完全性肺静脉异位引流病理谱及个体化手术治疗

Objective To delineate the morphological spectrum of total anomalous pulmonary venous connection (TAPVC) by building the "road map" and the " variation chart" of pulmonary veins,and to advance individualized surgical treatment Methods Between April,2006 to June,2009, 139 consecutive patients with TAPVC underwent operations. There were 61 supracardiac,55 intracardiac,6 infracardiac and 17 mixed types. Pathological diagnosis was made by echocardiogram,magnetic resonance imaging, computerized tomography,or the cardiac catheterization and operative findings during operation. The option of procedure was determined by findings. Results (1) Pathological spectrum:①" the road map"of the pulmonary veins: the patients with supracardiac type were divided into 4 subtypes according to the course of vertical veins:left course (47 cases),right course (9 cases),posterior course(2 cases) and double courses (3 cases). The patients with intracardiac type were divided into 3 subtypes according to the draining site of pulmonary veins: to coronary sinus (49 cases) ,to right atrium (5 cases) or to coronary sinus and right atrium (1 case),and the openings of pulmonary veins may be 4,2 or 1 in each subtype. The patients with infracardiac type were divided into 4 subtypes according to the draining site of vertical vein:to portal vein (1 case),to hepatic vein(3 cases),to portal vein and hepatic vein (1 case) and to inferior vena cava (1 cases). The patients with mixed type were divided into 3 subtypes: bilateral and symmetrical connections "2 + 2" pulmonary venous drainage pattern; bilateral and asymmetrical connections"3 +1" pulmonary venous drainage pattern and bizarre anatomic variants. ② The morphological "variation chart" of pulmonary veins individual pulmonary vein stenosis or excessive tributary veins (11 cases) showed: hypoplastic confluence veins (4 cases) and vertical veins distortion or elongation or forming hemodynamic vise or common pulmonary vein that drained to coronary sinus or right atrium through a narrowed short vertical vein or a small window(9cases).(2)Surgical results：early death was encountered in 6 cases(4.3％).The causes included 10W cardiac output syndrome in 1 case,and pulmonary veins stenosis and associated complications in the other 5 patients.Six patients with pulmonary restenosis underwent reoperation.All survived.Two patients had mild residual obstruction during Intermediate-term follow-up.Conclusions The patients with TAPVC had a wide spectrum of pulmonary veins with high inter-individual variation.It was useful to delineate the anatomy of pulmonary veins and to plan personalized procedures during operations according to the"road map"and"variation chart"of pulmonary veins.     

小儿睾旁横纹肌肉瘤的诊治

Objective To improve the diagnostic accuracy and therapeutic effect of paratesticu-lar rhabdomyosarcoma (PARA RMS). Methods Fourteen patients diagnosed with PARA RMS from 1977 to June 2008 were retrospectively analyzed. The patients aged from 1 to 15 years, with the mean age of 6 years. Of all the cases, 6 were younger than 5 years. Nine sinistral and 5 dextral PARA RMSs were diagnosed. All patients manifested as scrotum swollen and diagnosed with PARA RMS at stage Ⅰ by roentgenographic findings. Of all the patients, 10 underwent radical inguinal orchiectomy, and 4 patients who had undergone orchiectomy ( 1 case) or tumor enucleation (3 cases) through a trans-scrotal incision in other hospitals underwent inguinal exploration with removal of the testis or the remaining sperrnatic cord. Excision of the scar of scrotal incision were also performed on the 4 above-mentioned patients. Thirteen cases were diagnosed as the spindle cell type of embryonic RMS and one the as the gland alveolar type. Chemotherapy (VAC, vincristine + actinomycin + cyclophosphamide) was performed on all patients. Results Thirteen of 14 patients were followed up for 1 to 24 years (mean, 6 years). Eleven patients were followed up for at least 2 years, including 10 (91%) survivors without tumor recurrence. For the other one patient with the age of 8 years, who underwent only 6 periods of chemotherapy and whose parents gave up the following chemotherapy, had the recurrence of a retroperitoneal metastatic tumor with the diameter more than 8 cm at 2 years postoperatively. A 9 year old patient who was admitted in 1981 had no sperm in his semen at follow-up. Conclusions PARA RMS often shows as a unilateral scrotal swelling or mass, thus it could be diagnosed earlier than other genitourinary tumors. Most cases of early diagnosed with PARA RMS are at stage Ⅰ, with the pathologic diagnosis of spindle cell type which indicated a good prognosis. The curative rate for pa-tients in stage 1 can be up to 90% after radical inguinal orchiectomy combined with VAC chemothera-py. In recent years, only VCR+ ACTD is suggested for type 1 PARA RMS for 1 year, to avoid infer-tility.     

Two approaches for newborns with critical congenital heart disease: a comparative study

Background Prenatal diagnosis and planned peripartum care is an unexplored concept in China.This study aimed to evaluate the effects of the“prenatal diagnosis and postnatal treatment integrated model”for newborns with critical congenital heart disease.Methods The medical records of neonates(≤28 days)admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020.The patients were divided into“prenatal diagnosis and postnatal treatment integrated group”(n=47)and“non-integrated group”(n=69).Results The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group(5.2±7.2 days vs.11.8±8.0 days,P<0.001;11.9±7.0 days vs.16.5±7.7 days,P=0.001,respectively).The weight at surgery also was lower in the integrated group than in the non-integrated group(3.3±0.4 kg vs.3.6±0.6 kg,P=0.010).Longer postoperative recovery time was needed in the integrated group,with a median mechanical ventilation time of 97 h(interquartile range 51–259 h)vs.69 h(29–168 h)(P=0.030)and with intensive care unit time of 13.0 days(8.0–21.0 days)vs.9.0 days(4.5–16.0 days)(P=0.048).No significant difference was observed in the all-cause mortality(2.1 vs.8.7%,P=0.238),but it was significantly lower in the integrated group for transposition of the great arteries(0 vs.18.8%,log rank P=0.032).Conclusions The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns,and surgical intervention could be performed with a lower risk of death,especially for transposition of the great arteries.     

Selective coronary angiography in follow - up and technical study of Kawasaki disease - induced giant coronary artery aneurysm北大核心CSCD

Objective To investigate the characteristics of long - term changes in giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD) and the technical approach of using selective coronary angiography (SCAG) in children. Methods A retrospective analysis was made in 52 patients with KD and GCAA in acute phase, including 38 males and 14 females with an average age of (4. 1 ±2.5) years old ranging from 1 to 14 years old,from January 2008 to December 2018 at the Department of Cardiology, Guangzhou Women and Children's Medical Center. The selective coronary angiography (SCAG) was performed in the patients who were followed up for (10.6 ±3.6) years (1-19 years). The changes in coronary artery lesions were analyzed and the technical approach of SCAG was discussed in children. Results Among 52 patients investigated by SCAG, the location of coronary artery lesion was found the left - anterior descending branch in 21 cases(40.4%),20 cases(38. 5%) in the right coronary artery,8 cases (15.4%) in the left main trunk and 3 cases(5.7%) in circumflex. The left coronary artery in 2 cases and the right coronary artery in 4 cases were completely occluded,and collateral vessel formation was found in 12 cases. There were 21 cases of right coronary artery stenosis, including 7 cases of the right coronary artery occlusion and bridge - like neovascularization, and 4 cases of the right coronary artery woven lesions (thrombotic occlusion and clustered neovascularization). There were 27 cases of the left coronary artery stenosis with different degrees, including 5 cases of the left coronary artery occlusion and bridge - like neovascularization, and 2 cases of the left coronary artery woven lesions (thrombotic occlusion and clustered neovascularization). All of 52 patients with GCAA were followed up for 1 to 19 years. GCAA still existed in 40 cases. Regression to small coronary artery aneurysm was found in 8 cases,and mild - dilation regression was observed only in 4 patients. The resting electrocardiogram showed pathological Q wave and the left ventricular enlargement in 6 cases,and normal in the remaining 46 patients. The children had no subjective symptoms and their activities were not restricted.compared with SCAG,echocardiography detected 52 cases with proximal morphological changes in the coronary artery, but distal changes were found only in 3 cases. And 12 cases with coronary artery occlusion and neovascularization failed to be prompted by echocardiography. Conclusions GCAA induced by KD can persist for a long time, and has characteristics of coronary occlusion, stenosis, and collateral vessel formation. The conventional electrocardiogram and echocardiography are insensitive to the coronary artery morphology and dysfunction in KD sequelae. The SCAG is of great value in tracking this disease. For using SCAG in children,as long as the appropriate equipment and projection angle are selected, and the operation skills are mastered, satisfactory images and high success rate can be obtained. © 2019 Chinese Medical Journals Publishing House Co.Ltd. All rights reserved.     

Risk factors for death in children with severe adenovirus pneumonia in southwest Chongqing北大核心CSCD

Objective To investigate the risk factors for death in children with severe adenovirus pneumonia (SAP) in pediatric intensive care unit (PICU), and to provide reference basis for clinical reasonable treatment and reducing the adverse outcome. Methods The clinical data of 68 children with SAP hospitalized in PICU, Children′s Hospital of Chongqing Medical University from August 2018 to September 2019 were retrospectively analyzed.They were divided into the death group and the survival group according to their condition.The age, basic diseases, complications and laboratory examination results of children were collected for univariate analysis, and multivariate Logistic regression analysis was performed for those with significant univariate analysis. Results Among the 68 children with SAP, 50 were males and 18 were females, and 46 cases (67.6%) aged between 6 months and 2 years.Fifty-five cases(80.9%) of SAP occurred in spring and summer.There were 61 cases (89.7%) with the spike over 39 ? and 21 cases (30.9%) had fever for over 2 weeks; 42 cases (61.8%) were infected with mixed other pathogens.Intrapulmonary and extrapulmonary complications at varying severity were observed.There were 23 cases (33.8%) deaths.Univariable Logistic regression analysis showed that the rates of congenital heart disease(13 cases vs. 9 cases), alanine transaminase >100 U/L(12 cases vs. 8 cases), acute respiratory distress syndrome (ARDS)(13 cases vs. 9 cases) and severe extrapulmonary complications (19 cases vs. 14 cases) were significantly higher in the death group than those in the survival group (all P<0.05). Multivariate Logistic regression analysis showed that congenital heart disease, ARDS and severe extrapulmonary complications were independent risk factors for death in children with SAP (all P<0.05). Conclusions Children with SAP in PICU are mostly 6 months to 2 years old.SAP mainly occurs in spring and summer, which is featured by the high spike of fever, long duration of fever, easy to be infected with other pathogens, high incidences of systemic complications, and high mortality.The combination of congenital heart disease, ARDS and severe extrapulmonary complications increases the risk of death in children with SAP in PICU. © 2022 Chinese Medical Journals Publishing House Co.Ltd. All rights reserved.     

Mutation spectrum and clinical features of congenital long QT syndrome in 20 children: a single center study北大核心CSCD

Objective To explore the pathogenic genes, clinical characteristics and treatment follow-up of children with congenital long QT syndrome (LQTS). Methods Clinical data of 20 cases diagnosed with congenital LQTS and underwent gene testing from April 15, 2011 to April 15, 2021 in Department of Pediatric Cardiology, Shandong Provincial Hospital Affiliated to Shandong University were retrospectively collected and analyzed using independent sample t-test and Fisher's exact probability method. Results LQTS-related gene mutations were detected in all the 20 cases, and pathogenic or suspected pathogenic mutations were identified in 18 cases (90.0%). Five LQTS mutation genes were discovered, including KCNQ1, KCNH2, SCN5A, CACNA1C and AKAP9.Eighteen cases (90.0%) had positive symptoms, and 13 cases (65.0%) had definite inducements.The inducement of symptoms in children with LQTS type 1(LQT1) was related to exercise, the causes of syncope in LQT1 and Jervell-Lange-Nielsen syndrome type 1 (JLNS1) with complex heterozygous mutations were exercise or emotional agitation; the causes of syncope in LQTS type 2 (LQT2) were unrelated to exercise; severe exercise in LQTS type 3 (LQT3) resulted in symptoms; and seizure in LQTS type 8 (LQT8) was non-induced.The corrected QT(QTc) interval of 20 cases was (553.1±66.6) ms, with a range of 460-707 ms, among which 17 cases showed QTc≥480 ms.The electrocardiogram(ECG) manifestations of children with various types of LQTS were different.There was no significant difference in QTc between different genders, or between children with syncope and those without syncope (all P>0.05). The follow-up time was (3.4±2.3) years, ranging from 0 to 8.3 years.Seventeen children received treatment[beta blockers and implantable cardiovertor-defibrillator(ICD)] and 3 cases did not.By the end of the follow-up, 1 child died, 19 cases survived, and 2 cases of the surviving children lost consciousness. Conclusions There is a high consistency between genetic diagnosis and clinical diagnosis of congenital LQTS.The positive rate of gene detection is 90.0%.The clinical manifestations and ECG characteristics vary with genotypes.Beta blockers are protective.ICD therapy can prevent sudden cardiac death when oral medication does not respond. © 2022 Chin J Pathol, June. All rights reserved.     

Follow-up and prognosis analysis of rheumatic disease complicated with pulmonary arterial hypertension in children北大核心CSCD

Objective To explore the clinical features, follow-up characteristics and prognosis of rheumatic disease complicated with pulmonary arterial hypertension (PAH) in children, and to provide support for its clinical diagnosis and treatment. Methods A retrospective analysis was conducted on the data of rheumatic 24 patients complicated with PAH hospitalized in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to the Capital Institute of Pediatrics, Department of Rheumatology and Immunology, Jiangxi Children′s Hospital, Department of Pediatrics Ⅰ, the First Affiliated Hospital of Zhengzhou University and Department of Pediatrics, the Affiliated Hospital of Inner Mongolia Medical University from January 2013 to June 2022.The rheumatic patients complicated with PAH were followed up by telephone on June 30, 2022, and their clinical symptoms, treatment, follow-up, and prognosis data were collected.According to different treatment methods, the patients were divided into different clinical subgroups. The change of PAH was analyzed. The t-test was used for comparison between groups.P<0.05 was statistically significant. Results A total of 24 cases were enrolled, with 7 males and 17 females.The average onset age of PAH was (10.97±3.79) years old.The median duration of PAH was 6.00 (32.20) months.The average pulmonary artery pressure was (51.71±17.66) mmHg(1 mmHg=0.133 kPa). There were 9 cases of systemic lupus erythematosus, 5 cases of Takayasu′s arteritis, 3 cases of juvenile dermatomyositis, 3 cases of undifferentiated connective tissue disease, 2 cases of systemic juvenile idiopathic arthritis, 1 case of Behcet′s disease, and 1 case of Kawasaki disease.Among 24 cases, the common symptoms were fever (14 cases), fatigue (10 cases) and dyspnea (7 cases). Of the 24 cases, 10 cases were complicated with hydropericardium, 9 cases with valve regurgitation, and 5 cases with decreased systolic and/or diastolic function.Lung changes were observed in 17 cases.Eleven cases were tested for B-type natriuretic peptide (BNP), and the BNP levels were all elevated in them (11 cases), with a median BNP of 3 073 (10 645) ng/L.After the first occurrence of PAH, 12 cases were treated with Methylprednisolone therapy, 10 cases received Cyclophosphamide therapy, and 2 cases who were both systemic lupus erythematosus, underwent blood purification.In the treatment of PAH, 11 cases were treated with pulmonary artery pressure reduction, and 7 of the 11 cases took PAH-targeted drugs.The mean decrease of the average pulmonary artery pressure in children receiving the targeted therapy[(44.80±24.08) mmHg] was significant higher than that in children not receiving the targeted therapy [(16.15±17.25) mmHg] (t=2.661, P=0.016). Twenty children were reexamined and/or followed up, and the average course of PAH at the telephone follow-up was (36.29±26.67) months.The pulmonary arterial hypertension in 6 cases completely recovered, with median recovery time of 8.00 (13.47) months, but 2 of them died after the complete recovery.The pulmonary arterial hypertension improved in 11 children, 1 of whom died and the remaining children were in stable condition.The pulmonary arterial hypertension worsened in 2 children, 1 of them improved previously but aggravated recently, and the other child did not monitor pulmonary artery pressure and died during telephone follow-up. Conclusions Rheumatic diseases complicated with PAH are rare and most often diagnosed in severe rheumatic children.It can lead to death, and is commonly accompanied by notably elevated BNP levels.The patients who have early PAH detection, intensive treatment of the primary disease, symptomatic and targeted pulmonary artery pressure reduction show a better prognosis. © 2023 Journal of Chinese Agricultural Mechanization. All rights reserved.     

Clinical features,diagnosis and treatment of intussusception secondary to inverted Meckel's diverticulum in children北大核心CSCD

Objective To explore the clinical characteristics, diagnosis, treatment and prognosis of inverted Meckel's diverticulum in children, and to summarize the clinical experience. Methods Ten children who received surgical treatment in Beijing Children's Hospital, Capital Medical University from October 2018 to October 2021 were retrospectively analyzed. The children were diagnosed with Meeker's diverticulum. Their data were complete and their follow-up information was available. The clinical features, diagnosis, treatment and prognosis were summarized. There were 6 males and 4 females, whose age of onset ranged from 5 months to 12 years and 5 months. The clinical manifestations were abdominal pain in 8 cases, crying in 2 cases and bloody stool in 3 cases. B - ultrasound showed suspected diseases secondary to inverted Meckel's diverticulum, including small intestinal intussusception in 6 cases and back junction type intussusception in 4 cases. One case of small intestinal intussusception was misdiagnosed after laparoscopic exploration in other hospitals and suffered from recurrent intussusception. All patients underwent segmental bowel resection. Results Of the 10 children, 8 cases received laparoscopic exploration and 2 cases underwent open surgery for secondary intestinal necrosis. Postoperative pathology revealed Meckel's diverticulum. Ectopic tissue was accessible in 8 cases, including ectopic gastric tissue in 7 cases, and ectopic pancreas in 1 case. During the postoperative follow - up period (1 month - 3 years), the patients were generally in good condition with no complications such as intestinal obstruction. Conclusions Intussusception secondary to inverted Meckel's diverticulum is rare and difficult to diagnose before operation. The diagnosis should be confirmed by surgical exploration and postoperative pathology. However, careful surgical exploration is required since inverted Meckel's diverticulum is prone to be misdiagnosed during exploration. © 2022 ＣｈｉｎＪＡｐｐｌＣｌｉｎＰｅｄｉａｔ. All rights reserved.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

单纯性完全型肺静脉异位引流产前8例超声心动图诊断

摘要 目的 探讨胎儿单纯性完全型肺静脉异位引流（TAPVC）的产前超声心动图特点,提高对本病的产前诊断准确率。方法 回顾性分析2011年5月至2014年2月经新生儿超声心动图、手术或尸解证实的8例单纯性TAPVC的胎儿期超声心动图检查结果,总结超声心动图特征。结果 8例单纯性TAPVC胎儿中,心下型2例,心内型1例,心上型5例,5例存在垂直静脉或共同肺静脉腔与垂直静脉连接处梗阻;8例胎儿中,引产1例（心下型）,余7例均于出生后行手术治疗,其中1例术后死亡（心下型）,6例均恢复良好。TAPVC胎儿超声心动图特征为：①正常的左心房形态消失,呈圆形或椭圆形,左心房光滑并多变小,在孕后期较为明显;②降主动脉与左心房间距离明显增大,多数在左心房后方可见一异常的腔隙（即共同肺静脉腔）,可显示左、右侧上升（心上型）或下降（心下型）的垂直静脉;③妊娠早期左、右心系统比值早期多正常,但妊娠中、晚期（孕26周后）可出现右心系统轻度扩大;④引流入冠状静脉窦时（心内型）,冠状静脉窦可有扩张;引流入上腔静脉时（心上型）,上腔静脉扩张;引流入肝内血管时（心下型）,肝内血管可有不同程度的扩张;⑤彩色多普勒可显示引流途径及是否合并垂直静脉梗阻。结论 在孕早期左、右心系统比值正常时,TAPVC易被漏诊及误诊,应注意多角度、多切面扫查,孕晚期超声心动图检查可减少漏诊。     

Sensitivity,specificity, and accuracy of fetal echocardiography for high-risk pregnancies in a tertiary center in Egypt

BackgroundAdvances in fetal echocardiography training among pediatric cardiologists have led to substantial improvements in prenatal detection of congenital heart diseases (CHDs). Nevertheless, diagnostic accuracy varies among centers. Moreover, this subspecialty continues to evolve in developing countries, with limited studies assessing fetal echocardiography sensitivity, specificity, and accuracy in developing countries such as Egypt.Subject and methodsHigh-risk pregnancies referred for fetal echocardiography from January 2011 to January 2019 were analyzed retrospectively. All of the cases included had one of the high-risk indications for fetal echocardiography. Maternal age and gestational age at the prenatal diagnosis were determined, and detailed fetal and neonatal echocardiograms were documented. The results of fetal and postnatal echocardiography were compared to assess the sensitivity, specificity, and accuracy of fetal echocardiography. A minor lesion was defined when no postnatal intervention was needed and a major anomaly when postnatal cardiac therapy, surgery, or intervention was required.ResultsOut of 615 pregnant patients referred, comparisons between fetal and neonatal echocardiography were possible in 458 fetuses, with 157 patients excluded from the study. The mean maternal age in the study was 26.97 ± 5.871 years and the mean gestational age at referral was 27.24 ± 5.407 weeks. The most common indication for prenatal echocardiography in this cohort was a family history of CHD (142; 31%) followed by nonimmune hydrops (97; 21.18%) and abnormal obstetrical ultrasound screening (64; 13.97%). We had three false-positive minor diagnoses and four false-negative cases with only one requiring intervention. Prenatal diagnosis in this study was accurate in 98.47% of cases. The sensitivity, specificity, and accuracy of fetal echocardiography in the current work were 97.03%, 99.07%, and 98.47, respectively.ConclusionFetal echocardiography is considered a highly sensitive specific tool for prenatal detection of congenital heart diseases in high-risk pregnancies even in developing countries. However, minor fetal cardiac disorders are challenging to diagnose and family counseling should emphasize the difficulty of excluding or confirming such lesions.     

Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography

Background  Although echocardiography is the first-line imaging modality in the diagnosis of total anomalous pulmonary venous connection (TAPVC), multidetector CT (MDCT) could have advantages in the diagnosis of TAPVC in certain cases. Objective  To compare MDCT with echocardiography in the evaluation of TAPVC. Materials and methods  Enrolled in the study were 23 patients with surgically proven TAPVC. The echocardiography and MDCT findings were independently interpreted by a paediatric cardiologist and cardiac radiologist in terms of: (1) the drainage site of the common pulmonary vein, (2) stenosis of the vertical vein, and (3) the course of the atypical vessel into the systemic vein in the case of vertical vein stenosis. The findings from both modalities were correlated with the results obtained at surgery (n=22) or autopsy (n=1). Results  In all patients, MDCT correctly depicted the drainage site of the common pulmonary vein, stenosis of the vertical vein and the course of the atypical vessel into the systemic vein (sensitivity 100%, specificity 100%). The specificity of echocardiography was 100% for the three defined findings. The sensitivity of echocardiography, however, was 87%, 71% and 0%, respectively. Conclusion  MDCT can facilitate the diagnosis of TAPVC in certain cases.     

Associations Between Prenatal Food Insecurity and Prematurity,Pediatric Health Care Utilization,and Postnatal Social Needs

ObjectiveChildhood food insecurity endangers child development and health outcomes. Food insecurity will grow increasingly common in the economic wake of the coronavirus pandemic and prenatal care represents an early, clinical opportunity to identify families at risk. However, longitudinal relationships between clinically-identified prenatal food insecurity and prematurity, pediatric health care utilization, and postnatal social needs have not been described.MethodsWe examined longitudinal data from mother-child dyads who received prenatal and pediatric care and social needs screening at a large academically-affiliated safety net medical center between October 2018 and July 2019. Associations among household food insecurity and premature birth, pediatric inpatient and outpatient utilization, missed immunizations, and postnatal social needs were estimated using adjusted regression.ResultsAmong the 268 mothers, those who experienced prenatal household food insecurity had 3 times higher odds of having a child born prematurely (95% confidence interval [CI] 1.0–8.9, P = .05) and had children with higher inpatient hospitalizations (incidence rate ratio [IRR] 2.4, 95% CI 1.0–5.6, P = .04) and missed immunizations (IRR 3.4, 95% CI 1.1–10.3, P = .03) in the first 6 months of the child's life. These mothers also had higher odds of having any social needs in the pediatric setting (odds ratio 3.4; 95% CI 1.5–8.0, P = .004).ConclusionsPrenatal household food insecurity was linked to future adverse perinatal and pediatric outcomes in low-income mother-child dyads. Food insecurity identifies children at social and medical risk, providing an early clinical opportunity to intervene.     

混合型完全性肺静脉异位连接17例的诊断与治疗

目的探讨儿童混合型完全性肺静脉异位连接(TAPVC)的诊治要点及影响预后的主要因素。方法近5 a来,在广东省心血管研究所确诊为混合型TAPVC的患儿共17例,收集该17例患儿的临床资料和随访结果,进行回顾性分析。结果 17例混合型TAPVC患儿中心上型+心内型14例,心上型+心下型3例,并肺静脉回流梗阻6例。17例术前全部行超声心动图检查,其中13例行多排螺旋CT扫描,14例行手术治疗。行CT扫描者,10例经手术探查证实此10例患儿CT能够准确显示所有肺静脉回流径路。行超声检查者,12例超声报告了所有4条肺静脉回流径路,5例超声未能报告肺静脉全部回流径路。超声报告所有肺静脉回流径路者,经CT或手术探查,证实其中7例超声显示的所有肺静脉回流径路均准确,4例超声准确显示了3条肺静脉回流径路,1例超声显示的肺静脉回流径路完全错误。并肺静脉回流梗阻的6例患儿,超声心动图发现6例,而CT仅发现1例。14例行手术治疗者,急诊手术6例,择期手术8例。围术期死亡2例,均为急诊手术者。12例康复出院者,随访时间2～53(28.8±17.4)个月,中位随访时间30个月。术后3例发生吻合口狭窄,其中1例术后2个月死于严重吻合口狭窄导致的急性肺水肿。结论混合型TAPVC以心上型+心内型最多见。结合超声心动图和多排螺旋CT扫描是术前评估的最佳策略。对肺静脉回流径路的显示多排螺旋CT优于超声心动图,对肺静脉回流梗阻的显示超声心动图优于多排螺旋CT。急诊手术可能是围术期死亡的危险因素。术后随访主要并发症为吻合口狭窄。     

The current state of surgical practice for neonatal torsion: A survey of pediatric urologists

ObjectiveThe evaluation and treatment of perinatal testicular torsion is controversial. We performed a survey to assess practice patterns among pediatric urologists regarding treatment of perinatal torsion.MethodsAn internet survey was administered to members of two pediatric urology societies. Cases of prenatal, postnatal and bilateral prenatal torsion were outlined. Respondents were asked about use of ultrasound, timing of surgery, incision, and management of the contralateral testicle. A case with a non-palpable testicle and blind ending vessels was also presented.ResultsWe had 121 respondents. In a neonate with prenatal torsion, 34% percent would operate immediately, 26% urgently within 72 h, 28% electively and 12% would not explore; 93% would perform a contralateral orchiopexy. In a neonate with postnatal torsion, 93% would operate immediately, 5% urgently, 1% electively and 1% would not explore; 96% would perform a contralateral orchiopexy. In both cases, 75% would use a scrotal incision and 25% would use an inguinal incision. When presented with bilateral prenatal torsion, 90% would operate immediately, 1% urgently, 2% electively and 7% would not operate. In the case of a non-palpable testicle with blind ending vessels 28% would perform a contralateral orchiopexy, 12% would explore the ipsilateral canal for a “nubbin”, 56% would perform no intervention and 4% would perform some other form of management.ConclusionWe documented variability of timing for intervention of prenatal torsion and confirmed that most view postnatal torsion as a surgical emergency. Most perform a contralateral orchiopexy for prenatal torsion despite the fact that most cases are extravaginal. The surgical approach via a scrotal incision appears to be preferred at this time.     

Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection

Background

Obstructed total anomalous pulmonary venous connection (TAPVC) is frequently misdiagnosed as pulmonary disease and without operative correction early death is common. It is important to make a correct diagnosis before surgery.

Objective

The purpose of this study was to describe the chest radiographic features of obstructed TAPVC and compare CT angiography with transthoracic echocardiography in the evaluation of obstructed TAPVC.

Materials and methods

Eighteen children with obstructed TAPVC were assessed. Their clinical and imaging data were retrospectively reviewed. The characteristic radiographic findings were analyzed and compared with surgical results, and the diagnostic accuracy of CT angiography and transthoracic echocardiography was evaluated in terms of pulmonary venous drainage and obstruction detection.

Results

The common radiographic features included pulmonary venous congestion or edema or both (16 of 18 cases, 89%), and absence of cardiomegaly (12 of 18 cases, 67%). CT angiography correctly diagnosed TAPVC and clearly revealed the draining sites in all children (five with supracardiac TAPVC, three with cardiac TAPVC, eight with infracardiac TAPVC and two with mixed TAPVC). The diagnostic agreement between CT angiography and surgery was 100%. Transthoracic echocardiography only correctly revealed the draining sites in 11 children (5 with supracardiac TAPVC, 2 with cardiac TAPVC and 4 with infracardiac TAPVC). The diagnostic agreement between transthoracic echocardiography and surgery was 61%. The diagnostic accuracy of CT angiography was higher than that of transthoracic echocardiography (P?=?0.0156). Thirty-four sites of obstruction were correctly detected by CT angiography (11 in the mediastinum, 1 at the diaphragmatic level, 9 below the diaphragm and 13 stenotic individual pulmonary veins in the lung). The diagnostic agreement between CT angiography and surgery was 92%. Transthoracic echocardiography only correctly detected 15 sites of obstruction (11 in the mediastinum, 1 at the diaphragmatic level and 3 below the diaphragm). The diagnostic agreement between transthoracic echocardiography and surgery was 41%. The rate of detection for sites of obstruction with transthoracic echocardiography was much lower than that of CT angiography (P?=?0.0002).

Conclusion

In children with obstructed TAPVC, plain radiographs usually show a characteristic pattern of pulmonary venous congestion or edema, or both, and a normal cardiac silhouette. CT angiography is superior to transthoracic echocardiography in the evaluation of pulmonary venous drainage and obstruction, especially in children with infracardiac and mixed TAPVC.     

Obstructed total anomalous pulmonary venous connection

Summary With the advent of echocardiography, total anomalous pulmonary venous connection (TAPVC) can be readily diagnosed without much difficulty. However, noninvasive detection of the presence of pulmonary venous obstruction in TAPVC remains a difficult issue. During a 5.5-year period, 42 patients were found to have TAPVC by catheterization, surgery, and/or autopsy: 17 had supracardiac drainage, 13 paracardiac drainage, nine infracardiac drain-age, and three mixed drainage. Obstruction to pulmonary venous drainage was found in 24 patients (57%). Patients with right isomerism tended to have a higher incidence of pulmonary venous obstruction than those with the usual atrial arrangement (80% vs. 44%,p<0.05). Color Doppler combined with cross-sectional echocardiography provided accurate delineation of drainage sites in 93% cases (39 of 42). Among the 39 cases with correct echocardiographic delineation of the drainage site, obstruction was detected by echocardiography in 22 cases with a sensitivity of 100% (22 of 22) and a specificity of 85% (17 of 20). Therefore, complete echocardiography, including cross-sectional images and color Doppler proved to be a reliable tool in the detection of drainage sites and pulmonary venous obstruction in TAPVC.     

Structural congenital brain disease in congenital heart disease: Results from a fetal MRI program

ObjectiveTo identify the type and incidence of fetal brain pathology in fetuses with a prenatal diagnosis of congenital heart disease (CHD).Patients and methods67 pregnant women underwent a fetal MR-examinations between 20 and 38 gestational weeks. MR was done on a 1.5 T superconducting system. The type of cardiac malformation was defined by fetal echocardiography. Fetuses with a chromosomal abnormality or an extracardiac anomaly were excluded.ResultsFetal MRI scans in the final study cohort (53 fetuses) yielded normal results in 32 fetuses and a brain abnormality in 21 fetuses. Congenital brain disease (CBD) was found in 39% of the final study cohort of fetuses with CHD. MRI findings were classified into malformations, acquired lesions and widening of the ventricles and/or outer CSF spaces (malformations: 7 fetuses, acquired lesions: 5 fetuses, changes in CSF spaces: 9 fetuses). Asymmetry of the ventricles was the most common finding in the CSF group.ConclusionsOur data suggest that fetal MRI can be used to characterize structural CBD in CHD. Advanced MRI techniques such as diffusion tensor imaging and proton spectroscopy are tools that, in the future, will certainly shed light on the spectrum of structural and functional CBDs that are associated with CHD.     

Fetal Echocardiography in Ectopia Cordis

Ectopia cordis is an extremely rare congenital abnormality occurring in 5.5 to 7.9 per 1 million live births with high lethality. Between January 1995 and October 1997 eight cases of ectopia cordis were diagnosed at our institute before birth. On the basis of echocardiography the fetal heart anatomy was categorized as either normal heart anatomy (NHA; n= 3) or congenital heart defect (CHD; n= 5). In the majority of cases (seven of eight) other abnormalities were present. Some reports have described ectopia cordis being diagnosed in the first trimester of pregnancy. In our study group the average gestational age at diagnosis was 26 weeks. The prenatal diagnosis of isolated ectopia cordis is easy; counseling the patient, the perinatal management including term, place, and method of delivery, and optimal care of the newborn are more difficult. Ectopia cordis is a malformation that pediatricians rarely encounter, even at pediatric cardiology centers. Much more frequently it is a problem for sonographers and obstetricians; however, pediatric cardiologists should be aware of diagnostic algorithm for such cases, especially when additional abnormalities are present.