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Sleep-disordered breathing is one of the complications commonly seen in patients with adult congenital heart disease (ACHD) due to multiple causes including complex underlying cardiac defects, cardiomegaly, previous thoracotomies, obesity, scoliosis, and paralysis of the diaphragm. It is often hard to determine its main cause and predict the efficacy of each treatment in its management. We herein report a 30-year-old woman after biventricular repair of pulmonary atresia with intact ventricular septum diagnosed as sleep-related hypoventilation disorder. Simultaneous treatment targeting obesity, paralysis of the diaphragm, and cardiomegaly followed by respiratory muscle reinforcement through non-invasive ventilation resolved her sleep-related hypoventilation disorder. Such management for each factor responsible for the hypoventilation is expected to provide synergetic therapeutic efficacy and increase daily activity in a patient with ACHD.

Pulmonary agenesis is a complete absence of the pulmonary parenchyma, airways, and vasculature unilaterally or bilaterally. Although bilateral cases are lethal, the outcome of unilateral cases remains not well described. We performed a comprehensive literature review to assess the clinical features of pulmonary agenesis.Four database sources were searched on October 10, 2021 and two cases were included from our institution. Studies related to the clinical impact of comorbidity and intervention on the survival outcome in pulmonary agenesis were included for full-text review.We identified 259 patients-with right-sided (59%), left-sided (34%), and bilateral agenesis (7%)-among 195 articles and our two cases. Additional anomalies included cardiovascular (40%), skeletal (30%), gastrointestinal (20%), tracheal (20%: all stenoses), and genitourinary (14%) anomalies. Fifty-seven (24%) individuals in unilateral pulmonary agenesis had isolated disease. Outcomes related to survival were not uniformly reported, but the 2-year overall survival rate of unilateral agenesis was 62% and no subsequent death was reported until 13 years of age. The right-sided agenesis was more frequently associated with tracheal stenosis (27% vs. 11%, p = 0.003) than the left-sided disease. A multivariable analysis indicated that tracheal stenosis (hazard ratio [HR]: 2.2, 95% confidence interval [CI]: 1.3-4.1, p = 0.003) and gastrointestinal anomalies (HR: 2.0, 95% CI: 1.1-3.3, p = 0.010) were prognostic factors for mortality.The poor prognostic factors were tracheal stenosis, right agenesis, and gastrointestinal anomalies. Treatment for these comorbidities is a key point for improving the survival of unilateral pulmonary agenesis.

Purpose To investigate the usefulness of commercially available fat-containing agents in magnetic resonance imaging (MRI) based on bone mineral measurement.Methods The proximal femurs obtained from 14 volunteers were analyzed by 0.3T MRI with a fat-containing nutrient solution (based on soybean oil, 10% and 20%), 100% soybean oil and saline as reference substances. Fat content was estimated based on the relationship between the intensities of the signals of the reference substances. Since this was an approximate value, it was set as the estimated fat fraction based on signal intensity (SIeFF, %). The SIeFF values of the femoral bone marrow, including the femoral head, neck, shaft, and trochanter area, were measured. Reference substances were set as close as possible to the outside of both proximal femurs. MRI data were compared in terms of bone mineral content (BMC) and bone mineral density (BMD) by dual-energy X-ray absorptiometry (DXA) in the proximal femur. MRI and DXA data were obtained on the same day.Results According to Pearson’s correlation coefficient, the SIeFF and total BMC and BMD data revealed strong and moderate inverse correlations in the femoral head (r r = − 0.66 to − 0.45), respectively.Conclusion Commercially available fat-containing agents may be useful in estimating the bone marrow fat content for bone mineral measurement by MRI. SIeFF and BMC and BMD showed a strong inverse correlation in the femoral head. Nevertheless, a more thorough study is warranted before this method can be used as an alternative to DXA.

Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed. Methods and Results The cohort of the Z‐Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving 44 institutions in Japan including 1006 patients with KD, was examined. CAAs were classified by the z score of their internal diameter in the acute phase: small ( z z z ≥10). The lower limit of small CAA was based on the Japanese Ministry of Health, Labour and Welfare criteria. In the right coronary artery, the CAA regression rates 10 years after diagnosis were 95.5% for small, 83.2% for medium, and 36.3% for large. In the proximal left anterior descending artery, the regression rates 10 years after diagnosis were 95.3% for small, 80.1% for medium, and 28.8% for large. Cox regression analysis showed that diagnosis under the age of 1 year and onset of KD in 2010 to 2012 for the right coronary artery and the left anterior descending artery, and female for the right coronary artery were significantly associated with a high regression rate, whereas large CAAs for the right coronary artery and the left anterior descending artery were significantly associated with a low regression rate. Conclusions The current study, the largest Japanese study of its kind, found that small aneurysm, recent onset, and diagnosis under the age of 1 year predict regression, and that even giant aneurysms could regress. These data may contribute to long‐term management of coronary aneurysms. Registration URL: https://www.umin.ac.jp/ctr/ ; Unique identifier: UMIN000010606.

Background Chemotherapy in childhood leukemia potentially induces brain lesions and neurological sequelae. Paroxysmal sympathetic hyperactivity (PSH) is known as a treatment-associated complication; however, the full clinical spectra of PSH remain to be elusive. Case report A 5-year-old girl was diagnosed of acute myeloid leukemia (AML) M5. After the intensification therapy, she developed recurrent symptoms of episodic tachycardia, hypertension and perspiration lasting for several hours per day. The low-frequency-high-frequency ratio on Holter electrocardiography was rapidly increased from 0.84 to 2.24 at the onset of the paroxysmal event, whereas the video-monitoring electroencephalography (EEG) never identified ictal patterns of epileptiform discharges during the episodes. Thus, the diagnosis of PSH was given at 7 years of age. Myoclonic and generalized tonic-clonic seizures frequently appeared from 10 years of age, which poorly responded to anticonvulsants. EEG showed diffuse slow-wave bursts with multifocal spikes. Serial head magnetic resonance imaging (MRI) revealed diffuse cerebral and hippocampal atrophy, but not inflammatory lesions in the limbic system. Conclusion We first demonstrate a pediatric case with PSH who developed drug-resistant epilepsy 3 years after the onset of PSH. Our data suggest the pathophysiological link of persistent PSH with chemotherapy-associated brain damage.

Background Infective endocarditis (IE) in patients with adult congenital heart disease (ACHD) remains a diagnostic challenge due to difficulties in detecting endocardial lesions by echocardiography. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has shown good diagnostic performance in prosthetic valve IE. This study aimed to assess its additional diagnostic value in ACHD-associated IE and to characterize its advantages.Methods and Results:Overall, 22 patients with ACHD and clinical suspicion of IE were retrospectively studied. 18F-FDG PET/CT was performed in addition to conventional assessment based on the modified Duke criteria. The final IE diagnosis was determined by an expert team during a 3-month clinical course, resulting in 18 patients diagnosed with IE. Seven patients (39%) were diagnosed with definite IE only by initial echocardiography. An 18F-FDG PET/CT assessment revealed endocardial involvement in the other 9 patients, resulting in the diagnosis of definite IE in 16 in total (88%). Right-sided endocardial lesions were more common (n=12, 67%) but rarely identified by echocardiography, whereas 18F-FDG PET/CT revealed right-sided lesions in 9 patients. A negative 18F-FDG PET/CT (n=7, 39%) assessment was associated with a native valve IE (71% vs. 0%). In 4 patients who were identified with not-IE, neither echocardiography nor 18F-FDG PET/CT detected any suspicious cardiac involvement. Conclusions In the diagnosis of ACHD-associated IE, characterized by right-sided IE, 18F-FDG PET/CT assessment should be useful.

Non-reentrant fascicular tachycardia (NRFT) developed in a 6-year-old Japanese boy. Because of the drug-resistant recurrences, he received catheter mapping and ablation at age 10 years. An electrocardiogram exhibited a superior left axis deviation, a right bundle branch block-type configuration, and relatively narrow QRS with sharp R wave. It suggested verapamil-sensitive ventricular tachycardia (VT), but showed no sensitivity to verapamil or reentrant characteristics in the electrophysiological study. Detailed VT mapping determined the earliest presystolic Purkinje potential on the left posterior fascicle at the mid-ventricular septum. Radiofrequency current applications to the lesion led to his NRFT-free life without restriction for 16 months.

Background Pregnant women with repaired tetralogy of Fallot (rTOF) are at increased risk of adverse outcomes. Although pre-pregnancy pulmonary valve replacement (PVR) may be considered in some women to attenuate risk, published data to support this practice are lacking. Our objective was to explore the impact of pre-pregnancy PVR on pregnancy outcomes in rTOF. Methods Women with rTOF and cardiovascular magnetic resonance imaging (CMR) before and after pregnancy were included if CMR studies were completed within 3 years of pregnancy. Subjects were compared according to presence (+) or absence (−) of PVR at pre-pregnancy CMR. Pregnancy outcomes (cardiovascular, obstetric, and fetal/neonatal) were documented. Results Of the 29 study women identified, 7 were PVR+ and 22 were PVR-. Post-pregnancy, the PVR- group demonstrated interval increase in indexed right ventricular end-diastolic volumes (RVEDVi) (157 ± 28 versus 166 ± 33 ml/m2, p = 0.003) and end-systolic volumes (RVESVi) (82 ± 17 versus 89 ± 20 ml/m2, p = 0.003) as compared with pre-pregnancy, but no significant change in RV ejection fraction, RV mass, or left ventricular measurements. In the PVR+ group, there were no interval changes in RV measurements pre-versus post pregnancy. Interval rate of change in RVESVi of PVR- exceeded PVR+ women (+3.7 ± 5.0 versus −2.2 ± 5.0 ml/m2/year, p = 0.03). Pregnancy outcomes did not differ in PVR+ versus PVR- women. Conclusions Pregnancy outcomes did not differ according to PVR status in our cohort. While RV volumes remained unchanged in PVR+ women, interval RV dilation was observed in PVR- women. Additional study of a larger population with longer follow-up may further inform clinical practice regarding pre-pregnancy PVR.

We performed a second puncture of the extracardiac conduit in an 11-year-old Fontan patient to assess the patency of the stent previously deployed in the left pulmonary vein. For the first puncture, a mechanical Brockenbrough needle was selected to puncture the Gore-Tex conduit, an electrical insulator. For the second puncture, the location of that previous hole was detected as an indentation covered with atrial tissue, which is an electrical conductor. The second puncture was performed safely using a radiofrequency transseptal needle.

BackgroundPatients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients.MethodsPrimary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated.ResultsA total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age ConclusionsShort-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

Although cardiovascular magnetic resonance (CMR) imaging measurements are known to differ between sexes and according to age in the normal heart, little is known about measurement variability in patients with congenital heart disease (CHD). Yet, aggregate CMR data, without adjustment for sex or age

ObjectivesAlthough a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects.MethodsAdults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode.ResultsTen Fontan subjects (50% male, 24.5 years (IQR 20.8–34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10–0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (−0.11–0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20–0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21–0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2–0.84) vs −0.27 L/min/m2 (−0.55–0.13), p=0.001; and 0.47 L/min/m2 (0.21–0.71) vs 0.07 L/min/m2 (−0.47–0.33), p=0.034, respectively).ConclusionExternal ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

Pulmonary veno-occlusive disease (PVOD) and idiopathic/heritable pulmonary arterial hypertension (I/HPAH) cause progressive PH on the distinct genetic impact. A 29-month-old boy presented with a loss of consciousness. He had severe PH refractory to pulmonary vasodilators. Hypoxemia and ground-glass opacity on the chest computed tomography were present, and significant pulmonary edema developed after the introduction of continuous intravenous prostaglandin I

The number of women with congenital heart disease (CHD) reaching reproductive age has been increasing. Many women with CHDs are desirous of pregnancy, but they face issues regarding preconception, antepartum, and postpartum management. On the other hand, the fetal diagnosis of CHD has improved with advances in the technique and equipment for fetal echocardiography. Recently, experiences with fetal intervention have been reported in patients with severe CHD, such as critical aortic stenosis. Nevertheless, some types of CHD are challenge to diagnose prenatally, resulting in adverse outcomes. Medical care is part of the transitional care for women and fetuses with CHD during the perinatal period. Pre-conceptional and prenatal counseling play an important role in transitional care. Sex and reproductive education need to be performed as early as possible. We herein review the current status, important issues to be resolved, and the future of maternal and fetal CHD to relevant caregivers.

With advances in medical care, the majority of infants and children with chronic diseases are now able to reach adulthood. However, many of them still need special health care because of their original diseases, sequelae, and complications. The transition from the child health care system to the adult health care system is a crucial step for these patients. The goal of transitional care is to maximize the lifelong function and potential of these patients by uninterruptedly providing appropriate health-care services. To achieve this goal, we should (i) coordinate the transfer to adequate medical institutions and departments for adults, (ii) educate patients to improve self-management, and (iii) support the transition to social and welfare services for adults. Transitional care in pediatric cardiology has been a step ahead of such care in other diseases because of the relatively high incidence and the long history of adult congenital heart disease. Education of the patients to establish autonomy reduces dropping out and unexpected hospitalizations and it is the most important part of transitional care. To achieve this goal, we should provide explanations to pediatric patients according to their age and level of understanding from their first visit, rather than waiting until they reach a certain age. Tools for education and readiness checks are also being developed. To achieve a situation in which pediatric patients with chronic disease can take care of their own health and fully utilize their abilities at the growing step, transitional care plays a crucial role not only in pediatric cardiology but also in other subspecialties.

Introduction: Pulmonary agenesis is a complete absence of the pulmonary parenchyma, bronchi beyond the bifurcation, and pulmonary vasculature unilaterally or bilaterally. Because of the rare occurrence, its pathophysiology and outcome remain elusive. We evaluate the clinical features and risk factors for mortality due to pulmonary agenesis. Methods: Two patients we experienced are presented as index cases. All reported cases of pulmonary agenesis were collected from online and publication databases between 1955 and 2020. We assessed the impact of comorbidity and intervention on the survival outcome. Results: We identified 230 patients—138 (60%) with right-sided, and 14 (6%) with bilateral agenesis—among 164 articles and our cases. There were 93 (40%) cardiovascular, 70 (30%) skeletal, and 48 (21%) gastrointestinal anomalies; 47 (20%) tracheal stenoses; and 33 (14%) genitourinary anomalies. Fifty-two (23%) patients had isolated pulmonary agenesis. The 2-year overall survival (OS) rate was 66% and there was no subsequent death until 13 years of age. The right agenesis was significantly associated with a lower 2-year OS rate (58% vs. 78%, p=0.019) or more frequent tracheal stenosis (28% vs. 12%, p=0.006) than left-sided disease. A multivariable analysis indicated that tracheal stenosis (hazard ratio [HR] 2.4, 95% confidence interval [CI] 1.5-4.1, p=0.004) and gastrointestinal anomaly (HR 1.9, 95% CI 1.1-3.3, p=0.018) were prognostic factors for mortality. Conclusions: The poor prognostic factors were tracheal stenosis, right agenesis and gastrointestinal anomaly. Tracheal and gastrointestinal controls are targeted at birth and in infancy for the improved survival of unilateral pulmonary agenesis.

Aims Coronary arteritis is a life-threatening complication that may arise in the acute stage of Kawasaki disease (KD), the leading cause of systemic vasculitis in childhood. Various microorganisms and molecular pathogens have been reported to cause KD. However, little is known about the key molecules that contribute to the development of coronary arteritis in KD. Methods and results To identify causative molecules for coronary arteritis in KD, we prospectively recruited 105 patients with KD and 65 disease controls in four different parts of Japan from 2015 to 2018. During this period, we conducted lipidomics analyses of their sera using liquid chromatography–mass spectrometry (LC-MS). The comprehensive LC-MS system detected a total of 27 776 molecules harbouring the unique retention time and m/z values. In the first cohort of 57 KD patients, we found that a fraction of these molecules showed enrichment patterns that varied with the sampling region and season. Among them, 28 molecules were recurrently identified in KD patients but not in controls. The second and third cohorts of 48 more patients with KD revealed that these molecules were correlated with inflammatory markers (leucocyte counts and C-reactive proteins) in the acute stage. Notably, two of these molecules (m/z values: 822.55 and 834.59) were significantly associated with the development of coronary arteritis in the acute stage of KD. Their fragmentation patterns in the tandem MS/MS analysis were consistent with those of oxidized phosphatidylcholines (PCs). Further LC-MS/MS analysis supported the concept that reactive oxygen species caused the non-selective oxidization of PCs in KD patients. In addition, the concentrations of LOX-1 ligand containing apolipoprotein B in the plasma of KD patients were significantly higher than in controls. Conclusion These data suggest that inflammatory signals activated by oxidized phospholipids are involved in the pathogenesis of coronary arteritis in KD. Because the present study recruited only Japanese patients, further examinations are required to determine whether oxidized PCs might be useful biomarkers for the development of coronary arteritis in broad populations of KD.

Fat suppression is a technique used to suppress the signals from adipose tissues, during clinical evaluation of the tissues near the fat-tissue boundary. However, in cases where the scan area has a complicated shape, the effect of fat suppression may demonstrate poor uniformity, resulting in diagnosis-related difficulties. To improve the uniformity of fat suppression, phantom studies are more suitable than volunteer studies. In this study, we evaluated the reliability of the region of interest (ROI) dependency using an unevenness phantom, to develop a method to assess the uniformity of fat suppression while using whole magnetic resonance imaging by masking the surrounding phantom. We modulated different ROI sizes, which were eroded from 100% to approximately 50%, and observed that the normalized absolute average deviation and error increased with decreased ROI. Using our method, more objective, concrete, and accurate data could be obtained by including the whole-body phantom (whole poor uniformity area).

Prostaglandin I2 (PGI2) causes hyperthyroidism, a critical complication in patients with pulmonary arterial hypertension (PAH). However, it remains unknown whether PGI2 may have unfavorable effects on thyroid function in children with congenital portosystemic venous shunt syndrome (CPSVS). We present a boy with CPSVS who developed PAH at seven years of age. During ongoing PGI2 therapy, he experienced thyrotoxicosis at 17 years of age. The literature review showed that the reported 12 patients with PAH (median 11 years of age) developed hyperthyroidism during between one and 11 years of PGI2 treatment. Only one patient survived the acute PAH crisis due to hyperthyroidism. These data provide evidence that prophylactic intervention for hyperthyroidism is indicated for children with CPSVS during PGI2 treatment.

A long duration of Fontan circulation can cause fibrosis, cirrhosis of the liver and liver cancer. There have been some reports of hepatocellular carcinoma occurring after the Fontan procedure (FP), and we encountered a case of intrahepatic cholangiocarcinoma (ICC) after the FP. We herein report the case of 33-year-old man who developed ICC after receiving the FP. At five years of age, the patient underwent the original FP. At 16 years of age, he received total cavopulmonary connection conversion. Sixteen years later, he suffered right heart failure. Thereafter his symptoms showed repeated exacerbation and remission. One year later, he was admitted to the hospital due to the aggravation of congestive heart failure symptoms. He developed contrast nephropathy, and his renal function rapidly weakened. Computed tomography revealed multiple mass lesions in the liver. He ultimately died four months after his admission. An autopsy revealed the hepatic tumor to be ICC. Patients who have undergone the FP need to undergo a checkup with ultrasound on a regular basis. Keywords: Fontan procedure, Intrahepatic cholangiocarcinoma, Liver tumor

Objective To search the predictive factors of infliximab resistance in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients. Study design. Twenty-seven patients with KD who received infliximab after 4–5 g/kg of IVIG therapy from 2013 to 2015 were consecutively recruited in this study. They were divided into two groups: patients who responded to infliximab (infliximab-responsive group, n = 15) and patients who required additional therapy for the disease control (infliximab-resistant group, n = 12). We analyzed the clinical and laboratory parameters just before the infliximab treatment including serum levels of procalcitonin and cytokines with respect to the infliximab response. Results Serum procalcitonin concentration (P = 0.017), neutrophils to lymphocytes ratio (P = 0.013), and % neutrophils (P = 0.004) were higher, and serum sodium concentration (P = 0.017) was lower in infliximab-resistant group than those of infliximab-responsive group, respectively. Multivariate logistic regression analyses indicated that higher procalcitonin concentration (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.00–5.00, P = 0.046) and lower sodium levels (OR 0.64, 95% CI 0.32–1.00, P = 0.047), but not other variables, were associated with infliximab-resistance. Serum procalcitonin concentrations positively correlated with the serum levels of interleukin-6, soluble tumor necrosis factor receptor type 1 and type 2, respectively. Analyses of the receiver operating characteristic (ROC) curve showed that the cut-off value of procalcitonin 2.0 ng/ml had 58.3% of sensitivity and 93.3% of specificity. ROC analysis yielded an area under the curve (AUC) of 0.739 to predict infliximab-resistance. Conclusion Serum procalcitonin might be an effective biomarker to predict infliximab resistance in severe KD patients who are refractory to IVIG treatment.

Objectives: To investigate changes in right ventricular (RV) strain after pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (TOF) by applying a novel postprocessing technique to conventional short-axis cine MRI to measure RV strains before and after PVR.Methods: Twenty-four patients with repaired TOF who underwent PVR and 16 healthy controls were enrolled. Global maximum and minimum principal strains (GPSmax, GPSmin) and global circumferential and longitudinal strains (GCS, GLS) were measured from short-axis cine images reconstructed radially along the long axis. Strain parameters before and after PVR were compared using paired t-tests. One-way ANOVA with Tukey post-hoc analysis was used for comparisons between the before and after PVR groups and the control group. Results: There were no differences in strain parameters before and after PVR. The GPSmax before PVR was lower than that in the control group (P=0.002). Before and after PVR, GCSs were higher and GLSs were lower than those in the control group (before and after GCSs: P=0.002 for both, before and after GLSs: PP=0.0003). Conclusions: RV strains from radially reconstructed short-axis cine images revealed impaired myocardial motion after PVR. When compared to the control group, changes in GCS and GLS in TOF patients before and after PVR might be due to RV remodeling.

The causal effects of vaccines on Kawasaki disease (KD) remain elusive. We aimed to examine the association between vaccines administered during infancy and the development of KD in Japan. We conducted a multicenter prospective case-control study using questionnaires and compared the vaccination status of infants (age: 6 weeks to 9 months) who developed KD (KD group, n = 102) and those who did not develop KD (non-KD group, n = 139). Next, we performed a case-crossover study of 98 cases in the KD group and compared the status of vaccinations between the case and control periods. We also compared the incidence of KD in children for each 5-year period before and after the addition of new vaccines (2012–2013) using data from the Nationwide Survey of KD. In the case-control study, the vaccination status of the KD and control groups did not differ to a statistically significant extent. Multivariable analysis of the vaccination status and patient backgrounds showed no significant association between vaccination and KD development. In the case-crossover study, the status of vaccinations during the case and control periods did not differ to a statistically significant extent. In the analysis of data from the Nationwide Survey of KD, the incidence of KD in children of ages subject to frequent vaccination showed no significant increases in the latter five years, 2014–2018. Based on these prospective analyses, we confirmed that vaccination in early infancy did not affect the risk of KD.

Pregnancy is hazardous with pulmonary arterial hypertension, but maternal mortality may have fallen in recent years. We sought to systematically evaluate pulmonary arterial hypertension and pregnancy-related outcomes in the last decade. We searched for articles describing outcomes in pregnancy cohorts published between 2008 and 2018. A total of 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated. Results showed that out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28 ± 2 years, mean pulmonary artery systolic pressure on echocardiogram was 76 ± 19 mmHg. Etiologies include idiopathic pulmonary arterial hypertension (22%), congenital heart disease (64%), and others (15%). Majority (74%) had good functional class I/II. Only 48% of women received pulmonary arterial hypertension-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34 ± 1 weeks, most (76%) had Cesarean section. Maternal mortality rate was 12% overall ( n = 26); even higher for idiopathic pulmonary arterial hypertension etiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, pulmonary arterial hypertension crises, pre-eclampsia, and sepsis; 61% of maternal deaths occur at 0–4 days postpartum. Stillbirth rate was 3% and neonatal mortality rate was 1%. In conclusion, pulmonary arterial hypertension in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.

Infective endocarditis (IE) in patients with adult congenital heart disease (ACHD) remains a diagnostic challenge due to difficulties in detecting endocardial lesions by echocardiography.In the diagnosis of ACHD-associated IE, characterized by right-sided IE