Kawasaki disease (KD) is one of the acute vasculitis diseases, and its most common complications are coronary artery lesions and myocarditis.Different degrees of myocardial injury can be observed in the acute phase of KD, and different abnormal waveforms can be detected in the electrocardiogram (ECG). As a noninvasive electrophysiological examination, ECG is of great significance for both early diagnosis and prognosis evaluation of KD.The advance in research enables ECG manifestations and electrophysiological significance in the early phase of KD to be gradually re-cognized.In this article, the foreign and domestic research on the changes and clinical significance of ECG in acute stage of Kawasaki disease in recent years was reviewed and summarized, and recent advance in the application of ECG in the acute phase of KD was also summarized.

Objective:To investigate the clinical manifestations, pathological characteristics, treatment and prognosis of pediatric rheumatic carditis with the initial symptom of acute heart failure, and to improve the clinical understanding of the disease.Methods:The clinical data, laboratory examination results, imaging findings, treatment and prognosis data of 10 cases with acute rheumatic carditis were collected and analyzed retrospectively.The patients pre-sented with acute heart failure as the first symptom and were treated in Shenzhen Children′s Hospital from January 2015 to February 2022.Results:There were 4 males and 6 females in this study.The mean age was 9.1 years (3 years and 1 month to 11 years and 3 months), and the average onset age was (9.3 ± 3.2) days (4-14 days). All the 10 cases had circulation symptoms.Besides, 4 cases were also complicated with joint symptoms, 3 cases with neurological symptoms and 2 cases with skin symptoms.Echocardiography revealed mitral valve diseases in all cases.More specifically, combined valve diseases were found in 5 cases, pure mitral regurgitation in 3 cases, and mixed mitral valve diseases (mitral regurgitation complicated with mitral stenosis) in 2 cases.After receiving antibiotic therapy, anti-infection treatment and anti-heart failure therapy, symptoms improved in all patients and valve lesions were alleviated.All patients were followed up for 6 months to 6 years.Six cases had persistent rheumatic heart disease during the follow up.Conclusions:Pediatric rheumatic carditis with the initial symptom of acute heart failure is characterized by early onset and the typical manifestation of rheumatic fever.Mitral valve disease is the commonest lesion.Echocardiography is of great value for the early diagnosis of the disease.Medical treatment is effective, but the incidence of chronic rheumatic heart disease is still high.

Objective:To explore the clinical characteristics of intellectual developmental disorder with cardiac arrhythmia syndrome (IDDCA) in a family caused by GNB5 gene variation and to review the literature.Methods:The clinical and genetic data of an infant with IDDCA, who visited Shenzhen Children′s Hospital in September 2018, were collected and analyzed. His parents′ and brother′s gene analysis was also done by the next-generation sequencing and confirmed by Sanger sequencing. Related literature up to March 2020 was searched in Online Mendelian Inheritance in Man (OMIM), PubMed, CNKI and Wanfang databases with “GNB5” “IDDCA” “LADCI” “intellectual developmental disorder with cardial arrhythmia” “language delay and attention deficit-hyperactivity disorder or cognitive impairment with or without cardiac arrhythmia” as the key words. The related papers were retrieved and analyzed to summarize the clinical and genetic characteristics of this disorder.Results:The proband was an 11-month-old boy who presented with mental and motor developmental retardation, accompanied with convulsion and muscle weakness. Sinus arrest was also detected. His electroencephalogram (EEG) and flash visual evoked potential (FVEP) were both abnormal. Genetic analysis identified the homozygous frameshift variation of GNB5 gene (c.136delG, p.Glu46Argfs*8) in this infant and heterozygous variation in his parents, confirmed the diagnosis of IDDCA. The same GNB5 variation was identified in his brother, who was 4 years and 8 months old and had developed the similar clinical manifestations after birth. There were only 7 papers reporting this disease in the literature review, with a total of 27 patients from 14 families. Including these 2 cases, there were 29 patients in total, whose age of diagnosis ranged from 5.5 months to 23 years. Among all the patients, 20 cases (69%) were diagnosed as IDDCA, while 8 cases (28%) as LADCI; and 11 (38%) were males while 18 (62%) females. Regarding the clinical features, 66% (19/29) had mental retardation, 41% (12/29) had seizures, 79% (23/29) developed language delay and 62%（18/29） had sinus node dysfunction. Genetic tests showed that 4 patients from 3 families had complex heterozygous variation, and 25 patients (86%) from 12 families had homozygous variation. Seventeen patients from 8 families were consanguineous. Among the total 12 variations, there were 4 nonsense, 3 frameshift, 2 missense and 2 shear mutations, and 1 shear disorder caused by synonymous mutation.Conclusions:IDDCA caused by GNB5 gene variations mainly manifests as general developmental delay or severe mental retardation, and sinus node dysfunction. GNB5 associated syndromes have phenotypic heterogeneity and are inherited in an autosomal recessive manner.

Objective:To evaluate the effectiveness of interventional treatment for neonatal critical pulmonary stenosis(NCPS).Methods:Clinical data of 12 neonates with NCPS who received percutaneous balloon pulmonary valvuloplasty (PBPV) from January 2016 to December 2019 in Department of Cardiology, Shenzhen Children′s Hospital were summarized and analyzed.The collected data included transthoracic echocardiography (TTE), percutaneous oxygen saturation (SPO 2), relevant data on interventional surgery, and follow-up results. Results:All 12 neonates with NCPS received PBPV successfully.The postoperative pressure difference between the right ventricle and the pulmonary artery ranged from 8 to 35 mmHg[(20±7) mmHg, 1 mmHg=0.133 kPa]. The postoperative SPO 2 ranged from 74%-100%[(93.0±5.9)%]. Three neonates with NCPS received Blalock-Taussig (B-T) shunt.One neonate with NCPS developed supraventricular tachycardia during the operation.There was no death for these 12 neonates with NCPS. Conclusions:Interventional treatment of neonates with NCPS could achieve a better effect and be employed as the first treatment option.Some neonates with NCPS would require cardiac B-T shunt or patent ductus arteriosus stent implantation.