Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Tamizaje de cardiopatías congénitas en el neonato mediante oximetría de pulso en el Hospital Materno Infantil José Domingo de Obaldía. Agosto 2014- febrero 2015. / Screening of congenital heart disease in neonates through pulse oximetry. Hospital Materno Infantil José Domingo de Obaldia. August 2014 - February 2015

Introducción. Existen evidencias sólidas que demuestran la utilidad de realizar de manera sistemática una prueba de saturación de oxígeno a todos los recién nacidos aparentemente sanos después de 24 horas de nacido y antes de 48 horas; a esta prueba se le ha llamado "tamiz neonatal para cardiopatías congénitas" y sirve para detectar oportunamente anomalías cardiacas estructurales congénitas que cursan con hipoxemia. El tamiz de cardiopatías congénitas puede ayudar a reconocerlas tempranamente y posibilitar su mejor evolución. Objetivos. Evaluar el uso de la oximetría de pulso como herramienta de detección de las cardiopatías congénitas en los recién nacidos asintomáticos. Resultados. Se realizó la prueba de tamizaje cardíaco mediante oximetría de pulso a 2236 recién nacidos sanos asintomáticos, nacidos en el Hospital Materno Infantil José D. de Obaldía (HMIJDDO) en un período de seis meses (agosto 2014-febrero 2015). De esta muestra se obtuvo un tamiz positivo en 16 de los recién nacidos evaluados (0.72%). A los cuales se les realizó ecocardiograma que reportó: 6 casos (37.5%) con un corazón estructuralmente normal, 6 (37.5%) con Ductus Arteriosos Persistentes, 3 (18.75%) Conexiones Anómalas de Venas Pulmonares, y sólo en un caso (6.25%) se presentó un Síndrome de Ventrículo Derecho Hipoplásico, Hipoplasia de Válvula Tricúspide, Atresia pulmonar vs Estenosis Crítica de Válvula en Domo, Conducto Arterioso Grande y Tortuoso, Sospecha de Fístulas Ventrículo-Coronarias. Conclusión. Los padres y los médicos deben saber que una prueba de saturación normal, no descarta la presencia de otras cardiopatías congénitas que no cursan con hipoxemia. Una vez tengamos capacitado un personal que realice este tamizaje, podemos poner la oximetría como control para todos, pero no como un tamiz aislado, sino como un dato más para una fisiopatología que estemos pensando.

Introduction. There is solid evidence that demonstrate the utility of testing in a systematic way of oxygen saturation all the healthy newborn infants apparently after 24 hours old and within 48 hours; This proof has been called "screening neonatal for congenital heart disease" and serves to opportunely detect congenital structural cardiac abnormalities that present with hypoxemia. Sieve of congenital heart disease may help recognize them early and facilitate their better development. Objectives. Evaluate the use of pulse oximetry as a tool for detection of congenital heart diseases in the newborn asymptomatic. Results. The cardiac screening using pulse oximetry test was performed to healthy newborns 2236 asymptomatic, born in the Hospital Materno Infantil José Domingo de Obaldía (HMIJDDO) in a period of six months (August 2014-February 2015). This sample was obtained a positive screen in 16 of the newborns assessed (0.72%). Which was les echo reported: 6 (37.50%) with a structurally normal heart, 6 (37.50%) Arteriosus persistent ductus, 3 (18.75%) Anomalous pulmonary venous connections. And only in one case (6.25%) presented a right hypoplastic ventricle, tricuspid valve, pulmonary Atresia hypoplasia syndrome vs valve stenosis-criticism in dome, large patent ductus arteriosus and tortuous, suspicion of ventricle-coronary fistula. Conclusion. Parents and physicians should know that a normal saturation test, doesn´t rule out the presence of other congenital heart disease who dont present with hypoxemia. Once we have trained staff who perform this screening, we can put the oximetry as a control for all, not as an isolated sieve, but one fact more for a pathophysiology that we are thinking.

Adolescentes con cardiopatía congénita: conocimientos sobre su enfermedad, sexualidad y posibles complicaciones de un embarazo / Adolescents with congenital heart disease: knowledge about their disease, sexuality and possible complications during pregnancy

Introducción. Las jóvenes tienen su primera experiencia sexual en edades tempranas; si padecen una cardiopatía, el riesgo de morir se eleva cinco veces durante el parto. Objetivo. Describir los conocimientos que tienen las adolescentes sobre su cardiopatía congénita (CC), sexualidad, embarazo y las complicaciones que esto conlleva. Material y métodos. Estudio descriptivo, transversal y prospectivo realizado previo consentimiento informado de los padres. Se aplicó cédula ex profeso a 30 mujeres hospitalizadas de 12 a 18 años de edad con alguna CC. Cédula con información sociodemográfica y sobre conocimientos de su enfermedad, sexualidad y embarazo, con respuestas dicotómicas, opción múltiple y abiertas. Datos analizados con medidas de dispersión y tendencia central. Resultados. Edad promedio 14.6 años; 10% con vida sexual activa iniciada entre 13-17 años. Todas conocen su tratamiento, 90% sabe qué enfermedad padece. 77.3% tiene confianza con su madre para platicar de sexualidad; 60% considera que es por falta de información el embarazo; 73.4% conoce dos o más métodos anticonceptivos, principalmente condón, aunque 29.9% piensa erróneamente que todos protegen de enfermedades de transmisión sexual; 73.3% considera poderse embarazar aún con cardiopatía. Conclusiones. La mayoría de adolescentes está informada, pero es imperativo el desarrollo de estrategias educativas que disminuyan la problemática.

Introduction. Today, young people have their first sexual experience at early ages. In addition, if they suffer from heart disease, the risk of dying raises five times during childbirth. Objective. Describe the knowledge they have about their adolescent congenital heart disease (CHD), sexuality, pregnancy and the complications that entails. Material and methods. We performed a descriptive, transversal and prospective study prior informed consent of the parents. A charter expressly 30 woman 12 to 18 years of age with some kind of CC, hospitalized was applied. The schedule consisted of sociodemographic information and knowledge about his illness, sexuality and pregnancy with dichotomous response, multiple choice and open. We analyzed the data with measures of central tendency and dispersion. Results. Average age 14.6 years; 10% initiated sexual activity between 13 and 17 years. All know their treatment, 90% know what disease you have and what stage. 77.3% have confidence with her mother to talk about sexuality; 60% believe that teenage pregnancy is due to lack of information; 73.4% know two or more birth control. The best known is the condom, while 29.9% mistakenly think that some contraceptive methods protect against sexually transmitted diseases; and 73.3% consider being able to still pregnant with heart disease. Conclusions. Most teens know about their disease, contraception and sexuality; however, it is imperative to develop educational strategies to reduce the problem.

The clinic report of 566 consecutive cases of Fontan operation and analysis early risk factors / 中华胸心血管外科杂志

Objective To summarize 566 consecutive cases of Fontan operation in the treatments of complex congenital heart diseases and to analyze the risk factors of early surgical outcome.Methods We reviewed all 566 consecutive patients who underwent a Fontan procedure in Shanghai Children's Medical Center from January 2006 to May 2014.Preoperative records of staged surgeries,perioperative data and imaging results were obtained from medical records.Results A total of 566 patients(M/F =345/221) underwent a Fontan operation,with 166 cases of single stage Fontan operation and 400 cases of multi-staged operation.The median age at the time of the Fontan procedure was 5 years,and the median weight was 15.4 kg.In our series,there were 278 extracardiac conduits,110 intracardiac conduits,114 lateral tunnels,36 intracardiac/extracardiac conduits,and 27 direct cavopulmonary conduits.There were 36 cases of early Fontan failure,with overall early postoperative survival rate being 93.6％.The median ventilation duration was 8 h,with the median postoperative intensive care unit stay being four days,the average oxygen saturation being 0.90 at the end of ICU stay,the median chest drainage time being 10 days,and the median postoperative hospital stay being 19 days.Besides the CICU oxygen saturation,the rest postoperative results had no obvious differences in five surgical groups.Heterotaxy syndrome,earlier age at the time of the Fontan procedure,bilateral Glenn shunt,moderate AV valve regurgitation and severe and right ventricular-type morphology were risk factors of early Fontan failure,P ＜ 0.05.Conclusion In order to improve the early outcome of Fontan operation,closely follow-up should be taken directly after Glenn operation in high-risk patients.Doctors should also have an in-depth understanding of the of postFontan physiological and pathological features,and select the operation time and Fontan type most suited to the of the individualized characteristics.

Outcome assessment of different surgeries for neonates with pulmonary atresia and ventricular septal defect / 中国胸心血管外科临床杂志

@#Objective To explore the feasibility and option of different surgeries for neonates with pulmonary atresia and ventricular septal defect (PA/VSD) through assessing the effect of common surgeries. Methods Fourteen neonates who underwent their first surgery in our center from July 2004 to October 2014 were included. Their basic characteristics, operation and pre- and postoperative clinical information were extracted. Follow up was conducted and the last visit was on October 10, 2016. Short- and midterm survival and total correction rate were compared among different surgeries. Results Among the 14 patients, there were 4 (28.6%) patients, 6 (42.9%) and 4 (28.6%) who underwent one-stage repair, right ventricular outflow tract (RVOT) reconstruction, and systemic to PA shunt operation respectively. The overall in-hospital mortality after the first operation was 28.6% (4/14). At last visit, no death occurred resulting the 5-year survival rate of 71.4% (10/14). The overall total correction rate for all neonates was 64.3% (9/14). Although no statistical difference was found in the mortality among the one-stage repair , RVOT reconstruction and systemic to PA shunt group(50.0% vs. 33.3% vs. 0.0%, P=0.280), the survival and hazard analysis implied better outcomes of the systemic to PA shunt palliation operation. There was no statistical difference in the total correction rate and months from the first palliative operation to correction between those who underwent RVOT reconstruction and systemic to PA shunt (75.0% vs. 50.0%, P=0.470; 32.0 months vs. 18.0 months, P=0.400). Conclusion Performing surgeries for neonates with PA/VSD is still a great challenge. However, the midterm survival rate was optimistic for the early survivors. Systematic to PA shunt seemed to be a better choice with lower mortality for the neonates with PA/VSD who need the surgery to survive.

One-stage surgical repair of interrupted aortic arch in neonates and young infants:surgical experiences, follow-up re-sults and subsequent treatments / 中华胸心血管外科杂志

Objective To discuss one-stage surgical repair of interrupted aortic arch ( IAA) in neonates and young in-fants, summarize follow-up results and subsequent treatments.Methods From September 2010 to December 2014, a series of 38 consecutive neonates and young infants ( M/F =26/12 ) with IAA were admitted in our surgical group to receive surgical treatment, with a median age of 11 days(1 day to 5 months) and a median body weight of 4.1 kg(1.8 to 5.8 kg).IAA was type A in 24 cases, type B in 13 cases and type C in 1 case.Associated cadiovascular anomalies were common except 2 cases, including atrial septal defect(n=23), ventricular septal defect(n=36), left ventricular outlet tract obstruction(LVOTO)(n=7), anomalous left pulmonary artery from aorta(n=1), truncus arteriosus(n=1).There were 5 cases of airway stenosis in 28 cases of CT angiography and 3D airway reconstruction.All the 38 cases were admitted with differential cyanosis.36 cases were recieved one-stage surgical repair except 2 cases died before operation.35 cases of IAA associated with intracardiac anom-alies were repaired through a median sernotomy.Cardiopulmonary bypass(CPB) was performed with two canulations in ascend-ing aorta and main pulmonary artery.Selective cerebral perfusion(n=21) or circulatory arrest(n =14) was carried out with body temperature of 18℃-23℃.After the patent ductus arteriosus tissue was completely resected , a continuous end-to-end or end-to-side suturing with 7/0(8/0) prolene was performed.In 1 type B cases with expected excessive anastomotic tension, the left subclavian artery was cut off to release the aortic arch .The associated anomalies were repaired during the cooling or re-warming period.Chest close was delayed to carry out no more than 24-72 h postoperatively in 16 cases.The other one type A IAA case without intracardiac anomaly was repaired through a standard left posterior lateral incision .Patients were strictly fol-lowed up with a standard protocol.Complications such as aortic arch restenosis, new LVOTO, new airway stenosis, and so on, were specialy focused on during the follow-up period.If needed, reoperation or transcatheter intervention was applied to treat the complications.Results Two cases died before operation and another 2 died in the early period postoperatively because of pulmonary hypertension crisis and severe tracheal stenosis, respectively.Sudden death was in 1 case during the follow-up peri-od.Median CPB time was 138 min(105-208 min) and median selective cerebral perfusion or circulatory arrest time was 24 min(16-35 min) .Mechanical ventilation time was 2-25 days.Blood pressure difference between the upper and lower limbs was less than 20 mmHg in all the patients before discharge.29 cases(85.29%) was followed up for 28 months(9 -60 months).3 cases were received reoperation in 5 cases of newly detected LVOTO.One case received reoperation in the 7 preop-erative LVOTO cases because of newly detected LVOTO(blood pressure gradient more than 40 mmHg).Two cases with aortic arch restenosis received percutaneous transcatheter ballon dilation .One case with new left main bronchus stenosis after opera-tion was strictly followed up.Conclusion IAA is a rare and severe congenital heart disease.Positive surgical repair should be performed after definite diagnosis being made .Although satisfactory results can be obtained with one-stage primary operation , IAA is a kind of progressive chronic disease.New postoperative LVOTO should be focused on becasue it will need reoperation or even the third operation .

The diagnosis and treatment of scimitar syndrome in 6 cases / 中华胸心血管外科杂志

Objective Investigate the clinical features,diagnosis and treatment of the scimitar sydrome.Methods A retrospective analysis of clinical data of six children with scimitar syndrome from 2008 to 2014,including clinical symptoms and signs,chest X-ray,echocardiography,chest CT and cardiac catheterization,treatment outcome and follow-up and review of the literature.Results Six patients with scimitar syndrome were diagnosed between 2008 and 2014.There were 3 girls and 3 boys;5 cases ＜ 6 kg in weight.Scimitar syndrome was suspected in 5 cases because of extroversion,and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava.Computed tomography confirmed the diagnosis in all patients.One patient had horseshoe lung,3 had a unilobar right lung,3 had a hypoplastic right lower lobe.Three patients had severe pulmonary arterial hypertension,1 had moderate to severe pulmonary arterial hypertension,and 2 had moderate pulmonary arterial hypertension.All patients had lower respiratory tract infections,volume loss of the right lung,a normal or hyperinflated left lung,dextroversion of the heart,and scimitar arteries from the descending aorta.4 cases of pulmonary venous drainage correction surgery,3 underwent body-pulmonary embolism,1 case of 9-year-old asymptomatic children without surgery,and 1 patient with heart failure,severe pulmonary hypertension,pulmonary infection,septicemia died before surgery.Conclusion Clinically,found children with heart of dextrocardia position,growth retardation,recurrent lung infections,unexplained right heart failure,pulmonary hypertension,should consider the possibility scimitar syndrome.Early diagnosis and early treatment of pulmonary venous drainage and pulmonary vascular embolization,can significantly improve the clinical symptoms in children and reduce mortality.

The value of transcutaneous oxygen saturation in early diagnosis of newborns with critical congenital heart disease / 中国新生儿科杂志

Objective To study the value and significance of transcutaneous oxygen saturation monitoring from different parts in early diagnosis of neonatal critical congenital heart disease. Methods The transcutaneous oxygen saturation in right upper extremities and right lower extremities of neonates born in our hospital from July 2010 to July 2015 were detected at 2 hours to 24 hours after birth. If the transcutaneous oxygen saturation ≤ 0. 95 or difference between upper and lower extremities≥0. 03, color doppler ultrasound echocardiography were done. Results There were 24 385 cases of newborns in our study. 1 680 newborns with abnormal transcutaneous oxygen saturation ( ≤0. 95 or difference between upper and lower extremities ≥0. 03) were examined with color doppler ultrasound echocardiography. 204 cases of congenital heart disease (12. 1% ) contain 9 cases of critical congenital heart disease ( 0. 5% ) were detected. The critical congenital heart disease included complete transposition of great artery ( n = 3), tetralogy of Fallot ( n = 4), artery malformation ( n = 1), and pulmonary atresia with right ventricular dysplasia ( n = 1 ) . Conclusions Transcutaneous oxygen saturation monitoring from different parts has a high value for early detection of critical congenital heart disease in the newborn. It is recommended for clinical use in grass-roots hospitals.

Evaluating right heart function after right ventricle-pulmonary anastomosis for right ventricle outflow reconstruction / 中华胸心血管外科杂志

Objective To evaluate the right heart function with echocardiography after right ventricle-pulmonary artery (RV-PA) anastomasis for right ventricle outflow (RVOT) reconstruction in patients with different types of pulmonary atresia and ventricle septal defect(PA/VSD).Methods From Nov 2002 to Aug 2013,31 patients with PA/VSD had undergone right ventricle-pulmonary anastomasis to reconstruct RVOT for radical or palliative repair.Related echocardiography indexs including strain/rate etc.were used to evaluate the right heart function and the progress of the right heart valves regurgitation.Results There were 3 early hospital deaths.No later death during follow-up.The echocardiography suggested the pulmonary artery and tricuspid regurgitation were more serious,however,the right heart function was relatively fine.The regurgitation of tricuspid valve was positive correlation with duration of follow-up (P =0.016).Conclusion The right heart function in follow-up keeps relatively well,and tricuspid valve regurgitation needs a long-term follow-up.

Morphological and Functional Evaluation of Quadricuspid Aortic Valves Using Cardiac Computed Tomography

OBJECTIVE: The aim of this study was to identify the morphological and functional characteristics of quadricuspid aortic valves (QAV) on cardiac computed tomography (CCT). MATERIALS AND METHODS: We retrospectively enrolled 11 patients with QAV. All patients underwent CCT and transthoracic echocardiography (TTE), and 7 patients underwent cardiovascular magnetic resonance (CMR). The presence and classification of QAV assessed by CCT was compared with that of TTE and intraoperative findings. The regurgitant orifice area (ROA) measured by CCT was compared with severity of aortic regurgitation (AR) by TTE and the regurgitant fraction (RF) by CMR. RESULTS: All of the patients had AR; 9 had pure AR, 1 had combined aortic stenosis and regurgitation, and 1 had combined subaortic stenosis and regurgitation. Two patients had a subaortic fibrotic membrane and 1 of them showed a subaortic stenosis. One QAV was misdiagnosed as tricuspid aortic valve on TTE. In accordance with the Hurwitz and Robert's classification, consensus was reached on the QAV classification between the CCT and TTE findings in 7 of 10 patients. The patients were classified as type A (n = 1), type B (n = 3), type C (n = 1), type D (n = 4), and type F (n = 2) on CCT. A very high correlation existed between ROA by CCT and RF by CMR (r = 0.99) but a good correlation existed between ROA by CCT and regurgitant severity by TTE (r = 0.62). CONCLUSION: Cardiac computed tomography provides comprehensive anatomical and functional information about the QAV.

Effect of Dexmedetomidine Hydrochloride Injection on Awakening Quality in Children with Congenital Heart Disease Undergoing Fast_track Anesthesia and Video_assisted Thoracoscopic Surgery / 医药导报

Objective To study the effect of dexmedetomidine hydrochloride injection ( Dex) on awakening quality in Pediatric Patients with congenital heart disease undergoing fast_track anesthesia and whole Video_assisted thoracoscoPic surgery. Methods One hundred and twenty Pediatric Patients ( ASA I orIIleVel) with congenital heart diseases were randomly chosen for this study. They receiVed femoral artery and Vein cannulation to establish cardioPulmonary byPass and three small holes were obtained in the right chest for the PurPose of undergoing the whole Video_assisted thoracoscoPic surgery on rePairmen of atrial sePtal defect or simPle Ventricular sePtal defect. They were randomly diVided into two grouPs: Dex_assisted fast_track anesthesia grouP (D grouP,n=60) and general fast_track anesthesia grouP (C grouP,n=60). Each one in grouP D was injected with loading dose of Dex (1 μg·kg-1),then was intraoPeratiVely infused with maintenance dose of 0. 5 μg·kg-1·h-1. Children in grouP C were giVen the same dose of saline in the same way. Hemodynamic changes in the two grouPs at each time Point:before anesthesia ( t0 ) ,at the time of extubation ( t1 ) ,5 min Post_extubation ( t2 ) ,10 min Post_extubation ( t3 ) ,15 min Post_extubation ( t4 ) , 30 min Post_extubation (t5) and at the time of transferring out of CICU (t6),resPectiVely,were obserVed. Restlessness extent, incidence,time of eye oPening after calling, fully awaking time, extubation time, the time of transferring out of CICU and VAS scores,were also recorded. Results From t0 to t2-t5 ,SBP in D grouP was decreased from (114. 2±10. 5) mmHg to (107. 2±10. 3) -(105. 3±11. 3) mmHg,DBP decreased from (61. 3±9. 2) mmHg to (58. 8±7. 8) -(57. 3±6. 3) mmHg,and HR gradually decreased from (95. 2±15. 7) time·min-1 to (85. 7±13. 7)-(83. 3±12. 6) time·min-1,with significant differences (P0. 05). VAS at 30 min after extubation was significantly higher in grouP C than in grouP D(4. 7±0. 7 vs. 2. 4±0. 6,P<0. 05). Conclusion Injection of Dex with loading dose (1 μg·kg-1) and intraoPeratiVe infusion of maintenance dose of 0. 5 μg·kg-1·h-1 in children with congenital heart disease undergoing fast_track anesthesia and whole Video_assisted thoracoscoPic surgery could be conduciVe to maintain hemodynamic stability, reduce restlessness occurrence, enhance security during awakening Process, and alleViate Post_oPeratiVe Pain.

Effectiveness and safety of high frequency oscillation ventilation in serious pulmonary hemorrhaege after surgical repair of congenital heart disease / 中华胸心血管外科杂志

Objective Experience with high-frequency oscillatory ventilation (HFOV) after congenital cardiac surgery is limited.The aim of the present study was to investigate the effect and safety of high frequency oscillation ventilation for patients with serious pulmonary hemorrhaege after surgical repair of congenital heart disease (CHD).Methods The patients with serious pulmonary hemorrhaege after surgical repair of CHD using conventional mechanical ventilation or high frequency oscillation ventilation were retrospectively analyzed.From January 2010 to July 2012,there were fourteen patients suffered from serious pulmonary hemorrhaege after surgical repair of congenital heart disease in our hospital and all involved in this study.The mean age was (6.5 ± 5.9) months(ranged from 1 to 24 months) and the mean body weight was (5.8 ± 1.7) kg(ranged from 3.7 to 10 kg).Before May 2011 patients with serious pulmonary hemorrhaege after surgical repair of congenital heart disease were treated with conventional mechanical ventilation(CMV group,n =6),and after May 2011 high frequency oscillation ventilation (HFOV group,n =8) instead.The diagnoses were pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries 7 cases,tetralogy of Fallot with imbalance pulmonary arterial development 5 cases,and total anomalous pulmonary venous connection 2 cases.Heart rate,arterial blood pressure,central venous pressure(CVP),inostmpic score,the blood gas analysis,the oxygenation index(OI),the outcomes and complications during both mechanical ventilations were all recorded.Results The PaO2,OI and systolic blood pressure of patients in HFOV group were significant higher than those in CMV group after 2 hours ventilation.There was no statistical difference in PCO2,diastolic blood pressure and inostropic score after 1,2,4,8,24 hours ventilation.All cases had no significant changes in hemodynamics.CVP of patients in HFOV group increased slightly(P ＜0.05),but there was no statistical difference in arterial blood pressure.Four patients died in CMV group.The mortality of CMV group and HFOV group was 66.7％ (4/6) and 37.5％ (3/8,one with pulmonary venous obstrution,one with tracheobronchial blocked,the other with re-hemorrhaege) respectively.The mortality of HFOV group was no significantly different from that of CMV group.Conclusion Compared to CMV,using HFOV achieved greater oxygenation function in patients with serious pulmonary hemorrhaege after surgical repair of CHD rapidly,had no significant changes in hemodynamics and rare serious complications.HFOV was effective and safe for patients with serious pulmonary hemorrhaege after surgical repair of congenital heart disease.

Changes in Patient Characteristics of Infective Endocarditis with Congenital Heart Disease: 25 Years Experience in a Single Institution

BACKGROUND AND OBJECTIVES: The profile of infective endocarditis (IE) has changed and is now showing an increasing prevalence of IE among congenital heart disease (CHD) patients. We studied the change of clinical profiles of IE over the past 25 years in patients with CHD at a single institution. SUBJECTS AND METHODS: We reviewed medical records retrospectively for 325 patients diagnosed with IE between January 1, 1987, and March 31, 2012. We analyzed and compared the differences in patient characteristics and outcomes between 1987-2000 (group A) and 2001-2012 (group B). RESULTS: Over the 25-year period, 93 cases of IE in CHD patients were diagnosed (59 cases in group A and 34 cases in group B). Ventricular septal defect was the most common underlying cardiac disease observed during the entire period. The most common causative pathogen was Streptococcus in both groups. Group A contained 16 cases (27.1%) that had undergone cardiac surgery, whereas this number was 19 (55.8%) in group B. The number of patients who had undergone palliative care or surgery using prosthetic materials was higher among group B patients (p<0.001). Surgical procedures due to uncontrolled infection were performed in three cases in group A and 10 cases in group B. CONCLUSION: Infective endocarditis and CHD show a close correlation, and the profile of IE patients can change in line with an increase in the survival rate of patients with complex CHD and the improvement of surgical techniques. Ongoing reassessment and the systematic management of these patients is crucial in the prevention and treatment of IE.

Diagnostic methods and surgical treatment of the left superior vena cava draining into the left atrium / 中华胸心血管外科杂志

Objective To review our experiences of diagnostic methods and surgical treatment of the left superior vena cava (LSVC) draining into the left atrium.Methods Nineteen patients with LSVC draining into the left atrium were diagnosed and treated surgically from February 1998 to January 2012.All the cases were combined with other congenital heart diseases including patent ductus arteriosus,ventricular septal defect,atrial septal defect,single atrium,triatriatum,partial endocardial cushion defect,anomalous pulmonary venous drainage,right ventricle outflow stenosis,pulmonary valve stenosis,tetralogy of Fallot,double outlet right ventricle,complete endocardial cushion defect,tricuspid atresia.The patients were diagnosed through different methods including echocartiographic examination,cardiac catheterization,computer tomography,and explored during the operation,even postoperatively.All were treated surgically with four techniques including simple ligation to the LSVC,including ligation during a redo procedure; intra atrial rerouting to drainage the flow from the LSVC to the right atrium,atrial septum reconstruction to make the outlet of the LSVC lying in the right side of the patched atrial septal,and bidirectional Glenn shunt to get a physiological result.Results No mortality postoperatively.All the cases were uneventful postoperatively.And the main postoperative course was related only to the main diagnosis of congenital heart disease,not to the left superior vena cava draining into the left atrium.The echo examination result was satisfied before the discharge.The early and long term follow-up(1-11 years) results are excellent,no arrhythmia,no cardiac deficits after echo examination,including stenosis obstruction and residual shunt.No death.Conclusion The diagnostic methods of the LSVC draining into the left atrium are difficult,the only way to make the diagnosis clearly enough before the operation is depending on improving of more and more comprehending to this rare cardiac anomaly,by the pediatric cardiologists,the sinologist,the intensive care unit,and the pediatric cardiae surgeons.The choice of different surgical treatment is depending on the diagnosis and the findings during the operations,and the results are excellent after a suitable choice made.

Off-pump occlusion of trans-thoracic minimal invasive surgery on simple congenital heart diseases / 中华胸心血管外科杂志

ObjectiveReport an operation of off-pump occlusion of trans-thoracic minimal invasive surgery (OPOTTMIS) adopted on the therapy of 92 patients suffered with simple congenital heart diseases (CHD) of atrial septal defect ( ASD),ventricular septal defect ( VSD ) and patent ductus arteriosus (PDA) and summarize the experiences.Methods The clinical data of 92 CHD patients administrated with OPOTTMIS in our hospital during the time of July 2008 and July 2011 were analyzed retrospectively.Moreover,the relevant literatures were reviewed and the different kinds of treatments and their outcomes of CHD were compared.These patients were 3-56 years old and weighed 8.0-54.5 kg,with male and female 38 and 54 cases,including ASD 52 cases,VSD 26 cases and PDA 14 cases,respectively.Within the initial 3 months of postoperation,all the patients were followed up and managed to take the re-examination with electrocardiogram and echocardiogram one time for each month,and 3 months later,at least one time for every 3-6 months.Results91 cases were performed the OPTTMIS procedure successfully,1 child with sieve-like ASD transferred to open-heart surgery and 1 adult( 1.1％ ) PDA died on the second day after operation for the pulmonary hypertension crisis.4 cases appeared mild residual shunt including 2 of ASD,1 of VSD and 1 of PDA.2 cases showed Ⅱ stage atrial ventricular block ( AVB ) within ASD and VSD,respectively.Also,post-operation hemothorax happened to 1 case ASD and 1 case VSD for the active bleeding of cardiac puncture site and the exploration and hemostatic operation were performed.91 (98.9％) patients were performed the OPOTTMIS operation successfully,the total complications within 72 hours was 6.5％ (6/92),respectively.There were no heavy complications happened during peri-operation such as cardiac rupture,infective endocarditis,stroke,hemolytic and thrombus formation.90 patients outcomes of 3 months to 4 years follow-up with the ECG and UCG examination showed that there were no Ⅲ stage atrioventricular block,no obvious occluder shift and broken and no moderate cardiac valve regurgitation,no evident blood flow restriction of left ventricular outflow tract and descending aorta,only found mild residual shunt within 2 cases ( VSD and PDA each) and heart expansion compared to pre-operation within 2 PDA patients.However,all the patients'heart functions were in grade Ⅰ to grade Ⅱ according to NYHA standard.ConclusionOPOTTMIS is a safe,feasible and effective option on simple congenital heart diseases.

Surgical correction of supracardiac total anomalous pulmonary venous connection using sutureless technique / 中华胸心血管外科杂志

Objective Retrospectively analyze surgical correction supracardiac total anomalous pulmonary venous con-nection (TAPVC) using sutureless technique to prevent post-repair pulmonary vein stenosis. Methods Between December 2007 and December 2008, 25 children cases of total anomalous pulmonary venous connection underwent primary surgical cor-rection. The anatomic types of TAPVC were supra cardiac in nine patients, inha cardiac in one, mixed in 3 and intra cardiac in 12. Five of nine supra cardiac TAPVC underwent correction using sutureless technique. There were 2 male and 3 female. Their age at surgeries ranged from 2 months to 13 years, and the body weight were from 4.5 kg to 2.1 kg with an average of (7.9±6.4) kg. After median sternotomy and opening the pericardium, the heart was arrested by delivering cold crystal ear-dioplegia. The heart was then positioned toward the patient' s right and under the right henri sternum. A generous incision across the posterior wall of the left atrium and one on the common pulmonary vein was made. The latter was extended upwards to the midpoint of the vertical vein. The left atrium was subsequently connected to the pulmonary venous confluence by suturing the edge of the atrium to the posterior mediastinal pericardium that surrounding the common pulmonary vein and the vertical vein with 7-0 PDS. The vertical vein was partially ligated after conclusion of CPB, leaving a diameter of 5mm shunt. Routine follow-up with echocardiogram were at diacharging, 3 months, 6 months and 1 year after surgery. Results All 5 cases survived uneventfully excopt 1 baby on ventilation over 7 days after surgery, who had bilateral lung consolidation before the operation. Echocardiogram showed satisfactory results with maxium velocity acrossing the anastomosis of 0.65 -0.85 m/s. Conclusion Sutureleas technique can avoid trauma to the pulmonary venous endothelium and minimize the tension of anastomasis. It may play an important role to prevent post-repair pulmonary vein stenosis. More patients with long-term follow-up are necessary to draw a definite conclusion of this technique.

Color Doppler echocardiographic characteristics of bilocular heart and associated cardiac malformations / 中华超声影像学杂志

Objective To probe into the color Doppler echocardiographic characteristics of bilocular heart (BH) and associated malformations. Methods Twenty patients of BH were examined with color Doppler echocardiography (CDE) and the characteristics were observed. All the CDE results were compared with angiocardiography and 14 were confirmed by operation. Results Nineteen cases were properly diagnosed based on the CDE characteristics,1 case were misdiagnosed as mitral atresia. CDE characteristics of BH were obvious:① Apical four-chamber view of two-dimensional echocardiography (2DE) showed total echo dropout in both the interatrial septum and the interventricular septum and disappearance of the intracardiac "cross". The common atrioventricular valve closed during systole and appeared as figure "8", named 2DE "8" sign. During diastole, the common atrioventricular valve opened to a common ventricle. ② Color Doppler flow imaging (CDFI) showed common intra-atrial flow signal entereda common ventricle through common atrioventricular valve during diastole in all patients and colorful reflux through common atrioventricular valve during systole in 15 cases. ③ Among the 20 eases, 16 of the common atria were situs solitus,4 were situs inversus; 10 of the common ventricles were type A,2 were type B and 8 were type C. According to the spatial relationship of the great arteries,there were 4 type Ⅰ ,7 type Ⅱ and 9 type Ⅲ.There were 18 pulmonary stenosis and 2 pulmonary hypertension. ④ In the presence of pulmonary stenosis, CDFI showed colorful shunt signals through pulmonary artery during systole. Conclusions BH and associated malformations have obvious echocardiographic characteristics. CDE has a specific value in diagnosing BH and associated malformations.

One stage surgical correction of congenital heart disease and tracheal stenosis in infants / 中华胸心血管外科杂志

Objective To review and evaluate the effect of one stage surgical correction of congenital heart disease with tracheal stenosis in infants.Methods Between August 2001 and December 2005,six infants with congenital heart disease with tracheal steno- sis were repaired at one stage operation.The age was 24 d～3 y[mean(13.16?12.03)months]and the weight was 4.2～10.0 kg [mean(7.98?2.03)kg].There were 3 patients with tetralogy of Fallot,1 with pulmonary atresia;2 with ventricular septal defect, 1 with coarctation of aorta;and 1 with pulmonary sling.The congenital heart disease were repaired underwent low temperature and car- diopulmonary bypass,and the tracheal stenosis were corrected simultancously.Results There was one death postoperative because of right and left branch stenosis.One patient with pulmonary sling died of granulation tissue on the patch 3 months after operation.All other 4 patients recovered uneventfully.These patients were followed up from 6 month to 4 years.There were no complications.CT scan shows that the tracheal anastomosis was patent without any stenosis.Conclusion One stage repair of congenital heart disease with tracheal stenosis in infants may have a satisfactory result.To deal with the tracheal stenosis is difficult,the operative methods de- pends on the length of stenosis.The principles of surgical methods consist of using autologous material,maintaining its contour and function of respiratory epithelium,and maintaining its growth.

Diagnosis and surgery of left coronary artery abnormally originating from pulmonary artery (10 cases reports) / 中华胸心血管外科杂志

Objective To summarize clinical characteristics, diagnosis and surgery of abnormally origination of left coronary artery from the pulmonary artery. Methods Clinical data of 10 patients with left coronary artery abnormally originating from pulmonary artery were analyzed, including 5 men and 5 women, aged from 13 to 40 years. Definite diagnosis was made by ultrasonic cardiogram (UCG) and cardiac catheter examination. Three cases were simply abnormal origination, six cases combined with MI, and one case combined with both MI and ventricular aneurysm of left ventricular apex. Ligation of the abnormal coronary artery was done in four patients, three were given pulmonary artery inner tunnel plasty under extrocorporeal circulation. Open implantation of left coronary artery to ascending aorta were done in 3 patients, while plasty of mitral valve were performed in 5 and ventricular aneurysm resection in 1. Results One patient died postoperatively. The follow-up ranged from 1 month to 11 years. One patient received replacement of mitral valve 16 months after first surgery due to severe MI. All the followed-up patients presented no myocardial ischemia or infarction, no residual shunt or late death. Cardiac function was rehabilitated to grade 1. Conclusion Obvious blood dynamics and cardiovascular morphology changes existed in patients with left coronary artery abnormally originating from pulmonary artery. Early diagnosis and surgery should be done. Proper surgical approach is the key to success.

Changes of hydrogen sulfide and heme oxygenase-1 in blood of children with congenital heart disease and pulmonary hypertension before and after surgery / 中国病理生理杂志

0.05).There was a negative correlation between PASP and the level of H2S in 3 groups at time points of pre-operation,1 h and 24 h post-operation (pre-operation,r=-0.66,P