Objective To evaluate the diastolic function and relationship between diastolic function and early diastolic synchrony in patients with dilated cardiomyopathy (DCM) by real-time three-dimensional echocardiography (RT-3DE) volume-time curves (VTC) after cardiac resynchronization therapy (CRT).Methods Thirty-nine patients with DCM were enrolled by RT-3DE VTC before and 1 week,6 months,12 months after CRT,draw the left ventricular (LV) 16,12,6 segments LV diastolic early volume standard deviation of the time (Tedv-SD),and with the R-R interval normalized as early diastolic unsynchronized index (DDI) ;draw end-systolic volume (LVESV),LV end-diastolic volume (LVEDV),LV ejection fraction (LVEF) ;and calculate the diastolic peak filling rate(PFR),the ratio of early diastolic volume and enddiastolic volume(EDVearly/EDV).Results LVEDV,LVESV had no significant improvement.Compared with the before and 1 week after CRT,but the improvement was statistically significant after 6 months and 12 months (P ＜0.05,P ＜0.01); LVEF after 1 week,6 months and 12 months were statistically significant (P ＜0.05) ;Compared with before,PFR after 6 months was significantly increased (P ＜0.05),EDVearly/EDV at 12 months after CRT was significantly reduced (P ＜ 0.01); There was a significantly shortened in each segment (Tedv-SD)/R-R 1 week after CRT (P ＜0.01),but the parameters had no obvious improvement later.Correlation analysis:△ DDI and △ EDVearly/EDV reduction was significant positive correlation (r =0.52,P ＜0.01),△DDI and PFR has negative correlation (r =-0.40,P ＜ 0.05),△ EDVearly/EDV and △PFR also had a good relationship (r =-0.56,P ＜0.01).Conclusions The LV synchrony and diastolic function were improved after CRT in patients with DCM; PFR,EDVearly/EDV can be used as evaluation of left ventricular diastolic function effectively targets.

Objective To evaluate the clinical value of real-time 3 dimensional echocardiography (RT-3DE) for measurement of right ventricular function in the repaired tetralogy of Fallot (TOF) compared to cine magnetic resonance imaging (c-MRI).Methods Thirty-seven randomly selected patients with repaired TOF from Jan.2000 to Jun.2007 in Children's Hospital,Fudan University underwent both RT-3DE and c-MRI for the data of right ventricular enddiastolic volume (RVEDV),right ventricular end-systolic volume (RVESV),right ventricular ejection fraction (RVEF),which were compared to evaluate the reproducibility and correlation of two ways on right ventricular function.Results All the patients completed both RT-3DE and c-MRI on the same day.The correlation for RVEDV,RVESV,RVEF measured by 2 methods was high (r =0.933,0.943,0.911,P =0.000,0.000,0.105),and it showed that consistency existed in RVEF,as difference was only (1.2 ± 4.5) ％.Comparison with c-MRI,RVEDV and RVESV measured by RT-3DE was underestimated,in which difference was (-17.4 ± 16.9) mL,(-9.3 ± 10.5) mL.Conclusions RVEF with RT-3DE can be assessed with acceptable accuracy,but it is not so good for RVEDV and RVESV.Further study needs to be performed to make sure the value of RT-3DE on the assessment of right ventricular function in repaired TOF.

0.05 ) between the left ventricular stroke volume with RT-3DE ( 24.58 ? 3.44 )ml,the right ventricular stroke volume with RT-3DE ( 23.58 ? 3.18 )ml and those values measured with Swan-Ganz pulmonary thermodilution method ( 25.64 ? 3.18 )ml.Furthermore,there was well correlation between the left ventricular stroke volume and the right ventricular stroke volume with RT-3DE (r= 0.93 ).They were also well correlated with those values with Swan-Ganz pulmonary thermodilution method (r= 0.85 ,r= 0.92 ).However,there was significant difference (P

Objective:To analyze the distribution of clopidogrel metabolism-related gene variability in Kawasaki disease (KD) children with coronary artery lesions (CAL) across different age groups and the impact of genetic variability on the efficacy of clopidogrel antiplatelet therapy.Methods:A retrospective cohort study was conducted. Clinical data were collected from 46 KD children with CAL who were hospitalized in the Cardiovascular Center of Children′s Hospital of Fudan University between January 2021 and August 2022 and were treated with clopidogrel, including gender, age, body mass index, course of KD, CAL severity grade, and baseline platelet count. According to their age, the children were divided into ≥2-year-old group and <2-year-old group. Their platelet responsiveness was assessed by adenosine diphosphate-induced platelet inhibition rate (ADPi) calculated via thromboelastography, and children were categorized into high on-treatment platelet reactivity (HTPR) and normal on-treatment platelet reactivity (NTPR) groups. Genotypes of CYP2C19, PON1 and ABCB1 were detected. The t test, one-way analysis of variance and Chi-square test were used for intergroup comparison. Results:Among the 46 KD children with CAL, 34 were male and 12 were female; 37 were ≥2-year-old and 9 were <2-year-old; 25 cases were in the HTPR group and 21 cases were in the NTPR group, with 19 HTPR and 18 NTPR in the ≥2-year-old group, and 6 HTPR and 3 NTPR in the <2-year-old group. Genetic analysis showed that 92 alleles among the 46 children, with frequencies of CYP2C19*1, CYP2C19*2, CYP2C19*3, CYP2C19*17, PON1 192Q, PON1 192R, ABCB1 3435C, ABCB1 3435T at 59% (54/92), 32% (29/92), 9% (8/92), 1% (1/92), 36% (36/92), 64% (59/92), 63% (58/92) and 37% (34/92), respectively. Analysis of the impact of genotype on ADPi revealed that in children aged ≥2 years, those with CYP2C19*1/*3 genotype had significantly lower ADPi than those with CYP2C19*1/*1 genotype ((34±15)% vs. (61±29)%, t=2.18, P=0.036). There were also no significant difference in ADPi among children with PON1 192Q homozygous, PON1 192R heterozygote and PON1 192R homozygous genotypes ((40±22)% vs. (52±33)% vs. (65±27)%, F=2.17, P=0.130), or among those with ABCB1 3435C homozygous, ABCB1 3435T heterozygote and ABCB1 3435T homozygous genotypes ((55±34)% vs. (60±27)% vs. (49±24)%, F=0.33, P=0.719). In <2-year-old group, there were no significant differences in ADPi across CYP2C19*1/*1, CYP2C19*1/*2 and CYP2C19*2*2 genotypes ((40±20)% vs. (53±37)% vs. (34±16)%, F=0.37, P>0.05). There were no significant differences in ADPi across CYP2C19*1/*1 and CYP2C19*1/*3 genotypes ((44±27)% vs. (42±20)%, t=0.08, P>0.05). There were no significant differences in ADPi across PON1 192Q homozygous, PON1 192R heterozygote and PON1 192R homozygous genotypes (45% vs. (55±27)% vs. (24±5)%, F=1.83, P>0.05). There were no significant differences in ADPi across ABCB1 3435C homozygous, ABCB1 3435T heterozygote and ABCB1 3435T homozygous genotypes ((36±16)% vs. (50±35)% vs. 45%, F=0.29, P>0.05). The risk analysis of HTPR in different genotypes revealed that in children aged ≥2 years, carrying at least 1 or 2 loss-of-function alleles of CYP2C19 was a risk factor for HTPR ( OR=4.69, 10.00, 95% CI 1.11-19.83, 0.84-119.32, P=0.033, 0.046, respectively), and PON1 192R homozygosity and carrying at least one PON1 192R allele were protective factors against HTPR ( OR=0.08, 0.13, 95% CI 0.01-0.86, 0.01-1.19, P=0.019, 0.043, respectively). Conclusion:KD children aged ≥2 years carrying CYP2C19 loss-of-function alleles and PON1 192Q are more likely to develop HTPR.

OBJECTIVETo evaluate the efficacy and safety of evaluation,treatment and follow-up of Kawasaki coronary artery disease based on the clinical severity classification.

METHODThis study evaluated 52 patients admitted to the Children's Hospital of Fudan University between July 2005 and December 2013 who were diagnosed with Kawasaki Disease with coronary artery disease.Inclusion criteria were a disease course of more than two months, initial echocardiography showing severity of grade IV and above, and confirmation of disease severity by angiography. Of those studies, 44 were male and eight were female, aged 6 to 142 (average 41) months. Treatment was planned according to protocols in "Suggestions for Management of Kawasaki Coronary Artery Disease" with follow-up. Those patients with grade IV and above confirmed by angiogram were given oral low-dose asprin and warfarin, and those with grade Vb were given coronary artery bypass grafting (CABG) after comprehensive evaluation. Analysis was carried out for diagnosis, treatment, complications, and results of follow-up.

RESULT(1) Satisfied images were shown by the angiography of all 52 cases. Forty five patients (86%) had giant aneurysm or multiple aneurysms, with thrombosis found in 10 of 45 patients (22%). Coronary artery lesions occurred in 138 coronary branches, and more common in left anterior descending branch (47 branches, with incidence 34%) and right coronary artery (48 branches, with incidence 35%). There were no complications during or after angiography. (2) After angiography, 49 patients remained at grade IV or above, and three improved to grade III. Ultimately, clinical severities of coronary artery disease included three patients at grade III, 31 patients at grade IV, nine patients at grade Va, and nine patients at grade Vb. (3) Thirty-eight patients were properly using aspirin and warfarin, and two patients with severely elevated international normalized ratio (INR) levels presented with knee joint and gastric hemorrhage, both of which were treated successfully.Patients with INR levels between 1.5 and 2.5 did not show signs of hemorrhage. (4) In follow-up visits between 6 months and 8 years, one patient had representation of thrombosis on angiography, but did not lead to coronary stenosis; four patients were improved from grade IV to either grade III or II. The remaining showed no new thrombotic formation or stenosis. (5) Of the nine grade Vb patients, five underwent coronary artery bypass grafting. The youngest of these patients, a 22 months old girl, died intraoperatively. The remaining four recovered postoperatively and were followed up for 8 to 90 months. One patient had a preoperative left ventricular ejection fraction (LVEF) of only 32.8%, with LVEF remaining abnormal post-CABG, between 35% and 44%. The remaining three patients had normal heart size, cardiac function, and electrocardiogram.Of the other four grade Vb patients, two were contraindicated for surgery due to severe heart failure and loss of myocardial activity. Two other cases are being followed up closely due to their young age of 9 months.

CONCLUSIONCoronary angiography is safe and efficacious in children, and even in infants.It is the current gold standard tool for grading Kawasaki coronary artery disease. Proper anticoagulation therapy can markedly decrease the incidence of coronary artery occlusion in patients with Kawasaki coronary artery disease. Safe ranges of corrected INR should be between 1.5 and 2.5 after taking warfarin. CABG is an effective treatment for severe coronary artery disease with myocardial ischemia.

Aspirin ; therapeutic use ; Child ; Child, Preschool ; Coronary Angiography ; Coronary Artery Bypass ; Coronary Artery Disease ; complications ; therapy ; Disease Management ; Echocardiography ; Electrocardiography ; Female ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; therapy ; Treatment Outcome ; Ventricular Function, Left ; Warfarin ; therapeutic use