Interventional treatment and early-stage follow-up of pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective: To explore the application value of percutaneous peripheral interventional therapy in pulmonary atresia with intact ventricular septal (PA-IVS). Methods: Retrospective case summary. The data was collected from 25 children who were hospitalized at the Children's Hospital,Zhejiang University School of Medicine from August 2019 to August 2022, had been diagnosed with PA-IVS by echocardiography, and underwent interventional treatment. The sex, age, weight, operation time, radiation exposure time, and radiation dose of the patients were collected. The patients were divided into the arterial duct stenting group and the non-stenting group. Preoperative tricuspid annular diameters and Z scores, right ventricular length diameters, and right ventricular/left ventricular length-diameter ratios were compared by paired t-tests. Right ventricular systolic pressure difference, oxygen saturation, lactic acid before and after the surgery were compared for 24 children who received percutaneous balloon pulmonary valvuloplasty. Right ventricular improvement in 25 children after operation was analyzed. The correlation between postoperative oxygen saturation and postoperative right ventricular systolic blood pressure difference, the degree of pulmonary valve opening and the Z value of tricuspid valve ring in the non-stenting group were analyzed. Results: A total of 25 patients with PA-IVS were enrolled in the study, of whom 19 were males and 6 females, with an age at surgery of 12 (6, 28) days and a weight of (3.7±0.5) kg. One of them underwent only stenting of the arterial duct; 20 children underwent only percutaneous pulmonary valve perforation and balloon angioplasty; 4 children underwent both procedures. The Z-value of the tricuspid ring was -1.5±1.2 in the group with arterial duct stenting, and -0.1±0.4 in the group without stenting (t=2.77, P=0.010). The tricuspid regurgitant flow rate 1 month after surgery was significantly lower than the preoperative ((3.4±0.6) vs. (4.8±0.9) m/s, t=6.62,P<0.001). In the 24 children with percutaneous pulmonary valve perforation and balloon angioplasty, the preoperative right ventricular systolic blood pressure was (110±32) mmHg, and the postoperative systolic blood pressure was (52±19) mmHg (1 mmHg=0.133 kPa) (F=59.55, P<0.001). The factors that may affect postoperative oxygen saturation in 20 cases of non-stenting group were analyzed. The results suggested that the pre and post-operative right ventricular systolic blood pressure differences (r=-0.11, P=0.649), and the pulmonary valve orifice opening (r=-0.31, P=0.201) and tricuspid annulus Z value (r=-0.18, P=0.452) at 1 month after the operation were not significantly correlated with the postoperative oxygen saturation. Conclusions: Interventional therapy can be used as the first choice for one-stage operation of PA-IVS. Percutaneous pulmonary valve perforation and balloon angioplasty are more suitable for children with well-developed right ventricles, tricuspid annulus, and pulmonary arteries. While the smaller the tricuspid annulus, the more dependent it is on the ductus arteriosus and thus patients are more suitable for arterial duct stenting.

Recent research on platelet-leukocyte aggregates and their role in the pathogenesis of Kawasaki disease / 中国当代儿科杂志

Activated platelets may interact with various types of leukocytes such as monocytes, neutrophils, dendritic cells, and lymphocytes, trigger intercellular signal transduction, and thus lead to thrombosis and synthesis of massive inflammatory mediators. Elevated levels of circulating platelet-leukocyte aggregates have been found in patients with thrombotic or inflammatory diseases. This article reviews the latest research on the formation, function, and detection methods of platelet-leukocyte aggregates and their role in the onset of Kawasaki disease, so as to provide new ideas for studying the pathogenesis of Kawasaki disease.

A case -control study on the risk factors of congenital heart disease among perinatal infants / 预防医学

Objective To investigate the incidence of congenital heart disease (CHD)among perinatal infants in Hangzhou City and to explore risk factors of congenital heart disease in order to provide suggestions for CHD's prevention.Methods A hospital -based case -control study was carried out.Cases and controls were interviewed.By means of univariate and multiple logistic regression analysis,risk factors were analyzed.Results The incidence of CHD from 2009 to 2013 was 62.73 per 10,000.Of 176 perinatal infants with CHD,109 were single deformity and 67 were composite deformities of heart.The logistic regression analysis showed that maternal contact with harmful drugs during early pregnancy (OR =3.350,95%CI =1.024 -13.992),maternal respiratory infection during early pregnancy (OR =4.235,95%CI =1.275-18.735),abnormal childbearing history (OR =3.679,95%CI =1.102 -14.113),maternal smoking (OR =4.229, 95%CI =1.167 -15.782)and elderly parturient women (OR =2.974,95%CI =1.213 -16.372)were the risk factors of CHD.And maternal folic acid supplementation (OR =0.275,95% CI =0.079 -0.982 )was the protective factor. Conclusion It's of great importance to avoid risk factors and supply folic acid properly during pregnancy to prevent CHD.

Six Kawasaki disease patients with acute coronary artery thrombosis / 中华儿科杂志

<p><b>OBJECTIVE</b>To improve the awareness of acute coronary artery thrombosis in Kawasaki disease (KD).</p><p><b>METHOD</b>Six KD patients with acute coronary artery thrombosis (Jan. 2004 to Jan. 2013) were studied retrospectively. The basic information, clinical manifestations, laboratory data, echocardiography and electrocardiography (ECG), method and consequence of thrombolytic therapy were analyzed.</p><p><b>RESULT</b>The mean age of patients with coronary artery thrombosis (5 males and 1 female) was (17.2 ± 11.3) months.Five cases had thrombosis in left coronary artery (LCA), and four cases had thrombosis in aneurysm of left anterior descending artery (LAD). One case had thrombosis in both left and right coronary artery (RCA).One case died. Maximum thrombus was about 1.60 cm × 0.80 cm, locating in LAD. The diameter of LCA and RCA was (0.44 ± 0.07) cm and (0.45 ± 0.07) cm. Two patients showed abnormal ECG. Case 3 showed ST segment depression in lead V5. Case 6 showed myocardial infarction.In acute phase of KD, three patients received treatment with intravenous immunoglobin (IVIG), five patients were treated with aspirin.In sub-acute and convalescent phase of KD, all patients were treated with low-dose aspirin.Warfarin and dipyridamole were applied in 5 patients. All cases were treated with thrombolytic therapy using urokinase and/or heparin. After thrombolytic therapy, echocardiography showed thrombolysis in four cases and no change in one.One patient died of myocardial infarction.</p><p><b>CONCLUSION</b>Most of acute coronary thrombosis in KD occurred in LAD. KD patients with coronary artery thrombosis are at risk of sudden death due to myocardial infarction.</p>

Clinical features of recurrent Kawasaki disease in 20 children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical features of recurrent Kawasaki disease (KD) and the relationship of recurrent KD with coronary artery lesions.</p><p><b>METHODS</b>The medical data of 20 children with recurrent KD who were admitted to the Children's Hospital from January 1998 to May 2007 were retrospectively studied. Their clinical features were compared with those of children with initial KD.</p><p><b>RESULTS</b>The incidence of recurrent KD was 1.34% (20/1489). KD relapsed 2 months to 4.6 years (average: 1.2 years) after the first episode in the 20 children. Compared with the initial KD group, the clinical symptoms in the recurrent KD group were incomplete, complicated and less severe. The period of fever, platelet count, C-reactive protein and ESR were remarkably reduced in the recurrent KD group, but the incidence of coronary artery lesions increased significantly compared with the initial KD group (40% vs 25%; P<0.05).</p><p><b>CONCLUSIONS</b>The clinical symptoms of recurrent KD were incomplete in children. Recurrent KD was associated with an increased incidence of coronary artery lesions.</p>

Effect of intravenous immunoglobulin and vitamin C on progression of experimental autoimmune myocarditis in mice / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To evaluate the effect of intravenous immunoglobulin (IVIG) and vitamin C on the progression of experimental autoimmune myocarditis(EAM).</p><p><b>METHODS</b>Fifty-two Balb/c mice were randomized into six groups: The blank group received no treatment, the remaining 5 groups were immunized with 100mug emulsified porcine myosin at d 1 and d 7. Different agents were injected from d 1, SVitC group:150 mg/kg*d(-1)vitamin C; LVitC group: 300 mg/kg*d(-1)vitamin C; IVIG group: 1 g/kg*d(-1)IVIG; IVIG+VitC group: 1 g/kg*d(-1)IVIG and 150 mg/kg*d(-1)vitamin C; The control group same volume of normal saline. All mice were sacrificed at d 21, and serum TNF-alpha levels were detected with enzyme linked immunosorbent assay (ELISA). The ratio of heart to body weight(C/W), spleen to body weight(S/W) and kidney to body weigh(R/W) were calculated. The spleens and heart were examined pathologically and/or immunohistochemically.</p><p><b>RESULT</b>Compared with those of control group, inflammatory cells infiltration in the myocardium and calcification in the pericardiume in SVitC and LVitC groups were extenuated. There were inflammatory cells infiltrating in the myocardium sparely and no calcification in the pericardium in IVIG and IVIG+VitC groups. The size of spleens enlarged especially in IVIG and IVIG+VitC groups. White and red pulps of spleens were hyperplastic microscopically. The C/W of treatment groups decreased significantly compared with that of control group. The S/W of therapy groups and control group was significantly higher than that of blank group; and the S/W of IVIG and IVIG + VitC groups was significantly higher than that of SVitC and LVitC groups. The R/W in each groups had no significant difference. The TNF-alpha level in SVitC and LVitC groups was a little lower than that in control group; TNF-alpha level in IVIG and IVIG+VitC groups was significantly lower than that of control group. Wide fluorescence stripe was found along extracellular matrix surrounding the damaged cardiomyocytes of control group. Both density and intensity of fluorescence in SVitC and LVitC groups were lower than those of control group. There were much wider fluorescence stripe and strengthened intensity in IVIG and IVIG + VitC groups. The myofilaments were in wild disorder and sarcomere had severe breakage in control group. Moreover, chondriosome hypertrophy and vacuolar degeneration were found. The damage lessened in SVitC and LVitC groups. Both myofilaments and sarcomeres in IVIG and IVIG + VitC groups were almost normal, and the chondriosome was normal.</p><p><b>CONCLUSION</b>IVIG and vitamin C have some protective and therapeutic effect on the progression of EAM by decreasing pathological damage of myocardium and depressing TNF-alpha production, and IVIG combined with vitamin C is more effective.</p>

Patent Ductus Arteriosus and Pulmonary Valve Stenosis in A Patient with 18p Deletion Syndrome

We report on a patient with a partial deletion on the short arm of chromosome 18 (del 18p), who presented with dysmorphic features and delayed developmental milestones as well as with a patent ductus arteriosus (PDA) and pulmonary valve stenosis (PS). Several forms of congenital heart disease (CHD) are found in about 10% of patients with del (18p), but coexisting PDA and PS have not been reported. Del (18p) must be considered in patients with characteristic phenotypic abnormalities and congenital heart disease, including a combination of PDA and PS.

Cardiovascular manifestations in 40 patients with Williams syndrome / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the cardiovascular manifestations of Williams syndrome (WS) confirmed by fluorescence in situ hybridization (FISH).</p><p><b>METHODS</b>Between July 2004 and January 2007, FISH was used to confirm diagnosis in 71 suspected WS cases by detecting chromosome 7q microdeletion. Cardiovascular abnormalities were assessed by echocardiography and Doppler echocardiography.</p><p><b>RESULTS</b>Forty out of 71 patients were detected to have Elastin gene locus microdeletion, 25 patients (25/40, 62.5%) had at least one cardiac anomaly; among these patients, supravalvular aortic stenosis (SVAS) was diagnosed in 18 patients (18/25, 72%) and 6 of them had complex abnormalities. Patent ductus arteriosus was diagnosed in 3 patients (3/25, 12%, 1 was associated with other malformations), isolated pulmonary stenosis in 1 patient (1/25, 4%), isolated coarctation of aorta in 2 patients (2/25, 8%), and hypertension in 2 patients (2/25, 8%), mild aortic regurgitation in 2 patients, mild mitral regurgitation and moderate mitral regurgitation in 3 patients respectively.</p><p><b>CONCLUSION</b>A detailed cardiac evaluation should be performed in all patients with Williams syndrome due to the high frequency of cardiovascular abnormalities.</p>

Percutaneous balloon aortic valvuloplasty for congenital valvular aortic stenosis in children / 中华心血管病杂志

<p><b>OBJECTIVE</b>To assess the effect of the balloon valvuloplasty for congenital valvular aortic stenosis (AS) in children.</p><p><b>METHODS</b>A total of twenty one children with AS accepted the treatment of percutaneous balloon aortic valvuloplasty (PBAV). One of them complicated with PDA underwent PDA closure by using Amplatzer occluder in the mean time. PBAV could not be accomplished in 3 cases because the catheter could not be advanced into left ventricle and PBAV was not performed in 2 cases due to the gradient pressures across aortic valves less than 50 mm Hg (1 mm Hg = 0.133 kPa). The procedure was completed in 16 cases. The ratios of balloon/valve were 0.98 +/- 0.04 (0.92 - 1.10).</p><p><b>RESULTS</b>13 cases had more than 50% gradient reduction (81.25%), 2 had 40% - 50% gradient reduction. The follow up period ranged from 3 months to 5 years. The gradient pressures rose to more than 50 mm Hg after follow up in 3 cases and they underwent repeat balloon valvuloplasty procedure or were operated successfully. There was no moderate to severe aortic insufficiency (AI).</p><p><b>CONCLUSION</b>The result of balloon aortic valvuloplasty showed the significant hemodynamic improvement with relative safety in pediatric patients. PBAV provides another choice in comparison with surgery.</p>

Changes in CD69, CD25 and HLA-DR expressions in peripheral blood T cells in Kawasaki disease / 中华儿科杂志

<p><b>OBJECTIVE</b>The study was designed to investigate the changes in CD(69), CD(25) and HLA-DR expressions in peripheral blood T cell in Kawasaki disease (KD).</p><p><b>METHODS</b>The authors detected CD(69), CD(25) and HLA-DR expressions in peripheral blood T cell by using flow cytometry. The patients who met the diagnostic criteria for KD comprised sixteen boys and fifteen girls (4 - 60 months of age; mean, 26 +/- 18 months). All received intravenous gammaglobulin at a dose of 1 g/(kg.d), for 2 days and oral aspirin at a dose of 30 - 50 mg/(kg.d). In case of persistent fever, a repeated dose of intravenous gammaglobulin or I.V. methylprednisolone at a dose of 20 mg/(kg.d) for three daily doses was attempted. The authors tested blood samples from 17 healthy controls consisting of nine boys and eight girls (3 - 84 months of age; mean, 25 +/- 18 months) and the samples from 31 patients.</p><p><b>RESULTS</b>The percentage of peripheral blood CD(3)(+) T lymphocyte was (54.4 +/- 9.0)% in acute stage of KD and (65.0 +/- 7.0)% in healthy controls. There was a significant difference between the two groups (P < 0.001). The values of CD(69)(+) [(11.2 +/- 12.6)%, vs. (0.6 +/- 0.4)%], CD(25)(+) [(9.2 +/- 3.5)% vs. (3.9 +/- 1.8)%] and HLA-DR(+) [(8.3 +/- 5.0)% vs. (4.3 +/- 2.3)%] in KD patients were markedly increased compared to those of the healthy controls. After intravenous gammaglobulin treatment, the percentage of CD(3)(+)CD(69)(+) and CD(3)(+)CD(25)(+) significantly decreased [CD(3)(+)CD(69)(+): (14.0 +/- 13.0)% vs. (1.6 +/- 1.2)%, P < 0.05; CD(3)(+)CD(25)(+): (7.8 +/- 4.1)% vs. (2.0 +/- 0.6)%, P < 0.01]. However, the CD(3)(+) T lymphocytes increased [(50.8 +/- 5.0)% vs. (64.9 +/- 5.5)%, P < 0.01]. There was no significant difference in expression of CD(3)(+) T lymphocyte cell activating markers between coronary artery disease group and normal coronary artery group in KD cases (P > 0.05).</p><p><b>CONCLUSION</b>CD(3)(+) T cell activation in the early and middle stages is involved in the mechanism responsible for cardiovascular injury.</p>

Transcatheter therapy of combined congenital heart diseases in children / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To investigate the methods of interventional catheterization for combined congenital heart disease and to evaluate its efficacy in children.</p><p><b>METHODS</b>From March 1994 to December 2003, 15 cases (6 boys, 9 girls) underwent transcatheter intervention for combined congenital heart diseases. The procedure of transcatheter intervention was as follows: for pulmonary stenosis (PS) and atrial septal defect (ASD) or patent ductus arteriosus (PDA), PBPV first, occlusion of ASD or PDA later; for coarctation of aorta (COA) and PDA, dilation of COA first, occlusion of PDA 4-15 months later; for aortic stenosis (AS) and PDA, PBAV first, occlusion of PDA later; for ventricular septal defect (VSD) and PDA, all occlusions with detachable coils.</p><p><b>RESULT</b>Transcatheter intervention for combined congenital heart diseases was successful in all patients. There was no residual shunt after occlusion immediately apart from 2 cases of PDA which were little residual after occlusion immediately. Follow-up for (3.57 +/-2.61) years, the systolic pressure gradients across pulmonary valve and coarctation were normal by ultrasonic or transcatheter, except AS. There was 3 cases presented postoperative complications: 1 with mechanical haemolysis, 1 with fall off of coil and 1 with arterial embolism, respectively.</p><p><b>CONCLUSION</b>Transcatheter intervention for combined congenital heart diseases could obtain satisfactory results with appropriate indications and procedure manipulations.</p>

Pathophysiology of bleomycin-induced pulmonary hypertension in immature rabbits / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To investigate the evolution of pulmonary hypertension induced by intratracheal bleomycin (BLM) in immature rabbits.</p><p><b>METHODS</b>Immature rabbits were divided into control and BLM groups. Two and four weeks after intratracheal normal saline or BLM, the systolic, diastolic and mean pulmonary artery pressure (PASP, PADP, MPAP) were measured by micro-catheter, the pathological changes and the expression of VEGFmRNA and eNOSmRNA of endothelial cells in pulmonary arteries were evaluated by HE and in situ hybridization.</p><p><b>RESULTS</b>Pulmonary artery pressure was elevated 2 weeks and 4 weeks after intratracheal BLM. Two weeks after treatment PASP was (16.5 +/- 2.9 compared with 25.2 +/- 7.0) mmHg, PADP (8.8 +/- 4.2 compared with 13.1 +/- 3.8) mmHg, MPAP (12.1 +/-4.0 compared with 18.4 +/-4.7) mmHg in control and BLM groups, respectively; meanwhile 4 weeks after treatment, PASP was (16.7 +/-2.3 compared with 23.8 +/-7.1) mmHg, PADP (7.3 +/-1.5 compared with 13.8 +/-6.6) mmHg, MPAP (11.3 +/- 1.9 compared with 17.6 +/- 6.3) mmHg in control and BLM groups, respectively. The thickness of arterial wall increased and the cavity became narrow, and the thickness index (TI) and area index (AI) increased in middle and small pulmonary arteries 2 weeks and 4 weeks after intratracheal BLM. Two weeks after treatment TI was 0.52 +/- 0.16 compared with 0.65 +/- 0.16, AI 0.74+/- 0.17 compared with 0.84 +/- 0.14 in control and BLM groups, respectively; meanwhile 4 weeks after treatment TI was 0.52 +/- 0.11 compared with 0.64 +/- 0.15, AI 0.71 +/- 0.15 compared with 0.85 +/- 0.10 in control and BLM groups. The levels of VEGFmRNA and eNOSmRNA expression in pulmonary arterial endothelial cells decreased 2 weeks and 4 weeks after intratracheal BLM. Two weeks after treatment VEGFmRNA was 0.83 +/- 0.09 compared with 0.45 +/- 0.11, eNOSmRNA 0.79 +/- 0.12 compared with 0.45 +/- 0.12 in control and BLM groups, respectively; meanwhile 4 weeks after VEGFmRNA was 0.81 +/- 0.19 compared with 0.46 +/- 0.15, eNOSmRNA 0.89 +/- 0.14 compared with 0.44 +/- 0.12 in control and BLM groups, respectively.</p><p><b>CONCLUSION</b>Intratracheal bleomycin may induce the pathological changes of pulmonary arteries and decrease the expression of VEGFmRNA and eNOSmRNA in immature rabbits, which results in pulmonary hypertension.</p>

Effect of VEGF gene transfer on the bleomycin-induced pulmonary hypertension in immature rabbits / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To investigate the effect of vascular endothelial growth factor (VEGF) gene transfer on the bleomycin(BLM)-induced pulmonary hypertension in immature rabbits.</p><p><b>METHODS</b>Immature rabbits were divided into 4 groups; control, BLM, liposome and trans-gene groups. The systolic, diastolic and mean pulmonary artery pressure (PASP, PADP, MPAP) were measured by micro-catheter, the pathological changes and the expression of VEGFmRNA and eNOSmRNA of endothelial cells in pulmonary arteries were evaluated by HE stain and in situ hybridization.</p><p><b>RESULT</b>(1) The PAP of BLM and liposome groups was higher than that of control and trans-gene groups. The PASP was 16.5+/-2.9, 25.2+/-7.0, 24.4+/-6.0 and 18.3+/-2.7 mmHg; the PADP was 8.8+/-4.2, 13.1+/-3.8, 13.7+/-4.6 and 10.2+/-2.6 mmHg; the MPAP was 12.1+/-4.0, 18.4+/-4.7, 18.4+/-5.1 and 14.1+/-2.5 mmHg in control, BLM, liposome and trans-gene groups respectively. (2) The thickness of wall increased and the cavity became narrow, and the thickness index (TI) and area index (AI) increased in middle and small pulmonary arteries of BLM and liposome groups. The TI was 0.52+/-0.16, 0.65+/-0.16, 0.63+/-0.11 and 0.55+/-0.13; and the AI was 0.74+/-0.17, 0.84+/-0.14, 0.85+/-0.08 and 0.79+/-0.12 in control, BLM, liposome and trans-gene groups,respectively. (3) The level of VEGFmRNA and eNOSmRNA expression in pulmonary arterial endothelial cells decreased in BLM and liposome groups. The level of VEGFmRNA and eNOSmRNA expression in trans-gene group was higher than that in BLM and liposome groups, but lower than that in control group. VEGFmRNA was 0.83+/-0.09, 0.45+/-0.11, 0.45+/-0.13 and 0.65+/-0.18; eNOSmRNA was 0.79+/-0.12, 0.45+/-0.12, 0.50+/-0.14 and 0.56+/-0.08 in control, BLM, liposome and trans-gene groups, respectively.</p><p><b>CONCLUSION</b>VEGF gene transfer in immature rabbits with BLM-induced pulmonary hypertension could attenuate the increasing of PAP and wall thickness in middle and small pulmonary arteries, and increase the level of VEGFmRNA and eNOSmRNA expression in pulmonary arterial endothelial cells.</p>