Déficit funcional em crianças com cardiopatias congênitas submetidas à correção cirúrgica após alta da unidade de terapia intensiva / Functional deficit in children with congenital heart disease undergoing surgical correction after intensive care unit discharge

RESUMO Objetivo: Avaliar a funcionalidade de pacientes pediátricos submetidos à correção cirúrgica de cardiopatia congênita após a alta da unidade de terapia intensiva e as possíveis correlações com variáveis clínicas e risco cirúrgico. Métodos: Estudo transversal, que incluiu crianças entre 1 mês e 18 anos incompletos, que realizaram cirurgia para correção de cardiopatia congênita, no período de outubro de 2017 até maio de 2018. A avaliação da funcionalidade foi realizada por meio da Functional Status Scale, a avaliação do risco cirúrgico se deu pelo Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), e as variáveis clínicas foram obtidas do prontuário eletrônico. Resultados: A amostra foi composta de 57 crianças, com mediana de idade de 7 (2 - 17) meses, sendo 54,4% do sexo masculino. Dentre as crianças, 75,5% apresentaram alteração na funcionalidade, e 45,6% delas tiveram disfunção moderada. Cerca de 47% da amostra apresentou classificação RACHS-1 > 3, indicando maior risco cirúrgico. Maior déficit funcional foi associado a crianças mais novas, com maior duração da ventilação mecânica invasiva e do tempo de internação na unidade de terapia intensiva. Além disso, maior grau de disfunção foi observado entre aqueles classificados com RACHS-1 > 3. Conclusão: A prevalência de disfunção foi elevada em crianças e adolescentes com cardiopatia após cirurgia cardíaca. Maior risco cirúrgico, duração da ventilação mecânica invasiva, permanência na unidade de terapia intensiva e os mais jovens apresentaram associação com pior desempenho funcional.

Abstract Objective: To evaluate the functional status of pediatric patients undergoing congenital heart surgery after discharge from the intensive care unit, and to evaluate the correlations among clinical variables, functional status and surgical risk. Methods: Cross-sectional study including patients aged 1 month to less than 18 years undergoing congenital heart surgery between October 2017 and May 2018. Functional outcome was assessed by the Functional Status Scale, surgical risk classification was determined using the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and clinical variables were collected from electronic medical records. Results: The sample comprised 57 patients with a median age of 7 months (2 - 17); 54.4% were male, and 75.5% showed dysfunction, which was moderate in 45.6% of the cases. RACHS-1 category > 3 was observed in 47% of the sample, indicating higher surgical risk. There was a correlation between functional deficit and younger age, longer duration of invasive mechanical ventilation and longer intensive care unit stay. Moreover, greater functional deficit was observed among patients classified as RACHS-1 category > 3. Conclusion: The prevalence of functional deficit was high among children and adolescents with congenital heart disease after cardiac surgery. Higher surgical risk, longer duration of invasive mechanical ventilation, longer intensive care unit stay and younger age were correlated with worse functional status.

Prenatal ultrasonic diagnosis of fetal isolated levocardia / 中华超声影像学杂志

Objective To investigate the clinical application valve of prenatal ultrasound in diagnosis of fetal isolated levocardia ( IL ) . Methods Thirty-eight IL fetuses diagnosed in three prenatal diagnosis centers from 2000 to 2016 were reviewed with their prenatal ultrasound features and cardiac and extracardiac malformations . Results Thirty-eight IL cases included 19 cases of left isomerism ( LI) ,14 cases of right isomerism( RI) and 5 non-classified cases . The overall incidence of malformations in IL patients was 97 .4％with 94 .7％ in LI patients and 100％ in RI patients . The incidence of cardiac malformations was 92 .1％with 84 .2％ in LI patients and 100％ in RI patients . The most common congenital heart diseases were:right ventricle outlet obstruction , univentricular physiology and atrioventricular septal defect . Anomalous pulmonary venous connection was more commonly seen in LI patients than in RI patients . The most common extracardiac malformation was duodenal obstruction( 86 .9％ ) . Inferior vena cava interruption and polysplenia were more commonly seen in LI patients .Juxtaposition of aorta and inferior vena cava and asplenia were more commonly seen in RI patients . Conclusions IL is very rarely seen in population with high incidence of congenital heart diseases and extracardiac malformations .

Surgical Treatment of Single Ventricle With Total Anomalous Pulmonary Venous Connection in Relevant Patients / 中国循环杂志

Objective: To summarize surgical result of single ventricle with total anomalous pulmonary venous connection (TAPVC) in relevant patients. Methods:A total of 25 patients of single ventricle with TAPVC treated in our hospital from 2008-09 to 2016-03 were summarized. The patients were between 5 days to 14 years of age with body weight at (3.3-49.5) kg. There were 22 patients with supracardiac type TAPVC, 1 with infracardiac type TAPVC and 2 with mixed type TAPVC;in addition,17 cases having complete atrio-ventricular canal defect, 2 having tricuspid atresia, 4 having more than moderate atrio-ventricular valve regurgitation, 5 having pulmonary atresia and 9having pulmonary venous obstruction. Results:There were 5/25 (20%) patients died at the early stage for low cardiac output syndrome. Followed-up study was conducted in 20 survivals for (1-65) months and 7 patients had re-surgery including 5 with modified Fontan operation, 1 with atrio-ventricular valve replacement due to severe atrio-ventricular valve regurgitation, 1 with pulmonary venous stenosis repair+atrio-ventricular valve replacement due to pulmonary venousobstruction and severe atrio-ventricular valve regurgitation. 3 patients died during followed-up period because of low cardiac output syndrome, protein lost intestine disease and neurological injury respectively. The rest survivals had good general condition, no residualpulmonary venous obstruction was found. Conclusion: The patients of single ventricle with TAPVC were frequently associated with right atrial isomerism and complex cardiac anomaly;surgical treatment still having the high mortality.

Supraventricular Tachycardia in Special Population

Medical and catheter-based ablation therapies have been successfully applied in treating supraventricular tachycardia, supported by abundant evidences from clinical trials. However, Special populations such as pediatric populations, pregnant patients, or patients with congenital heart diseases are easily excluded from clinical trials; thus, caution is needed when adopting results from clinical trials conducted in the usual adult population.

The Cause of Rapid Thrombocytopenia and Organ Hemorrhage in Congenital Heart Disease Patients After Interventional Occlusion / 中国循环杂志

Objective: To study the cause of rapid thrombocytopenia and organ hemorrhage in congenital heart disease (CHD) patients after interventional occlusion. Methods: A total of 665 CHD patients received interventional occlusion in our hospital from 2011-01 to 2015-12 were enrolled. The patients were divided into 3 groups according to the defects: Atrial septal defect (ASD) group,n=100, Ventricular septal defect (VSD) group,n=100 and Patent ductus arteriosus (PDA) group,n=465. Pre- and post-interventional occlusion platelet levels, the relationship between PDA occluder diameter and platelet counts were compared. Pressure difference between both sides of occluder was randomly measured in a part of patients including 50 in ASD group, 50 in VSD group and 102 in PDA group. Based on occluder diameter, the 102 PDA patients were further divided into 2 subgroups: Giant PDA,n=42 and Medium-small PDA,n=60; pressure differences between both sides of occluder were compared between 2 subgroups. Results: No severe thrombocytopenia and organ hemorrhage occurred in ASD group or VSD group. PDA group had 36/465 (7.74%) patients with severe thrombocytopenia, 18 (3.87%) with organ hemorrhage and all of them occurred in giant PDA subgroup; the diameter of PDA occluder was negatively related to post-operative to platelet counts (r=-0.659,P=0.001). For pressure difference on both sides of occluder, compared with prior operation, PDA group showed increased systolic and diastolic pressure differences and increased mean pressure difference at immediately post operation, allP< 0.05; systolic pressure difference in VSD group was (56.57±15.33) mmHg, in Medium-small PDA subgroup was (58.33±26.65) mmHg and in Giant PDA subgroup was (94.66±27.62) mmHg which was much higher than those in VSD group and Medium-small PDA subgroup, allP<0.01. Conclusion: Rapid thrombocytopenia and organ hemorrhage in CHD after interventional occlusion only happened in giant PDA patients. High pressure difference formed high-speed ifltration blood lfow which may cause scouring damage on platelets and it was the main reason for thrombocytopenia occurrence.

Small Left Atrial Size Complicating Percutaneous Transcatheter Device Closure of Secundum Atrial Septal Defect with Conventional Approach

BACKGROUND AND OBJECTIVES: Transcatheter device closure becomes the first option for treating secundum atrial septal defect (ASD), but the conventional method is sometimes unsuccessful even when the defect size indicates the closure to be feasible. To increase the success rate, modified methods have been introduced and used. This study aimed to find predictors for using the modified methods in the device closure of secundum ASDs. SUBJECTS AND METHODS: Between October 2010 and December 2012, 92 patients with ASDs underwent the transcatheter device closure. We analyzed the sizes of the defect, the surrounding rims, and the ratios of the left atrium (LA) dimensions to the device size in the patients who underwent the procedure either using the conventional or modified methods. RESULTS: Among the 88 successful cases (95.7%), 22 patients (25%) required modified methods (12 using pulmonary vein and 10 using balloon). The modified method group had the larger size of ASDs and smaller posterosuperior rim. The mean ratios of the LA anteroposterior diameter, width, and length to the device size were all significantly smaller in the modified methods group than in the conventional group (1.20 vs. 1.56, 1.32 vs. 1.71, and 1.61 vs. 2.07, respectively). We found that the risk factors for the modified methods were smaller retroaortic rim, larger ASD, and smaller LA dimension/device size. CONCLUSION: In addition to larger defects and smaller retroaortic rim, the smaller ratios of the LA dimensions to the device size influenced the need for the application of modified methods in the transcatheter device closure of ASDs.

Delayed sternal closure following pediatric cardiac surgery / 中华胸心血管外科杂志

Objective A retrospective review of the use of delayed sterna closure (DSC) after pediatric cardiac operations.The purpose of this study is to access the morbidity of DSC and to analyze the risk factors that may predict the need for DSC.Methods Between January 2008 and December 2011,5 546 infants (2 843 males,2 703 females) underwent cardiac operations through midterm sternotomy in Shanghai Children' s Medical Center.Median age was 5 months(1 day to 11 months) and mean weight was 6.1 kg(2.0-12.5 kg).The pathologies included transposition of great artery(TGA),corctation of aorta (CoA),interruption of aortic arch (IAA),pulmonary atresia (PA),truncus arteriosus (Truncus),complete atrioventricular septal defect(CAVSD),total anormalous pulmonary vein connection (TAPVC),double outlet of right ventricle(DORV),tetralogy of Fallot(TOF),and ventricular septal defect(VSD),et al.All hospital records were reviewed and clinical data were studied and analyzed.Results A total of 313 patients had DSC (5.6％).There were 191 males and 122 females.The reasons for DSC are hemodynamic instability in 296,bleeding in 11,and ECMO(or VAD) in 6 patients.Two hundred and fiftyone (80.2％) patients were less than 5 kg,84 (26.8 ％) patients were neonates,and 125 (39.9％) patients aged between one and three months old.The incidences of DSC for these patients were 13.9％,34.4％,and 18.4％ respectively.The diagnosis of complex congenital heart defects had a high risk of DSC.The incidences of DSC for TGA,CoA,IAA,PA,Truncus,CAVSD,TAPVC,and DORV were 28.8％,17.8％,43.1％,9.0％,30.4％,6.5％,and 10.8％ respectively.The DSC patients had longer duration of CPB time(147 min versus 72 min) and clamping time(81 min versus 40 min).In 282 patients the sternums were closed 1-11 days after the initial operation.In 3 cases the trial of closure failed for the first time and succeeded a second time until several days later.Fouty-six patients died ultimately including 15 patients died after the closure of sternum with a total mortality of 14.7 ％.Surgical site infection occurred in 17 patients (5.4％),and surgical intervention were needed in 4 severe cases(1.3％).Conclusion Neonates,an age less than 3 months,weight less than 5 kg,long CPB time and clamping time,and the diagnosis of complex congenital heart defects were risk factors of the need for delayed sterna closure after pediatric cardiac operations.

Expression of serum connective tissue growth factor in children with congenital heart disease and pulmonary artery hypertension and its significance / 中华实用儿科临床杂志

Objective To investigate the expression of serum connective tissue growth factor(CTGF) in congenital heart disease(CHD) with pulmonary artery hypertension (PAH) and its significance.Methods Fifty children with CHD in the Affiliated Hospital of Luzhou Medical College from May 2009 to Feb.2012 were selected in the study.Pulmonary artery systolic pressure (PASP) were measured by right heart catheterization.They were divided into 3 groups according to PASP levels as follows:normal PAH group(PASP ＜ 3.99 kPa,n =21),mild PAH group(3.99 kPa ≤ PASP ＜ 6.65 kPa,n =17),moderate or severe PAH group (PASP ≥ 6.65 kPa,n =12) ; serum CTGF and transforming growth factor-β1 (TGF-β1) were detected by using double-antibody sandwich enzyme linked immunosorbent assay.The relationship among the 3 groups and their PASP were analyzed.Results In comparison to normal PAH group,serum CTGF and TGF-β1 were significantly elevated in mild PAH group and moderate or severe PAH group (all P ＜0.01) ;compared with mild PAH group,serum CTGF and TGF-β1 were higher in moderate or severe PAH group(all P ＜ 0.01) ;CTGF levels were positive correlated with PASP both in mild PAH group and moderate or severe PAH group(r =0.828,0.849,all P ＜ 0.01);there were positive correlations between CTGF and TGF-β1 level in mild PAH group and moderate or severe PAH group (r =0.714,0.713,all P ＜ 0.01) ; there were positive correlations between TGF-β1 and PASP in mild PAH group and moderate or severe PAH group (r =0.623,0.639,all P ＜ 0.05).Conclusions Serum CTGF levels are elevated in children with CHD and PAH,which may be an important factor for the formation and development of CHD with PAH.

Application of modified Blalock-Taussig shunts in patients with complex congenital heart malformations and follow-up of 110 cases / 中华胸心血管外科杂志

Objective The modified Blalock-Taussig shunt (MBTS) is considered a low-risk management option for palliation in patients with severely cyanotic heart anomalies in advanced heart centers in western countries.But the morbidity and mortality associated with MBTS remains challenge in developing countries.Methods 106 patients with severely cyanotic complex heart anomalies underwent 110 modified Blalock-Taussig shunts(B-T shunts) between October 2000 and August 2012.The mean age was (3.8 ± 1.1) months (1 day-37.2 months).The mean weight of the babies was (5.0 ± 2.1) kg (2.3 kg-12.0 k g).The cardiac anatomy was as follows:pulmonary atresia with intact ventricular septum in 25,pulmonary atresia with ventricular septum defects in 45,tricuspid atresia in 11,tetralogy of Fallot in 7,complex single ventricle physiology in 18.All patients were deeply cyanotic,and preoperative prostaglandin E1 was needed in 78 patients to ensure ductus patent and maintain oxygen saturations prior to the shunt operation.The shunts were accomplished with 3.0 mm polytetrafluoroethylene grafts in 8 patients,3.5 mm in 11,4 mm in 65,5mm in 26.Results The duration of mechanical ventilation was 4 hours-7 days,cardiac intensive care unit stay was 1-12 days,hospital stay after operation was 1-18 days.There were 3 deaths (2.6％),immediate post-operative shunt block in 1,severe hypoxidosis in 1 and heart failure in 1.Oxygen saturation increased from 68.5％ preoperatively to 79.2％ postoperatively.Four patients had shunt block,additional shunt was created respectively.There was 2 late death.Follow-up of 94 patients revealed satisfactory systemic oxygen saturation of 0.81 ± 0.10 (0.63-0.92).86 cases received further operations,including 7 in TOF radical operation,31 in Rastelli,25 in Glenn,18 in Fontan.Conclusion With an encouraging early shunt patent rate and oxygen saturation increasing,we can now adopt MBTS as an alternative in patients with severely cyanotic heart anomalies.Modified B-T shunt is a good palliation for patients with cyanoti cheart anomalies,which can increase pulmonary blood flow.Excellent surgical skills and perioperative treatment contribute to good operation results,and to low morbidity and low mortality.

Contrast research in the application of pulmonary artery banding in the single ventricle repair pathway in different age patients with pulmonary artery hypertension / 中华胸心血管外科杂志

ObjectiveContrast research the operative effect of the pulmonary artery banding (PAB) in the congenital heart disease with pulmonary artery hypertension,underwent the single ventricle repair procedure in different age.Methods 49 cases,male 31 and female 18.Age (7.8 ± 3.8 ) years old,weight ( 8.82 ± 4.24) kg,percutaneous blood oxygen saturation ( SPO2 ) 0.90 ± 0.04,preoperative mean pulmonary artery pressure (mPAP) ( 54.6 ± 16.8 ) mm Hg.single ventricle 13cases,tricuspid atresia 12 cases,double outlet of right ventricle with left ventricular dysplasia 11 cases,tricuspid stenosis 5 cases,ventricular imbalance type complete atrioventricular canal defect 5 cases and cross heart cases 3 cases.divided into three groups:≤0.5 years old of 17 cases,0.5 to 2.0 years old of 17 cases,≥2.0 years old 15 cases.All cases taken PAB under anesthesia and cpb,inhaled oxygen concentration 40％,SPO2 0.85,MPAP 20 mmHg.comparatively analysis postoperative SPO2,MPAP,Ventilator using time,ICU stay time and operation mortality of the three groups.ResultsThe postoperative PAP obviously decreased.Three groups of postoperative SPO2,mPAP,ventilator using time,ICU stay time were not significantly different.More than were followed up 6-72 months,1 cases ( 1-2 months) dead in aspiration two months postoperation.the other three cases had been completed Glenn and/or Fontan.ConclusionPAB can effectively reduce the pulmonary artery pressure in the different age children with congenital heart disease and pulmonary artery hypertension,the effect is good.more than 2 years old children still PAB feasible operation,and likely to complete the Glenn and/or Fontan procedure.

Recent Trends in Indications of Fetal Echocardiography and Postnatal Outcomes in Fetuses Diagnosed as Congenital Heart Disease

BACKGROUND AND OBJECTIVES: We hypothesized that fetal echocardiography (echoCG) is an accurate diagnostic tool reflecting well postnatal echoCG findings and outcomes. SUBJECTS AND METHODS: We reviewed the medical records of 290 pregnant women, including 313 fetuses, who were examined by fetal echoCG at the Seoul National University Children's Hospital from January 2008 through April 2011. RESULTS: The mean gestational age at diagnosis was 26.2+/-5.2 weeks. The mean age of mothers at diagnosis was 31.7+/-3.8 years. We identified indications for fetal echoCG in 279 cases. The most common indication was abnormal cardiac findings in obstetrical screening sonography (52.0%). Among the 313 echoCG results, 127 (40.6%) were normal, 13 (4.2%) were minor abnormalities, 35 (11.2%) were simple cardiac anomalies, 50 (16.0%) were moderate cardiac anomalies, 60 (19.2%) were complex cardiac anomalies, 16 (5.1%) were arrhythmias, and 12 (3.8%) were twin-to-twin transfusion syndrome. The most common congenital heart disease was tetralogy of Fallot (23 fetuses, 15.9%). One hundred forty-eight neonates were examined by echoCG. We analyzed differences between fetal echoCG and postnatal echoCG. In 131 (88.5%) cases, there was no difference; in 15 (10.1%), there were minor differences; and in only 2 (1.4%) cases, there were major differences. CONCLUSION: There is a recent increase in abnormal cardiac findings of obstetric ultrasonography screenings that indicate fetal echoCG. Fetal echoCG is still a good, accurate diagnostic method for congenital heart disease.

Cliuical analysis of surgical diagnosis and treatment of vascular ring in infants and children / 中华胸心血管外科杂志

Objective To explore the diagnosis and surgical treatment experience of vascular ring in infants and children.Methods Fourteen cases (9 boys and 5 girls,aged 2 months to 6 years,weighted 4.5 - 15.0 kg) with vascular ring were diagnosed and treated surgically in Children's Hospital of Chongqing Medical University from Sep.2009 to Dec.2010.All children underwent X-ray,echocardiography and spiral computed tomography examination preoperatively.Bronchoscopy and barium swallow was performed in 5 cases respectively.The pathological types of vascular rings included double aortic arch in 1 ( 7.1％ ),pulmonary artery sling in 7 (50.0％),right aortic arch with left patent ductus arteriosus or persistent left ligament in 6 (42.9％).Associated cardiac anomalies were present in 7 (50.0％) patients.Tracheal stenosis of different length ( 12％ -62％ ) and severity (45％ -74％ cross-sectional luminal narrowing) was observed in the group.Barium swallow in 5cases showed localized compression of the esophagus.12 cases underwent repair of vascular ring with cardiopulmonary bypass (CPB),and the associated congenital heart defects were repaired simultaneously.2 cases of right aortic arch with left patent ductus arteriosus or persistent left ligament underwent surgery without CPB.Results The median duration of CPB in 12 cases and aortic cross-clamp time in 7 patients were 77.5 minutes ( range:55 - 186 minutes) and 36 minutes ( range:22 - 110 minutes) respectively.The median duration of postoperative ventilation and ICU stay were 21 hours (range:7 -308 hours) and 79.5 hours (range:16-314 hours) respectively.One baby with pulmonary artery sling died on the postoperative 12th day ( in-hospital mortality 7.1％ ).Of the 13 cases discharged from the hospital,1 case were lost to follow up.In the follow-up ( 1- 15 months) of 12 cases,digestive symptoms were disappeared.Development,exercise tolerance and symptoms showed obvious improvement,although 5 (41.7％) patients had residual respiratory problems.Conclusion Prolonged or recurrent aerodigestive issues in children should alert the pediatrician to the possibility of a vascular ring.Multislice spiral CT scanning is the best imaging modality.All vascular rings should be surgically corrected,and the associated long-segment severe tracheal stenosis needs.The short to midterm outcomes of surgical division are excellent.

Application of inhaled nitric oxide in the early period after Fontan operation / 中国小儿急救医学

Objective To evaluate the effects of inhaled nitric oxide(INO) in the early period after Fontan operation. Methods Twenty children receiving INO [(10 ～ 25) × 10 -6] after fight heart bypass for congenital heart disease were evaluated. The changes of hemodynamic and respiratory parameters were observed at two different intervals. The levels of methemoglobin (MetHb)and nitrogen dioxide(NO2)were monitored. Results In INO therapy group,CVP decreased from (20.8±4.8) mm Hg to (14.3±1.8) mm Hg,TPG decreased from (16.3 ±3.0) mm Hg to (8.8±2. 1) mm Hg,PaO2/FiO2 increased from (70.3±16.9) mm Hg to (120.5 ±14.0) mm Hg,and arterial oxygen saturation increased from 0. 78 ±0. 14 to 0. 91 ±0. 09 ,blood lactic acid level decreased from (3.98 ± 0. 86) mmol/L to (1.29 ± 1.60) mmol/L. No toxic side effect was observed. Conclusion INO has no significant long-term effect on patients after Fontan operation, but it can improve respiratory function, and reduce the incidence of low cardiac output syndrome and high cava pressure caused by reactive elevation of pulmonary vascular resistance in the early postoperative period after Fontan operation.

Uso del "balón con cuchillas" para la ampliación de una comunicación interauricular restrictiva / Blade Balloon Atrial Septostomy to Enlarge a Restrictive Atrial Septal Defect

La presencia de una comunicación interauricular no restrictiva es obligatoria para mantener un gasto cardíaco adecuado o para lograr una mezcla de sangre aceptable en algunos pacientes portadores de cardiopatías congénitas complejas. Describimos un paciente en quien luego de un intento fallido de dilatación estática con balón de una comunicación interauricular restrictiva se utilizó un balón con cuchillas con éxito para su ampliación.

A nonrestrictive atrial septal defect is mandatory in order to maintain an adequate cardiac output or to achieve an acceptable blood mixing in some patients with complex congenital heart defects. We successfully performed a blade balloon septostomy in a patient with a restrictive atrial septal defect who had previously undergone a failed static balloon dilation of the atrial septum.

Electron-beam CT diagnosis of congenital cardiovascular diverticula / 中华放射学杂志

Objective To investigate the clinical application of electron-beam CT(EBCT)in the diagnosis of congenital cardiovascular diverticula. Methotis Retrospective analysis of 9 patients with congenital cardiovascular diverticula confirmed by operation and pathology was done.Of them,enhanced continuous volume scan was performed on 8 patients and enhanced single slice scan was performed on one patient with an Imatron C-150 scanner.Results The group of 9 pailents included one patient with diverticulum of the left ventricle.3 patients with diverticulum of the atria and 5 patients with diverticulum of the aorta.EBCT scan and three dimensional reconstruction could demonstrate not only the origin,size,shape,Location and adjacent structure of diverticula,but also other important complicated abnormalities such as ventrieuloarterial connection disorder,cardiac sepud defect,aortic coarctation and even dissection.Conclusion EBCT is an ideal noninvasive technique in the diagnosis of congenital cardiovascular diverticula

Diagnosis of Congenital Dextrocardia with Complex Anomalies by Echocardiography / 国际儿科学杂志

0.05),the specificity of transthoracic echocardiography in diagnosing the atrial situs,ventricular structure,the connection of the ventricle and arteries or auricle,arterial situs was more than 90%.In mirror-image dextrocardia,majority had concordant segmental connection and could present anomalies with simple anomalies(42.9%) including atrial septal defect and ventricular septal defect,in addition,the major complex anomalies such as pulmonary stenosis were tetralogy of Fallot,double outlet of right ventricle(50%);In dextroversion,majority had discordant segmental connection and mostly presented anomalies with pulmonary stenosis or pulmonary atresia,including single ventricle,double outlet of right ventricle,transpo sition of great arteries,pulmonary atresia and so on(88.6%).Conclusion Echoca rdiography can exactly diagnose the most congenital dextrocardia with complex cardiovascular anomalies,and can be the first or alternative screening choice for diagnosing dextrocardia.

Comparison of oral Sildenafil and intravenous prostaglandin E_1 in treatment of pulmonary hypertension associated with congenital heart defects after cardiac surgery / 中华急诊医学杂志

Objective To compare the effects of oral Sildenafil(SIL)and intravenous prostaglandin E_1(Alprostadil,PGE_1)in treatment of postoperative pulmonary hypertension(PH)associated with congenital heart defects(CHD).Methods Data were collected from 24 children with postoperative mPAP≥35 mmHg in our hospital between August 2004 and March 2005.These children were randomly divided into three groups:8 children(group A)initially received SIL(0.35 mg/kg,orally by nasal gastric tube),followed by the addition of intravenous PGE_1(20 ng/kg per min)at 40 min.The second group of 8 children(group C)initially received intravenous PGE_1 followed by the addition of SIL at 20 min(dose as above).And remains(group B)were placebo-controlled.The changes of hemodynamic variables,arterial blood gas,lung static compliance(C)and work of breathing(W)were measured after drugs were given.Results Compared with group B,the mPAP and mPAP/mSAP was significantly reduced(P

Surgical and Long Term Results for Double Outlet Right Ventricle by the Type of Ventricular Septal Defect / 대한흉부외과학회지

BACKGROUND: The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. MATERIAL AND METHOD: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1~44) and 86 patients (68.3%) were male. We classified and studied this disease by the type of VSD. RESULT: The locations of VSD were subaortic in 79 (62.7%), subpulmonary in 17 (13.5%), doubly committed in 16 (12.7%) and noncommitted in 14 (11.1%). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0+/-45.1 months. The methods of total correction were intraventricular baffling in 37 (29.4%), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 (38.9%), intraventricular baffling with Rastelli procedure in 15 (11.9%), arterial switch operation in 8 (6.3%) and REV procedure in 4 (3.2%), etc. Hospital mortality rate was 10.3% (13 patients) and 25 reoperations were performed in 24 patients (19.0%). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9+/-70.7 months. The percent survival and survival for freedom from reoperation at 15 years were 82.5% and 66.7%, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. CONCLUSION: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

Somatostatin for Postoperative Chylothorax after Surgery for Children with Congenital Heart Disease

Chylothorax is a rare but serious postoperative condition with a high rate of morbidity that may lead to death of children with congenital heart disease. Here we reviewed nine consecutive cases with chylothorax in infants and children following cardiac surgery from March 2002 to February 2003. Somatostatin was added to conservative treatment proctocol to increase effectiveness of therapy in all cases. The duration of somatostatin therapy varied from 7 to 32 days. All cases of chylothorax were successfully treated with intravenous infusion of somatostatin as an adjunctive treatment. Even though two cases showed rebound phenomena, we avoided any surgical procedure in the nine patients who treated with conservative management combined with somatostatin. No significant side effects of somatostatin were observed. It seems that somatostatin is effective, noninvasive and safe therapeutic modality. It can be used as an adjunctive treatment to conservative management to control postoperative chylothorax in children with congenital heart disease.