Follow-up study of fetal cardiac birth defects after prenatal diagnosis and graded counseling / 中华妇产科杂志

Objective:To explore accurate prenatal diagnosis, full-coverage graded counseling and follow-up for the fetus with cardiac birth defects (CBD).Methods:CBD fetus diagnosed prenatal by echocardiography from January 2018 to December 2020 in Guangdong Provincial People's Hospital were enrolled. Fetal CBD was graded (Ⅰ-Ⅵ) according to prognosis and possible operation time after birth, and the classification criteria and common diseases included were proposed. After the prenatal grading counseling, the outcome of the fetus was followed-up. The induced labor rate, live birth rate, prenatal and postnatal ultrasound diagnosis coincidence rate and other indicators were calculated. The disease composition ratio, prognosis of fetus with different grades and the outcome of integrated treatment were analyzed.Results:The detection rate of fetal CBD was up to 16.2% (1 971/12 188), 30 cases of which were excluded. A total of 1 941 cases were included in this study, including 196 cases (10.1%) of gradeⅠ, 433 cases (22.3%) of gradeⅡ, 615 cases (31.7%) of grade Ⅲ, 261 cases (13.4%) of grade Ⅳ, 388 cases (20.0%) of gradeⅤ, 48 cases (2.5%) of grade Ⅵ. Grade Ⅱ and gradeⅢ (the operation time was within 1 year after birth) accounted for 54.0% (1 048/1 941). The distribution of some diseases in different grades had obvious proportion advantage, which was representative. Among 1 747 CBD fetus, 736 cases (induced labor rate 42.1%) chose to terminate pregnancy due to CBD. Of the 1 010 live births, 975 cases (96.5%) had the same prenatal and postnatal diagnosis, 3 cases were missed diagnosis and 32 cases were misdiagnosed. The diagnostic accuracy of live births with severe and complex congenital heart disease was 383 out of 389 (98.5%). A total of 258 cases have received surgery or intervention. The age at the time of surgery or intervention was different among grades（ χ2 =47.3， P<0.001）. With the improvement of prognosis from gradeⅠ to Ⅴ, the live birth rate increased and the induced labor rate decreased accordingly; the difference between grades was significant（ χ2 =623.6， P<0.001）. Conclusions:Prenatal diagnosis and graded counseling is important in the integrated model. Fetal CBD grading could refine post-natal treatment strategies, guide delivery decisions and become an evaluation standard.

Clinical outcomes and follow-up results of 300 cases of transposition of great arteries intervened by the arterial switch operation / 中华实用儿科临床杂志

Objective:To summarize and analyze the efficacy, experience and follow-up results of 300 cases of transposition of the great arteries (TGA) intervened by arterial switch operation.Methods:It was a retrospective, single-center study involving 300 TGA patients intervened by arterial switch operation between January 2010 and December 2017 in Guangdong Provincial People′s Hospital.Their clinical data were retrospectively analyzed.There were 236 male patients and 64 females.Among them, 128 cases (42.7%) were TGA with ventricular septal defect (TGA/VSD), and 172 cases (57.3%) were TGA with intact ventricular septal defect (TGA/IVS). The mean age and weight at operation were (23.8±39.2) cases days, and (3.5±0.8) kg, respectively.There were 193 cases (64.3%) with usual coronary artery patterns, and 107 cases (35.7%) with unusual coronary artery patterns.Among the 107 cases with unusual coronary artery patterns, 21 cases (7.0%) were involved with the intramural coronary artery, and 17 (5.7%) presented the single-ostium coronary pattern.Non normal distribution data were used the Mann- Whitney U test.Categorical measures were compared by Chi- square test or Fisher′ s exact test.Survival probability and freedom from events were calculated by the Kaplan-Meier method, and difference in survival probability by the Log Rank test. Results:All patients were successfully intervened by arterial switch operation, 73.3% of patients with TGA/IVS underwent the surgery within 3 weeks after birth, and 85.9% of patients with TGA/VSD underwent surgery within 3 months.The mean cardiopulmonary bypass time and aortic occlusion time were (193±68) min, and (122±39) min, respectively.Twenty-five patients (8.3%) died in hospital.Thirty cases had low cardiac output syndrome, 1 implanted with a permanent pacemaker due to complete atrioventricular block.A total of 254 patients were followed up for 1 month to 10 years.Three patients with single-ostium coronary pattern died at the follow-up period.The 5-year and 10-year survival rates were both 90.7%.During the follow-up, 49 cases (49/254 cases, 19.3%) had pulmonary artery stenosis, 66 cases (66/254 cases, 26.0%) had aortic valve regurgitation, 47 cases (47/254 cases, 18.5%) had pulmonary valve regurgitation, and 4 (4/254 cases, 1.6%) had aortic anastomotic stenosis.Among the 21 patients (21/254 cases, 8.3%) requiring reintervention, 17 patients (17/254 cases, 6.7%) underwent a total of 18 reinterventions, including 12 interventions of pulmonary artery plasty, 4 of percutaneous balloon pulmonary valvuloplasty, 1 of aortic reconstruction at anastomosis and 1 of pacemaker exchange due to battery exhaustion.Conclusions:Arterial switch operation is the optimal treatment for TGA.The long-term follow-up results of arterial switch operation are satisfactory in TGA children, with a low risk of long-term reoperation.

Prenatal diagnosis and intrauterine and extrauterine management of a fetus with both critical pulmonary stenosis and hydronephrosis: a case report / 中华围产医学杂志

We report the management of a fetus diagnosed with critical pulmonary stenosis, right ventricular hypoplasia, severe tricuspid regurgitation and severe hydronephrosis. After echocardiography and multidisciplinary team consultation, fetal pulmonary valvuloplasty was performed at 30 +3 weeks of gestation, to facilitate the development of the right ventricle and tricuspid valve. Fetal transdermal renal puncture performed at 31 weeks of gestation showed that the hydronephrotic fluid was urine. The mother gave birth vaginally at 37 +3 weeks. Prostacyclin was given to the baby to keep the ductus arteriosus open and maintain oxygen saturation above 80%. Nephrostomy was performed on the 6th day after birth for improvement of the hydronephrosis and renal function. Biventricular correction (percutaneous balloon pulmonary valvuloplasty) and left dismembered pyeloureteroplasty were successfully performed on 16 and 37 d after birth, respectively. Ultrasound follow-ups showed the structure and function of both heart and kidney recovered. We summarized the characteristics and management of fetal critical pulmonary stenosis complicated by severe hydronephrosis in this case, aiming to enhance experiences for appropriate treatment of similar cases in the future.

Research on relevant factors and the value on prenatal ultrasound diagnosis for coarctation of the aorta / 中华超声影像学杂志

To improve the accuracy of prenatal diagnosis for coarctation of the aorta ( CoA ) by comparatively analyzing the relevant factors and their application value . Methods Cases of disproportion of ventricles and great vessels were selected between January 2011 and July 2018 . Only liveborn fetuses with complete postnatal follow‐up were included in the study . One hundred and twelve cases were retrieved and analyzed . According to the postnatal ultrasound and CT results ,the patients were divided into gruop A and goup B . Different fetal echocardiography parameters and features were selected to evaluate the diagnostic value . Logistic regression analysis was used to select the best predictors of CoA and optimal cut‐offs for these parameters were identified by ROC analysis . Results One hundred and thirty eight fetuses were suspected to be CoA ,and 112 of them were born . T hey included 59 cases ( 52 .9% ) with CoA ( Group A ) and 53 cases ( 47 .3% ) without CoA ( Group B ) . T here were statistically significant differences between the two groups in gestational age ,Z‐score of diameter of left ventricle ,ascending aorta ( AAO ) and aortic arch isthmus ,main pulmonary artery ( M PA )/AAO diameters ratio ,and arterial duct/isthmus diameters ratio . T he parameters most predictive of postnatal CoA selected by logistic regression and the cut‐off values identified by ROC analysis were :gestational age at first diagnosis ≤34 .5 weeks ,Z‐score of diameter of left ventricle ＜ －1 .8 ,Z‐score of diameter of isthmus ＜ －2 .7 ,M PA/AAO diameters ratio＞1 .6 . AUC yielded by these parameters in combination was 0 .94 ( 95% CI :0 .89 －0 .99) . T he study group were divided into two subgroups by gestational age at first diagnosis .T he parameters and cut‐off values in subgroups were selected by the same analysis as above :Group Ⅰ ( ≤ 34 .5 weeks ) ,Z‐score of diameter of right ventricle ＞2 .1 ,M PA/AAO diameters ratio＞1 .6 ; Group Ⅱ( ＞34 .5 weeks) ,M PA/AAO diameters ratio ＞ 1 .7 ,arterial duct/isthmus diameters ratio ＞ 2 .3 . Meanw hile ,some ultrasound features ( including aortic arch hypoplasia ,retrograde blood flow of aortic isthmus ) should also be considered to improve diagnostic accuracy . Conclusions Combined with the use of different ultrasound parameters and features at different gestational weeks can improve the accuracy of the prenatal diagnosis of CoA in the right heart dominant fetus .

Prenatal interventional therapy in two cases with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objectives@#Two cases who underwent fetal pulmonary valvuloplasty (FPV) for pulmonary atresia with intact ventricular septum (PA-IVS) or critical pulmonary stenosis with intact ventricular septum (CPS-IVS) successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus.@*Methods@#One case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Parameters of right ventricle development and hemodynamics from echocardiography included tricuspid/mitral annulus (TV/MV), right ventricle/left ventricle long-axis (RV/LV), pulmonary/aortic annulus (PV/AV), tricuspid inflow duration/cardiac cycle, degree of tricuspid regurgitation (TR), blood flow direction of arterial duct and ductus venosus. Multidisciplinary team including the maternal-fetal cardiology, pediatric cardiology, cardiac surgery, obstetrics, neonatology and anesthesiology was summoned to discuss the indications and timing of PFV. Two cases underwent ultrasound-guiding trans-abdominal PFV at the 28 weeks of gestational age. Echocardiography was performed to observe the opening and closing of the pulmonary valve, and to evaluate the development of right ventricle and improvement in hemodynamics every 2-4 weeks until delivery.@*Results@#From the technical perspective, pulmonary balloon valvuloplasty was successfully performed in these two cases. The opening of pulmonary valve improved in these two cases at 2-4 weeks after FPV. However, an obvious restenosis was detected in the first case at 5-8 weeks after FPV. In the first case, the echocardiography parameters including TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle increased from 0.56, 0.42, 0.85,0.26 to 0.59, 0.51, 0.87, 0.32 at 5-8 weeks after FPV, respectively. However, the direction of blood flow through the arterial duct was still reverse. In the second case, TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle ratio increased from 0.70, 0.63, 0.91,0.35 to 0.80, 0.80, 0.97, 0.42 at 5-8 weeks after FPV, respectively. The direction of blood flow through the arterial duct changed to bidirectional. Both fetuses were born alive. The first case underwent pulmonary valve commissurotomy and modified Blalock-Taussig shunt on the 8th day after delivery and received follow-up for 6 months. The strategy for the next-step therapy was still pending. The second case underwent transcutaneous pulmonary balloon valvuloplasty on the 19th day after delivery and received follow-up for 3 months. The opening of pulmonary valve improved obviously and the cardiac function was normal in the second case.@*Conclusions@#FPV is safe and effective for fetus during the second and third trimester of pregnancy, and FPV is beneficial for the development of fetal ventricle, valve and large artery. In addition, FPV may help to avoid the postnatal surgery for isolated single ventricle, improve fetal heart failure and prevent fetal death.

Anomalous origin of the left coronary artery from the pulmonary artery in infants: clinical features and the perioperative treatment strategies / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).</p><p><b>METHOD</b>Data of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed. The patients' cardiac function was evaluated with echocardiography during follow-up.</p><p><b>RESULT</b>Most patients presented with symptoms of heart failure, such as tachypnea, diaphoresis, poor feeding, failure to thrive etc. Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients, endocardial hyperplasia in 5 patients, dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients. The left ventricular ejection fraction (LVEF) was (45.5±13.9)% (25%-77%). The left ventricular fractional shortening (LVFS) was (22.0±7.3)% (12%-38%). Twenty one patients underwent cardiovascular CT scan. Left coronary artery originated from left posterior sinus in 9 patients, from right posterior sinus in 1 patient, from lower main pulmonary artery in 5 patients, from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital, only 1 case who underwent angiography was diagnosed correctly. Two patients were misdiagnosed in 8 patients first-presented in our hospital. Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan. The preoperative therapies included using inotropic agents, diuretics and vasodilators according to cardiac function. Two patients underwent left coronary artery orifice ligation. Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system. Patients of NYHA IV with moderate mitral regurgitation (MR) and NYHA III with severe MR underwent mitral annuloplasty. If LVEF was less than 30% after weaning from cardiopulmonary bypass, blood pressure could not be maintained with medication, or lactates increased progressively, extracorporeal membrane oxygenation (ECMO) was demanded. Two patients showed low cardiac output syndrome immediately after surgical procedures, died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries, duration of ventilation was 7-500 hours, 11 of them were supported with ventilator for less than 60 hours. The mean length of hospital stay was (23.4±13.9) d (8-65 d). The follow-up duration ranged from 1-91 months (median 28.5 months). One case was lost to follow up. The patient died from infection 3 months after discharge. The cardiac functions of the remaining 22 patients were improved. The size of left ventricle of 14 patients recovered to normal. LVEF increased to the normal level in 20 cases. No patient underwent redo procedure.</p><p><b>CONCLUSION</b>The accurate diagnosis can be made based on history, electrocardiogram, echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants. Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.</p>

Analysis of complications during and post to interventional therapy of common congenital heart disease in children / 临床儿科杂志

Objective To analyze the incidence of complications during and after interventional therapy for common con-genital heart disease (CHD) in children. Methods From January 2011 to December 2013, interventional therapy of common congenital heart disease which include ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA) and pulmonary valve stenosis (PS) were performed in 2356 patients. Among them, 159 patients who developed complications during and post to interventional therapy were retrospectively analyzed. Results The overall complication rate was 6.75%(159/2356) (11.40% post VSD occlusion, 7.50% post ASD occlusion, 3.09% post PDA occlusion, 1.63% post percutaneous balloon pulmonary valvuloplasty (PBPV) ).The rate of arrhythmia was 4.41%(102/2356). The severe complication rate was 2.71%(64/2356) (3.62%post VSD occlusion, 2.21%post ASD occlusion, 2.53%post PDA occlusion, 1.63%post PBPV). The intraoperative severe complication rate was 0.51%(12/2356);the early severe complication rate was 1.99%(47/2356);the late severe complication rate was 0.21%(5/2356). Interventional therapy rate was 0.13%(3/2356); cardiovascular surgery rate was 0.64%(15/2356);conservative treatment rate was 1.95%(46/2356). The mortality rate was 0.08%(2/2356). Conclusions The complications and mortality rate of interventional therapy for CHD in children are relatively low, but cannot be ignored. The complication could be reduced by choosing proper indications, following the operational procedures and careful operative follow-up.