Natural excretion of a metallic susceptor originating from an ingested heated tobacco stick.

Key Clinical Message: Clinicians should not only consider the presence of metallic foreign bodies within the digestive tract but also contemplate the possibility of nicotine poisoning during the diagnostic process. Abstract: When clinicians encounter cases of accidental ingestion of some types of heated tobacco, they must consider not only nicotine poisoning but also the possibility of a metallic foreign body within the digestive tract during diagnosis. In children, even sharp or relatively large ingested foreign bodies can spontaneously pass below the esophagus. Considering that this 12-mm metal piece is small, natural excretion may be considered rather than endoscopic removal.

Being Overweight Is Related to Neoaortic Sinus Dilatation After Arterial Switch Operation.

BACKGROUND: Neoaortic sinus dilatation is a late comorbidity after an arterial switch operation in patients with dextro-transposition of the great arteries. We aim to explore whether neoaortic sinus dilatation is related to overweight or obesity, as these affect aortic remodeling in such patients. METHODS: We measured neoaortic diameters including those for the annulus, sinus, sinotubular junction, and ascending aorta by echocardiography and studied the relationship between these diameters and body mass index in patients aged 15 years and older after an arterial switch operation. RESULTS: A total of 45 patients were studied. Median (interquartile range) age, body mass index, and systolic blood pressure at echocardiography were 20 (17-28) years, 21.2 (18.4-24.2) kg/m2, and 120 (112-127) mm Hg, respectively. Echocardiogram revealed the following median values (interquartile ranges): neoaortic annulus diameter, 22.2 (19.8-23.8) mm; sinus diameter, 34.6 (31.8-39.8) mm; sinotubular junction diameter, 27.5 (22.4-30.0) mm; and ascending aortic diameter, 20.4 (19.4-22.7) mm. The neoaortic sinus diameter was significantly correlated with body mass index (r = 0.41, P = .004) and was significantly larger in patients with concomitant ventricular septal defect (P < .001) and those who were over-weight (body mass index ≥25 kg/m2; P = .006). CONCLUSION: Neoaortic sinus dilatation after an arterial switch operation is associated with being overweight during adolescence and young adulthood. It is important to prevent obesity in patients after an arterial switch operation and educate them on a healthy lifestyle.

Progress of pulmonary hypertension and high manganese levels through congenital portosystemic shunt closure.

A 6-year-old girl with congenital portosystemic shunt presented with abnormal manganese levels and improving pulmonary hypertension even 1 year after shunt vascular ligation. As the progress after portal vein blood flow recovery varies among individuals, long-term follow-up of patients with congenital portosystemic shunt is needed.

Overgrowth of the Amygdala in Children with Single Ventricle Congenital Heart Disease.

Aim: Early life stress is associated with overgrowth of the amygdala, which plays a key role in the processing and memory of emotional responses. Herein, we aimed to explore the amygdala volume in children with single-ventricle congenital heart disease who experience repeated admissions during the neonatal period and infancy. Methods: We compared the amygdala volume measured using brain magnetic resonance imaging (MRI) between 40 patients after completion of the Fontan procedure and 40 age- and sex-matched control subjects Results: Age at the MRI study were 9.2 (8.5-11.1) and 10.2 (9.2-10.3) years in the Fontan and control groups, respectively. The maximum amygdala volume in the Fontan group was significantly larger than in the control group (1232 [983-1392] mm3/m2 vs. 980 [728-1166] mm3/m2, P < 0.001). The amygdala volume did not correlate to cardiac index (r = 0.260) and central venous pressure (r = -0.107) in the Fontan group. Conclusions: Children with single-ventricle congenital heart disease exhibited amygdala overgrowth.

Coronary arterial dominance in patients with congenital heart disease.

Coronary arterial dominance is concerned in the management of ischemic heart disease. In particular, right coronary arterial dominance is having a risk for three-vessel coronary artery disease. Thus, this study aimed to explore coronary arterial dominance in patients with congenital heart disease. The study involved 250 patients, of which 105 patients were with tetralogy of Fallot (TOF), 100 patients with ventricular septal defect (VSD), and 45 patients with Kawasaki disease (KD). We retrospectively reviewed their ascending aortography to determine their coronary arterial dominance, Z-scores of coronary artery diameter, and the ascending aortic curvature, which pertained to the angle between the aortic annulus plane and ascending aortic plane. We identified relevant factors that contribute to having right coronary arterial dominance. Age and weight of the 250 subjects were 2.9 (1.0-8.7) months and 7.7 (5.0-9.4) kg, respectively. The Z-scores of right coronary and anterior descending arteries significantly differed among patients with TOF, VSD, and KD (P < 0.001, P = 0.001). However, there were no significant differences in the Z-scores of left main trunk and circumflex arteries. Right coronary arterial dominance occurred in 89%, 49%, and 61% in patients with TOF, VSD, and KD, respectively (P < 0.001). The presence of TOF was the most powerful predictor for right coronary arterial dominance (odds ratio: 10.31, 95% confidence interval: 4.11-27.2, P < 0.001). We found the robust relationship between right coronary arterial dominance and TOF. Patients with TOF may have an increased risk for the development of coronary artery disease during adulthood.

Home High-Flow Nasal Cannula Therapy in Children with Congenital Heart Disease.

High-flow nasal cannula (HFNC) therapy has been applied in the perioperative respiratory care for children with congenital heart disease and respiratory problems. However, the information about the feasibility of home HFNC therapy remains lacking among them. We retrospectively reviewed 5 children with congenital heart disease and respiratory problems who underwent home HFNC therapy, and evaluated their feasibility and safety. Age and weight at the introduction of home HFNC therapy were 19 (2-119) months and 5.3 (3.1-11.4) kg, respectively. All subjects had chromosomal anomaly including trisomy 18 in 3 and trisomy 21 in 2 subjects. Cardiac diagnoses included ventricular septal defect in 3, tetralogy of Fallot with complete atrioventricular septal defect in one, and pulmonary atresia with ventricular septal defect in another subject. Other comorbidities involved pulmonary hypertension in 4, micrognathia in 4, West syndrome in one, and bronchial asthma in one subject. Respiratory manifestations involved cyanosis due to upper airway obstruction in 2 and central hypopnea in 2, and recurrent pneumonia in one subject. After home HFNC therapy, systemic oxygen saturation significantly increased from 60 (40-78)% to 83 (83-96)% (P = 0.04), while heart rate and blood partial pressure of carbon dioxide were significantly decreased. There was no adverse event relevant to home HFNC during the follow-up period of 12 (5-49) months. Among them, one patient subsequently underwent tracheotomy at 11 years of age, and two patients weaned to conventional home oxygen therapy at 7 and 23 months of age. Home HFNC is safe and feasible in children with congenital heart disease and respiratory problems.

Pulmonary vascular resistance and compliance in individuals with trisomy 18.

Individuals with trisomy 18 (T18) usually have congenital heart disease, often with pulmonary hypertension, which is associated with poor outcomes. This study aimed to explore the characteristics of pulmonary circulation including pulmonary vascular resistance (Rp) and compliance (Cp) among them. We retrospectively reviewed cardiac catheterization data in subjects with T18, trisomy 21 (T21), and without chromosomal anomaly (control group) who were referred due to heart failure associated with ventricular septal defect between 2000 and 2020. Pulmonary hemodynamic parameters including Rp and Cp were compared between these groups. We studied 20 subjects with T18, 88 subjects with T21, and 240 control subjects. There was no significant difference in age (T18: 4.6 [3.0-6. 9] vs. T21: 2.8 [1.9-4.0] vs. control: 2.9 [1.6-3.2] months, p = 0.06) and mean pulmonary arterial pressure (T18: 41 [33-49] vs. T21: 35 [30-41] vs. control: 36 [28-43] mmHg, p = 0.121) between the groups. The pulmonary to systemic blood flow ratio (Qp/Qs) (p = 0.983), Rp (p = 0.449), and Cp (p = 0.195) did not differ between T18 and control groups. However, Qp/Qs and Cp in T18 group were significantly greater than that in T21 group (T18: Qp/Qs: 3.4 [2.3-5.2] vs. T: 21 2.3 [1.7-3.7], p = 0.001. Cp: 3.5 [2.3-5.5] vs. 2.3 [1.6-3.1] mmHg/mL/m2 , p = 0.007), while Rp was identical between the groups (T18: 2.0 [1.6-3.3] vs. T21: 2.3 [1.7-3.7], p = 0.386). The pulmonary circulation in T18 subjects differed from that observed in T21 subjects, and identical to that observed in control subjects. Pulmonary hypertension is expected to be normalized after reasonable corrective surgery in T18 patients with congenital heart disease.

Distensibility of the ductus arteriosus in neonates and young infants undergoing transcatheter closure.

Device migration is one of serious complications in neonates and infants undergoing transcatheter closure of the patent ductus arteriosus (PDA). We hypothesize that neonates and young infants possess the distensibility of the ductus, which may be related to device migration. We retrospectively reviewed angiographic findings in 41 neonates and infants who underwent transcatheter closure of PDA. We measured diameters of the ductus at the pulmonary (PA) side, the center, and the aortic (AO) side before PDA closure, and the device center diameter after device closure. The distensibility index was defined as the ratio of the device center diameter after device deployment to the diameter at the center of the ductus before PDA closure. Age and weight at the procedure were 168 (117-260) days and 5.3 (4.3-6.9) kg, respectively. Thirty-seven subjects accomplished the successful device closure, and four subjects were declined because of the device instability or migration. Implanted devices included Amplatzer Duct Occluders in 33 subjects and Amplatzer Vascular Plug-2 in 8 subjects. The PDA diameters at PA side, at the center, AO side, and the device center diameter were 3.2 (2.2-4.3) mm, 4.7 (3.6-5.7) mm, 7.7 (6.3-9.4) mm, and 5.8 (4.2-6.9) mm, respectively. The PDA diameter before closure was not correlated age and weight. The distensibility index was 1.28 (1.06-1.64), which was significantly correlated to age (r = - 0.49, P = 0.001) and weight (r = - 0.53, P < 0.001). Infants with the younger age and the lower weight have the more distensible PDA, which may be a risk for device migration.

Late Adverse Events in Patients With Pulmonary Atresia With Intact Ventricular Septum After Valvuloplasty.

BACKGROUND: We aimed to explore the relation between temporal right heart growth and long-term outcomes in patients with pulmonary atresia with an intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvuloplasty (BPV). METHODS: We performed echocardiography to measure pulmonary valve diameter, right atrial end-systolic area (RAA), and right ventricular end-diastolic area (RVA) before BPV 1 and 5 years after BPV. Primary and secondary end points were to explore temporal changes in right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs). RESULTS: In 31 patients, pulmonary valve diameter significantly increased after BPV, whereas tricuspid valve diameter remained unchanged throughout the 10.0-year follow-up (range, 5.8-14.0 years). After BPV, RAA temporally decreased, whereas RVA significantly increased. There were LAEs in 6 patients (19%), arrhythmias in 2, heart failure in 1, reintervention of the right ventricular outflow tract in 1, and reintervention for residual cyanosis in 2. The rate of freedom from LAEs at 5 and 10 years was 92% and 82%, respectively. Right atrial end-systolic area temporally decreased in patients without LAEs (P < .01); however, RAA remained unchanged throughout the period in patients with LAEs (P = .16). Moderate or severe pulmonary regurgitation (hazard ratio = 23.0; 95% confidence interval, 1.3-385; P = .03) and the ratio of RVA to RAA at 1 year after BPV (hazard ratio = 6.3 × 10-11; 95% confidence interval, 2.1 × 10-20 to 0.19; P = .03) were independent risk factors for LAEs. CONCLUSIONS: Disproportional right heart growth was observed in patients with pulmonary atresia with an intact ventricular septum after BPV. Pulmonary regurgitation and increased RAA are crucial for identifying the burden of LAEs among them.

Long-Term Outcomes After an Individualized Strategy in Patients with Pulmonary Atresia and Intact Ventricular Septum.

This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.

Characteristics of the pulmonary circulation in infants with complete atrioventricular septal defect.

OBJECTIVE: Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. METHODS: We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. RESULTS: Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of -0.70 (-1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). CONCLUSIONS: The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.

Headache and Left Ventricular Efficiency After Transcatheter Closure of Atrial Septal Defect.

OBJECTIVE/BACKGROUND: This study aimed to clarify the relationship between migraine-like headache and ventriculo-arterial coupling after transcatheter closure of the atrial septal defect in children. We hypothesized that migraine headache after defect closure would be related to an abnormal hemodynamic response against an increased left ventricular filling. DESIGN: A retrospective cohort study. METHODS: We calculated the end-ventricular systolic elastance (Ees), effective arterial elastance (Ea), and ventricular energy efficiency approximated based on echocardiography before and after defect closure, and compared these parameters between the subjects with and without headache after defect closure. RESULTS: A total of 167 subjects were studied. Age at the procedure, defect diameter, and pulmonary to systemic blood flow ratio were 11 (9-17) years, 12.8 (9.2-16.0) mm, and 1.8 (1.6-2.3), respectively. We identified 47 (28%) subjects with migraine headache after defect closure. Although there was no significant difference in the Ees, Ea, and ventricular energy efficiency before defect closure between the groups, the Ees (4.0 [3.4-4.9] vs 4.8 [3.7-6.1], P = .014) and ventricular energy efficiency (0.79 [0.76-0.82] vs 0.83 [0.79-0.85], P = .001) after defect closure in subjects with headache were significantly lower than those in subjects without headache. Migraine headache after defect closure was significantly associated with age (odds ratio: 0.97, 95% confidential interval: 0.94-1.00, P = .036) and a decrease in the ventricular energy efficiency after defect closure (odds ratio: 6.42, 95% confidential interval: 2.76-14.90, P < .001). CONCLUSION: A loss of ventricular energy efficiency was common in pediatric subjects with migraine-like headache after transcatheter closure of the atrial septal defect, which suggested that the left ventricular function maladaptation was related to headache development after defect closure. We advocate that an impaired ventriculo-arterial coupling may be one of the mechanisms for developing attacks in not only this population but also in other patients with migraine.

Sub-clinical myocardial ischaemia due to cardiac strangulation by an epicardial pacemaker lead.

We present the case of a 10-year-old boy with congenital complete atrioventricular block who had cardiac strangulation by an epicardial pacemaker lead placed during infancy. Coronary angiography and Tc99m tetrofosmin myocardial perfusion scintigraphy suggested sub-clinical myocardial ischaemia.

Effects of intracerebroventricular administration of 2-hydroxypropyl-ß-cyclodextrin in a patient with Niemann-Pick Type C disease.

Niemann-Pick Type C disease (NPC) is an autosomal recessive lysosomal storage disorder characterized by progressive neurological deterioration. Previously, we reported that intravenous administration of 2-hydroxypropyl-ß-cyclodextrin (HPB-CD) in two patients with NPC had only partial and transient beneficial effects on neurological function. The most likely reason for HPB-CD not significantly improving the neurological deficits of NPC is its inability to cross the blood-brain barrier. Herein, we describe the effects of intrathecal HPB-CD in an eight-year-old patient with a perinatal onset of NPC, administered initially at a dose of 10 mg/kg every other week and increased up to 10 mg/kg twice a week. Clinically, the patient maintained residual neurological functions for two years, at which time nuclear magnetic resonance spectroscopy showed a decreased choline to creatine ratio and increased N-acetylaspartate to creatine ratio, and positron emission tomography revealed increased standardized uptake values. Total-tau in the cerebrospinal fluid (CSF) was also decreased after two years. No adverse effects were observed over the course of treatment. The CSF concentrations of HPB-CD during the distribution phase after the injections were comparable with those at which HPB-CD could normalize cellular cholesterol abnormality in vitro. Further studies are necessary to elucidate the mechanisms of action of HPB-CD in NPC, and to determine the optimal dose and intervals of HPB-CD injection.

Acute alcohol intoxication in a 15-day-old neonate.

We describe a 15-day-old newborn girl who was fed with formula milk that was accidentally diluted with sake (Japanese wine prepared from fermented rice). The clinical features were flushed skin, tachycardia and low blood pressure indicating circulatory failure, somnolence and metabolic acidosis without hypoglycemia. The serum ethanol concentration was 43.0 mg/dL at 3 h after intake. The patient recovered under intravenous fluid replacement without complications. Follow-up examinations at 1, 2, 3, 6 and 12 months confirmed normal psychomotor development.