Incidence of non-benign arrhythmia in neonatal intensive care unit: 18 years experience from a single center.

Background: Non-benign arrhythmias, which require urgent recognition and care in neonatal intensive care unit (NICU) settings, are rare but can severely impact neonates. We aimed to clarify the epidemiology and characteristics of non-benign arrhythmias and their influence on neonates. Methods: This single-center retrospective study included patients admitted to the NICU at Kurashiki Central Hospital between January 2001 and December 2019. Only patients with structurally normal hearts were included. The use of direct cardioversion (DC), antiarrhythmic agents, and the presence of risk factors was reviewed from medical records. Results: Of the 8082 admissions, 2919 patients (36.1%) were low birth weight infants (LBWI) weighing less than 1500 g. There were 23 patients with arrhythmias (nine of them were LBWIs) with an incidence of 0.28%. There were 16 patients with tachyarrhythmia (eight supraventricular tachycardia [SVT] cases, three atrial flutters [AFL] cases, three ventricular tachycardia cases, two junctional ectopic tachycardia cases), and seven with bradyarrhythmia (all with complete atrioventricular [AV] block). For tachyarrhythmia cases, seven patients required DC, and eight were on antiarrhythmic agents at the time of discharge. Two patients (28.5%) with complete AV block required pacemaker implantation before discharge. The treatment strategy was dependent on the type of arrhythmia. All patients were discharged without significant morbidities. Conclusions: The incidence of non-benign arrhythmias was as low as 0.28%. Arrhythmias can be managed successfully in neonates, yet risk factors related to mortality warrant further study.

Recurrent Meningitis in an 11-year-old Girl with a Petrous Apex Cystic Lesion.

Cases of recurrent meningitis in elderly patients with a spontaneous cerebrospinal fistula have been reported, and in some of these patients, cystic lesions were thought to be the underlying cause. We report a case of recurrent meningitis in an 11-year-old Japanese girl with an arachnoid cyst in the petrous apex. Pulsation of the cystic lesion was thought to cause bone erosion, leading to the formation of a fistula. Magnetic resonance imaging was useful in evaluating the arachnoid cyst and fistula. During 2 years of follow-up, the osteolytic lesion enlarged and the rate of bone erosion was higher than expected.

Postoperative Lung Perfusion with Anomalous Origin of One Pulmonary Artery from the Aorta.

Pulmonary artery damage is difficult to estimate in a patient with one pulmonary artery from the aorta, and the pulmonary artery of anomalous origin is usually damaged. We describe a newborn patient with anomalous origin of the right pulmonary artery from the aorta who presented with significant lung perfusion at the anastomotic site 6 months postoperatively; the left/right perfusion ratio was 10:90 on a scintigram. The unbalanced left/right lung perfusion gradually improved over a number of years. In a newborn patient with anomalous origin of one pulmonary artery from the aorta, unbalanced lung perfusion may improve.

Differential diagnosis of nonepileptic twilight state with convulsive manifestations after febrile seizures.

BACKGROUND: Nonepileptic twilight state with convulsive manifestations (NETC) is a nonepileptic state following a febrile seizure (FS), which may be misdiagnosed as a prolonged seizure and result in overtreatment. We aimed to describe clinical manifestations of NETC and to determine characteristics that are helpful to distinguish NETC from other pathological conditions. METHODS: We conducted a retrospective chart review from January 2010 to December 2016 and selected the patients who presented with symptoms resembling status epilepticus with fever and a confirmed diagnosis using an electroencephalogram (EEG). We compared the NETC clinical features and venous blood gas analysis results with those of other conditions that mimic NETC. We also compared the characteristics of NETC with past reports. RESULTS: Our NETC patients presented with short durations of the preceding generalized convulsions followed by tonic posturing, closed eyes, no cyanosis, responsiveness to painful stimulation, and no accumulation of CO2 in the venous blood gas. Most of these characteristics were consistent with past reports. Prolonged FS or acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) showed several of these features, but all the characteristics were not consistent with our study. CONCLUSIONS: Prolonged FS and AESD need to be differentiated from NETC, and close clinical observation makes it possible to partially distinguish NETC from the other conditions. EEG is recommended for patients with symptoms that are inconsistent with these features.

Left main coronary artery atresia in a child: a case report and review of the literature.

Congenital atresia of the left coronary artery is a very rare condition. We report a case of a 13-year-old boy who was followed-up at our outpatient clinic from 6 months of age because of heart murmur. He had no symptoms for more than 10 years, and his left ventricular contraction was normal. Exercise electrocardiogram was performed at 7 and 10 years of age, and ST depression was not observed. He presented with chest pain and syncope on exertion at the ages of 11 and 12 years, respectively, and ST depression on exercise electrocardiogram was detected for the first time at 13 years of age. Cardiac echocardiography showed a high-choic antero-lateral papillary muscle of the mitral valve, prolapse of the mitral anterior leaflet, and mild mitral valve regurgitation. Coronary angiography did not demonstrate antegrade left coronary flow or dilation of the right coronary artery. We performed coronary artery bypass grafting using the left internal thoracic artery. Postoperatively, the ST depression resolved, and his exercise tolerability improved.

A New Z Score Curve of the Coronary Arterial Internal Diameter Using the Lambda-Mu-Sigma Method in a Pediatric Population.

BACKGROUND: Several coronary artery Z score models have been developed. However, a Z score model derived by the lambda-mu-sigma (LMS) method has not been established. METHODS: Echocardiographic measurements of the proximal right coronary artery, left main coronary artery, proximal left anterior descending coronary artery, and proximal left circumflex artery were prospectively collected in 3,851 healthy children ≤18 years of age and divided into developmental and validation data sets. In the developmental data set, smooth curves were fitted for each coronary artery using linear, logarithmic, square-root, and LMS methods for both sexes. The relative goodness of fit of these models was compared using the Bayesian information criterion. The best-fitting model was tested for reproducibility using the validation data set. The goodness of fit of the selected model was visually compared with that of the previously reported regression models using a Q-Q plot. RESULTS: Because the internal diameter of each coronary artery was not similar between sexes, sex-specific Z score models were developed. The LMS model with body surface area as the independent variable showed the best goodness of fit; therefore, the internal diameter of each coronary artery was transformed into a sex-specific Z score on the basis of body surface area using the LMS method. In the validation data set, a Q-Q plot of each model indicated that the distribution of Z scores in the LMS models was closer to the normal distribution compared with previously reported regression models. Finally, the final models for each coronary artery in both sexes were developed using the developmental and validation data sets. A Microsoft Excel-based Z score calculator was also created, which is freely available online (http://raise.umin.jp/zsp/calculator/). CONCLUSIONS: Novel LMS models with which to estimate the sex-specific Z score of each internal coronary artery diameter were generated and validated using a large pediatric population.

Circumstances and outcomes of out-of-hospital cardiac arrest in elementary and middle school students in the era of public-access defibrillation.

BACKGROUND: Circumstances and outcomes of out-of-hospital cardiac arrest (OHCA) in elementary and middle school students while at school in the era of public-access defibrillation are unknown. METHODS AND RESULTS: We conducted a nationwide hospital-based survey of elementary and middle school students who had had OHCA of cardiac origin and received prehospital resuscitation in 2005-2009. Among 58 cases recruited, 90% were witnessed by bystanders; 86% had ventricular fibrillation as the initial rhythm; 74% were resuscitated by bystanders; 24% were defibrillated by bystanders; 55% occurred at school; 66% were exercise-related; 48% were followed up before the event; 67% had structural heart disease. In total, 53% of overall patients and 79% of those initially defibrillated by bystanders had a favorable neurological outcome. Patients were more likely to be defibrillated by bystanders (38% vs. 8%, P=0.012) and had a more favorable neurological outcome in schools (69% vs. 35%, P=0.017) than in other locations. The majority of arrests in schools were exercise-related (84% vs. 42%, P=0.001), occurred at sports venues, and students were resuscitated by teachers; half of the cases at school occurred in patients with a pre-event follow-up. CONCLUSIONS: After OHCA, children were more likely to be defibrillated by bystanders and had a better outcome in schools than in other locations, which may be relevant to the circumstances of events.

Long-term outcome of transcatheter polytetrafluoroethylene-covered stent implantation in a giant coronary aneurysm of a child with Kawasaki disease.

We report the long-term outcome of a child with Kawasaki disease (KD), who had a giant aneurysm with stenotic lesions in the right coronary artery, resulting in the transcatheter implantation of a polytetrafluoroethylene (PTFE)-covered stent at the age of 8. Quantitative coronary angiography analysis later showed the increase of the diameter stenosis at both stent edges for the first 10 months after implantation; however, the stenosis did not develop thereafter. Coronary perfusion was still well maintained at follow-up coronary angiography 5.5 years after implantation (diameter stenosis was 36.5% at proximal and 37.4% at distal edge, respectively). Although further follow-up is necessary, the long-term outcome of a PTFE-covered stent implantation for a KD patient is satisfactory in this instance. Implantation of a covered stent in a giant aneurysm is considered a promising treatment option.

Clinical features of adult patients with Eisenmenger's syndrome in Japan and Korea.

BACKGROUND: There are few articles on mortality and morbidity of adult patients with Eisenmenger's syndrome (ES) in the current era when disease targeting therapy (DTT) has been available. METHODS AND RESULTS: 198 patients (a median age 35 years, 64% female) with ES who visited the 16 participating institutes in Japan and Korea from 1998 to 2009 were enrolled. Clinical data during adulthood were collected from each institutional chart and analyzed centrally. During a median follow-up of 8 years, 30 patients died including 14 sudden deaths. 89 patients took oral medication of DTT and clinical improvement was observed in 54 of them. However, survival rate in patients taking DTT was not different from those without (87% vs 84%, p=0.55). When the clinical data in between first and last clinic visits were compared in 85 patients, the patients with NYHA >/=III increased from 24% to 48% (p<0.001), SpO2 decreased from 89% to 85% (p=0.008) and hematocrit increased from 51.4% to 52.9% (p=0.04). Non-survivors had poorer NYHA function class, lower body weight (BW), lower body mass index (BMI), and higher serum level of Cr at the first visits than survivors. CONCLUSIONS: Long term survival and clinical status of adult patients with ES remains unsatisfactory even in the current era of DTT. Poor NYHA functional class, low BW, low BMI and high serum level of Cr were related to mortality. DTT therapy improved clinical status in many patients with Eisenmenger's syndrome, but no significant impact on survival could be shown.

Comparison of the clinical presentation, treatment, and outcome of fulminant and acute myocarditis in children.

BACKGROUND: Myocarditis (MC) is an important cause of cardiac dysfunction in children. Fulminant MC is sometimes fatal, and sequelae may develop during follow-up. We conducted a nationwide survey to determine the clinico-epidemiological features of MC in Japanese children and adolescents. METHODS AND RESULTS: Survey questionnaires were mailed to 627 hospitals, which were asked if they had treated MC patients aged between 1 month and 17 years during the period from January 1997 through December 2002. Responses were collected until December 2005, and data were collected and analyzed until January 2008. A total of 169 patients were reported: 64 fulminant cases, 89 acute cases, and 8 chronic cases. Incidence was 43.5 cases/year and 0.26 cases/100,000. Pathogens were identified in 37 patients; coxsackie virus accounted for 60%. Major cardiovascular manifestations at onset were congestive heart failure, refractory arrhythmia, and syncope in 70, 37, and 17 patients, respectively. Intravenous immunoglobulin was administered to 73 patients. Mechanical support seemed to be effective and life-saving. Among the 169 patients, 123 survived. Cardiovascular sequelae were reported in 49 patients. CONCLUSIONS: The survival rate for children with fulminant MC was disappointing. Overall, two-thirds of survivors had no sequelae. Mechanical support may reduce the mortality and the risk of clinical worsening.

[Paroxysmal automatic movements mimicking neonatal seizures induced by midazolam].

We have observed paroxysmal automatic movements including drum-beating and pedaling motions in three full-term neonates following intravenous bolus injections (0.1-0.3 mg/kg/dose) or drip infusions (0.2 mg/kg/h) of midazolam used for sedation. In one patient, abnormal movements were also induced by a bolus injection of midazolam during the EEG recording, and no change was revealed in the EEG during the episode. In another patient, abnormal movements were further worsened by an injection of diazepam. Interictal EEGs of all patients were normal. The clinical manifestations of these paroxysmal automatic movements and the mode of their appearance were quite similar in all patients. It is quite likely that abnormal movements in the patient without ictal EEG change do not have epileptic origin but brainstem release phenomenon induced by midazolam. Because the abnormal movements in the other two cases had similar clinical manifestations and mode of appearance, we suspected that these movements were also non-epileptic though ictal EEGs were not recorded in theses cases. When we encounter paroxysmal automatic movements mimicking neonatal seizures following intravenous midazolam administration, ictal EEG recordings are recommended. If there are no ictal changes, we should avoid treatment with anticonvulsant drugs for these movements. Since midazolam is frequently used in neonates for sedation during various examinations, future investigations on the selection of appropriate drugs and dosage for sedation in neonates, including the usage of midazolam, are necessary.

Three-dimensional echocardiographic pictures of isolated double-orifice mitral valve.

We present a case of a 12-year-old boy with a rare form of cardiac anomaly, isolated double-orifice mitral valve. He was referred to our hospital at 1.5 years old because of heart murmur. Two-dimensional echocardiography showed double-orifice mitral valve without any associated cardiac anomalies. He has been followed carefully without any medication for 11 years. He has had no symptoms and an excellent natural course thus far.

Standard method for ultrasound imaging of coronary artery in children.

The Child Coronary Arterial Diameter Reference Study Group of the Japan Kawasaki Disease Society recommends ultrasound imaging as the standard method for measuring the diameter of the coronary artery in children. The patient is examined in a supine or right decubitus position by using a sector probe (≥ 5 MHz). The coronary arterial diameter measured at the minimum gain setting is the distance between the internal echo edge and the internal echo edge. The diameter is measured during the early diastolic phase at the end of the T wave. The left main coronary artery and the proximal right coronary artery are approached from the precordial short axis at the level of the aortic valve. The proximal and mid-right coronary arteries are observed on the atrioventricular groove, anterior to the tricuspid valve ring. The right coronary artery of the acute margin of the heart runs along the right side of the tricuspid valve ring. The distal right coronary artery is observed on the posterior atrioventricular groove, and the posterior descending branch of the right coronary artery is observed on the posterior interventricular groove. The right coronary artery is also well observed from the right sternal border in the right decubitus position. Proximal and mid-anterior descending arteries are observed on the anterior interventricular groove. The proximal left circumflex coronary artery is observed in the atrioventricular groove, anterior to the mitral valve ring.

An elderly case of right ventricular outflow obstruction precisely diagnosed and successfully treated with surgical repair.

Congenital diseases causing an obstruction of the right ventricular outflow are difficult to precisely diagnose, especially in elderly patients. Here, we describe a 76-year-old woman who presented to our hospital with shortness of breath on exertion and was finally diagnosed as infundibular pulmonary stenosis by multiple modalities. Surgery was successfully performed and the symptom was relieved. This case underscored the usefulness of multiple modalities for the precise assessment of the right ventricular outflow obstruction and the effectiveness of surgery even in elderly patients with infundibular pulmonary stenosis.

Analysis of Kawasaki disease showing elevated antibody titres of Yersinia pseudotuberculosis.

AIM: To elucidate a clinical difference between patients with Kawasaki disease documented with Yersinia pseudotuberculosis infection and patients with Kawasaki disease without Yersinia pseudotuberculosis infection. PATIENTS AND METHODS: From January 1985 to July 2004, 452 patients were diagnosed with Kawasaki disease. Forty-two patients had elevated antibody titres of Yersinia pseudotuberculosis and/or positive stool culture (Yersinia-positive group). Three hundred and thirty patients had no elevated antibody titres (Yersinia-negative group). We compared the clinical characteristics retrospectively. RESULTS: The age of onset in the Yersinia-positive group (3.05+/-2.20 y) was significantly higher than that in the Yersinia-negative group (2.31+/-2.05 y) (p=0.03). The age-adjusted statistical analysis demonstrated that the incidence of coronary artery lesions (dilatations plus aneurysms) in the Yersinia-positive group (22/42, 52.4%) was significantly higher than in the Yersinia-negative group (105/330, 31.8%) (p=0.001), and the incidence of additional administration of immunoglobulin in the Yersinia-positive group (13/36, 36.1%) was significantly higher than in the Yersinia-negative group (41/256, 16.0%) (p=0.004). CONCLUSION: Our data suggest that Yersinia pseudotuberculosis infection might play a role in the developing mechanism of poor response to therapy and the tendency to develop coronary artery lesions in Kawasaki disease patients.

Successful release of recurrent pulmonary venous obstruction after repair of totally anomalous pulmonary venous connection by transcatheter implantation of stents.

We report a 3-month-old female infant, in whom pulmonary venous obstruction occurred after repair of totally anomalous pulmonary venous connection, and which was successfully released by a transcatheter implantation of a stent using the transseptal approach. Close follow-up is required, since the long-term outcome is still unclear. Nevertheless, transcatheter implantation of stents is a promising option for treating this challenging lesion.

Changes in causes of sudden deaths by decade in patients with coronary arterial lesions due to Kawasaki disease.

Over a 25-year period, we encountered 12 patients who died suddenly with coronary arterial lesions due to Kawasaki disease. We report their clinical course, and analyze the happenings of their deaths. Of the 12 patients, 10 were dead on arrival at hospital. Their age at death ranged from 13 months to 27 years, with a median of 16 years, and the interval from the onset of Kawasaki disease to death ranged from 2 months to 24 years. In 4 patients, death was found to be due to myocardial infarction, while in the remaining 8, it could not be determined. In 7 patients, coronary angiograms obtained less than 4 months after the acute onset of Kawasaki disease showed lesions bilaterally, most being giant aneurysms. Myocardial infarction had occurred in 6 patients prior to their death. In 1 patient of the late 1970s, who collapsed after running, cardiac sequels had not been suspected prior to autopsy. During the 1980s, 3 infants with bilateral giant aneurysms died within a year of the initial onset of Kawasaki disease, with acute myocardial infarction being the cause in 2 of them. In the late 1990s, and the 2000s, 5 patients died suddenly with left ventricular dysfunction, their ejection fractions being less than 40 percent more than 20 years after the initial onset of Kawasaki disease. Prior to their sudden deaths, they had had no cardiac events for many years, but had suffered previous myocardial infarctions. Multifocal premature ventricular contractions, and non-sustained ventricular tachycardia, are probable risk factors in such patients. Careful follow-up, checking for ventricular arrhythmia, is needed to prevent sudden death in patients suffering left ventricular dysfunction in the setting of Kawasaki disease.

Impact of noncoronary cusp prolapse in addition to right coronary cusp prolapse in patients with a perimembranous ventricular septal defect.

BACKGROUND: Few data are available with regard to the impact of aortic cusp herniation on the evolution of aortic regurgitation (AR) in patients with a perimembranous ventricular septal defect (VSD). METHODS: One hundred and two patients with a perimembranous ventricular septal defect with right coronary cusp prolapse were divided to two groups depending on the development of aortic regurgitation. The original defect diameter, the right coronary cusp deformity index (RCCD), and the right coronary cusp imbalance index were obtained as we reported previously. RESULTS: Mild aortic regurgitation was detected in 35 patients, and moderate in three. No aortic regurgitation was observed in 64 patients. A significantly larger number of patients had noncoronary cusp prolapse and the right coronary cusp imbalance index >/=1.30 in the aortic regurgitation group than in the no regurgitation group. Relative risk and odds ratio of noncoronary cusp prolapse and the right coronary cusp imbalance index >/=1.30 for development of aortic regurgitation were 3.69 (95% CI, 0.91-15.03) and 27.90 (95.94-130.85), and 2.23 (0.83-5.98) and 4.70 (1.44-15.27), respectively. Surgical closure was performed in 29 patients. All patients with no noncoronary cusp prolapse underwent simple patch closure of the ventricular septal defect, while five patients with noncoronary cusp prolapse and aortic regurgitation underwent aortic valvuloplasty. Among these, one patient needed aortic valve replacement. CONCLUSIONS: The additional complication of noncoronary cusp prolapse is a strong risk factor for the development of aortic regurgitation in patients with a perimembranous ventricular septal defect with right coronary cusp prolapse.