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Objectives To mitigate the shortage of homograft sources, the use of handmade trileaflet expanded polytetrafluoroethylene valves in pulmonary valve replacement has shown excellent results from multicentre studies conducted in Japan. However, world-wide data outside Japan is relative insufficient. This study presents the long-term results of a single surgeon’s use of flipped-back trileaflet method in a 10-year case series. Methods We have developed an efficient way to make a trileaflet-valved conduit utilizing flipped-back method for pulmonary valve replacement and have employed the technique since 2011. Retrospective data were studied between October 2010 and January 2020. Echocardiography, electrocardiogram, Pro-Brain Natriuretic Peptide, and Magnetic Resonance Imaging data were analyzed. Results 55 patients were reviewed and median follow-up duration was 2.9 years. The majority of diagnoses was Tetralogy of Fallot (n = 41), and these patients subsequently underwent secondary pulmonary valve replacement at a median age of 15.6 years. Survival was 92.7% with the longest follow-up period being 10 years. There was no need for reoperation, and freedom from reintervention was 98.0% at 10 years. There were 4 deaths (3 in-hospital and 1 outpatient). One patient eventually received transcatheter pulmonary valve implantation. Postoperative echocardiography showed mild or less pulmonary stenosis and pulmonary regurgitation degree in 92.2% and 92.0% of patients, respectively. Comparable magnetic resonance imaging data (n = 25) showed significant reduction in right ventricular volumes but not in ejection fractions. Conclusion Our series showed satisfactory long-term function of handmade flipped-back trileaflet valved conduit used in our patients. The simple design is efficiently reproducible without complex fabrication process.

Objective: To present an accurate prenatal diagnosis of coarctation of the aorta with ventricular septal defect and to illustrate how early diagnosis in prenatal period with proper referral and counseling can optimize management. Case report: A case with coarctation of the aorta with ventricle septal defect was found to have an abnormal three vessel view at 12 weeks, and with close follow-ups, coarctation of the aorta with ventricle septal defect was diagnosed at 24 weeks. Following the support from a multidisciplinary team that provided counseling, diagnosis, and follow-ups, the pregnant woman decided to continue with the pregnancy and had a vaginal delivery at a medical center. The newborn made an uneventful recovery after undergoing cardiac surgery on day 9. Conclusion: The case demonstrates the role a fetal medicine team plays in diagnosing, supporting, and seamlessly transferring the congenital heart disease case from the first line obstetrician to the cardiac surgeon. A multi-disciplinary team approach was able to lead to improved perinatal outcome of the congenital heart disease case. Keywords: Aorta, Fetal, Prenatal, Ultrasound, VSD

OBJECTIVES Many surgeons develop unique techniques for unmet needs for right ventricular outflow reconstruction to resolve pulmonary regurgitation after corrective surgery for congenital heart diseases. Expanded polytetrafluoroethylene (ePTFE) stands out as a reliable synthetic material, and clinical results with handmade ePTFE valves have been promising. This review focuses on the historical evolution of the use of ePTFE in pulmonary valve replacement and in the techniques for pioneering the translation of the handmade ePTFE trileaflet design for the transcatheter approach. METHODS We searched for and reviewed publications from 1990 to 2020 in the Pubmed database. Nineteen clinical studies from 2005 to 2019 that focused on ePTFE-based valves were summarized. The evolution of the ePTFE-based valve over 3 decades and recent relevant in vitro studies were investigated. RESULTS The average freedom from reintervention or surgery in the recorded ePTFE-based valve population was 90.2% at 5 years, and the survival rate was 96.7% at 3 years. CONCLUSIONS Non-inferior clinical results of this ePTFE handmade valve were revealed compared to allograft or xenograft options for pulmonary valve replacement. Future investigations on transferring ePTFE trileaflet design to transcatheter devices should be considered.

Objectives A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geometries has been reported in patients with repaired tetralogy of Fallot (rTOF). We aimed to investigate the associations between RVOT/PA geometries and outcome indicators in a large rTOF cohort receiving non-conduit repair. Methods Three-dimensional magnetic resonance angiographic images of 206 patients with rTOF who had a pulmonary regurgitation (PR) fraction ≥20% were reviewed. Patients' RVOT geometry was quantitatively classified into 4 distinct shapes (tubular, hourglass, pyramid, and inverted trapezoid). Bilateral PA size discrepancy was defined as the diameter of the smaller side being less than 70% of that of the bigger side. Results Based on lateral projection of the 3-dimensional images, patients with an inverted trapezoid-shaped RVOT had the smallest RV end-diastolic volume index (EDVi) (108.7 ± 24.3 mL/m2) and pulmonary valve annulus diameter, and shortest QRS duration, whereas those with a pyramid-shaped RVOT had the largest RV EDVi (161.0 ± 44.6 mL/m2) and pulmonary valve annulus diameter. Similar trends of differences were also observed if such classifications were based on the frontal projections. Multivariable analysis revealed that RVOT shapes, subvalvular diameter, PR fraction, QRS duration, and the presence of bilateral PA size discrepancy were independent determinants of RV EDVi. Furthermore, having bilateral PA size discrepancy (25.2%) was independently associated with lower peak oxygen consumption (P = .041). Conclusions Distinct RVOT morphologies and branch PA size discrepancy are associated with variations in RV remodeling and exercise capacity in patients with rTOF. These findings may aid decision-making regarding reintervention for PR and branch PA size discrepancy.

OBJECTIVES Handmade trileaflet expanded polytetrafluoroethylene valved conduit developed using the flip-over method has been tailored for pulmonary valve reconstruction with satisfactory outcomes. We investigated the in vitro performance of the valve design in a mock circulatory system with various conduit sizes. In our study, the design was transformed into a transcatheter stent graft system which could fit in original valved conduits in a valve-in-valve fashion. METHODS Five different sizes of valved polytetrafluoroethylene vascular grafts (16, 18, 20, 22 and 24 mm) were mounted onto a mock circulatory system with a prism window for direct leaflets motion observation. Transvalvular pressure gradients were recorded using pressure transducers. Mean and instant flows were determined via a rotameter and a flowmeter. Similar flip-over trileaflet valve design was then carried out in 3 available stent graft sizes (23, 26 and 28.5 mm, Gore aortic extender), which were deployed inside the valved conduits. RESULTS Peak pressure gradient across 5 different sized graft valves, in their appropriate flow setting (2.0, 2.5 and 5.0 l/min), ranged from 4.7 to 13.2 mmHg. No significant valve regurgitation was noted (regurgitant fraction: 1.6–4.9%) in all valve sizes and combinations. Three sizes of the trileaflet-valved stent grafts were implanted in the 4 sizes of valved conduits except for the 16-mm conduit. Peak pressure gradient increase after valved-stent graft-in-valved-conduit setting was CONCLUSIONS The study showed excellent in vitro performance of trileaflet polytetrafluoroethylene valved conduits. Its valved stent graft transformation provided data which may serve as a reference for transcatheter valve-in-valve research in the future.

OBJECTIVES We report our surgical experience in congenitally corrected transposition of great arteries (CCTGAs) and the long-term follow-up result. METHODS From January 1995 to February 2012, 56 patients with CCTGA received definite surgical repair; 15 patients received conventional repair (Group I), 18 patients received anatomical repair (Group II) and 23 patients received single ventricular palliation (Group III). They were followed for early and late mortality, long-term survival, postoperative morbidity and reintervention or reoperation. RESULTS The overall survival rate was 80% at 16 years in Group I, 53% at 13 years in Group II and 100% at 13 years in Group III. After excluding the early surgical mortality, the long-term survival rate was 92% at 16 years in Group I, 64% at 13 years in Group II and 100% at 13 years in Group III. Patients with significant tricuspid valve regurgitation showed the worst outcome after surgery. CONCLUSIONS Our series showed good results with single ventricular palliation (SVP) in CCTGA with complex anatomy, but the long-term result should be followed. Anatomical repair is the choice of operation only for those with favourable anatomy. The more complicated intracardiac repair may result in late left ventricular outflow tract obstruction, various degrees of atrioventricular block, systemic or pulmonary venous return obstruction and the lack of an ideal conduit (e.g. homograft) for Rastelli reconstruction. Therefore, we preferred SVP in patients with complex and unfavourable anatomy.

Background Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. Methods Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3). Results All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). Conclusions Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.

Genetic syndromes, especially 22q11 deletion (del22q11) syndrome, are common in patients with pulmonary atresia and ventricular septal defect (PA-VSD), but their association with long-term outcomes varies. The purpose of this study was to evaluate the long-term outcome after complete repair of PA-VSD and to determine the impact of genetic syndromes.We reviewed our experience of 125 patients with PA-VSD who received primary or staged repair between 1978 and 2010. Evaluations for genetic syndromes included clinical features, cytogenetic analysis, and fluorescence in situ hybridization or multiplex ligation-dependent probe amplification.Genetic syndromes were documented in 26 patients (20.8%), including del22q11 in 16 patients, trisomy 21 in 2 patients, and other syndromes in 8 patients. The prevalence of hypoplastic pulmonary arteries was not significantly different between the syndromic and nonsyndromic groups. After 1,069 patient-years of follow-up, 20-year survival was 90% ± 6% in patients without syndromes and 14% ± 23% in patients with syndromes (p0.01). Multivariate analysis identified the presence of a genetic syndrome as an important risk factor for hospital and late mortality. Subgroup analysis showed that genetic syndromes other than del22q11 were associated with worse outcome. The rate of 10-year freedom from cardiac reintervention after repair was 53% ± 11%, with hypoplastic pulmonary arteries before repair as a major risk factor (p = 0.02).Genetic syndromes significantly affect survival after repair of PA-VSD, whereas genetic syndromes do not represent additional risk for reintervention. Repair is feasible in patients with syndromes, but suboptimal long-term outcome should be addressed when counseling parents.

Background Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which the connection between the pulmonary vein (PV) and left atrium needs to be surgically created. This study investigated the spectrum and outcome of a Taiwanese cohort. Methods Isolated TAPVC cases were identified from our institutional database between 1995 and 2009. We reviewed the medical chart and conducted telephone interviews with those lost to follow-up. Results There were 78 patients (52% male). The anomalous drainage sites were mainly supracardiac type (42.3%) and cardiac type (39.8%). Before operation, PV stenosis was found in 100% of infracardiac type, and in 69.7% of supracardiac type. Among the 75 patients undergoing operation, the surgical mortality was 9% (7/75). Perioperative arrhythmias (mainly of atrial origin) occurred in 35% of the patients. Of the 68 patients who survived the first operation, 28 (41%) developed pulmonary vein restenosis. Half of them progressed to severe PV stenosis, which required reintervention or resulted in mortality. Preoperative PV stenosis was the most significant predictor for postoperative PV restenosis and PV re-intervention. For the cohort, the 1-year and 5-year survivals were 78.9% and 74.2%, respectively, and the predictor for survival was again preoperative PV stenosis. Conclusion The surgical mortality of isolated TAPVC is now low. Preoperative PV stenosis not only increased the risk of late PV restenosis and its reintervention, but also the overall mortality. The spectrum of PV drainage, per se, was not associated with worse outcome. PV restenosis remained the most important issue after correction of TAPVC.

Objectives Pulmonary artery sling is frequently associated with tracheal and/or bronchial stenosis. A number of patients receive only re-implantation or relocation of the left pulmonary artery (LPA) while other patients may require tracheoplasty for stenosis of the airway. This study used computer tomography (CT) scans for the evaluation of airways and to evaluate the trachea diameter and the clinical outcomes with or without tracheoplasty. Methods A total of 15 patients with PA sling who received CT scans were included in this study. We reviewed the clinical outcomes and the severity of trachea stenosis, and evaluated various surgical strategies. Results Among the 15 patients, one received only conservative treatment, 14 (93%) patients received LPA re-implantation, and eight (53%) received tracheoplasty. For the eight patients that received LPA reimplantation and tracheoplasty, five received slide tracheoplasty and all survived, two patients had pericardial patch augmentation and both died, and one patient received a resection and end-to-end anastomosis and survived. The diameter of the trachea in survivors without tracheoplasty was significantly larger than those who died or received tracheoplasty (3.2 ± 0.3 mm vs. 2.2 ± 0.4 mm, P = 0.003). Establishing 3.0 mm as the threshold, the positive predictive value for tracheoplasty or mortality was 89% and the negative predictive value was 83%. Conclusions In this study cohort, approximately 60% of the patients with PA sling received tracheoplasty. A diameter of the trachea

Background Infected aneurysm of the suprarenal abdominal aorta is rare and can be fatal without surgery. There have been only sporadic case reports or small case series. We review our experience with 14 patients over 13 years. Methods Retrospective chart review. Results Between 1997 and 2010, 14 cases of infected aneurysms of the suprarenal abdominal aorta were treated at our hospital. There were 11 men with median age of 75.5 years (range, 35-88). Of the 13 pathogens isolated, the most common responsible microorganism was nontyphoid Salmonella in eight (62%) followed by Staphylococcus aureus in three (23%) and Streptococcus in two patients (15%). At the first admission, six patients had medical treatment alone, five patients underwent early open in situ graft repair, and three patients underwent hybrid endovascular stenting and visceral debranching. Of the six medically treated patients, two patients died in the hospital because of aneurysm rupture, and two patients underwent late open in situ graft repair because of aneurysm progression or rupture. Of the five open surgically treated patients, one patient died in the hospital because of nosocomial sepsis, and four patients were alive without major postoperative complication. Of the three endovascularly treated patients, one patient died in the hospital because of intestinal ischemia, one patient died 6 months later because of postoperative complication, and one patient was alive with complications of paraplegia, renal failure, and permanent dialysis. The aneurysm-related mortality rate was 33% (2/6) in medical treatment alone, 20% (1/5) in open in situ grafting, and 67% (2/3) in hybrid endovascular stenting. Conclusions Infected aneurysm of the suprarenal abdominal aorta was rare. Nontyphoid Salmonella was the most common responsible microorganism. Open in situ graft repair remained a preferred and durable treatment strategy.

article i nfo Objectives: Uncorrected congenital heart defects (CHD) with severe pulmonary hypertension (sPH, systolic pulmonary arteryN70% of systolic pressure) are usually considered inoperable. We are curious to know if some selected patients might benefit from palliative operation for those sPH with uncorrected CHD. Methods: Adults or adolescents with sPH associated with ventricular septal defect (VSD) with/without great artery anomalies were selected for pulmonary artery banding (PAB) to reduce sPH. The target pulmonary pressure was less than half of the systolic blood pressure after arch or great arteries reconstruction. Repeated catheterization was performed to evaluate the feasibility of defect closure. Results: Consecutively, 8 patients (age 26±9 years) received PAB as a palliative procedure in the past 8 years without mortality. The pre-PAB systolic pulmonary pressure was 119±9 mmHg. Additional PAB had been applied in 4 of them. All patients showed significant improvement in function class (III to I or II). The mean post-PAB pulmonary pressure decreased significantly (77.5±9.2 mmHg to 42.0±9.0 mmHg) and 6-minute walk test was also found to have great improvement (270±86 m to 414±49 m), but the saturation at rest did not show a difference. Three of them received corrective surgery to close defects over 3-5 years. Conclusion: For some selected adult sPH with uncorrected CHD, PAB can work as a palliative procedure to improve their functional class and even provide a chance of total repair.

OBJECTIVE Malignancy is the leading cause of death among heart-transplant recipients. There is a higher incidence of post-transplant malignancy in heart-transplant recipients than in kidney-transplant recipients. This study sought to assess the incidence of malignancy in heart-transplant recipients in Taiwan. METHODS This is a retrospective chart review. RESULTS From 1987 to 2008, 291 patients who underwent heart transplantation and survived for more than 1 month were enrolled. Seventeen patients (5.8%) developed de novo malignancies including skin cancers (three), post-transplant lymphoproliferative diseases (seven) and solid-organ malignancies (seven). Solid-organ malignancies affected prostate, liver, urinary bladder, kidney, lung, larynx, pancreas and brain in seven patients. Malignancy was responsible for 7% and 13% of all death for heart-transplant recipients who lived for more than 1 year and more than 5 years. The cumulative incidence of 1.03% at 1 year, 4.2% at 5 years and 8.1% at 10 years in our patients was much lower than the incidences reported in the multicentre registry of the International Society for the Heart and Lung Transplantation and in the Western series. The incidence was especially low for skin cancers. Compared with previous reports of kidney-transplant recipients in Taiwan, the incidence of post-transplant malignancy was not significantly increased. CONCLUSIONS The incidence of post-transplant malignancy was low in Chinese heart-transplant recipients compared with heart-transplant recipients in Western countries. It resulted from a relative rarity of skin cancers in the Chinese population.

Pulmonary regurgitation after repair of tetralogy of Fallot has always been an important issue for pediatric cardiac surgeons. Some of the patients may need pulmonary valve replacement in the future. Conventional prosthesis valves were seldom custom made for the pulmonary valve and were often inadequate to use because of variable age and size of these patients. Through evolution of techniques, we have developed a simple, efficient, and theoretically anatomic way of constructing trileaflet polytetrafluoroethylene valved conduit used for right ventricle outflow tract reconstruction. It was surprisingly straightforward, time efficient, and capable of being produced in various sizes.

Late cardiovascular complications after intervention for coarctation of the aorta (CoA) might be common. Such data, especially in Asian populations, are still limited. A total of 169 patients with CoA who survived balloon (n = 41) or surgical (n = 128) intervention from 1986 to 2004 were enrolled. The total follow-up was 1,776 patient-years, and the patient age at last follow-up was 14.7 +/- 8.5 years. Reintervention for recoarctation, systemic hypertension, and left ventricular outflow tract obstruction (LVOTO) was subsequently noted in 57 (34%), 36 (21.3%), and 24 (14.2%) patients, respectively, without significant differences between the balloon and surgical groups. The 20-year freedom from reintervention, systemic hypertension, and LVOTO rate was 52%, 78.6%, and 80.8% in the balloon group and 59%, 51%, and 81.9% in the surgical group, respectively. The age at CoA intervention and the aortic geometry after CoA intervention of gothic type were common risk factors for reintervention for recoarctation and late systemic hypertension. In contrast, the presence of bicuspid aortic valves and gothic arch geometry after CoA intervention increased the risk of LVOTO. Plasma renin/aldosterone activities were not elevated in those with or without systemic hypertension. In conclusion, the profile of reintervention for recoarctation and late systemic hypertension after CoA interventions in our Asian cohort with CoA was similar to that observed in white patients. Asian patients might have a lower risk of LVOTO.

Congenital left main coronary artery (LMCA) atresia is a rare anomaly and surgical revascularization is recommended once the diagnosis is confirmed. In some cases, LMCA atresia will coexist with other cardiac anomalies, mostly in pediatric patients. A 32-year-old woman had congenital LMCA atresia associated with patent ductus arteriosus (PDA), aortic regurgitation (AR), and moderate pulmonary hypertension. The PDA and AR were repaired surgically and the patient's symptoms improved. Coronary revascularization was not performed because the LCA was supplied by 2 large conus arteries, as "naturally occurring" bypass arteries. The patient remained asymptomatic at the 2.5-year follow-up visit. Given the positive outcome in this patient, surgical repair of the associated cardiac defect without coronary revascularization may be a reasonable approach for patients with congenital LMCA atresia, in which the left coronary arterial tree is supplied by other "naturally occurring" bypass arteries.

Background Infected aneurysm of the aorta is almost always fatal without undergoing aortic resection. Medical treatment was attempted selectively in patients who were considered too high risk for surgery. We review our experience with 22 patients treated without undergoing aortic resection over 12 years. Methods Retrospective chart review. Results Between 1995 and 2007, 22 cases of infected aortic aneurysms treated without undergoing aortic resection during the first admission were included. There were 17 men with a median age of 76 years (range, 35 to 88 years). Of 18 pathogens isolated, the most common responsible microorganism was nontyphoid Salmonella in 11 followed by Staphylococcus aureus in five. The site of infection was thoracic in eight and abdominal in 14. The hospital mortality rate was 50%, and the aneurysm-related mortality rate after long-term follow-up was 59%. The event-free survival rate at one year was 32%. Of 11 patients with Salmonella infection, eight patients have lived beyond 30 days and six were event-free after one year. Of 11 patients with non-Salmonella, four patients have lived beyond 30 days and only one was event-free after one year. The overall aneurysm-related mortality rate was 36% in Salmonella infected patients and 82% in non-Salmonella infected patients. Conclusion Clinical results of medical treatment using current antibiotics in patients with infected aortic aneurysm were poor. Traditional surgical excision of infected aortic aneurysms with revascularization remains the gold standard and should be attempted except in high risk patients.

Background/Purpose: The optimal management of interrupted aortic arch (IAA) with ventricular septal defect is controversial. The aim of this study was to evaluate our 12 years of experience of surgical outcomes of one-stage and rapid two-stage total corrections of IAA with ventricular septal defect and to delineate the management of postoperative complications. Methods: We reviewed the medical charts of all patients from 1996 to 2007. Neonates with inherent complex anatomy were excluded. There were 26 patients in our series, with 11 type A and 15 type B IAA. Nineteen patients received one-stage repair and seven patients received rapid two-stage total correction. Rapid twostage total correction was defined as two operations performed within 1 week. Results: The 1-month postoperative survival rate was 81% (21/26), with 79% (15/19) in the one-stage group, and 86% (6/7) in the rapid two-stage group. The rapid two-stage group had a shorter cardiopulmonary bypass time (160.1 ± 58.4 vs. 216.8 ± 73.7 minutes, p = 0.054) and aortic cross clamp (AXC) time (65.6 ± 24.4 vs. 91.8 ± 22.4 minutes, p = 0.022) than the one-stage group. Postoperative left ventricular outflow tract obstruction (LVOTO) and aortic arch restenosis were common in survivors, with frequencies of 48% (10/21) and 71% (15/21) respectively. Within the postoperative arch stenosis subgroup, nine out of 15 patients received balloon angioplasties, which proved effective after only one treatment. The overall late survival rate was 73% (19/26), with 68% (13/19) in the one-stage group, and 86% (6/7) in the rapid two-stage group. Conclusion: The outcome of rapid two-stage repair is comparable to that of one-stage repair. Rapid twostage repair has the advantages of significantly shorter cardiopulmonary bypass duration and AXC time, and avoids deep hypothermic circulatory arrest. LVOTO remains an unresolved issue, and postoperative aortic arch restenosis can be dilated effectively by percutaneous balloon angioplasty. [J Formos Med Assoc 2008;107(11):876–884]

Ebstein's anomaly is a rare, congenital cardiac anomaly that may result in cyanosis, right heart failure, and tachyarrhythmia during the newborn stage or after adolescence. This study investigated the data of 77 patients diagnosed between 1980 and 2006 at a tertiary care center in Taiwan. Patients were grouped into either an early group or a late group. Survival declined rapidly within the newborn stage in the early group, but declined only during the third decade in the late group. Surgical results were poor (20% success rate) for neonatal systemic-to-pulmonary shunts in those cases with associated pulmonary atresia, but were satisfactory for other surgical modes. Supraventricular tachyarrhythmia occurred in 31 (41%) patients at a median age of 10 years and could be eliminated by radiofrequency ablation (81% success rate), though the recurrence rate was high (41%). In conclusion, other than those cases requiring shunts at the newborn stage, the long-term outcome was favorable.

AIM: The aim of this study was to define the improvement in short-term outcome and risk factors of Norwood stage one reconstruction for hypoplastic left heart syndrome (HLHS) in Taiwan, after implementing new perioperative management strategies. METHODS: Data were retrieved from a retrospective chart review of patients with HLHS treated between July 1997 and July 2007. Since we implemented new perioperative strategies in 2004, we divided our patients into two groups, early era (1997 - 2003) and late era (2004 - 2007), and compared the outcome. RESULTS: We enrolled 48 patients. In the early era group (n = 28), the diagnosis was confirmed by cardiac catheterization and controlled ventilation was used to manipulate the balance between systemic and pulmonary blood flow. The survival rate was only 17.9 % (5/28). Surgery was performed at 15.2 ± 10.7 days, which was significantly later than in the late era group (4.6 ± 4.0 days, n = 20). A lower preoperative shock and more prenatal diagnoses were recorded for the late era group. RV-PA conduit was used in 17 patients in the late era group of which 12 (70.6 %) survived to be discharged from hospital. The risk factor was significant TR (triscupid regurgitation). CONCLUSIONS: With our contemporary perioperative management and change in surgical strategy, survival after first-stage palliation has improved. We believe that our HLHS experience is valuable for low volume centers and also for Asian cohorts.

OBJECTIVE: To describe survival and neurologic outcome and identify the factors associated with survival among pediatric patients following extracorporeal cardiopulmonary resuscitation (ECPR) for in-hospital cardiac arrest. DESIGN: Retrospective study. SETTING: A university-affiliated tertiary care hospital. PATIENTS: Eligible patients were < or = 18 yrs of age and received extracorporeal membrane oxygenation during active cardiopulmonary resuscitation for in-hospital cardiac arrest. INTERVENTIONS: Extracorporeal membrane oxygenation (ECMO) during active cardiopulmonary resuscitation. MEASUREMENTS AND MAIN RESULTS: The primary outcome was survival to hospital discharge. The secondary outcome was neurologic status after ECPR at hospital discharge and late follow-up. Good neurologic outcome was defined as Pediatric Cerebral Performance Categories 1, 2, and 3. Continuous variables were expressed as medians (interquartile range). We prospectively defined the early cohort (January 1999 to December 2001) and late cohort (January 2002 to January 2006) and compared the survival rates. We identified 27 ECPR events. The survival rate to hospital discharge was 41% (11 of 27). The nonsurvivors had higher pre-cardiopulmonary resuscitation serum lactate levels (14 [10.2-19.6] mmol/L vs. 8.5 [4.4-12.6] mmol/L, p < .01), longer durations of cardiopulmonary resuscitation (60 [37-81] mins vs. 45 [25-50] mins, p < .05) with longer activating time for ECMO (12.5 [7.5-33.8] mins vs. 5 [0-10] mins, p < .01), and more renal failure after ECPR (68% [11 of 16] vs. 9% [1 of 11], p < .01). The survival rate of the late cohort was better than that of the early cohort (58% [11 of 19] vs. 0% [0 of 8], p < .05). By exact multiple logistic regression analysis, the early cohort and renal failure after ECPR were two independent risk factors for mortality. Among the 11 survivors, ten had good neurologic outcomes. CONCLUSIONS: ECPR successfully rescued some pediatric patients who failed rescue with conventional in-hospital CPR. Good neurologic outcomes were achieved in the majority of the survivors. Early cohort and post-ECPR renal failure were associated with mortality.

This study is based on a database of 16 years; we sought to define the incidence and outcome of scimitar syndrome. Of 8,771 patients, 5 (0.057%) with scimitar syndrome were identified and constituted the study population. Follow-up ranged from 1 to 16 years (median: 10 years). Diagnosis was assured by computed tomography in four patients and by cardiac catheterization in one. Two patients presented with respiratory distress soon after birth and required early pneumonectomy in one case and coil embolization of the abnormal feeding arteries to the right lower lung followed by surgical rerouting of the abnormal pulmonary vein and repair of the atrial septal defect in the other case. The former was supported by ventilator therapy for 3 years after pneumonectomy, but was finally weaned from the ventilator. Among the other three, two had repeated pneumonia that resolved after rerouting of the abnormal right pulmonary vein and cardiac repair. The asymptomatic child did not receive any intervention. In spite of the abnormal orientation of the airways, none of the four patients with detailed computed tomography imaging showed any significant compression of the airways. All five patients were doing well as of the last follow-up. In conclusion, scimitar syndrome is a very rare disease in this Asian country and the varied symptoms, such as tachypnea and repeated infection, could be improved after interventions.

Heart transplantation is indicated for children with end-stage heart failure or complex inoperable congenital defects. Due to the shortage of pediatric donor hearts, various bridge techniques have been used for pediatric recipients to prolong patient survival until a heart is available. This study evaluates long-term outcome of bridge and nonbridge support for pediatric heart transplantation. Between March 1995 and June 2004, 18 pediatric patients underwent heart transplantation. Six patients (33.3%) underwent biological or mechanical bridge techniques before transplantation. Eight patients (44.4%) required perioperatively extracorporeal membrane oxygenation (ECMO) support. Patient data and records were retrospectively reviewed. Causes of death and long-term outcome were analyzed. Five of eight patients in the ECMO group (62.5%) were successfully decannulated and discharged home with excellent functional classes. No differences in rejection rate, survival rate, and functional class existed between the bridged and nonbridged groups. Overall 1-year and 5-year survival rates were both 83.3% and all have a good functional class. Pediatric heart transplantation can be accomplished with excellent early survival despite multiple prior cardiac operations and relatively severe illness. For the variety in small, low-body-weight pediatric patients, mechanical circulatory support using ECMO is suitable for managing sudden collapse while waiting for heart transplantation, and graft dysfunction after cardiac transplantation. The mortality rate is acceptable in this very high-risk group of patients and long-term outcome is good.

Summary. Unilateral lung agenesis is a rare congenital defect and could be associated with multiple abnormalities. The patients usually have poor long-term outcomes especially in those with right lung agenesis. We reviewed the 10-year experience in our hospital to describe special clinical features and try to delineate the causes of poor outcomes. From 1995 to 2005, 14 patients less than 18 years of age with unilateral lung agenesis (4 with left agenesis, 10 with right agenesis) were enrolled. Medical records reviewed included diagnosis, presentation, chromosome anomalies, cardiovascular anomalies and interventions, outcomes. We found that the mechanisms of severe airway disease in right lung agenesis included (1) trachea compression by the aortic arch, (2) the presence of ‘‘pseudo–ring-sling complex,’’ (3) distended pulmonary artery due to left to right shunt which impinged the only bronchus, and finally (4) the persistent LSVC that restricts the growth of trachea. The etiologies of airway complication in left lung agenesis included anomalous aortic arch compression on trachea and the coexisting heart disease with significant left to right shunt, which impinged on the bronchus. In conclusion, unilateral lung agenesis has frequently associated airway problems due to its surrounding vessels. Satisfactory airway intervention remains challenging. This disease still requires great effort to improve patient outcomes. Pediatr Pulmonol.

Background Ventricular septal rupture (VSR) is an uncommon but well-recognized mechanical complication of acute myocardial infarction (AMI). The outcome of VSR remains poor even in the era of reperfusion therapy. We reviewed our experience with surgical repair of post-infarction VSR and analyzed outcomes in an attempt to identify prognostic factors. Methods From October 1995 to December 2013, data from 47 consecutive patients (mean age, 68 ± 9.5 years) with post-infarction VSR who underwent surgical repair at our institute were retrospectively reviewed. The preoperative conditions, morbidity and surgical mortality were analyzed. Multivariate analysis was subsequently carried out by constructing a logistic regression model in order to identify independent predictors of postoperative mortality. Long term survival function were estimated using the Kaplan-Meier method and compared using the log-rank test. Results Percutaneous coronary intervention was performed in 17 (36.2%) patients, intra-aortic balloon pump (IABP) was used in 34 (72.3%), and six (12.8%) were supported with extracorporeal membrane oxygenation (ECMO) preoperatively. Forty-one (87.2%) patients received emergent surgical treatment. Concomitant coronary artery bypass grafting was performed in 27 (57.4%) patients. Operative mortality was 36.2% (17 of 47). The survival rate was 59.3% with concomitant CABG and 70% without concomitant CABG (p = 14). Multivariate analysis revealed that the survivors had higher preoperative left ventricular ejection fractions (LVEFs) compared with those who died (51 ± 13.7% vs. 36.6 ± 6.4% , respectively; p

Summary Background : Intraoperative transesophageal echocardiography (TEE) is useful in evaluating the repair of lesions in patients with congenital heart disease. But the use of TEE in infants with total anomalous pulmonary venous connection (TAPVC) remains unclear. We reviewed the safety and efficacy of intraoperative TEE during TAPVC repair. Methods : Twenty-eight consecutive 1 day to 7 month-old infants with TAPVC (14 supracardiac, six intracardiac and eight infracardiac type) had surgical repair with intraoperative TEE monitoring. Results : Four patients received immediate surgical revision after primary surgery for residual anastomotic stenosis diagnosed by TEE. In addition, two unsuspected ventricular septal defects and three persistent ductus arteriosus were detected before surgery. Eight infants (29%) had hypotension and hypoxemia associated with TEE probe insertion before surgery, but this hemodynamic disturbance returned to baseline value after withdrawing the TEE probe from the esophagus. However, these eight patients had uneventful TEE probe insertion following sternotomy. The mechanism was probably because of the reduction of intrathoracic pressure when the chest was opened. Conclusions : TEE probe insertion in TAPVC patients may pose a potential risk of compression of pulmonary venous confluence resulting in hemodynamic instability. Therefore, we suggest that the use of TEE in such TAPVC patients appears to be safer after sternotomy.

Patients receive methylprednisolone pulse therapy (MPT) when acute cardiac rejection occurs. Although the regimen is generally safe and effective, severe complications occasionally develop. From 1997 to 2007, there were 210 cardiac transplantation procedures performed at our hospital. Among these patients, there were 23 episodes of acute rejection treated with MPT, 10 mg/kg/d. Two patients in our series had cardiac arrest within 36 hours after initiating the therapy. Endomyocardial biopsy specimens showed International Society for Heart Transplantation grade 1B allograft rejection in both cases. Emergent intubation and cardiopulmonary resuscitation were performed. Venoarterial extracorporeal membrane oxygenation (ECMO) was used to rescue the patients. The cardiac function in both patients recovered gradually. Left ventricular ejection fraction increased from 16.2% to 47% in one patient and from 27% to 30% in the other patient. One patient was successfully weaned from ECMO after 2 days of support. The other patient was discharged against medical advice because of hypoxia-related brain death after 3 days. Both patients had a history of tachyarrhythmias before initiation of MPT. Although the relationship between mechanisms of cardiac arrest and MPT is uncertain, the risk of cardiac arrest cannot be overlooked when initiating MPT, especially in patients with a history of tachyarrhythmia. Meanwhile, ECMO can serve as a rescue method if cardiac arrest occurs.

BACKGROUND Cardiac surgery performed in patients with low body weight is a challenge for surgeons. Currently, such outcomes are mainly reported from European or North American centers. In this study, we review our cardiac surgery experience with neonates and infants weighing

AB Introduction: Submaximal exercise parameters are associated with an increased risk of hospitalization in patients with heart failure, but the implication in patients with Fontan circulation remains unknown. We investigated the prognostic value of these parameters in a Fontan cohort, in whom maximal exercise effort is often limited. Methods: Fifty-two Fontan patients received cardiopulmonary exercise tests at least 12 months after Fontan completion. We evaluated two maximal parameters (peak oxygen consumption (V[spacing dot above]O2) and HR reserve) and two submaximal parameters (oxygen uptake efficiency slope (OUES) and minute ventilation (V[spacing dot above]E) to carbon dioxide elimination (V[spacing dot above]CO2) slope). Results: The peak V[spacing dot above]O2 and OUES were 58.0% +/- 11.2% and 56.6% +/- 14.5% of the age- and sex-related predicted values. In the subsequent follow-up (median, 22.7 months), 11 patients (21%, including one death) exhibited cardiac morbidity, defined as cardiac-related hospitalization. Time-dependent receiver operating characteristic curve analysis demonstrated that only submaximal parameters were related to 2-yr cardiac morbidity (area under the curve for OUES 0.781, P = 0.018; for V[spacing dot above]E/V[spacing dot above]CO2 slope 0.714, P = 0.04), even in the subgroup achieved maximal exercise effort. The optimal threshold value for OUES was 45%, and for the V[spacing dot above]E/V[spacing dot above]CO2 slope, it was 37. Furthermore, the OUES conveyed independent prognostic information beyond resting oxygen saturation and a history of heart failure or protein-losing enteropathy. Conclusion: Submaximal exercise parameters provide superior prognostic information to maximal exercise data for predicting cardiac morbidity in Fontan patients. Moreover, the association between the OUES and cardiac morbidity is independent of relevant baseline clinical information

Objectives There are limited options for mechanical circulatory support to treat end-stage heart failure in pediatric patients. Although extracorporeal membrane oxygenation is commonly used in infants and children, ventricular assist devices (VAD) provide a longer duration of support with fewer complications before recovery or as a bridge to heart transplantation (HTx), as described herein. Methods This retrospective chart review of eight patients transplanted from April 2008 to December 2011, after left ventricular assist device (LVAD) implantation due to end-stage heart failure. Their mean age was 12 years (9–15 y) and mean body weight, 48 kg (20–78). All were New York Heart Association functional class IV with mean left ventricular ejection fractions less than 15%. Results The six patients (75%) received HTx after a mean LVAD support duration of 43.2 days; 2 (25%) died before a suitable heart became available. Their mean duration of LVAD support was 30 days. There were 4 (50%) who experienced clinically evident thromboembolic events: 3 (37.5%) cerebrovascular with 1 mortality and 1 (12.5%) as acute limb ischemia. Transient hemodialysis was performed in 4 (50%). Bloodstream infection identified in 6 (75%) was controlled with intravenous antibiotics. Driveline infection identified in 4 (50%) was treated successfully with local wound dressing changes and intravenous antibiotics. One 9-year-old boy died of rejection at 16 months after transplantation. Conclusions Because of the organ shortage, pediatric patients have a low chance to undergo HTx. VAD provides long-term support for children with end-stage heart failure before a suitable heart becomes available. A thromboembolic event remains a major complication influencing their survival.

Background— Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in Taiwan. This study investigates the long-term survival and risks of TOF in an Asian cohort. Methods and Results— This study enrolled 819 consecutive patients with TOF (61.1% male), who received total correction between 1970 and 2002, as participants. Patient medical records were reviewed, and the survival status of those out of contact was confirmed by death records retrieved from the National Health database. The mean (±SD) patient age at cardiac repair was 6.5±7.6 years, and a prior shunt operation was performed in 119 (14.5%) of the patients. At cardiac repair, a transannular patch for right ventricle outlet reconstruction was required in 444 (54.2%) of the patients. After 13 808 patient-years of follow-up, the 30-year survival rate was 90.5%. The annual mortality rate increased from 0.123% in the initial 15 years after repair to 0.395% thereafter ( P P =0.028). Conclusions— In this Asian TOF cohort, except for a late decrease after 15 years, long-term survival after cardiac repair was satisfactory. Although cardiac death was the most common cause of late death, accidents or suicide may also be associated with late mortality, suggesting a potential role for psychosocial support.

Background: The most common type of left ventricular-to-right atrial shunts are those associated with perimembranous ventricular septal defect when the defects diminish in size via aneurysmal transformation. This study is to characterize the echocardiographic features and to determine the possible mechanisms. Methods and results: From January 1986 to December 1992, of 930 consecutive patients with isolated perimembranous ventricular septal defect, 692 showed evidence of aneurysmal transformation. Excluding those with subaortic ridge, echocardiographic analysis was based on 664 of them. Of these, 94 patients had left ventricular-to-right atrial shunts, 115 had their defects spontaneously closed and in 455 only interventricular shunt persisted. Four types of echocardiographic findings featured in the aneurysmal transformation process: type A, both the anterior and septal leaflets of tricuspid valve contributed to the process; type B was similar to type A, but the arc-like structure from the anterior leaflet is much less evident; in type C, only the septal leaflet, and in type D, other adjacent tissues, contribute to the aneurysmal transformation. Type A morphology was closely associated with the development of left ventricular-to-right atrial shunt while types C and D were most commonly seen in those spontaneously closed defects and those with only interventricular shunt. The vegetations of infective endocarditis were located at the atrial side of the tricuspid valve in patients with left ventricular-to-right atrial shunts, while they were in the right ventricle in those with only interventricular shunt. Conclusion: The morphology of the aneurysmal transformation in perimembranous ventricular septal defect can be characterized by echocardiograms. Although the septal leaflet of the tricuspid valve is usually involved in this process, it is the incorporation of the anterior leaflet that predisposes to the development of left ventricular-to-right atrial shunts.

The study aims to describe 11 years of experience with extracorporeal cardiopulmonary resuscitation (ECPR) for in-hospital paediatric cardiac arrest in a university affiliated tertiary care hospital.Paediatric patients who received extracorporeal membrane oxygenation (ECMO) during active extracorporeal cardiopulmonary resuscitation (ECPR) at our centre from 1999 to 2009 were included in this retrospective study. The results from three different cohorts (1999-2001, 2002-2005 and 2006-2009) were compared. Survival rates and neurological outcomes were analysed. Favourable neurological outcome was defined as paediatric cerebral performance categories (PCPC) 1, 2 and 3.We identified 54 ECPR events. The survival rate to hospital discharge was 46% (25/54), and 21 (84%) of the survivors had favourable neurological outcomes. The duration of CPR was 39±17 min in the survivors and 52±45 min in the non-survivors (p=NS). The patients with pure cardiac causes of cardiac arrest had a survival rate similar to patients with non-cardiac causes (47% (18/38) vs. 44% (7/16), p=NS). The non-survivors had higher serum lactate levels prior to ECPR (13.4±6.4 vs. 8.8±5.1 mmol/L, p0.01) and more renal failure after ECPR (66% (19/29) vs. 20% (5/25), p0.01). The patients resuscitated between 2006 and 2009 had shorter durations of CPR (34±13 vs. 78±76 min, p=0.032) and higher rates of survival (55% (16/29) vs. 0% (0/8), p=0.017) than those resuscitated between 1999 and 2002.In our single-centre experience with ECPR for paediatric in-hospital cardiac arrest, the duration of CPR has become shorter and outcomes have improved in recent years. Higher pre-ECPR lactate levels and the presence of post-ECPR renal failure were associated with increased mortality. The presence of non-cardiac causes of cardiac arrest did not preclude successful ECPR outcomes. The duration of CPR was not significantly associated with poor outcomes in this study.

Objective: Bloodstream infection (BSI) is a well-recognized problem and it affects 10—50% of solid organ transplant recipients. The purpose of this study was to assess the incidence and prognosis of BSI in heart transplant recipients at our hospital. Methods: The study is a retrospective chart review. Results: We diagnosed 101 episodes of BSI in 73 out of 306 heart transplant patients (24%) during the 12-year study period. BSI occurred at a median of 191 days (range 1—3376) after transplant and 50% occurred within 6 months after transplant. Most BSI episodes were nosocomial (73%), especially those occurring within the first month (94%). As far as pathogen was concerned, Gram-negative bacteria predominated (57%), followed by Gram-positive bacteria (34%), fungus (5%), anaerobics (2%), and cryptococcus (2%). Overall 30-day mortality rate was 30%. Death occurred in 36% (13/36) of the patients with early-onset BSI, 14% (2/14) of the patients with BSI in months 2—6, and 29% (15/ 51) of the patients with late-onset BSI. Mortality rate was over 50% in those patients with Pseudomonal infection, fungal infection, cryptococcal infection of central nervous system, lung infection, and severe sepsis. Compared to Western series, there was a high incidence of infections caused by Enterobacter species and Acinetobacter baumannii. Conclusions: There was a high incidence of BSI after heart transplantation in Taiwan, especially infections caused by Enterobacter species and A. baumannii. Mortality was high in patients with infection caused by Pseudomonas, Candida, and Cryptococcus and in patients with severe sepsis. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Aortic rupture is a rare but potentially catastrophic complication following a balloon aortoplasty for recoarctation. The treatment of aortic ruptures remains challenging. We present here a 9-year-old girl with Turner syndrome who experienced a life-threatening rupture in her aorta after a balloon aortoplasty for recoarctation. She was successfully rescued by the antegrade deployment of a commercially available iliac limb extension stent-graft via an ascending aortic conduit. Prudent balloon aortoplasty for recoarctation in patients with Turner syndrome is important due to their inherent aortopathy and previous surgical repairs. Possible reasons for an aortic rupture are oversized ballooning and the choice of balloon diameter based only on an angiographic measurement. In agreement with earlier reports, our case also confirms the importance of keeping a commercially available stent graft available to treat this complication that has potentially fatal consequences.

Surgical repair of tetralogy of Fallot (TOF) in countries with sound medical care systems is seldom delayed until adolescence. This study investigated the clinical profile and the surgical outcomes in such a population from Taiwan. Between 1970 and 2009, 179 TOF patients (56% male) received total repair at 19.2 ± 8.3 (10-49) years of age. We reviewed the medical records and interviewed the patients concerning their current status. The survival was ascertained in all by a link to our national health database. Major morbidities before cardiac repair included atrial arrhythmia (1.1%), ventricular arrhythmia (3.9%), infective endocarditis (6.7%), brain abscess (4.6%) and pulmonary tuberculosis (3.3%). Ventricular arrhythmia and pulmonary tuberculosis occurred mainly after 20 years of age. Thirty patients (16.8%) received a palliative shunt. The preoperative QRS duration increment was 0.6 ms/year. Early mortality occurred in 4 (2.2%) and was related to previous shunt surgery (OR = 16.5, p < 0.05) and coronary artery crossing RVOT (OR 17.6, p < 0.05). After repair, the functional class improved in all patients. The median age at latest follow-up was 31.8 (32.8 ± 12.3) years. The survival was 92.7 and 89.3% at 20 and 30 years after operation, respectively. Late cardiovascular death could be predicted by the length of postoperative intensive care unit stay (OR = 1.3, p < 0.001). The freedom from ventricular arrhythmia 30 years after repair was 84.1% and was associated with a final QRS longer than 160 ms. Unrepaired TOF patients were at high risk of infective endocarditis, brain abscess, pulmonary tuberculosis and arrhythmias during their adolescence and adulthood. Cardiac repair in this age group was still safe and effective.

This 16-month-old girl (weight 7 kg) was a patient with a complete form of endocardial cushion defect, and she received total correction at the age of 2 months. Severe mitral regurgitation persisted despite surgery, and mechanical valve replacement was performed at the age of 4 months. Two episodes of mitral valve thrombosis with severe mitral stenosis complicated her recovery, and she underwent 2 more operations for mitral valve replacement and double valve replacement with aortic root enlargement at the age of 6 months and 1 year, respectively. Impaired heart function and complete atrioventricular block complicated the operation, and a pacemaker was also inserted. She received warfarin therapy and was followed up regularly at our clinic. Mitral valve thrombosis occurred again, with a presentation of progressive dyspnea and poor activity. Chest radiography and transthoracic echocardiography showed severe mitral stenosis with lung edema (Figures 1 and 2A). Pulmonary hypertension was also found, with estimated systolic pulmonary artery pressure of 75 mm Hg. Intubation was provided with ventilator support, and inotropic agents were given, accompanied by heparin infusion. Fluoroscopy then showed poor motion of both leaflets of the mitral valve (Movie IA in the online-only data supplement). Because of the history of repeated operations and the family’s decision, fibrinolytic therapy with tissue plasminogen activator (tPA) was attempted. We discontinued warfarin first, checked the fibrinogen level, and then discontinued heparin and started tPA treatment. We gave a loading dose of 0.1 mg/kg first and then 6 courses of escalating doses of tPA at 0.1 mg kg 1 h 1 for 6 hours and then 0.15, 0.2, 0.3, 0.3, and 0.4 mg kg 1 h 1 . The fibrinogen level was checked after

Objectives: Coronary artery (CA) anatomy of anatomically corrected malposition of the great arteries (ACM) has not been well elucidated. We studied the CA pattern and their association with the aortopulmonary rotation (APR). Methods: CA anatomy and APR of ACM were identified by image or surgical intervention. The degree of APR was defined by the pattern of the aortic sinus of Valsalva on the lateral angiogram or computed tomography (CT). Results: Four CA types were presented in 10 patients and literatures. Six patients had the same CA type as the usual pattern in transposition of the great arteries (type 1); and two had left anterior descending (LAD) from the left-hand facing sinus that also gave rise to the rightCA(RCA)(type0).OnepatienthadLADfromtheleft-handfacingsinuswhileRCAandcircumflexarteryfromtheright-handfacingsinus(type 10); and single CA from the anterior left-hand sinus (type 3c) was identified in another. APR was left anterior in type 1 and more lateral in types 0 and 3c; and left posterior in type 10 (p = 0.001). The usual pulmonary trans-annular incision can only be performed in case of type 10 CA after posterior APR (10%, 1/10). In the majority (90%, 9/10 with the CA type 1, 0, and 3c), the RCA is in front of the pulmonary annulus, and the atrioventricular groove patch plasty posterior to the RCA had to be adopted for a trans-annular patch. Conclusions: The observed CA pattern was correlated with APR. APR identified by the aortic sinus pattern could implicate the CA patterns and the surgical options in ACM. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objectives: The great arteries are in a non-spiral relationship in their transposition, even after arterial switch operation (ASO) with the Lecompte manoeuvre. We aim to restore the natural spiral great arteries and compare such ASO with conventional ASO with the Lecompte manoeuvre to clarify the functional implications of the spiral great arteries. Methods: The early survivors of ASO (n = 130) were included in this retrospective study. In spiral ASO (n = 48), the main pulmonary artery ran alongside the aorta and gave rise to its branches posteriorly. Patients who underwent non-spiral ASO with the manoeuvre (n = 82) were compared. The survival, re-operation-free ratio and the anatomic details for re-intervention after ASO were reviewed. Results: Average follow-up was 5.6 ± 3.4 years (range: 2 months to 11 years). The estimated 10-year survival was similar (92.6% spiral vs 92.1% non-spiral, respectively). Significant pulmonary stenosis (PS) (>40 mmHg) was noted in five (10.4%) in the spiral group and seven patients (9.7%) in the non-spiral (p = NS). The re-operation-free survival at 10 years was not significantly different (87.4% vs 90.1%). The reasons for re-operation were subvalvular PS (n = 3) and residual defect (n = 2) in the spiral group, while supravalvular PS (n = 3), neo-aortic regurgitation (n = 1) and aortic neo-coarctation (n = 2) in the non-spiral group. Supravalvular PS and aortic neo-coarctation that occurred in the non-spiral group were not seen after spiral ASO. Conclusions: Intermediate-term results of spiral ASO were satisfactory. Transposition is not a mere reversal of the great arteries; therefore, recognition of non-spiral relationship should be appreciated. Spiral reconstruction would be beneficial to reduce supravalvular PS and neo-aortic kinking. Further recognition of spiral function and refined modification might justify the application of spiral ASO in the future.

We report an unusual cause of prolonged chylothorax drainage after Norwood stage one reconstruction. This 1-month-old girl's chylous drainage was refractory to medical treatment. Echocardiography revealed thrombosis of the superior vena cava. Upon sternotomy to remove the thrombus, we were surprised to find the ePTFE (expanded polytetrafluoroethylene) tube previously used for selective cerebral perfusion compressing the innominate vein and the pericardium-based aortic arch. We performed a superior vena cava thrombectomy and shortened the ePTFE tube. Her chylothorax subsided gradually. We suggest that external compression of the venous drainage system should be considered in patients with prolonged chylothorax drainage. Once medical treatment fails, early surgical exploration may be helpful to stop the chylothorax.

Objective: There are no guidelines to select isolated heart transplantation or simultaneous heart and kidney transplantation in patients with renal dysfunction. We sought to assess the effect of simultaneous kidney transplantation on heart-transplantation outcome in patients with renal dysfunction. Methods: Retrospective case review. Results: Between 1993 and 2006, 45 patients with preoperative serum creatinine 2m g dl 1 underwent heart transplantation, including 32 isolated heart transplantation and 13 simultaneous heart and kidney transplantation. The survival of83.3 10.8%at30days,58.3 14.2%at1yearand50.0 14.4%at3 yearsinsimultaneousheartandkidneytransplantationdidnotdifferfrom the survival of 81.8 6.7% at 30 days, 66.7 8.2% at 1 year and 45.1 9.3% at 3 years in isolated heart transplantation. The dialysis-free and patient survival of 66.7 13.6% at 30 days, 58.3 14.2% at 1 year and 50.0 14.4% at 3 years in simultaneous heart and kidney transplantation also did not differ from the rate of 81.8 6.7% at 30 days, 66.7 8.2% at 1 year and 31.4 8.9% at 3 years in isolated heart transplantation. Conclusions: Simultaneous kidney transplantation is an effective therapy for patients depending on dialysis pretransplant, reducing postoperativeriskofmortalityintheseverysickpatientstothelevelofpatientswithless severerenaldiseasenotrequiringdialysisbeforetransplant. # 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: Liver cirrhosis is considered to be a contraindication to heart transplantation. However, the clinical outcome of heart transplantation in patients with liver cirrhosis has not been reported. Here, we sought to evaluate the clinical outcome of heart transplantation in cirrhoticpatients.Methods:Datawere collectedby retrospective chartreview. Patientswithliver cirrhosisatthe timeof transplantationwere included. Results: Between 1987 and 2007, 12 patients with liver cirrhosis underwent heart transplantation at the authors’ hospital. Diagnosis of liver cirrhosis was based on preoperative abdominal sonography in five, autopsy in five, and laparotomy in two patients. Causes of heart failure were dilated cardiomyopathy in four, coronary artery disease in three, congenital heart disease in three and valvular heart disease in two patients. Causes of liver cirrhosis were alcoholism in two, cardiac in seven, and unknown in three patients. The Child classification was class A in three, class B in five and class C in four patients. Overall, the hospital mortality rate was 50% and major in-hospital complications occurred in nine patients (75%). Patients with non-cardiomyopathy diagnosis, previous sternotomy, and massive ascites had a high hospital mortality rate. The median follow-up duration was 33.5 months. There was no late death. Late post-transplant complications occurred in four patients and there was no event of liver dysfunction. All survivors were in Child class A at outpatient follow-up. Conclusions: Although there was high mortality and morbidity, patients with end-stage heart failure and liver cirrhosis can be considered for heart transplantation with careful case selection. # 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Background We retrospectively reviewed cases of left pulmonary artery (LPA) sling complex with computed tomography to explore its possible meaning in embryogenesis. Methods Eighteen children (6 girls and 12 boys aged 5 days to 17.5 years; mean, 1.5 years) were identified as having LPA sling during the period of October 1996 to March 2006. Another 2,364 cardiac computed tomography scans that had no LPA sling were included as control. The associated bronchopulmonary and vascular anomalies were identified. Chi-square tests were used to compare the probabilities of coexistence of these anomalies, with or without LPA sling. Results All patients with LPA sling presented with tracheal stenosis (100%), and there was a high incidence of combined right tracheal bronchus (22%), underdeveloped right lung (22%), persistent left superior vena cava (22%), and left patent ductus arteriosus (39%). These associated anomalies were more highly associated with subjects who had LPA sling than with those who did not (p Conclusions Owing to the spatial coexistence of LPA sling with the associated anomalies in the embryonic foregut mesoderm, we propose that a "space available" hypothesis in the local environment appears to be important in the embryogenesis of an LPA sling complex.

Intracardiac repair for complex congenital heart defects in premature neonates with very low birth weight (VLBW) is still a challenge to pediatric cardiac surgeons. We report the successful rerouting of cardiac type total anomalous pulmonary venous return (TAPVR) in a premature newborn (36 th gestational week) with VLBW (1250 g). She had severe hypoxemia and low cardiac output despite medical treatment. Rerouting surgery of TAPVR was performed under deep hypothermia circulatory arrest at the age of 20 days. The sternum was left open and approximated 2 days later. Follow-up echocardiography showed good ventricular function without pulmonary venous obstruction. The endotracheal tube was removed 7 days postoperatively. She was then discharged without complication. In conclusion, with improved cardiopulmonary bypass technique and perioperative care, open heart surgery can be performed in premature newborns with VLBW. [ J Formos Med Assoc 2007;106(2 Suppl):S23-S26]

We were glad to receive the comments from Ma et al. [1], regarding our recently published article [2]. This is a retrospective study with chart review using our patients’ medical records. In our data, there was no moderate to severe tricuspid regurgitation (TR) in our patients receiving single ventricle palliation (SVP) [2]. We chose to perform SVP in this group of patients due to the presence of severe pulmonary stenosis or pulmonary atresia combined with other anatomical restrictions (e.g. remote type ventricular septal defect (VSD), chordae straddling, unusual coronary artery pattern and unbalanced ventricle) [2]. There was only 1 patient with moderate TR receiving valve repair before the SVP, and the postoperative echo showed no aggravation of valve regurgitation [3]. Why was there no patient receiving SVP with severe TR in our study? We believed severe tricuspid valve regurgitation is not an indication for SVP, and anatomical repair might be helpful, despite the suboptimal long-term result [4]. We think the progress of TR severity is slow and insidious. When TR becomes apparent, it might be due to structural defects (i.e. Ebsteinoid anomalies) or be regarded as a sign of morphological RV dysfunction. We performed SVP in the early years of the patients’ lives (mean age of 8.6 years) to prevent TR progression in the near future and preserve ventricular function [2, 4–6]. Our article presented only the shortand mid-term results; the long-term follow-up results are still unknown. Although there is no consensus for the timing of performing surgical repair in patients without severe TR or cyanosis, we preferred to perform the operation earlier, before right ventricular failure [2, 5, 6]. Our patients with congenitally corrected transposition of the great arteries typically presented with cyanosis or other heart failure symptoms. Patients without symptoms usually do not seek medical help and are diagnosed accidentally for other medical diseases. If there were no major cardiac anatomical anomalies such as large VSD, severe pulmonary stenosis or atresia, and atrioventricular valve defects with severe valve regurgitation that would lead to heart failure in the future, surgical intervention would not be beneficial for these patients [6].

A 30-year-old man who is a heroin addict was diagnosed with uncontrolled tricuspid valve endocarditis and repeated lung abscesses. He underwent tricuspid valvectomy for the endocarditis. After surgery the patient had severe tricuspid regurgitation and hypoxemia develop. Due to severe tricuspid regurgitation-induced ventricular distension and persistent low cardiac output, reimplantation of the tricuspid valve was planned for 2 weeks after the first operation. To avoid lung injury caused by the cardiopulmonary bypass and to preserve right ventricular function, a self-made superior and inferior vena cava shunt was connected to the pulmonary artery. The tricuspid valve was implanted without cardiopulmonary bypass.

Background Progression of aortic regurgitation (AR) in repaired outlet ventricular septal defects (VSDs) remains unclear, especially for muscular outlet and perimembranous outlet VSDs. We tried to identify the risk factors for AR progression and aortic valve replacement (AVR) at long-term follow-up. Methods Four hundred patients with complete follow-up after the repair of their outlet VSD between 1987 and 2002 were studied. Results Juxta-arterial VSD, perimembranous outlet VSD, and muscular outlet VSD were noted in 190, 148, and 62 patients, respectively. There were 377 patients with none to mild AR (group I) and 23 with moderate to severe AR (group II) preoperatively. Aortic valve replacement was performed on 11 patients (all from group II), with 10 having received AVR concomitantly with VSD repair and 1 having received it 4 years later. Only severity of preoperative AR and older age (>15 years) at VSD repair were significant predictors of AVR. With a total follow-up of 2230 person-years, the 10-year freedom from AVR after VSD repair for group I was 100% and that for group II was 50.2%. In group I, AR progressed in 4 patients only (1.2%, 2 juxta-arterial and 2 perimembranous outlet) and aortic valvular (aortic valve prolapse or ruptured sinus Valsalva aneurysm) or subvalvular anomalies were present in all. The event-free (AR or AVR) survival rates among the 3 outlet-type VSDs however showed no difference. Conclusions Aortic regurgitation progression modes after surgical VSD repair were similar among the 3 outlet-type VSDs. Aortic valve replacement was rarely necessary for patients who were operated on when they were younger than 15 years. Aortic regurgitation of a less-than-moderate degree preoperatively rarely progressed after VSD repair.

Extracorporeal life support (ECLS) had been successfully used in neonatal respiratory failure, but cardiac ECLS has been used increasingly in recent years. The purpose of this study was to review our experience in pediatric patients supported by ECLS for postoperative circulatory failure and to analyze the factors associated with mortality. Between January 1999 and December 2004, 68 pediatric patients (< 18 years old) who received ECLS within 7 days after cardiac surgery at the National Taiwan University Hospital were included in this study. The overall survival rate of this cohort was 32.4%. Age and gender did not affect survival. Patients with separate biventricular physiology had a higher probability of survival than those with systemic-pulmonary shunt or cavopulmonary anastomosis (41.3% vs. 13.6%, p < 0.05). Acute renal failure during ECLS was significantly associated with mortality (83% vs. 33.5%, p < 0.001). After ECLS initiation, the lowest lactate levels on the second to fourth days were lower in survivors than in nonsurvivors (2.4 vs. 3.3 mmol/L, p < 0.05). There was a trend toward a better survival in the most recent 2 years in comparison with the previous 4 years (47.6% vs. 25.5%, p = 0.07), although this trend did not reach statistical significance. In conclusion, nonbiventricular physiology, acute renal failure, and high blood lactate levels after ECLS increased the risk of mortality for pediatric patients requiring ECLS for postoperative cardiac support.

Background B-type natriuretic peptide (BNP) is a marker of heart failure. In adult patients with heart failure, decreased BNP levels after implantation of ventricular assist devices may be indicative of recovery. However, BNP levels among pediatric patients receiving mechanical support are largely unknown. Methods Fifteen pediatric patients with cardiogenic shock who were supported by extracorporeal membrane oxygenation (ECMO) were evaluated. The BNP levels were determined before ECMO initiation, during ECMO support, and after ECMO removal. Results All patients had elevated BNP levels before initiation of ECMO (median, 1,430 pg/mL; range, 361 to 5,000 pg/mL). Among the 15 patients, 1 received heart transplantation. Extracorporeal membrane oxygenation was withdrawn in 2 patients, and the other 12 patients were weaned from ECMO. Four patients died after initially successful weaning from ECMO. The BNP levels of the nonsurvivors (median, 3,685 pg/mL; range, 2,494 to 5,000 pg/mL) were higher than those of the survivors (median, 1,127pg/mL; range, 108 to 3,030 pg/mL) on the next few days after ECMO removal ( p = 0.016). The BNP levels on the fourth day after removal of ECMO among the survivors (median, 498 pg/mL; range, 108 to 890 pg/mL) were lower than those among the nonsurvivors (median, 3,900 pg/mL; range, 3,230 to 5,000 pg/mL; p = 0.017). Conclusions Among pediatric patients supported with ECMO, the survivors had lower BNP levels than those who did not survive. We suggest that serial blood BNP levels can be potential markers for monitoring pediatric patients on mechanical circulatory support, and the concept merits further study.