis of total anomalous pulmonary venous connection.

INTRODUCTION: Total anomalous pulmonary venous connection (TAPVC) has a low prenatal diagnostic rate. Therefore, we investigated whether Doppler waveforms with a low pulsatility in the pulmonary veins can indicate fetal TAPVC. METHODS: This retrospective study included 16 fetuses with TAPVC, including 10 with complex congenital heart disease and 104 healthy fetuses that underwent fetal echocardiography. Pulmonary venous S and D wave flow velocities and the valley (representing the lowest velocity between the S and D waves) were measured. Valley indices I and II were then calculated as (velocity of valley/ greater of the S and D wave velocities) and (velocity of valley/lesser of the S and D wave velocities), respectively. RESULTS: Supra/infracardiac TAPVC cases exhibited significantly greater valley indices than that of the healthy group. After adjusting for gestational age at fetal echocardiography, valley index I (odds ratio [OR] 7.26, p < 0.01) and II (OR 9.23, p < 0.01) were significant predictors of supra/infracardiac TAPVC. Furthermore, valley indices I and II exhibited a high area under the curve for detecting supra/infracardiac TAPVC, regardless of the presence of pulmonary venous obstruction. CONCLUSION: The valley index may be a useful tool for the detection of fetal TAPVC.

Initial national investigation of the prenatal diagnosis of congenital heart malformations in Japan-Regional Detection Rate and Emergency Transfer from 2013 to 2017.

BACKGROUND: Investigation into the detection rate (DR) of congenital heart diseases (CHDs) in fetuses is important for the assessment of fetal cardiac screening systems. OBJECTIVES: We highlight issues of fetal cardiac screening in Japan. METHODS: We performed an initial national survey of fetal diagnosis of CHDs from the data of the national registry for congenital heart surgery from 2013 to 2017. Subjects were neonates and infants with moderate or severe CHDs. We investigated DR in each prefecture in Japan and emergency transfer (ET) for neonates by analyzing distance and admission day of ET with or without fetal diagnoses. RESULTS: The overall average DR in Japan was 0.41 (0.02 increase every year). No regional significant relationship was found between DR and population in each prefecture. ET was performed in 12% of neonates with prenatal diagnosis and in 63% of neonates without resulting in significant risk for ET in fetuses without a fetal diagnosis [OR 13.3 (11.6-15.3), p<0.001]. The distance of ET was shorter and admission was earlier in the neonates with a prenatal diagnosis than in those without [median 6.6 km (IQR: 4.1-25.7) vs 17.0 km (IQR: 7.4-35.3), median 0.0 day (IQR: 0.0-0.0) vs 0.0 day (IQR: 0.0-1.0), p<0.001, p<0.001, respectively] CONCLUSIONS: Prenatal cardiac diagnosis reduces geographic and chronological risks of ET for moderate to severe CHDs. DR is still developing and periodic official surveillance is required for improving prenatal cardiac diagnosis in Japan.

Paclitaxel-coated drug-eluting balloon for pulmonary vein stenosis after repair of total anomalous pulmonary venous return with asplenia.

We used a paclitaxel-coated drug-eluting balloon (DEB) for pulmonary vein stenosis (PVS) after repair of total anomalous pulmonary venous return with asplenia. No adverse effect of paclitaxel was revealed and plasma concentration of paclitaxel was not elevated after intervention. Although progression of PVS slowed after dilatation with the DEB, stenosis recurred relentlessly, resulting in patient demise. This strategy may be safe for infantile cases, but requires further effective strategy, such as usage of larger sizes of DEBs. .

Association of Preoperative Mixed Venous Oxygen Saturation with Postoperative Segmental Pulmonary Hypertension in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals.

In patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), segmental pulmonary hypertension is common; however, its pathophysiology remains to be elucidated. This study aimed to identify preoperative hemodynamic factors associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs. This study included 12 patients with PA/VSD/MAPCAs who underwent unifocalization and intracardiac repair from 2009 at our institution. Hemodynamic measurements of preoperative and postoperative cardiac catheterization were collected. Patients were considered to have pulmonary hypertension if the mean pulmonary pressure measured postoperatively at any peripheral pulmonary artery was ≥ 20 mmHg. The data were compared between patients with and without postoperative segmental pulmonary hypertension; p value < 0.05 was considered statistically significant. Intracardiac repair was performed at the age of 2.1 years (range 0.5-28.2 years). Unifocalization was performed separately prior to intracardiac repair in two patients and concomitantly in 10 patients. Postoperative cardiac catheterization, performed 1.2 years (range 0.2-8.7 years) after intracardiac repair, revealed that five patients had segmental pulmonary hypertension. Patients with postoperative pulmonary hypertension had significantly lower preoperative mixed venous oxygen saturation than those without pulmonary hypertension (57% [55-63%] vs. 65% [53-75%], p < 0.05). No significant differences were found between the groups in terms of age at intracardiac repair, preoperative arterial oxygen saturation, and preoperative peripheral pulmonary arterial mean pressure. Lower preoperative mixed venous oxygen saturation was associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs.

Meropenem pharmacokinetics during extracorporeal membrane oxygenation and continuous haemodialysis: a case report.

OBJECTIVES: Pharmacokinetic (PK) parameters can change significantly during extracorporeal membrane oxygenation (ECMO) and continuous haemodialysis. This case report describes the pharmacokinetics of a 3-h meropenem infusion in an infantile anuric patient on ECMO with continuous haemodialysis. CASE: A 19-month-old female patient with asplenia syndrome was admitted to the paediatric intensive care unit for postoperative management of an extracardiac total cavopulmonary connection procedure. Veno-arterial ECMO and continuous haemodialysis were initiated on postoperative Day 2 for circulatory insufficiency due to septic shock and thrombosis of the inferior vena cava extending to the pulmonary artery. Blood and ascites cultures were positive for extended-spectrum ß-lactamase-producing Escherichia coli, and 3-h meropenem infusions [120-300 mg/kg/day divided every 8 h (q8h)] were commenced. Following dose escalation to 300 mg/kg/day q8h, sustained negative blood cultures were confirmed. The estimated meropenem clearance and volume of distribution (Vd) were 2.21 mL/kg/min and 0.59 L/kg, respectively. These patient-specific PK parameters were used to predict the PK profile of various dosing regimens. Both 1-h and 3-h infusions of meropenem at 60, 120 and 200 mg/kg/day q8h predicted that the free drug concentration would remain above the minimum inhibitory concentration (fT>MIC) at an MIC of 1 µg/mL for >40% of the dosing interval. However, when the target was set at 100% fT>MIC, only a 3-h infusion of 200 mg/kg/day q8h could achieve the target in this patient despite the presence of anuria. CONCLUSION: To optimise meropenem dosing in paediatric patients on ECMO and continuous haemodialysis, further study and PK monitoring are warranted.

Effect and Complications of Everolimus Use for Giant Cardiac Rhabdomyomas with Neonatal Tuberous Sclerosis.

Most cardiac rhabdomyomas with tuberous sclerosis (TS) are asymptomatic and spontaneously regress. However, some cases require surgical intervention due to arrhythmia and severe obstruction of cardiac inflow or outflow. We report herein a neonatal case of giant cardiac rhabdomyomas with TS and insufficient pulmonary blood flow from the right ventricle. Lipoprostaglandin E1 was necessary to maintain patency of the ductus arteriosus. We used everolimus, a mammalian target of rapamycin inhibitor, to diminish the cardiac rhabdomyomas. After treatment, the rhabdomyomas shrank rapidly, but the serum concentration of everolimus increased sharply (maximum serum trough level: 76.1 ng/mL) and induced complications including pulmonary hemorrhage, liver dysfunction, and acne. After the everolimus level decreased, the complications resolved. Everolimus may be a viable treatment option for rhabdomyomas, but its concentration requires close monitoring to circumvent complications associated with its use.

Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome.

A 5-month-old girl with single ventricle, interrupted inferior vena cava and polysplenia syndrome palliated with bilateral Blalock-Taussig shunts developed severe cyanosis despite apparently increased pulmonary blood flow. Angiography revealed diffuse pulmonary arteriolar capillary dilatation and early pulmonary venous filling, suggesting the presence of pulmonary arteriovenous malformations. Abdominal angiography at 6 months demonstrated a large extrahepatic portosystemic shunt, which was percutaneously closed with a vascular plug. Kawashima operation was performed 2 weeks after portosystemic shunt closure. Although cyanosis improved temporarily, the patient suffered from deteriorating cyanosis at 9 months of age and underwent Fontan completion. Thereafter, her oxygen saturation gradually improved to 95% over the course of 3 weeks. Both the congenital extrahepatic portosystemic shunt and Kawashima operation contributed to the development of pulmonary arteriovenous malformations.

Vascular ring associated with d-transposition of the great arteries: when should we suspect aortic arch anomalies?

A male neonate with d-transposition of the great arteries was diagnosed with the concomitant anomaly of left circumflex aortic arch and right-sided ductus arteriosus, which formed a vascular ring. Initial postnatal echocardiography had demonstrated an obscured aortic isthmus mimicking coarctation of the aorta, which could be a diagnostic clue to circumflex aortic arch.

Echocardiographic assessment of ventricular contraction and synchrony in children with isolated complete atrioventricular block and epicardial pacing: Implications of interventricular mechanical delay.

AIMS: To assess the correlations between interventricular mechanical delay (IVMD) and cardiac function in children with isolated complete atrioventricular block and epicardial pacing. METHODS AND RESULTS: We enrolled 13 children (six boys) with an epicardial dual-chamber pacemaker due to isolated complete or advanced atrioventricular block. The patients were 9.8 (range, 6.8-15.3) years old, and none had symptomatic heart failure at the follow-up visit. Ventricular pacing sites, which remained the same for 7.2 (1.6-12.3) years, were the left ventricle (LV) in two patients, right ventricle (RV) in four patients, and both ventricles in seven patients. IVMD was ≤40 ms in six patients (short IVMD group) and >40 ms in seven patients (long IVMD group). Compared with the long IVMD group, the short IVMD group was associated with better LV longitudinal strain (-20% [-24% to -18%] vs -16% [-20% to -13%], P < .05). The short IVMD group had superior LV mechanical synchrony than the long IVMD group (septal to lateral delay of the time to peak longitudinal strain 15 [-16-78] ms vs 78 [13-93] ms, P < .05; standard deviation of the time to peak radial strain 13 [9-34] ms vs 35 [18-64] ms, P < .05). CONCLUSION: In children with epicardial pacing at LV, RV, or both ventricles, a left-sided contraction delay was associated with poorer LV contraction and impaired LV synchrony. IVMD will help to stratify patients during follow-up.

Flow Energy Loss as a Predictive Parameter for Right Ventricular Deterioration Caused by Pulmonary Regurgitation After Tetralogy of Fallot Repair.

The optimal timing for pulmonary valve replacement after Tetralogy of Fallot (TOF) repair remains controversial. In this study, we estimated the feasibility of using flow energy loss (FEL) to predict right ventricular (RV) deterioration due to pulmonary regurgitation after TOF repair. We examined RV outflow tract (RVOT) flow in nine patients who underwent TOF or double-outlet right ventricle repair in the intervention group (Group I) and compared them with three healthy children in the control group (Group C). We evaluated flow across the RVOT and pulmonary valve by vector flow mapping (VFM) on echocardiography and by phase contrast-magnetic resonance imaging (PC-MRI). Next, we calculated FEL and analyzed the relationship between FEL and clinical parameters of RV function. The mean FEL was significantly greater in Group I than in Group C (p = 0.002). Flow pattern and FEL were comparable by VFM and PC-MRI at the same phase 14.6 years after TOF repair. There was a significant positive correlation for the cardiothoracic ratio with both the mean FEL [correlation coefficient (r) = 0.78; p = 0.012] and the diastolic peak FEL (r = 0.75; p = 0.021) in Group I. There was also a significant positive correlation between the serial change in QRS duration with both the mean FEL (r = 0.82; p = 0.014) and the diastolic FEL (r = 0.70; p = 0.052) in Group I. FEL by VFM is an effective tool for evaluating ventricular deterioration caused by RV workload.

Effect of i.v. immunoglobulin in the first 4 days of illness in Kawasaki disease.

BACKGROUND: Although early treatment of Kawasaki disease (KD) with i.v. immunoglobulin (IVIG) is expected to prevent coronary artery abnormalities, the effectiveness of IVIG by day 4 of illness remains to be determined. METHODS: This was a multi-institutional, retrospective cohort study. Patients diagnosed with KD at ≤4 days of illness were divided into two groups: those who received initial IVIG before and on day 5 of illness. Baseline characteristics were adjusted using propensity scores. The primary endpoint was the need for additional treatment. RESULTS: Of 339 patients diagnosed with KD by day 4, 181 and 158 received IVIG before and on day 5 of illness, respectively. Patients in the early treatment group had more adverse prognostic factors: infancy, early onset of the principal symptoms, and abnormal laboratory data. We thus adjusted baseline characteristics before treatment decisions using propensity scores. Propensity score matching of the two groups yielded 100 observations. More patients required additional treatment in the matched early treatment group: 37% vs 24% (adjusted OR, 1.7; 95%CI: 1.06-2.8; P = 0.047). The difference was more pronounced for risk of relapse after initial resolution of fever: 14% vs 5.0% (adjusted OR, 3.2; 95%CI: 1.3-7.7; P = 0.02). The risk of coronary artery lesion did not differ significantly. CONCLUSIONS: IVIG treatment by day 4 of illness is associated with the requirement for additional treatment even after adjustment of baseline characteristics. Increased resistance to IVIG when given by day 4 should be considered in order to improve the treatment regimen for early-diagnosed KD.

Novel Risk Assessment Tool for Immunoglobulin Resistance in Kawasaki Disease: Application Using a Random Forest Classifier.

BACKGROUND: Resistance to intravenous immunoglobulin (IVIG) therapy is a risk factor for coronary lesions in patients with Kawasaki disease (KD). Risk-adjusted initial therapy may improve coronary outcome in KD, but identification of high risk patients remains a challenge. This study aimed to develop a new risk assessment tool for IVIG resistance using advanced statistical techniques. METHODS: Data were retrospectively collected from KD patients receiving IVIG therapy, including demographic characteristics, signs and symptoms of KD and laboratory results. A random forest (RF) classifier, a tree-based machine learning technique, was applied to these data. The correlation between each variable and risk of IVIG resistance was estimated. RESULTS: Data were obtained from 767 patients with KD, including 170 (22.1%) who were refractory to initial IVIG therapy. The predictive tool based on the RF algorithm had an area under the receiver operating characteristic curve of 0.916, a sensitivity of 79.7% and a specificity of 87.3%. Its misclassification rate in the general patient population was estimated to be 15.5%. RF also identified markers related to IVIG resistance such as abnormal liver markers and percentage neutrophils, displaying relationships between these markers and predicted risk. CONCLUSIONS: The RF classifier reliably identified KD patients at high risk for IVIG resistance, presenting clinical markers relevant to treatment failure. Evaluation in other patient populations is required to determine whether this risk assessment tool relying on RF has clinical value.

Echocardiographic assessment of prosthetic mitral valves in children.

AIMS: We studied how Doppler-derived hemodynamic parameters in children change as the relative prosthetic mitral valve (PMV) size decreases with somatic growth and evaluated the diagnostic utility of the parameters for detecting PMV obstruction in children. METHODS AND RESULTS: We reviewed 26 echocardiographic examination results of 15 mechanical bileaflet PMVs in 12 children. The median age at echocardiographic examination was 6.6 (0.6-18.1) years. The PMV functioned normally in 24 examinations but was obstructed due to thrombosis in two cases. PMV sizes ranged between 16 and 25 mm, which were standardized to body surface area (BSA) at the examination with z-score calculations. We assessed the peak E velocity, mean pressure gradient (PG), and pressure half time (PHT) of the transprosthetic flow, the velocity-time integral (VTI) ratio of the PMV inflow to the left ventricular outflow, and the BSA-indexed effective orifice area (iEOA) of the PMV calculated with the continuity equation. Linear regression analysis revealed statistically significant correlations between all parameters of normally functioning PMVs and the PMV size z-scores (Pearson correlation coefficients: peak E velocity, -0.68; mean PG, -0.71; PHT, -0.82; VTI ratio, -0.76; iEOA, 0.79). Compared with the predictive values derived from the regression equations, the VTI ratio and iEOA exceeded ± 2 standard errors in both patients with obstructive PMVs. CONCLUSION: To assess PMV function in children, Doppler-derived hemodynamic parameters should be compared with their predictive values based on relative PMV sizes. The deviation of the VTI ratio and iEOA from their predictive values may indicate prosthetic obstruction.

Functional total anomalous pulmonary venous connection via levoatriocardinal vein.

We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the "dead-end" left atrium, the pulmonary venous flow had a to-and-fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left-side heart obstruction.

Clinical implications of mitral valve geometric alterations in children with dilated cardiomyopathy.

We aimed to elucidate the relationship between severity of secondary mitral regurgitation and mitral valve geometry in children with dilated cardiomyopathy. The medical records of 16 children with dilated cardiomyopathy (median age, 1.2 years; range, 0.4-12.3 years) were reviewed. Mitral valve geometry was evaluated by measuring coaptation depth using echocardiographic apical four-chamber views at the initial presentation. Patients were dichotomised according to the mitral regurgitation severity: patients with moderate or severe secondary mitral regurgitation (n=6) and those with mild secondary mitral regurgitation (n=10). A total of 58 healthy children were considered as normal controls, and a regression equation to predict coaptation depth by body surface area was derived: coaptation depth [mm]=4.37+1.34×ln (body surface area [m2]) (residual standard error, 0.49; adjusted R2, 0.68; p<0.0001). Compared with patients with mild secondary mitral regurgitation, those with moderate or severe secondary mitral regurgitation had significantly larger coaptation depth z-scores (6.4±2.3 versus 1.9±1.4, p<0.005), larger mitral annulus diameter z-scores (3.6±2.6 versus 0.9±1.8, p<0.05), higher left ventricular sphericity index (0.89±0.07 versus 0.79±0.06, p<0.005), and greater left ventricular fraction shortening (0.15±0.05 versus 0.09±0.05, p<0.05). In conclusion, geometric alteration in the mitral valve and the left ventricle is associated with the severity of secondary mitral regurgitation in paediatric dilated cardiomyopathy, which would provide a theoretical background to surgical intervention for secondary mitral regurgitation in paediatric populations.

Fever pattern and C-reactive protein predict response to rescue therapy in Kawasaki disease.

BACKGROUND: Evidence to guide rescue therapy in refractory Kawasaki disease (KD) is lacking. The aim of this study was to determine the most important variables in predicting non-response to rescue therapy in refractory KD. METHODS: We retrospectively analyzed 171 patients diagnosed with refractory KD resistant to initial i.v. immunoglobulin (IVIG). Participants received rescue therapy consisting of IVIG monotherapy or IVIG plus prednisolone. Characteristics and laboratory variables were compared between rescue therapy non-responders and responders. Multivariate logistic regression analysis was performed to determine the independent predictors of non-response to rescue therapy. RESULTS: Among the 171 participants, 54 (31.6%) were non-responders to rescue therapy. On univariate analysis, fever pattern after initial IVIG, day of illness at rescue therapy, rescue therapy regimen and six laboratory variables (pre-IVIG sodium, C-reactive protein [CRP]; post-IVIG white blood cell count, platelet count, sodium, CRP) were useful in discriminating between non-responders and responders. These nine variables were included in multivariate logistic regression analysis. Persistent fever after initial IVIG (aOR, 2.39; 95%CI: 1.07-5.37) and post-IVIG CRP (aOR, 1.09; 95%CI: 1.02-1.17, per 1 mg/dL increase) were identified as independent predictors of non-response to rescue therapy. IVIG rescue monotherapy (aOR, 3.05; 95%CI: 1.05-8.84) also predicted non-response after adjusting for fever pattern and post-IVIG CRP. CONCLUSIONS: Persistent fever and elevated CRP after initial IVIG are predictive of non-response to rescue therapy for refractory KD. For patients at high risk of non-response, IVIG plus prednisolone, or even further intensified rescue therapy regimens may be preferable.

Total anomalous pulmonary venous connection with ccTGA and VSD: Can pulmonary artery banding avert pulmonary venous obstruction?

We describe a rare case of infracardiac total anomalous pulmonary venous connection (TAPVC), associated with congenitally corrected transposition of the great arteries (ccTGA) and ventricular septal defect, in which the patient had undergone pulmonary artery banding (PAB) at 16 days of age. She began to have episodes of severe cyanosis while crying, 2 weeks after PAB. Cardiac catheterization at 34 days of age showed severe pulmonary hypertension and a transhepatic pressure gradient of 7 mmHg. The infant underwent TAPVC repair and conventional repair for ccTGA at 35 days of age. Although PAB might have the provisional effect of delaying the manifestation of pulmonary venous obstruction (PVO), it is unable to prevent the development of PVO due to the high resistance of the hepatic sinusoids. Signs of PVO should be closely monitored so that TAPVC can be repaired in a timely fashion.

Dissipative energy loss within the left ventricle detected by vector flow mapping in children: Normal values and effects of age and heart rate.

BACKGROUND: Vector flow mapping (VFM) is a novel echocardiographic technology which enables visualization of the intraventricular flow velocity vector. Dissipative energy loss (EL) derived from the velocity vector field of intraventricular blood flow is considered to reflect the efficiency of blood flow, and could be an indicator of left ventricular function. We aimed to determine the reference values of the EL derived from VFM within the left ventricle. METHODS: VFM analysis was performed using echocardiography in 64 healthy children. The velocity vector fields of the intra-left ventricular blood flow were obtained from the apical 5-chamber view, and the EL values during systole and diastole were calculated. The measurements were averaged over three cardiac cycles, and indexed to body surface area (BSA). RESULTS: The mean subject age was 6.8±4.3 years. The mean EL was 4.10±2.35mW/m/m(2) BSA during systole and 16.24±11.63mW/m/m(2) BSA during diastole. On multivariate analysis, age and heart rate (HR) were independent predictors of systolic EL, whereas age, HR, and E wave peak velocity were independent predictors of diastolic EL. The regression equations used to predict the BSA-indexed systolic and diastolic EL were as follows: log10 (systolic EL)=-0.0332-0.00213×age (months)+0.00789×HR (beats/min) (adjusted R(2), 0.833; p<0.0001); and log10 (diastolic EL)=0.277-0.00346×age (months)+0.00570×HR (beats/min)+0.00564×E wave peak velocity (cm/s) (adjusted R(2), 0.867; p<0.0001). CONCLUSIONS: The systolic and diastolic EL were positively correlated with HR and negatively correlated with age. Moreover, the diastolic EL was positively correlated with the E wave peak velocity. The present study provides reference values for the systolic and diastolic EL that can be used in future studies examining patients with heart disease.

Serum hyaluronic acid concentration in Fontan circulation: correlation with hepatic function and portal vein hemodynamics.

Although liver fibrosis causes significant morbidity in the late postoperative period of the Fontan procedure, the diagnostic value of hyaluronic acid (HA), a serum marker of liver fibrosis, has not been established in Fontan patients. The purpose of this study was to determine whether increased serum HA concentration in Fontan patients is associated with an increase in inspiratory-to-expiratory flow rate ratio (Qin/Qex) of the portal vein (PV), which is indicative of liver fibrosis. We retrospectively studied 28 consecutive patients with Fontan circulation who underwent cardiac catheterisation for various indications. The median age at examination was 5.5 years (range 2.2-5.6). The median HA concentration was 17.7 ng mL(-1) (range 10.0-82.1), which was used to divide our 28 patients into two groups. Patients in the high-HA group had significantly greater Qin/Qex of the PV than those in the low-HA group (1.25 ± 0.12 vs. 1.12 ± 0.11, p < 0.05). Platelet counts were significantly lower in the high-HA group (216 ± 74 vs. 294 ± 104 × 10(9) L(-1), p < 0.05). No significant difference was found in inferior vena caval pressure. In conclusion, increase of HA concentration in Fontan patients accompanies the change in PV hemodynamics peculiar to liver cirrhosis and might be an early indicator of liver fibrosis.