Normal values and distribution of ventricular global longitudinal strain in 513 healthy fetuses measured by two-dimensional speckle-tracking echocardiography: a multi-institutional cohort study.

This study aimed to determine the normal reference values and distribution of global longitudinal strain (GLS) in the right and left ventricles of healthy Japanese fetuses during pregnancy. This multi-institutional cohort study included healthy Japanese fetuses during normal pregnancies without maternal or fetal complications between 18 and 40 weeks of gestation. Two-dimensional fetal echocardiographic images of the four-chamber view with a high frame rate were acquired and stored as DICOM clips. Data were collected and analyzed in a central laboratory to measure the left ventricular (LV) and right ventricular (RV) GLS using two-dimensional speckle tracking. In total, 513 fetuses were enrolled. The mean LV-GLS and RV-GLS were - 24.3% ± 3.5% and - 23.5% ± 3.7%, respectively. The magnitude of the GLS, with normal limits in both ventricles, decreased with advancing gestation. LV values were r = 0.34 (95% confidence interval, 0.27-0.42) and p < 0.0001; RV values were r = 0.33 (95% confidence interval, 0.25-0.41) and p < 0.0001. The normal values of healthy Japanese fetuses in healthy pregnancies is the first to be established by the large-scale, multi-institutional cohort study as LV-GLS of 24.3% ± 3.5% and RV-GLS of - 23.5% ± 3.7%, respectively. This can serve as a basic reference for assessing the cardiac functions in Japanese fetuses with various heart diseases.

Multiple atrial septal defects closure with a single device using radiofrequency energy-assisted wire atrial septostomy: A new method.

Percutaneous closure of multiple atrial septal defects can be more challenging. It is often discussed whether a single or dual device closure is appropriate for two or more large atrial septal defects with insufficient distance between defects. In this case, we used radiofrequency energy-assisted wire atrial septostomy to break intervening tissue between two adjacent oval fossa defects, thereby combining them into a single hole and facilitating device closure using a single device. This technique could be considered in patients with multiple adjacent secundum defects separated by intervening tissue.

Pulmonary veno-occlusive disease after respiratory syncytial virus infection in a post hematopoietic stem cell transplantation patient.

Background: Pulmonary veno-occlusive disease (PVOD) is a rare but fatal complication of hematopoietic stem cell transplantation (HSCT). Although literature on PVOD post-HSCT is scarce, a recent study has indicated that this condition may be underestimated. Respiratory syncytial virus (RSV) is a common respiratory pathogen that causes common cold in healthy individuals but may lead to severe lower respiratory infection accompanied by respiratory distress in infants and immunocompromised individuals, such as post-HSCT patients. However, little is known about the relationship between PVOD and RSV infections. Case report: A 4-year-old boy was diagnosed with metastatic neuroblastoma and underwent intensive chemotherapy, autologous HSCT, and allogeneic cord blood transplantation (CBT). He experienced PVOD on day 194 following CBT after displaying upper respiratory symptoms and positive RSV antigen test results approximately one month prior. Pathological examination of a lung biopsy specimen revealed lung injury suspected to be associated with viral infection in addition to PVOD-related findings, suggesting that RSV infection might have contributed to the onset of PVOD. Conclusions: The patient's clinical history and histological findings indicated that RSV could have triggered the development of PVOD under potential endothelial damage caused by HSCT and other prior treatments. Common respiratory viral infections, such as RSV infection, may evoke the development of PVOD.

Assessment of left atrial deformation in patients with total anomalous pulmonary venous connection by two-dimensional speckle-tracking echocardiography.

BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by impaired left ventricle growth and diastolic dysfunction. We hypothesized that the patients with TAPVC reduced blood flow into the left heart prenatally could affect left atrium (LA) not just growth but function. We compared the age-related changes in LA deformation using two-dimensional speckle-tracking echocardiography (2DSTE) in Patients with TAPVC. METHOD: This single-center, retrospective cohort study was conducted on consecutive isolated TAPVC patients who underwent neonatal surgery between January 1, 2009 and January 1, 2022. The LA datasets in TAPVC patients were analyzed before surgery (n = 28) and follow-ups at 1-2 (n = 24) and 5-7 years of age (n = 13) and compared with those of age-matched healthy controls (January 2009-2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) strains. LA pressure was evaluated by periodic follow-up catheterization after repair. RESULTS: Compared to the controls, the TAPVC patients had significantly smaller LA maximum volume preoperatively, and with age, the LA maximal volumes reached normal levels, while the LA minimal volumes were larger. All 2DSTE-determined LA strains showed significant reductions at all time points in the TAPVC group compared to those in the control (median εR, εCD, and εCT; before surgery: 17.0% vs. 26.0%, 12.9% vs. 15.9%, and 6.3% vs. 10.4%; follow-up at 1-2 years: 30.0% vs. 45.7%, 23.2% vs. 29.6%, and 6.1% vs. 16.3%; follow-up at 5-7 years: 31.2% vs. 43.1%, 25.0% vs. 31.2%, and 5.2% vs. 10.8%, respectively; p < 0.05). Only εCT did not represented a significant change over time even though after correction of blood flow (median εCT: 6.0% → 5.9%). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. CONCLUSION: This study showed that nevertheless maximum volume of LA was recovered within the normal range, reduced LA strains, especially contractile function lasted from birth even after repair in Patients with TAPVC.

Survey results: status report on problems caused by sexual mismatch between sonographer and patient during echocardiography-a 2020 report of the Japanese Society of Echocardiography.

OBJECTIVES: Ultrasonography is an essential examination performed in various clinical fields. The number of clinical sonographers has been increasing. However, the working environments and conditions at each facility are different, leading to diverse problems. Among them, the emerging issue is the sexual disagreement between the sonographer and patient at the time of echocardiography. Since the patient must expose their breast during echocardiography, female patients may refuse to undergo the examination when conducted by a male sonographer. This study aimed to conduct a questionnaire survey to understand the measures for sonographer-patient gender mismatch at different facilities. METHODS: A questionnaire on the implementation of echocardiography by male sonographers for female patients was answered by representatives and specialist technicians of the Japanese Society of Echocardiography. RESULTS: Questionnaire responses were obtained from 50 facilities (59 participants). A total of 70% of the facilities restricted male sonographers from conducting echocardiography examinations for female patients. Among them, 81% of the facilities serviced female patients aged 60 years or younger. CONCLUSIONS: It has become clear that the gender selection of echocardiographic examiners varies from facility to facility, and providing a sufficient explanation before echocardiographic examination is necessary to avoid causing uncomfortable situations for female patients.

Automated analysis method to assess pulmonary blood flow distribution using conventional X-ray angiography.

Quantitative assessment of the right-to-left ratio of pulmonary blood flow distribution is important for determining the clinical indications for treating pulmonary arterial branch stenosis. A novel theory was recently proposed that can be used to quantitatively assess the right-to-left ratio on conventional X-ray angiography images. In the proposal, further developments were indicated, especially automated calculation. In this study, a new automated algorithm was developed. In the X-ray image, regions of interest were set in right and left lung, and time-signal intensity curves were measured. The new automated algorithm is applied to determine the optimal time window for the analysis of the time-signal intensity curve and to calculate the slope of the curve in the optimized time window. The right-to-left ratios in seven consecutive patients calculated by the new automated algorithm were compared to those calculated by lung perfusion scintigraphy. The ratios were in good agreement with linear regression with a slope of 1.27 and a Pearson correlation coefficient of 0.95. The processing time was less than 10 s, which is one-eighth of the manual processing time. The new automated algorithm is accurate, stable, and fast enough for clinical use in the real world.

Control of Heart Rate in Infant and Child Tachyarrhythmia With Reduced Cardiac Function Using Landiolol (HEARTFUL)　- Results of a Prospective, Multicenter, Uncontrolled Clinical Study.

BACKGROUND: The prospective Control of HEART rate in inFant and child tachyarrhythmia with reduced cardiac function Using Landiolol (HEARTFUL) study investigated the effectiveness and safety of landiolol, a short-acting ß1 selective blocker, in children.Methods and Results: Twenty-five inpatients aged ≥3 months to <15 years who developed supraventricular tachyarrhythmias (atrial fibrillation, atrial flutter, supraventricular tachycardia, and inappropriate sinus tachycardia) were treated with landiolol. The primary endpoint, the percent of patients with a reduction in heart rate ≥20% from the initial rate of tachycardia, or termination of tachycardia at 2 h after starting landiolol, was achieved in 12/25 patients (48.0%; 95% CI 28.4-67.6), which exceeded the predetermined threshold (38.0%). At 2 h after starting landiolol administration, heart rate had decreased by ≥20% in 45.8% (11/24) and recovery to sinus rhythm was achieved in 40.0% (6/15) of the patients. Adverse reactions (ARs) occurred in 24.0% (6/25) of patients, and the study was discontinued in 4.0% (1/25) of the patients; however, none of these ARs were considered serious. The most common AR was hypotension (20.0% [5/25] of patients). CONCLUSIONS: The HEARTFUL study has demonstrated the efficacy of landiolol, by reducing heart rate or terminating tachycardia, in pediatric patients with supraventricular tachyarrhythmias. Although serious ARs and concerns were not identified in this study, physicians should be always cautious of circulatory collapse due to hypotension.

A geometrical pitfall of Area-Length method; -Is left ventricle volume evaluation of repaired Tetralogy of Fallot by angiocardiography accurate?

Biplane Area-Length (AL) method by left ventriculography (LVG) has been widely adopted as a standard method to estimate left ventricular volume. However, we have experienced difficulties in adopting the value by AL method for the children with Tetralogy of Fallot (TOF) due to the discrepancy among volumetric modalities. This study validated some limitations of AL method, considering the basic principles of its formulation. A single center retrospective cohort study was conducted for 1 year. The confirmed 22 cases with repaired TOF at our hospital were enrolled. The clinical characteristics, some cardiac MRI analyses, and all the cardiac catheterization studies were collected. Angiographic data were compared with historic cohorts of Kawasaki disease without any coronary artery lesions by using AL method. Cardiac MRI analyses of ten TOF patients were additionally available. LVG studies showed that the length of the long axis on anteroposterior view (AP) was not equal to that on lateral view (LT) due to anatomically apical elevation in TOF, followed by a significant difference found in the sagittal lengths of the LV long axis between AP and LT (P = 0.003). Because the difference critically affected the formula depending on biplane AL method, the calculated LVEDV of TOF group appeared overestimated, compared with the control group (TOF vs control group: 119.5% ± 6.3% vs 96.4 ± 3.5% of Normal, P = 0.006). Available cardiac MRI analyses of some patients in TOF group revealed 55% increase of LVEDV by AL method (angiocardiography 116 ± 7.0 vs CMR 75 ± 3.7 ml/m2, P = 0.0025). A pitfall exists when applying biplane AL method to measure LV volume especially for TOF patients, because the long axis on AP view is not always identical to that on LT view.

Predictors of long-term mortality among perioperative survivors of Fontan operation.

AIMS: The criteria for 'good' Fontan haemodynamics have been poorly defined in relation to long-term outcomes. The aim of this study was to identify the risk factors for mortality among haemodynamic parameters obtained early after the Fontan operation. METHODS AND RESULTS: Clinical data of all perioperative survivors of the Fontan operation performed before 2011, from nine institutions, were collected through a retrospective chart review. In total, 1260 patients were included. The median age at the time of Fontan operation was 3.6 years. Post-operative cardiac catheterization was conducted in 1117 patients at a median period of 1.0 years after the operation. During the median follow-up period of 10.2 years, 107 patients died. The mortality rates at 10, 20, and 25 years after the operation were 5%, 12%, and 22%, respectively. On multivariable analysis, older age at the time of the operation {≥15 years, hazard ratio (HR) [95% confidence interval (CI)]: 3.2 (1.7-5.9)} and haemodynamic parameters obtained at post-operative catheterization, such as low ejection fraction [<30%, HR (95% CI): 7.5 (3.2-18)], low systemic oxygen saturation [<80%, HR (95% CI): 3.8 (1.6-9.1)], high central venous pressure [≥16 mmHg, HR (95% CI): 2.3 (1.3-3.9)], and low mean systemic arterial pressure [<60 mmHg, HR (95% CI): 3.0 (1.4-6.2)] were identified as independent predictors of mortality. The predictive model based on these parameters had a c-index of 0.75 at 10 years. CONCLUSIONS: Haemodynamic parameters obtained at a median period of 1.0 years, post-operatively, can accurately identify patients with a high mortality risk, who may need intensive management to improve long-term outcomes.

Identification of metabolomic profile related to adult Fontan pathophysiology.

BACKGROUND: Metabolic disorders are important pathophysiologies that can cause multiple organ dysfunction and worsen prognosis in Fontan patients. This study aimed to comprehensively evaluate the metabolomic profile of adult Fontan patients and characterize its pathophysiology in relation to 2 control groups. METHODS AND RESULTS: We performed metabolomic analysis of 31 plasma samples using capillary electrophoresis time-of-flight mass spectrometry. This observational cross-sectional study compared plasma metabolites of 14 heterogeneous adult Fontan patients with those of control groups, including 9 patients with congenital heart disease after biventricular repair and 8 normal healthy controls. Fontan patients exhibited significant differences in intermediate metabolite concentrations related to glycolysis, the tricarboxylic acid (TCA) cycle, and the urea cycle. The plasma concentrations of lactic acid, 2-oxoglutarate, isocitric acid, malic acid, cis-aconitic acid, arginine, citrulline, and the ratio of ornithine/citrulline showed significantly differences among the groups. Multiple logistic regression analysis with a stepwise selection-elimination method identified 2-oxoglutaric acid (odds ratio [OR] 1.98, 95% confidence interval [CI] 1.05-3.76) and cis-aconitic acid (OR 2.69, 95% CI 1.04-6.99) as independently associated with Fontan patients. After adjustment for the covariates of age and gender, 2-oxoglutaric acid (OR 1.97, 95% CI 0.98-3.93) and cis-aconitic acid (OR 3.88, 95% CI 0.99-15.2) showed remarkable relationships with Fontan patients. CONCLUSIONS: The present findings suggest that abnormalities in the TCA cycle and amino acid metabolism are distinguishing features in the pathophysiology of Fontan patients. Future metabolomic studies will assist in developing biomarkers for the early prediction of "silent" Fontan pathophysiologies.

A novel diagnostic approach for assessing pulmonary blood flow distribution using conventional X-ray angiography.

OBJECTIVE: Quantitative assessment of pulmonary blood flow distribution is important when determining the clinical indications for treating pulmonary arterial branch stenosis. Lung perfusion scintigraphy is currently the gold standard for quantitative blood flow measurement. However, it is expensive, cannot provide a real-time assessment, requires additional sedation, and exposes the patient to ionizing radiation. The aim of this study was to investigate the feasibility of a novel technology for measuring pulmonary blood flow distribution in each lung by conventional X-ray pulmonary angiography and to compare its performance to that of lung perfusion scintigraphy. METHODS: Contrast-enhanced X-ray pulmonary angiography images were acquired at a frame rate of 30 frames per second. The baseline mask image, obtained before contrast agent injection, was subtracted from subsequent, consecutive images. The time-signal intensity curves of two regions of interest, established at each lung field, were obtained on a frame-to-frame basis. The net increase in signal intensity within each region at the torrent period during the second cardiac cycle before contrast agent enhancement over the total lung field was measured, and the right-to-left ratio of the signal intensity was calculated. The right-to-left ratio obtained with this approach was compared to that obtained with scintigraphy. Agreement of the right-to-left ratio between X-ray angiography and lung scintigraphy measurements was assessed using linear fitting with the Pearson correlation coefficient. RESULT: The calculation of the right-to-left ratio of pulmonary blood flow by our kinetic model was feasible for seven children as a pilot study. The right-to-left ratio of pulmonary blood flow distribution calculated from pulmonary angiography was in good agreement with that of lung perfusion scintigraphy, with a Pearson correlation coefficient of 0.91 and a slope of linear fit of 1.2 (p<0.005). CONCLUSION: The novel diagnostic technology using X-ray pulmonary angiography from our kinetic model can feasibly quantify the right-to-left ratio of pulmonary blood flow distribution. This technology may serve as a substitute for lung perfusion scintigraphy, which is quite beneficial for small children susceptible to radiation exposure.

A case report: Twin sisters with restrictive cardiomyopathy associated with rare mutations in the cardiac troponin I gene.

Restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children, and its prognosis until now, has been poor. Recently some sarcomeric mutations have been reported as disease-causing genes of RCM. However, the genotype-phenotype correlation is not fully understood. Additionally, prognostic factors including sudden death in patients with RCM have not been elucidated. We report our experience in treating twin sisters with RCM or hypertrophic cardiomyopathy with RCM phenotype, both carriers of the same mutation in TNNI3, which encodes one of the major sarcomeric proteins in myofibrils. They were both diagnosed with RCM by cardiac catheterization at the age of 11 years. Despite appropriate follow-up and medical treatment, one died suddenly at the age of 11 years and the other also died at the age of 15 years due to heart failure while awaiting heart transplantation. In addition to our cases, other reports of younger fatal cases with RCM carrying TNNI3 mutations may suggest it as one of the prognostic factors. Genetic diagnosis is important in the clinical diagnosis, management, and treatment of cardiomyopathy. .

Ventricular myocardial response to exercise in patients with Fontan circulation.

BACKGROUND: Exercise stress echocardiography has been used to assess myocardial reserve in various heart diseases. This study examined the ventricular myocardial response to exercise in Fontan patients using exercise stress echocardiography. METHODS: Twenty-five Fontan patients and 19 control subjects underwent semi-supine bicycle exercise stress echocardiography in this prospective, single-center, cross-sectional study. Pulsed-wave Doppler tissue imaging peak systolic (s') and diastolic (e') velocities, longitudinal strain and systolic strain rate, and early diastolic strain rate data at rest and at peak exercise were obtained for the systemic ventricle. The myocardial reserve of functional parameters was calculated as the difference between peak exercise and rest. RESULTS: Inter- and intra-observer reliability were both high for exercise stress echocardiography measurements. Compared with controls, Fontan patients had significantly lower s', e', longitudinal systolic strain and strain rate, and early diastolic longitudinal strain rate at rest and at peak exercise as well as reduced myocardial reserve. CONCLUSIONS: Fontan patients have markedly reduced myocardial reserve during exercise. The use of exercise stress echocardiography assessment may improve the clinical management of Fontan patients.

Establishment of a Healthcare System for Patients With Adult Congenital Heart Disease in Collaboration With Children's Hospital　- The Nagano Model.

BACKGROUND: Despite the best efforts of pediatricians, healthcare for adult patients with congenital heart disease (ACHD) has proven challenging because of the increased numbers. This study presents the process of establishing an ACHD care system as a collaborative effort between Shinshu University Hospital and Nagano Children's Hospital. Methods and Results: Establishing an outpatient clinic for transition, a cooperation agreement for in-patient care between the 2 hospitals, and quality management of diagnostic imaging and educational meetings for adult cardiologists were the 3 major challenges. Of the 99 patients who visited the transition clinic in the children's hospital between May 2014 and December 2016, 3 returned to the pediatrician's clinic. Between June 2013 and December 2017, 273 patients visited the ACHD center in Shinshu University Hospital. Until December 2017, mortality and fatal arrhythmia were noted in 3 and 2 cases, respectively. Catheter ablation for arrhythmia was performed in 12 cases, and 4 cases of pregnancy with moderate/severe ACHD or estimated as high risk were managed with healthy livebirths. Surgical interventions for moderate/severe ACHD were performed in collaboration with the children's hospital or Sakakibara Heart Institute. CONCLUSIONS: Patients were successfully transferred to adult cardiology departments. Surgical and nonsurgical interventions for ACHD were provided. Collaboration between adult and pediatric cardiologists assists in the establishment of healthcare systems for ACHD.

Implication of Aortic Root Dilation and Stiffening in Patients with Tetralogy of Fallot.

Progressive aortic root dilation and stiffening have been reported in patients with tetralogy of Fallot (TOF), even after repair. However, the implications of such aortic dilation and stiffening have not been fully assessed. A stiff aorta has a negative effect on the coronary circulation. 15 patients with TOF after repair were enrolled in this study. Using a left ventriculogram and pressure waveform recorded by a pressure sensor-mounted catheter, we analyzed the diameter and distensibility of the ascending and descending aorta. We also calculated the subendocardial viability ratio (SEVR), which measures the cardiac blood supply-workload balance. These values were compared with those in one-to-one age-matched controls. Moreover, the correlation between the diameter ratio (aortic diameter of the patient/aortic diameter of the matched control subject) and the distensibility ratio (distensibility of the patient/distensibility of the matched control subject) was analyzed. The mean age of patients was 3.9 ± 1.6 years. In patients with TOF, the indexed diameter was larger (0.20 ± 0.02 vs. 0.15 ± 0.02 mm/cm, p < 0.0001) and the distensibility was lower (5.1 ± 2.2 vs. 7.7 ± 2.0 cm2 dynes-1 10-6, p = 0.0009) in the ascending aorta than in control subjects. There was no difference in the SEVR between patients and control subjects. The distensibility ratio was significantly negatively correlated with the indexed diameter ratio in the ascending aorta (r2 = 0.35, p = 0.019). Regardless of the aortic stiffening, the cardiac blood supply-workload balance was maintained. We speculate that the aortic root dilation might be a mechanism to functionally complement aortic stiffness in patients with TOF.

Significance of right atrial tension for the development of complications in patients after atriopulmonary connection Fontan procedure: potential indicator for Fontan conversion.

Elevated right atrial (RA) pressure and progressive RA dilation are thought to play pivotal roles in the development of late complications after atriopulmonary connection (APC) Fontan surgery. However, no clear cut-off value for RA pressure or RA volume has been determined for stratifying the risk of developing Fontan complications. We hypothesized that RA tension, which incorporates information about both RA pressure and volume, might help predict the risk of developing complications. We retrospectively studied 51 consecutive APC Fontan patients (median postoperative period 14 years). RA tension was computed from the RA pressure and RA radius, which was calculated from RA volume measured by RA angiography. The correlation between the cardiac catheterization hemodynamic data and the complications of APC Fontan was investigated. Of the 51 patients, 28 had complications, including liver fibrosis (n = 28), arrhythmia (n = 8), protein-losing enteropathy (n = 1), and RA thrombosis (n = 1). Among the hemodynamic data, RA volume and RA tension, but not RA pressure, were significantly higher in patients with complications than in those without (P = 0.004 and P = 0.001, respectively). The cut-off level for RA tension to predict Fontan complications was 26,131 dyne/cm by receiver operating characteristic curve (area under the curve 0.79, sensitivity 71.4%, and specificity 73.9%). The present study demonstrated the significance of RA tension rather than high venous pressure for the development of Fontan complications. Amid the uncertainty about clinical outcomes, the present results, subject to further validation, may contribute to the indications for Fontan conversion.

Frequency of malformed infants in a tertiary center in Hokkaido, Japan over a period of 10 years.

AIM: This retrospective study was performed to determine the frequency of malformed infants born at a tertiary center in Hokkaido, Japan. The accuracy of prenatal diagnosis rates was also investigated. METHODS: An observational study was performed using data of 1509 and 1743 newborn infants at a single center during two study periods, 2005-2009 (first) and 2010-2014 (second), respectively. Cases including minor anomalies (accessory auricle, nevus and fistula auris congenita) were not included. RESULTS: In total, 274 and 569 malformations were identified in 191 and 337 newborn infants in the first and second study periods, respectively. The number of malformed infants increased significantly over time (13% [191/1509] vs 19% [337/1743], respectively; P < 0.001), mainly as a result of an increase in cases of congenital heart disease (CHD), from 59 to 141 (31% [59/191] vs 42% [141/337] of all malformed infants in the first and second periods, respectively). The overall accurate prenatal diagnosis rate improved over time from 47% (128/274) to 58% (329/569) because of significant improvements in accurate prenatal diagnosis of CHD subtypes (23% [16/70] vs 65% [151/232] in the first and second periods, respectively, P < 0.0001). CONCLUSIONS: The frequency of malformed newborns was higher in the tertiary center than in the general population. The increased number of cases with prenatal suspicion and diagnosis of CHD contributed to the increased frequency of malformed infants during the study period.

Resolution of tachyarrhythmia-related fetal hydrops after corticosteroid administration for fetal lung maturation.

A case of hydrops fetalis (HF) that resolved after corticosteroid therapy despite persisting fetal tachycardia of 190 bpm is reported. The fetus with confirmed normal karyotype had HF in the presence of atrial flutter and sustained ventricular rate of 200-210 bpm at gestational week (GW) 31(-6/7). Aetiologies of HF other than fetal tachyarrhythmia were unlikely in this infant. The patient's family declined pharmacological treatment with maternal digoxin and desired to continue pregnancy with only intensive monitoring of fetal condition. Maternal transplacental ß-methasone was given to facilitate fetal lung maturation. The fetal tachyarrhythmia ameliorated to 190 bpm at GW 32(-5/7) and HF gradually disappeared by birth. The otherwise healthy female neonate weighing 3050 g born at GW 37(-0/7) was successfully treated with cardioversion performed 60 min after birth. This case suggested that corticosteroid therapy contributed to the resolution of HF associated with tachyarrhythmia.

Decreased baseline variability on fetal heart rate pattern in a fetus with heterotaxy syndrome.

In a fetus with suspected heterotaxy syndrome, a decreased/absent baseline variability of fetal heart rate pattern developed at gestational week 36(+5) and continued for 5 days until birth at gestational week 37(+2), while repeat biophysical profile scorings with ultrasound were consistently unremarkable. This neonate weighing 2404 g with Apgar scores of 7 (1-min) and 8 (5-min) and umbilical arterial cord blood pH of 7.28 with base deficit of 3.9 mmol/L, showed a heart rate of 120 b.p.m. for 3 h after birth, but subsequently developed sinus bradycardia (84 b.p.m.) unresponsive to crying. Isoproterenol initiated 9 h after birth was effective in the increase of heart rate to 120 b.p.m. in this neonate. Brain magnetic resonance imaging at 16 days of age was unremarkable. The decreased/absent baseline variability of fetal heart rate pattern was speculated to have been caused by sinus node dysfunction, and not by reduced fetal oxygenation in this case.

The cardiac blood supply-workload balance in children.

It is well known that the reflected pressure wave in small children returns earlier than that in adolescent. The reason of early return of the reflected pressure wave in infancy is their height. The short distance between heart and reflection point makes the reflected pressure wave returning to the heart earlier. In adult, the early return (during systole) of the reflected pressure wave means disadvantage to cardiac blood supply-workload balance. The purpose of this study was to clarify whether the early return of the reflected pressure wave in small children impairs the cardiac blood supply-workload balance. This study enrolled 37 small left-to-right shunt patients with normal aortic circulation below 15 years of age. The aortic pressure waveform was recorded using a pressure sensor mounted catheter, and augmentation index and subendocardial viability ratio were calculated. The age of patients was 6.1 ± 3.2 years. The augmentation index was 8.7 ± 14.3 % and the index had a negative correlation with patients' age (r = -0.6243, p < 0.0001). The subendocardial viability ratio, which means the cardiac blood supply-workload balance, was 0.92 ± 0.14 and the index had a positive relationship with patients' age (r = 0.6435, p < 0.0001). The cardiac blood supply-workload balance gradually improves from infancy to young adulthood. One of the causes of the unfavorable cardiac blood supply-workload balance in infancy would be the accelerated aortic pressure wave reflection due to their short height.