Retrospective analysis of infliximab in the treatment of Kawasaki disease / 中华儿科杂志

Objective: To investigate the efficacy and safety of infliximab (IFX) therapy for children with Kawasaki disease. Methods: Sixty-eight children with Kawasaki disease who received IFX therapy in Children's Hospital of Fudan University from January 2014 to April 2021 were enrolled. The indications for IFX administration, changes in laboratory parameters before and after IFX administration, response rate, drug adverse events and complications and outcomes of coronary artery aneurysms (CAA) were retrospectively analyzed. Comparisons between groups were performed with unpaired Student t test or Mann-Whitney U test or chi-square test. Results: Among 68 children with Kawasaki disease, 52 (76%) were males and 16 (24%) were females. The age of onset was 2.1 (0.5, 3.8) years. IFX was administered to: (1) 35 children (51%) with persistent fever who did not respond to intravenous immunoglobulin (IVIG) or steroids, 28 of the 35 children (80%) developed CAA before IFX therapy; (2) 32 children (47%) with continuous progression of CAA; (3) 1 child with persistent arthritis. In all cases, IFX was administered as an additional treatment (the time from the onset of illness to IFX therapy was 21 (15, 30) days) which consisted of second line therapy in 20 (29%), third line therapy in 20 (29%), and fourth (or more) line therapy in 28 (41%). C-reactive protein (8 (4, 15) vs. 16 (8, 43) mg/L, Z=-3.38, P=0.001), serum amyloid protein A (17 (10, 42) vs. 88 (11, 327) mg/L, Z=-2.36, P=0.018) and the percentage of neutrophils (0.39±0.20 vs. 0.49±0.21, t=2.63, P=0.010) decreased significantly after IFX administration. Fourteen children (21%) did not respond to IFX and received additional therapies mainly including steroids and cyclophosphamide. There was no significant difference in gender, age at IFX administration, time from the onset of illness to IFX administration, the maximum coronary Z value before IFX administration, and the incidence of systemic aneurysms between IFX-sensitive group and IFX-resistant group (all P>0.05). Infections occurred in 11 cases (16%) after IFX administration, including respiratory tract, digestive tract, urinary tract, skin and oral infections. One case had Calmette-Guérin bacillus-related adverse reactions 2 months after IFX administration. All of these adverse events were cured successfully. One child died of CAA rupture, 6 children were lost to follow up, the remaining 61 children were followed up for 6 (4, 15) months. No CAA occurred in 7 children before and after IFX treatment, while CAA occurred in 54 children before IFX treatment. CAA regressed in 23 (43%) children at the last follow-up, and the diameter of coronary artery recovered to normal in 10 children. Conclusion: IFX is an effective and safe therapeutic choice for children with Kawasaki disease who are refractory to IVIG or steroids therapy or with continuous progression of CAA.

Surgical Outcomes of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: An Echocardiography Follow-up / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography.</p><p><b>METHODS</b>From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up.</p><p><b>RESULTS</b>The mortality rate after the operation was 11.5%. Before repair, twenty patients (76.9%) presented with left ventricular (LV) dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation (MR) was present in all patients. Two patients (7.7%), both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months (range: 0.5-36.0 months). The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients. All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients (26.1%) showed supravalvar pulmonary stenosis and seven patients (30.4%) showed right pulmonary stenosis during follow-up.</p><p><b>CONCLUSIONS</b>Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.</p>

Diagnosis of noncompaction of the ventricular myocardium by echocardiography / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To evaluate the value of echocardiography in the diagnosis of noncompaction of the ventricular myocardium (NCVM) and to elucidate the echocardiographic characteristics of NCVM.</p><p><b>METHODS</b>This study included 53 patients (28 boys and 25 girls), with an age for initial diagnosis of 15 days to 18 years, who were diagnosed with NCVM by echocardiography between May 2006 and May 2015. Transthoracic two-dimensional echocardiography and color Doppler were performed for qualitative diagnosis, and the end-diastolic non-compacted layer/compacted layer (N/C) ratio measured in the parasternal ventricular short-axis sectional view was selected as the criterion for quantitative diagnosis.</p><p><b>RESULTS</b>The excessively prominent ventricular trabeculae and deep inter-trabecular recesses were all seen in 53 cases, and the blood flow in the cardiac chambers was connected to the inter-trabecular recesses. The areas involved in NCVM were mainly the apex (100%) and the middle segment of the left ventricular lateral wall (98%), followed by the middle segment of the left ventricular posterior wall (49%) and the middle segment of the left ventricular inferior wall (42%). In 53 children with NCVM, the N/C ratio was 4.3±1.9 (2.1-10.0). Cardiac insufficiency was found in 83% (44/53) of the children with NCVM, and the left ventricular ejection fraction for these children was (43±9)%.</p><p><b>CONCLUSIONS</b>Echocardiography can be used in the qualitative and quantitative diagnosis of NCVM and in the evaluation of cardiac function. The apex and the middle segment of the left ventricular lateral wall are often involved in NCVM, accompanied by decrease in the left ventricular ejection fraction.</p>

Assessment of cardiac output and volume load by transpulmonary thermodilution technique in immature pigs / 中国医学科学院学报

<p><b>OBJECTIVE</b>To assess the accuracy of cardiac output (CO) measured by transpulmonary thermodilution technique (TPTD)and explore the validity of intrathoracic blood volume index (ITBVI) for assessment of circulatory volume status.</p><p><b>METHODS</b>Ten immature pigs with a mean weight of (20.6±1.9)kg were studied during the conditions including normovolemia, hypervolemia, and hypovolemia. Simultaneous CO was measured in each condition using pulmonary artery thermodilution (PATD) method and TPTD. More specifically, CO (COPA) was determined with PATD, while CO (COTP) and ITBVI were determined with TPTD. All measurements were repeated 3 times. Central venous pressure (CVP) and heart rate were measured at the same time. The potential correlations of CVP and ITBVI with cardiac index (CI) and stroke volume index (SVI) in each blood volume status were analyzed.</p><p><b>RESULTS</b>A total of 90 simultaneous measurements of COPA and COTP in 3 different blood volume conditions were made. The correlation coefficient between the two measurements was 0.977 (P<0.001) and the mean difference was (0.25±0.26)L/min (95%CI:0.20-0.30 L/min, P<0.001). The coefficient of variation of COTP was 3.7%, while COPA was 5.4%. Compared with those in normovolemia, CVP and ITBVI in hypervolemia significantly increased (P=0.002, 0.019), ITBVI in hypovolemia decreased significantly (P<0.001), and CVP in hypovolemia decreased insignificantly (P=0.05). Correlation analysis revealed a significant correlation between ITBVI with CI and SVI in normovolemia (r=0.741, P=0.014; r=0.885, P=0.001). In contrast, correlations between CVP with CI and SVI were poor.</p><p><b>CONCLUSIONS</b>TPTD can accurately and precisely measure CO in different blood volume conditions. ITBVI measured by TPTD has better validity for the assessment of circulatory volume status than CVP.</p>

A comparative study of hemodynamics after cardiac surgery with venous-arterial modified ultrafiltration and aterial-venous modified ultrafiltration in infants / 中华胸心血管外科杂志

Objective Evaluate the effects of venous-arterial modified ultrafiltration on hemodynamics compared to arterial-venous in children undergoing cardiopulmonary bypass (CPB) for repair of congenital heart defects. Methods Forty patients underwent MUF randomly divided into two groups,group V-A MUF (n =20) and group A-V MUF (n =20) for 10 min after CPB. They were studied before CPB, after CPB, 10 min after CPB, and 30 min after CPB. Haemodynamic data including heart rate, blood pressure, central venous pressure and hematocrit were recorded. Transoesophaegeal echocardiography determined left ventricular posterior wall thickness in end-systole ( LVPWs) and end-diastole (LVPWd) , end diastolic volume (EDV) , end systolic volume (ESV) and ejection fraction (EF) were measured and compared in two groups. Results Patients in V-A MUF maintained better systolic arterial blood pressure at 10 min and 30 min compared with 0 min values after CPB. A significant decrease in EF were observed in both groups immediately after CPB ( P < 0.05 ). Significant increase in EF was observed at 10 min (60% ) and 30 min (46% ) after CPB compared with 0 min value after bypass in V-A MUF (P <0.001 ). In A-V MUF, no such increase in EF was observed. EF were significantly higher at 10 min and 30 min in V-A MUF as compared with A-V MUF (P < 0. 001). There was also significant improvement in posterior wall thickness in V-A MUF (P <0.05). Haematocrit values were not different in duration of postoperative between two groups. Conclusion Veno-arterial modified ultrafiltration is a safe and effective method of improving hemodynamics in children following cardiac surgery.

Medicinal Treatment of Pulmonary Hypertension / 实用儿科临床杂志

Pulmonary hypertension(PH) is a clinical hemodynamic syndrome characterized by elevation of pulmonary artery pressure and pulmonary vascular resistance,which was presumed mainly due to pulmonary vasoconstriction,remodeling of the pulmonary vessel wall,and thrombosis in situ.Endothelin,nitric oxide,and prostacyclin pathway were three major pathways involved in the pathogenesis.Current therapies interfered with these 3 pathways in combination with conventional measures have prolonged length of life of patients and improved their quality of life significantly.

Methodological study on real-time three-dimensional echo-cardiography and its application in the diagnosis of complex congenital heart disease / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Real-time three-dimensional echocardiography (RT-3DE) has made revolutionized improvements of cardiac imaging during the past few years. However, there is no standard examination method for RT-3DE so far. This study aimed to establish the diagnostic method of RT-3DE and evaluate its application in the diagnosis of complex congenital heart diseases (CHD).</p><p><b>METHODS</b>Fifty patients with complex CHD were examined by RT-3DE with modes of Live 3DE and Full Volume. A series of novel volumetric views combined with Van Praagh sequential segmental approach were introduced to reveal the pathological morphology of the hearts, which were compared with the findings of two-dimensional echocardiography (2DE), angiography and cardiac surgery.</p><p><b>RESULTS</b>In 50 patients, 190 image acquisitions of Full Volume were performed at several acoustic windows including subcostal, apical and parasternal regions. Among them, 94.2% (179/190) of image acquisitions were successful. Most sectional volumetric views could be clearly displayed in 92.6% of the successful image acquisitions. However, sectional volumetric views could not be clearly displayed in 7.4%, which was mainly due to poor perspective conditions of examination location, improper instrument multi-parameter setting and insufficient information of whole heart captured in Full Volume acquisitions. As compared with surgical findings and angiography, RT-3DE made correction to the diagnoses in 2 cases including 1 with corrected transposition of the great arteries and the other with single atrium and mitral cleft. The diagnoses initially made by 2DE for these 2 patients were double outlet right ventricle with transposition of the great arteries and complete atrio-ventricular septal defect.</p><p><b>CONCLUSIONS</b>RT-3DE can clearly display the pathological morphology of complex CHD by a series of novel volumetric views combined with sequential segmental approach through providing more spatial informative cardiovascular structures, which provides a practical method for RT-3DE diagnosis.</p>