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Adenotonsillectomy is a short surgical procedure under general anaesthesia in children. An ideal muscle relaxant for adenotonsillectomy would create an intense neuromuscular block while having a quick recovery time without postoperative morbidity. We compared the effect of different doses of rocuronium for the tracheal intubation in children under 5% sevoflurane and fentanyl.75 children (aged 3-10 years, ASA I) scheduled for adenotonsillectomy were enrolled. Anaesthesia was induced with propofol 2.5 mg/kg, followed by fentanyl 2 μg/kg. After mask ventilation with 5 vol% sevoflurane in 100% oxygen for 2 min, 2 ml of study drug was administered intravenously, i.e., either normal saline (S Group) or one of two doses (0.15 or 0.3 mg/kg) of rocuronium. We assessed conditions during tracheal intubation and also recorded the surgical condition, the time from discontinuation of sevoflurane to extubation and PAED scale, pain scores in PACU.Rocuronium groups (96% and 100%, respectively; P 0.01) showed statistically superior clinically acceptable intubating conditions than the saline group (72%). The 0.3 mg/kg rocuronium (80%) treatment clearly resulted in excellent intubating conditions compared with the 0.15 mg/kg group (44%; p = 0.028). There was no significant difference in the time to extubation and surgical condition, and in the postoperative measures of emergence delirium, pain, and recovery time among the three groups.A dose of 0.3 mg/kg rocuronium may provide optimal intubating conditions without delayed recovery in 5% sevoflurane anaesthesia with fentanyl in children undergoing adenotonsillectomy.NCT02467595.

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.

Objective The efficacy of chlorhexidine- and silver sulfadiazine-coated central venous catheters (CSS-CVC) against catheter-related infection remains controversial. We hypothesized that the loss of silver nanoparticles may reduce the antibacterial efficacy of CSS-CVCs and that this loss could be due to the frictional force between the surface of the CVC and the bloodstream. The objective of this study was to investigate whether the antimicrobial effect of CSS-CVCs decreases with increasing exposure time in a bloodstream model and quantitatively assay the antimicrobial effect of CSS-CVCs compared with polyurethane and antiseptic-impregnated CVCs. Methods Each CVC was subjected to 120 hours of saline flow and analyzed at intervals over 24 hours. The analyses included energy-dispersive X-ray spectroscopy, scanning electron microscopy, and optical density after a Staphylococcus aureus incubation test. Results The weight percentage of silver in the CSS-CVCs significantly decreased to 56.18% (44.10% ± 3.32%) with 48-hour catheterization and to 18.88% (14.82% ± 1.33%) with 120-hour catheterization compared with the initial weight percentage (78.50% ± 6.32%). In the S. aureus incubation test, the antibacterial function of CSS-CVCs was lost after 48 hours [3 (N/D) of OD]. Similar results were observed in a pilot clinical study using 18 CSS-CVCs. Conclusions We found that the efficacy of CSS-CVCs decreased over time and that the antibacterial function was lost after 48 hours of simulated wear-out. Therefore, antibiotic-impregnated CVCs may be a better option when longer (>48 hours) indwelling is needed.

Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.

Transannular patching (TAP) frequently accompanies primary repairs (PRs) in symptomatic neonates with tetralogy of Fallot (TOF). If a systemic-pulmonary shunt (SPS) facilitates the growth of the pulmonary valve annulus (PVA), patients with a marginally small PVA could benefit from a staged repair in terms of lowering the risk of TAP.Among 216 infants with TOF who underwent surgical intervention between January 2004 and December 2013, 29 infants underwent SPS with a subsequent repair (SPS group), whereas 187 infants received a PR (PR group). Median age and the Z-score of the PVA (PVA [Z]) at SPS were 32 days and -3.5, respectively. There was one late death and one follow-up loss after SPS, and preservation of the PVA was achieved on repair in 16 patients (16 of 29; 55%).Multiple regression analysis showed that performance of SPS was the only indicator of the increase in the PVA (Z) in the entire cohort (n = 216). On mixed linear regression, the PVA (Z) increased significantly after the placement of an SPS (-3.6 + 0.2*duration in months, p = 0.001), whereas the prerepair changes in the PVA (Z) were not statistically significant in the PR group (p = 0.7), with a significant intergroup difference (p0.001). Receiver operating characteristic curve analysis showed that placement of TAP is expected when the preshunt PVA (Z) is smaller than -4.2 (area under the curve: 0.82; 95% confidence interval: 0.62 to 1.00; sensitivity, 100%; specificity, 73%).SPS facilitates outgrowth of the PVA over somatic growth in patients with TOF. However, preservation of the PVA may not be achieved on staged repair if the initial PVA is too small.

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.

Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.

Background Surgical techniques currently used for the repair of Ebstein's anomaly comprise reconstruction of the tricuspid valve mechanism at the level of the true annulus with or without plication of the atrialized right ventricle. However, performing this procedure for patients with a dysmorphic anterior leaflet (i.e., insufficient leaflet tissue and decreased mobility due to tethering) may necessitate technical modifications. Methods A retrospective review was performed of 31 patients (seven males and 24 females, median age at operation 31 years) with Ebstein's anomaly, who underwent tricuspid valve repair between March 2002 and December 2014. The original Hetzer technique (annulus to annulus approximation) was employed for six patients with a well-formed anterior leaflet. In 25 patients, the tricuspid valve mechanism was restored at the displaced septal leaflet by approximating the anterior leaflet attachment in the true annulus to the displaced septal leaflet attachment in the mid-septum. A bidirectional superior cavopulmonary anastomosis was added in 27 of 31 (87%) patients. Results No early or late death occurred during the median follow-up of 66 months (1–138 months). Immediate postoperative tricuspid regurgitation was trivial to mild in 22 patients, and the median preoperative, immediate postoperative, and last follow-up tricuspid regurgitation jet areas in 21 adult patients were 23.3 cm2, 10.4 cm2, and 7.0 cm2, respectively. Two patients underwent reoperation at 81 and 119 months postoperatively. Five-year freedom from severe tricuspid regurgitation or reoperation was 93.2%. Conclusion Restoration of the tricuspid valve mechanism at the level of displaced septal leaflet leads to excellent long-term outcomes. The addition of the bidirectional superior cavopulmonary anastomosis has contributed to the success of this technique.

Objective Thoracic duct mass ligation (TDML) through a right thoracotomy (RT), regardless of the side of the pleural effusion, is a standard procedure for chylothorax that is refractory to medical treatment. This procedure may be unsuccessful in patients with complex congenital heart disease, which necessitates additional left thoracotomy (LT) for left periaortic mass ligation. We hypothesized that failure of the right-sided approach is attributable to the anatomic variation of the path of the thoracic duct. Methods Of the children who underwent surgery for congenital heart disease between 1992 and 2014, a total of 70 of 8880 (0.8%) underwent TDML by RT (n = 57) or LT (n = 13; LT after RT in 10, and primary LT in 3). Results Persistent chylothorax was successfully resolved in 65 patients (65 of 70; 93%) within 15 days (2-79 days) after the first or second TDML; 5 patients died with a chest-tube(s) in situ. After excluding mortality without chest-tube removal, we sought to identify the risk factor(s) necessitating LT in 65 patients (RT group: 54; LT group: 11). On logistic regression analysis, the LT group was more likely to have dextrocardia (odds ratio: 6.38; 95% confidence interval: 1.09-37.25; P = .04). The incidence of abnormal atrial situs, great arterial malposition, right descending thoracic aorta, and bilateral superior vena cavae were comparable in the 2 groups. Conclusions The path of the thoracic duct may vary in pediatric patients with complex congenital heart disease. Left periaortic mass ligation should be considered in patients with chylothoraces that persist after the right-sided approach, especially in patients with dextrocardia.

We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

OBJECTIVES Pericardial effusion can cause haemodynamic compromise after heart transplantation. We identified the effects of soft drains on the development of pericardial effusion. METHODS We enrolled 250 patients ≥17 years of age who underwent heart transplantation between July 1999 and April 2012 and received two conventional tubes (n = 96; 32 French), or two tubes with a soft drain (n = 154; 4.8 mm wide). The development of significant pericardial effusion or the need for drainage procedure during 1 month after heart transplantation was compared with the use of the propensity score matching method to adjust for selection bias. RESULTS At 1 month after transplantation, 69 patients (27.6%) developed significant pericardial effusion. Among these, 13 patients (5.2%) underwent pericardial drainage. According to multivariate analysis, history of previous cardiac surgery [odds ratio (OR) = 0.162; 95% confidence interval (CI) = 0.046-0.565; P = 0.004] and placement of a soft drain (OR = 0.186; 95% CI = 0.100-0.346; P < 0.001) were significant factors that prevented pericardial effusion or the need for drainage during the early postoperative period. For the 82 propensity score matched pairs, patients receiving an additional soft drain were at a lower risk of the development of significant pericardial effusion or the need for a pericardial drainage procedure during 1 month (OR = 0.148; 95% CI = 0.068-0.318; P < 0.001) compared with those receiving only two conventional tubes. CONCLUSIONS Pericardial soft drainage is a simple and safe procedure that reduces pericardial effusion and decreases the need for pericardial drainage after heart transplantation.

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.

Various surgical techniques have been developed for the repair of an interrupted aortic arch. However, tension and Gothic arch formation at the anastomotic site have remained major problems for these techniques: Excessive tension causes arch stenosis and left main bronchus compression, and Gothic arch configuration is related to cardiovascular complications. To resolve these problems, we adopted a modified surgical technique of distal aortic arch augmentation using an autologous main pulmonary artery patch. The descending aorta was then anastomosed to the augmented aortic arch in an end-to-side manner. Here, we report two cases of interrupted aortic arch that were repaired using this technique.

Most thoracopagus twins present with cardiac fusion and associated congenital cardiac defects, and assessment of this anatomy is of critical importance in determining patient care and outcome. Cardiac CT with electrocardiographic triggering provides an accurate and quick morphological assessment of both intracardiac and extracardiac structures in newborns, making it the best imaging modality to assess thoracopagus twins during the neonatal period. In this case report, we highlight the diagnostic value of cardiac CT in thoracopagus twins with an interatrial channel and complex congenital cardiac defects.

Backgrounds A pegylated form of recombinant granulocyte-colony stimulating factor (G-CSF) was developed for prophylactic use in breast cancer. The aim of this study was to evaluate the efficacy and safety of once-per-cycle DA-3031 in patients receiving chemotherapy for breast cancer. Methods A total of 61 patients receiving docetaxel, doxorubicin, and cyclophosphamide (TAC) chemotherapy were randomized in cycle 1 to receive daily injections of filgrastim (100 μg/m2) or a single subcutaneous injection of pegylated filgrastim DA-3031 at a dose of either 3.6 mg or 6 mg. Results The mean duration of grade 4 neutropenia in cycle 1 was comparable among the treatment groups (2.48, 2.20, and 2.05 days for filgrastim, DA-3031 3.6 mg and 6 mg, respectively; P = 0.275). No statistically significant differences were observed in the incidence of febrile neutropenia between the treatment groups (9.5 %, 15.0 %, and 5.0 % for filgrastim, DA-3031 3.6 mg and 6 mg, respectively; P = 0.681) in cycle 1. The incidences of adverse events attributable to G-CSF were similar among the treatment groups. Conclusions Fixed doses of 3.6 mg or 6 mg DA-3031 have an efficacy comparable to that of daily injections of filgrastim in ameliorating grade 4 neutropenia in patients receiving TAC chemotherapy.

OBJECTIVE: Regarding neo-aortic valve regurgitation (neo-AR) after the arterial switch operation (ASO), the ‘trap-door’ technique was supposed to be a risk factor due to a distortion of the sinotubular junction (STJ) geometry. Here we report our results of the ‘trap-door’ technique with a special emphasis on root geometry including the ratio of STJ to annulus. METHODS: From August 1991 to March 2010, 240 patients with transposition of the great arteries underwent the ASO and who had at least 1 year of follow-up were included in this study. The medical records were retrospectively reviewed. RESULTS: The median age and body weight at the time of operation were 11 (0–1213) days and 3.4 (1.30–18.75) kg, respectively. The median follow-up duration was 79 months (range 12 months—19.5 years). At the latest echocardiographic follow-up, only six patients had neo-AR greater than Grade II (6 of 240, 2.5%). We found no relationship between neo-AR greater than Grade II and perioperative factors. The actual sizes of the neo-aortic annulus, mid-sinus and STJ were observed as having increased over time. However, most zscores of STJ at the latest echocardiography varied between �2 and 2 and, more importantly, the ratio of STJ to neo-aortic annulus was 0.93 ± 0.20, which was near normal at the latest echocardiographic follow-up. CONCLUSIONS: Our results showed a very low incidence of significant neo-AR, which was relatively attributable to the preserved z-score of STJ and the normal range of STJ/annulus ratio. Therefore, we propose that it is important to maintain these factors adequately during the reconstruction of the neo-aortic root in the ASO.

Background and Objectives: Pulmonary arterial stenosis is a relatively common complication after corrective operation of congenital heart disease. Unilateral stenosis of pulmonary arteries could result in decrease perfusion of affected lung, pulmonary regurgitation, or elevation of right ventricular pressure. Eventually there are increasing risks of right ventricular failure, arrhythmia, or sudden death. However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. This study aimed at validating the effectiveness and investigating complications of pulmonary arterial stent implantation in a single institution during mid-term follow up period. Subjects and Methods: A total of 42 patients (50 stents) were implanted for treating branch pulmonary arterial stenosis. We used cardiac catheterization for comparing diameter after stent implantation directly and lung perfusion scan indirectly. We also investigated any adverse effect relating the procedure. Results: Percent stenosis of stenotic lesions were decreased from 54.1±10.7% to 22.8±12.5% (p

Massive pulmonary hemorrhage and other serious cardiopulmonary diseases in patients with fulminant hepatitis result not only in graft failure but also mortality after LT. ECMO is used to treat children with cardiorespiratory failure refractory to conventional intensive care. We describe a five-yr-old girl with genetically confirmed fulminant Wilson disease and severe pulmonary hemorrhage who underwent successful primary LT following veno-arterial ECMO. To our knowledge, this is the first report of successful primary LT in a patient using veno-arterial ECMO. The present case demonstrates that ECMO, as a bridging modality to LT, may be necessary to manage both massive pulmonary hemorrhage and possible graft loss because of hypoxemia.

Background We sought to determine whether longer duration before delayed sternal closure (DSC) increases the risk of mortality, postoperative infection, or wound dehiscence. Methods A retrospective review was performed of 154 patients who underwent DSC between January 1999 and April 2009. Median body weight and age at operation were 3.6 kg (1.5 to 80 kg) and 25 days (2 days to 20 years), respectively. Palliative procedures were performed in 58 patients (58/154, 37.7%). Sternal wound problems were categorized according to the consensus-based definitions. Multivariate analysis was conducted encompassing various preoperative and intraoperative variables to identify risk factors for adverse surgical outcomes. The mean comprehensive Aristotle score (CAS) was 13.2 ± 3.1. Results There were 28 hospital mortalities (28/154, 18.2%). Excluding patients who died before sternal closure (4/154, 2.6%), the median duration of sternal opening was 3.5 days (1 to 182 days). Postoperative infection (sternal wound infection or systemic infection) and sterile wound dehiscence (SWD) occurred in 17 and 14 patients, respectively. Multivariate analysis revealed that duration of ventilatory support increased the risk of mortality ( p = 0.004), and postoperative infection/SWD ( p = 0.001). CAS also correlated with postoperative infection/SWD ( p = 0.026). Duration of sternal opening however was associated with none of the outcome variables. Conclusions Long ventilatory support and complexity of the cardiac anomaly increase the risk of adverse outcomes after procedures to repair congenital cardiac anomalies . After adjusting these variables, longer duration before DSC does not seem to be a risk factor for surgical mortality, postoperative infection, or wound dehiscence.

Heart transplantation is a standard treatment for end-stage heart disease. Pediatric heart transplantation, however, is not frequently performed due to the shortage of pediatric heart donors. This is the first report of pediatric heart transplantation in Korea. Our retrospective study included 37 patients younger than 18 yr of age who underwent heart transplantation at Asan Medical Center between August 1997 and April 2009. Preoperative diagnosis was either cardiomyopathy (n = 29, 78.3%) or congenital heart disease (n = 8, 22.7%). Mean follow up period was 56.9 ± 44.6 months. There were no early death, but 7 late deaths (7/37, 18.9%) due to rejection after 11, 15, 41 months (n = 3), infection after 5, 8, 10 months (n = 3), suspicious ventricular arrhythmia after 50 months (n = 1). There was no significant risk factor for survival. There were 25 rejections (25/37, 67.6%); less than grade II occurred in 17 patients (17/25, 68%) and more than grade II occurred in 8 patients (8/25, 32%). Actuarial 1, 5, and 10 yr survival was 88.6%, 76.8%, and 76.8%. Our midterm survival of pediatric heart transplantation showed excellent results. We hope this result could be an encouraging message to do more pediatric heart transplantation in Korean society.

Although the outcome of neonatal cardiac surgery has dramatically improved, low body weight (LBW) is still considered an important risk for open heart surgery. The factors contributing to poor outcomes in LBW infants, however, are still unclear. We investigated risk factors for poor outcomes in infants weighing

A 17-year-old male presented with two episodes of syncope, cyanosis, and exercise intolerance 13 years after the Fontan operation. Echocardiography and magnetic resonance imaging showed dilated intrahepatic collaterals, which drained into the atrium through the left hepatic vein, and 24-hour Holter monitoring revealed sinus node dysfunction. We performed ligation of the left hepatic vein using intrahepatic collaterals as channels draining hepatic venous blood into the Fontan pathway, and implanted an epicardial dual chamber pacemaker. At one-year follow-up, the patient remained asymptomatic and his cardiac performance was much improved. doi: 10.1111/jocs.12317 (J Card Surg 2014;29:564–566)

Background Preoperative risk analysis for Fontan candidates is still less than optimal in that patients with apparently low risks may have a poor outcome, such as prolonged pleural drainage, protein-losing enteropathy, pulmonary thromboembolism and death. We hypothesized that low pulmonary vascular compliance (PVC) is a risk factor for persistent pleural effusion after the Fontan operation. Methods A retrospective review of 85 patients who underwent the extracardiac Fontan procedures (median age: 3.87 years) was performed. Fontan risk score (FRS) was calculated from 12 categorized preoperative anatomical and physiological variables. PVC (mm 2 /m 2 ·mmHg) was defined as pulmonary artery index (mm 2 /m 2 ) divided by total pulmonary resistance (Wood Unit·m 2 ) and pulmonary blood flow (L/min/m 2 ), based on the electrical circuit analogy of the pulmonary circulation. Chest tube indwelling time was log-transformed (log indwelling time, LIT) to fit normal distribution, and the relationship between perioperative predictors and LIT was analyzed by multiple linear regression. Results Preoperative PVC, chest tube indwelling time and LIT ranged from 6 to 94.8 mm 2 /mmHg/m 2 (median: 24.8), 3 to 268 days (median: 20 days), and 1.1 to 5.6 (mean: 2.9, standard deviation: 0.8), respectively. FRS, PVC, cardiopulmonary bypass time (CPB) and central venous pressure at postoperative 12 h were correlated with LIT by univariable analyses. By multiple linear regression, PVC ( p =0.002) and CPB ( p =0.003) independently predicted LIT, explaining 22% of the variation. The regression equation was LIT=2.744−0.016 PVC+0.007 CPB. Conclusion: Low pulmonary vascular compliance is an important risk factor for prolonged pleural effusion drainage after the extracardiac Fontan procedure.

Plastic bronchitis is a rare cause of acute obstructive respiratory failure in children. Life-threatening events are much more frequent in patients with repaired cyanotic congenital heart disease, and most frequent following a Fontan operation. Commonly, the diagnosis is not made until bronchial casts are expectorated. Detailed CT findings in plastic bronchitis have not been described.To describe the CT findings in plastic bronchitis in children after a Fontan operation.Three children with plastic bronchitis after a Fontan operation were evaluated by chest CT. Bronchial casts were spontaneously expectorated and/or extracted by bronchoscopy. Airway and lung abnormalities seen on CT were analyzed in the three children.CT demonstrated bronchial casts in the central airways with associated atelectasis and consolidation in all children. The affected airways were completely or partially obstructed by the bronchial casts without associated bronchiectasis. The airway and lung abnormalities rapidly improved after removal of the bronchial casts.CT can identify airway and lung abnormalities in children with plastic bronchitis after a Fontan operation. In addition, CT can be used to guide bronchoscopy and to monitor treatment responses, and thereby may improve clinical outcomes.

Although several previous studies have reported that a ventricular septal defect (VSD) can be closed safely through the detached tricuspid valve (TV) without impairing TV function, this has not been demonstrated for infants younger than 3 months.We enrolled 296 infants younger than 3 months who underwent VSD closure through the right atrium between January 2004 and December 2013. In the study group of 49 patients (16.6%), the TV was detached for better exposure of the defect.The median age and weight were 63.5 days (14-90 days) and 4.4 kg (2.1-6.7 kg), without intergroup differences. Abnormal chordal attachment of the TV in preoperative echocardiography was associated with detachment of the TV during surgery (P = .001). Cardiopulmonary bypass (P = .001) and aortic crossclamp (P .001) times were significantly longer in the study group. A permanent pacemaker was required for 2 patients in the control group. Follow-up echocardiography was available for 282 patients at a median of 21 months (1-108 months) after the operation. On final echocardiography, tricuspid regurgitation greater than grade 2 was observed in 1 patient in the study group and residual VSD existed in 4 patients (1 in the study group), without statistical significance.Detachment of the TV can be used safely for better exposure of the VSD without increased risk of tricuspid regurgitation in infants younger than 3 months. Preoperative echocardiography might be useful for predicting the possibility of detachment of the TV for enhancing exposure of the VSD.

Indications of sutureless repair (SR) for pulmonary vein anomalies have evolved from re-operational SR for pulmonary vein stenosis after the repair of total anomalous pulmonary venous drainage (TAPVD) to primary SR for TAPVD associated with right atrial isomerism or isolated TAPVD with small individual pulmonary veins (IPVs) and an unfavorable pulmonary vein anatomy. We sought to determine whether small IPVs outgrow somatic growth after primary SR. Between 2004 and 2013, 21 children underwent primary SR for TAPVD: 13 with a functionally single ventricle, 11 with right atrial isomerism, six with isolated TAPVD, and 13 with a pulmonary venous obstruction. TAPVD types were supracardiac in nine, infracardiac in 10, and mixed in two. Utilizing cardiac computed tomography (CT), the maximal diameter of each IPV was measured, and pulmonary vein index (PVI, summation of cross-sectional areas of all four IPVs divided by body surface area) was calculated. There were five early deaths after SR. Among survivors, 10 had both preoperative and postoperative cardiac CT at a 3.6-month median interval. On postoperative cardiac CT, IPVs were patent in all patients except one who developed a left lower pulmonary vein obstruction. There was a 71 ± 48 % postoperative increase in the actual diameter of all four IPVs, and PVI increased significantly from 215 ± 55 to 402 ± 117 mm(2)/m(2) (P value = 0.005). IPVs outgrew somatic growth after primary SR of TAPVD. Primary SR may be a useful measure in TAPVD patients whose IPVs are small.

Conjoined twinning is one of the most uncommon congenital anomalies. Spencer has reported that while the incidence of conjoined twinning is close to one in 200,000 live births, 1% of all conjoined twins are stillborn and 40%–60% die shortly after birth [1]. The separation of conjoined twins presents a unique challenge to many pediatric surgical specialties. A multidisciplinary approach is essential for successful twin separation. A variety of methods for providing soft-tissue coverage have been reported in the literature and include the use of skin grafts, skin substitute products, local skin flaps, and tissue expansion. Here, we report a case of the transplantation of a latissimus dorsi musculocutaneous (LDMC) flap between separated thoraco-omphalopagus conjoined twins. A set of male thoraco-omphalopagus conjoined twins were born with joined manubrium and upper abdomen (Fig. 1). They had a fused liver, and each had a congenital heart anomaly. Computed tomography (CT) revealed that conjoined twin 1 had supracardiac total anomalous pulmonary venous return, dextrotransposition of the great arteries, and a perimembranous ventricular septal defect, while conjoined twin 2 had supracardiac total anomalous pulmonary venous return. Fig. 1 A 13-day-old thoraco-omphalopagus conjoined twins who shared the heart and the liver. Although the literature recommends that separation should be delayed until the twins gain weight, the unstable hemodynamics and deteriorating conditions of the infants prompted early surgical separation on the 14th day after birth. During the separation, an atrial connection was identified and conjoined twin 2 took most of the fused atrium. After separation of the fused heart and liver, a chest and abdominal wall defect resulted, which was larger than expected. Closure of the defect was not performed immediately because the cardiac status of the babies required further correctional surgery. The next morning, however, the heart of conjoined twin 1 ceased to beat and resuscitation for 5 hours failed. Then, to utilize the tissue of conjoined twin 1 for coverage of conjoined twin 2, emergency tissue transplantation was elected. An LDMC flap was chosen because of its widest dimension. An extended LDMC flap 11 cm×8 cm was designed on the donor baby's right side back, incorporating the skin from the scapula to the pelvis. After skeletonization of the thoracodorsal artery and concomitant veins, the flap was harvested from conjoined twin 1 (Fig. 2). The right third rib cartilage of conjoined twin 2 was partially removed to expose the internal mammary artery and vein. Anastomosis of the vessel was achieved with 10-0 nylon sutures. The estimated ischemic time was longer than 8 hours. After applying acellular dermal matrix over the pericardium and intestine, we attempted to cover the whole defect with the flap, but an abrupt drop of the blood pressure by the cardiac tamponade effect followed. Then, we decided to leave part of the defect open and fixed the flap along the right margin of the defect (Fig. 3). The flap showed serious congestion immediately after the anastomosis but improved gradually. Fig. 2 Tissue transplantation was decided, and the latissimus dorsi musculocutaneous flap was chosen to cover the thoraco-abdominal defect. The latissimus dorsi musculocutaneous flap measuring 11 cm×8 cm was designed on the back of 15-day-old conjoined ... Fig. 3 The thoracoabdominal wall could not be totally covered with the latissimus dorsi myocutaneous free flap; therefore, Permacol was applied to the remaining defect measuring about 12 cm×7 cm. (A) Immediately after flap transfer, generalized congestion ... After 3 weeks, two-thirds of the flap was found to survive after debridement of the necrotized margin and functioned as a covering of the heart. The rest of the defect was planned to be closed later, but the baby could not recuperate and failed to survive until day 100. The cause of death was the eventual failure to maintain the cardiac output. The role of plastic surgeons in the separation of conjoined twins is to provide sufficient soft tissue coverage to the body wall. Skin grafting from one twin who had died to the other twin was reported by Lehr et al. [2]. Currently, various skin substitute products are available and can be used to cover deficient areas of the skin and even to reconstruct fascial defects. Random pattern or arterialized flaps of skin or skin and muscle can be used for coverage. Jackson et al. [3] reported the use of bipedicled rotational flaps of the expanded skin and local tissue adjacent to the defect when the expanded soft tissue alone proved to be insufficient for closure by simple advancement. After the first report of the successful use of tissue expansion by Zuker et al. [4] in 1986 for the separation of an ischiopagus conjoined twin, numerous reports have followed using tissue expansion in both the intraperitoneal and subcutaneous positions. To the best of our knowledge, our case is the first case of free flap transfer for a soft tissue defect from one twin to the other twin after the separation of conjoined twins. A case of free flap transfer in neonates was reported by Van Landuyt et al. [5]. They used a thoracodorsal artery perforator free flap successfully for the skin necrosis of an 11-week-old premature boy who weighed 1,160 g. We performed free flap transfer for the 15-day-old baby who weighed 2,410 g. Unfortunately, however, the defect after the separation of the thoraco-omphalopagus type of conjoined twin could not be successfully covered with a unilateral LDMC free flap. The defect could have been closed if we had used bilateral LDMC free flaps. There were two limitations in our case. First, elevation of the LDMC flap was done after failed resuscitation of the donor baby. During the elevation of the flap, the heart of the donor baby ceased to beat and there was no blood flow onto the LDMC flap. The other limitation was that flap elevation and recipient preparation were not performed simultaneously due to the unavailability of the operating room and equipment. Therefore, the ischemic time of the LDMC flap was more than 8 hours, which might be one of the reasons for the initial flap instability and partial flap loss. We were able to successfully transplant a musculocutaneous flap with microvascular anastomosis in the 15 day-old newborn baby weighing 2,410 g. This case also revealed the graveness of the defect after separation of the thoraco-omphalopagus conjoined twins and the importance of addressing and discussing calculative planning for the utilization of the available soft tissue, as well as that of vital organs such as the heart and the liver, before the separation of conjoined twins.

Coronary arteriovenous fistula (CAVF) is a rare condition defined as an anomalous termination of the coronary arteries. The etiology of CAVF is either congenital or acquired, and iatrogenic CAVF is most commonly caused by cardiovascular surgery or percutaneous intervention. Most of the prenatally diagnosed CAVFs were related to complex heart disease, and only few cases of an isolated CAVF have been reported to date. We successfully diagnosed an isolated CAVF by fetal echocardiography at 25.3 weeks of gestation. Accurate prenatal diagnosis resulted in the prompt decision for postnatal surgical correction, and the neonate thrived well without any complications.

Status asthmaticus is a rare, fatal condition, especially in children. Sometimes respiratory support is insufficient with a mechanical ventilator or medical therapy for patients with status asthmaticus. In such situations, early extracorporeal membrane oxygenation application is a useful method for treating refractory respiratory failure. We report on a case of a six-year-old, male child who underwent venovenous extracorporeal membrane oxygenation support for refractory status asthmaticus.

BACKGROUND For patients undergoing general anesthesia, adequate warming and humidification of the inspired gases is very important. The aim of this study was to evaluate the differences in the heat and moisture content of the inspired gases with low-flow anesthesia using 4 different anesthesia machines. METHODS The patients were divided into 11 groups according to the anesthesia machine used (Ohmeda, Excel; Avance; Drager, Cato; and Primus) and the fresh gas flow (FGF) rate (0.5, 1, and 4 L/min). The temperature and absolute humidity of the inspired gas in the inspiratory limbs were measured at 5, 10, 15, 30, 45, 60, 75, 90, 105, and 120 minutes in 9 patients scheduled for total thyroidectomy or cervical spine operation in each group. RESULTS The anesthesia machines of Excel, Avance, Cato, and Primus did not show statistically significant changes in the inspired gas temperatures over time within each group with various FGFs. They, however, showed statistically significant changes in the absolute humidity of the inspired gas over time within each group with low FGF anesthesia (P

Background . The Lecompte procedure for correcting transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. In this study, we evaluated the effectiveness and the application of the Lecompte procedure based on our experiences. Methods . A retrospective review was conducted of the records of 45 patients who underwent the Lecompte procedure during the past 11 years to achieve direct right ventricle to pulmonary artery continuity. Mean age at operation was 2.4 ± 1.7 years (range 3.5 months to 6.9 years). The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Results . Early mortality was 4.4% (2 of 45 patients) and late mortality was 4.7% (2 of 43). The mean follow-up was 4.9 ± 3.1 years. Fourteen patients (34.1% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mm Hg), the main reason for which was a calcified monocusp valve (n = 10, 71.4%). Eight of 45 patients (17.8%) underwent reoperation: 2 for residual ventricular septal defect, 1 for recurrent septic vegetation, and 5 for pulmonary stenosis. The cumulative survival rates were 91.1% ± 4.2% at 10 years. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 93.8% ± 4.3% and 71.4% ± 11.8% at 5 and 10 years, respectively. Conclusions . Our review suggests that the Lecompte procedure is an effective treatment modality for anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

Introduction: To report our experience in fetuses prenatally diagnosed with common arterial trunk (CAT) and to evaluate the postnatal outcomes. Material and Methods: This was a retrospective study conducted at Asan Medical Center, Seoul, Korea, between 2003 and 2012. Maternal medical and fetal echocardiographic records regarding fetuses were prenatally diagnosed with CAT were reviewed. Postnatal outcomes of survivors were also assessed during the mean follow-up of 36 months. Results: Of the 17 fetuses that were prenatally diagnosed with CAT and had a final diagnosis by postnatal echocardiography or autopsy, 12 were confirmed to have CAT and 5 were incorrectly diagnosed with CAT. Of the latter, 3 had pulmonary atresia and 2 had aortic atresia. The diagnostic accuracy was 71%. The median gestational age at prenatal diagnosis of confirmed CAT was 24.4 weeks (range 21.1-34.3). The truncal valve was prenatally thickened in 8 fetuses. All but 1 of the 12 confirmed CAT cases had associated heart and/or extracardiac anomalies. Of the 8 liveborn cases of postnatally confirmed CAT, 2 died before or after surgery respectively. The remaining 6 remained alive after successful corrective surgery. Conclusion: Prenatal diagnosis of CAT can be difficult in some cases and other diseases should be excluded before diagnosing CAT. Isolated CAT can be repaired by postnatal corrective surgery with a good outcome.

Compared with mitral repair, mitral valve replacement is an uncommon procedure in children due to associated high mortality and morbidity rates. The present study investigated early and late outcomes after MVR with mechanical prostheses in children at our institution. Between January 1994 and December 2009, 19 children underwent MVR. Mean patient age was 7.6 ± 5.5 years (range 3 months–16 years), and mean body weight was 23.7 ± 15.1 kg (range 5.0–58.1 kg). Mean prosthesis size was 25.8 ± 4.2 mm (range 19–31 mm). There were no operative or late mortalities. Three patients showed decreased left-ventricular function before surgery, and one of them underwent successful heart transplantation due to progressive LV dysfunction at 10 months after MVR. The proportion of patients with freedom from reoperation at 10 years was 94.7 ± 5%. There were no major thromboembolic or bleeding episodes. Although the small number of patients in our study was a limitation, MVR in children was found to result in excellent early and long-term outcomes. It appears that MVR could be considered in children before LV dysfunction develops.

Background Protein-losing enteropathy (PLE) is a life-threatening and poorly understood complication after the Fontan operation. We sought to determine the pre-operative risk factors for PLE which developed after the extracardiac conduit Fontan operation. Methods Two hundred thirty-five patients who underwent the extracardiac conduit Fontan operation as an initial Fontan type procedure (median age at operation: 3.5years) were enrolled in this cross-sectional retrospective study. Pre-operative and peri-operative variables were surveyed through a review of medical records. Results Within the median follow-up duration of 5years, 12 patients developed PLE (12/234, 5.1%) at a median interval of 2.2years after the Fontan procedure, and 4 died of PLE at a median interval of 1.2years (range 0.21–7.62) after diagnosis. Factors found to be related to the time to the development of PLE on univariate analysis were pulmonary vascular compliance (Cpv) (p=0.0019), central venous pressure at postoperative 12hours (p=0.0026), days of ICU stay (P=0.0449), days of hospitalization (p=0.0135), and days of chest tube indwelling (p=0.0493). Multivariate analysis, however, showed that only Cpv (p=0.0367) remained significant. The range of Cpv was 8.8–26.1mm 2 /m 2 /mmHg (median 17.9) in patients with PLE, and 6.6–122.3mm 2 /m 2 /mmHg (median 26.8) in patients without PLE. Conclusions Low pulmonary vascular compliance is associated with the development of PLE after the extracardiac conduit Fontan operation.

Background Optimal management of muscular ventricular septal defects (MVSD) is still not determined in the current era. Moreover, long-term left ventricular function after closure of MVSD is not well known. Thus, we investigated surgical outcomes including long-term left ventricular function after closure of MVSD through left ventriculotomy. Methods We conducted a retrospective review of medical records of 20 children who underwent MVSD closure between March 1993 and August 2010. There were 10 boys (50%) and 10 girls (50%). Patient age ranged from 1.6 to 103.4 months (median, 26.4 months), and body weight from 2.8 to 31.5 kg (median, 11.9 kg). Electrocardiogram results were normal sinus rhythm in all except 1 patient with congenital complete atrioventricular block. There were 16 patients who previously had palliative pulmonary artery banding procedures before closure of MVSD. There were 13 patients (65%) with Swiss-cheese type VSD. Results There was 1 hospital death of a patient with congenital complete atrioventricular block with pacemaker malfunction (5%). There was 1 late death of a patient with del 22q with adenoviral pneumonia. There was no reoperation. Median follow-up duration was 85.9 months (range, 4.7 to 166.7). The location of MVSD was apical portion in 10 patients (50%) and midtrabecular portion in 9 patients (45%). There were 6 Dacron patch closures and 13 direct closures of MVSD through left ventriculotomy. There was no complete atrioventricular block. Last follow-up echocardiographic data showed normal ejection fraction with 65.2% ± 8.2% after closure of MVSDs. There was no leakage in 8 patients; 11 patients had insignificant leakage, which disappeared spontaneously in 4 patients 17.9 months (median value) after operation. Conclusions Our acceptable long-term results of left ventricular function after left ventriculotomy proved that this technique might be a viable option in the management of MVSD.

Background Although mild mitral regurgitation (MR) associated with atrial septal defect (ASD) is believed to improve by ASD closure alone, new-onset MR or aggravation of preexisting mild MR may also develop after ASD closure. We sought to determine the preoperative risk factors for the postoperative deterioration of mitral valve competence in adults with ASD. Methods Retrospective review of 286 adults with ASD who had undergone surgical closure of ASD between January 2004 and December 2009 was performed. Patients with significant preoperative MR (≥III/IV) that necessitated mitral valve intervention were excluded. Preoperative MR grades were 0 in 204 patients, I in 67 patients, and II in 15 patients. Results After ASD repair, MR degree did not change or improved in most of the patients (204 of 286, 71%), whereas 82 patients (82 of 286, 29%) showed aggravated or new-onset MR, including 18 patients whose MR degree increased by grade 2 or more. Univariable analysis revealed older age, preoperative atrial fibrillation, large left atrial size, large indexed ASD size, high peak tricuspid regurgitation velocity, and associated tricuspid valve surgery or Maze procedure as risk factors for new-onset or aggravated MR, but only older age (odds ratio, 1.058; 95% confidence interval, 1.025 to 1.092; p = 0.0005) and indexed ASD size (odds ratio, 1.120; 95% confidence interval, 1.042 to 1.205; p = 0.0022) remained significant on multivariable analysis. The ratio of postoperative left ventricular end-diastolic volume to preoperative left ventricular end-diastolic volume was greater in patients with new-onset or aggravated MR (p = 0.016). Conclusions Mitral valve competence may deteriorate after ASD closure in older patients with large ASD.

Objective: The surgical approach for treating supravalvar aortic stenosis (SVAS) has evolved from a plain patch technique to a threedimensional patch repair, which has some drawbacks. Here, we report on the midterm outcomes after using our modified simple sliding aortoplasty preserving sinotubular junction without foreign material for surgical correction of SVAS. Methods: Between June 2001 and February 2010,18children(medianage,6.2years;range0.5—2.2years)with discreteSVASunderwentsurgicalrepair. Afterastandardmediansternotomy, cardiopulmonary bypass and cardioplegic arrest, the aorta was transected obliquely just distal to the point of stenosis. An incision was then made into the non-coronary sinus of the proximal aorta, and a counterincision was made into the lesser curvature of the ascending aorta, after which, the proximal and distal aorta were anastomosed directly with a running suture. Peak pressure gradients were estimated using echocardiography and the data were reviewed retrospectively.Results:The median follow-up periodwas 39.6 months(range, 1—104.5 months). There was no early or late death. No patients required re-operation. Ten patients had Williams—Beuren syndrome.There were eight cases of concomitant pulmonary artery angioplasty. The mean pressure gradient decreased from 65.9 18.4 mmHg preoperatively to 15.2 8.9 mmHg at the final follow-up (P = 0.01). There was no significant, more than mild aortic regurgitation. Conclusions: Our modified simple sliding aortoplasty showed excellent surgical results, and may be a good option for discrete SVAS. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Background: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. Materials and Methods: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 ㎏, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was 79.2±14.8 months. Results: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. Conclusion: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.

ObjectiveAortic translocation has received growing attention in the management of complete transposition with ventricular septal defect and pulmonary stenosis, but the criteria regarding pulmonary stenosis for selecting this option have yet to be established. The aim of this study is to evaluate the significance of pulmonary annulus size with the outcome after the arterial switch operation.MethodsBetween November 1996 and September 2008, 250 patients underwent the arterial switch operation for complete transposition. Among them, 8 patients with a pressure gradient greater than 30 mm Hg, bicuspid pulmonary valve, and an aortic Z-score of the pulmonary annulus less than 0 were included in this retrospective study. The median age was 19.1 months (range, 0.5–80.0 months). The median follow-up was 39.7 months (range 9.1–139.5 months).ResultsAt latest follow-up, the Z-score of the neoaortic annulus increased from −1.50 ± 1.13 (range, −3.42 to −0.35) to 1.10 ± 1.15 (range, −0.8 to 2.10) (P < .01). No patient had a significant pressure gradient across the left ventricular outflow tract. There was 1 early death and there were no late deaths. Two reoperations were performed in 1 patient for neoaortic stenosis at 81 months and 110 months after the operation. Latest echocardiogram revealed grade 0 or 1 neoaortic insufficiency.ConclusionIt was possible to extend the indication for the arterial switch operation with acceptable outcome to the patient with a Z-score of about −3 of the pulmonary annulus despite bicuspid pulmonary valve. Inasmuch as the arterial switch operation has benefits over the other options, a large-scale study is required for more reasonable triage in this group of patients.

From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure. She could survive by 100 days of extracorporeal membrane oxygenation support and finally, underwent heart-lung transplantation. This is the first successful pediatric heart-lung transplantation carried out in Korea., Graphical Abstract

Background Patients with arch obstruction and intracardiac defects have a high probability of abnormal aortopulmonary space geometry, which provides airway compression. The tissue-to-tissue technique arch repair could result in real airway problems. This report describes our experience with the perioperative evaluation and management of airway problems. Methods We retrospectively reviewed the medical records of 90 patients with arch obstruction and intracardiac defects who underwent computed tomography (CT) and corrective surgery in our institution between January 2000 and January 2007. Results Of the 77 patients who underwent preoperative CT (group 1), 21 were found to have airway compression (27.2%).Of those 21 patients, 5 underwent concomitant airway relieving procedures. In group 1, 2 patients required subsequent secondary surgery for airway problems after the initial arch repair. Of the 13 patients who underwent postoperative CT only (group 2), 6 underwent subsequent secondary surgery for airway relief. For airway relief, several procedures were additionally performed (eg, right pulmonary artery translocation anterior to the aorta, aortopexy, peribronchial dissection, and tissue augmentation). In terms of the type of arch repair, 48 patients underwent end-to-side anastomosis, 39 underwent extended end-to-end anastomosis, and 3 underwent end-to-end anastomosis. End-to-side was the repair type most commonly associated with airway compression requiring additional procedure (10 of 15, 66.6%). Conclusions Patients with arch obstruction and intracardiac defects had a rather high incidence of airway compression preoperatively and postoperatively. Preoperative CT and intraoperative complementary bronchoscopy were useful for identifying and fixing the airway problems. Additional procedures for relieving airway compression were required more frequently after end-to-side type arch repair than after extended end-to-end anastomosis. More meticulous intraoperative evaluation and management are recommended in this type of repair.

ObjectivesRecurrent or newly developing aortic regurgitation is a critical problem after the repair of ruptured sinus of Valsalva aneurysm.MethodsA retrospective review of 56 patients who underwent surgical repair of ruptured sinus of Valsalva aneurysm between June 1990 and August 2006 was performed. Rupture of the right coronary sinus into the right ventricle was the most common anatomic type (39/56, 69.6%). Preoperative aortic regurgitation equal to or greater than grade II (n = 8, 17.9%) was managed by repair (aortic valvuloplasty, n = 5) or replacement (n = 3). Ruptured sinus of Valsalva aneurysm was repaired primarily (n = 7) or by patching (n = 10) through an aortotomy in 17 patients (transaortic group). In the remaining patients (n = 39), ruptured sinus of Valsalva aneurysm was repaired primarily from the chamber into which the corresponding aortic sinus ruptured, and the aneurysmal sac was reinforced with a supporting patch (non-transaortic group).ResultsMedian follow-up duration was 46 months (0.4–177 months). There were 2 late deaths. Excluding 3 patients with aortic valve replacement on aneurysm repair, 11 patients (11/53, 21%) had recurrent or new-onset significant aortic regurgitation (≥II/IV) during the follow-up period. By multivariable analysis, aortic valvuloplasty at initial operation was the only significant risk factor for postoperative aortic regurgitation (P < .001). After adjustment, the non-transaortic approach appeared to be associated with a lower risk of postoperative aortic regurgitation, with marginal significance (hazard ratio 0.28; P = .058). Five-year freedom from significant aortic regurgitation in the transaortic and non-transaortic groups was 68% ± 12% and 94% ± 4%, respectively.ConclusionTransaortic repair of ruptured sinus of Valsalva aneurysm may cause postoperative aortic regurgitation by progressive distortion of the aortic sinus geometry.

We report a woman with atrial septal defect and severe pulmonary hypertension with 25.0 Wood unit.m(2) of indexed total pulmonary vascular resistance. She underwent successful corrective repair of atrial septal defect after 2 years of treatment with sildenafil, and has been monitored for 4 years after repair. This case supports a "treat and repair" approach using advanced pulmonary vasodilator therapy in selected patients with inoperable severe pulmonary hypertension associated with atrial septal defect.

PAGOD syndrome is an extremely rare congenital malformation complex involving multiple organs. These include pulmonary artery and lung hypoplasia, diaphragm defects, omphalocele, sex reversal or ambiguous genitalia, and complex cardiac defects. Eight cases have been reported to date. We report a case of PAGOD syndrome that is manifested by right diaphragm eventration, horseshoe lung with right lung hypoplasia, hypoplastic left heart syndrome (mitral atresia, aortic atresia), scimitar syndrome, agonadism with ambiguous genitalia. A karyotype revealed normal 46-XY. This patient received a modified Norwood procedure for hypoplastic left heart syndrome as an initial palliation and bidirectional cavopulmonary anastomosis as a second stage of operation. The postoperative courses were uneventful. This patient is waiting for Fontan operation.

【Extracorporeal membrane oxygenation (ECMO) has been utilized in congenital diaphragmatic hernia (CDH) patients with severe respiratory failure unresponsive to conventional medical treatment. We retrospectively reviewed 12 CDH patients who were treated using ECMO in our center between April 2008 and February 2011. The pre ECMO and on ECMO variables analyzed included gestational age, sex, birth weight, age at the time of ECMO cannulation, arterial blood gas analysis results, CDH location, timing of CDH repair operation, complications and survival. There were 9 boys and 3 girls. All patients were prenatally diagnosed. Mean gestational age was $38.8{\pm}1.7$ weeks and mean birth weight was $3031{\pm}499$ gram. Mean age at the time of ECMO cannulation was $29.9{\pm}28.9$ hours. There were 4 patients who survived. Survivors showed higher 5 min Apgar scores ( $8.25{\pm}0.96$ vs. $7.00{\pm}1.20$ , p=0.109), higher pre ECMO mean pH ( $7.258 {\pm}0.830$ vs. $7.159{\pm}0.986$ , p=0.073) and lower pre ECMO $PaCO_2$ ( $48.2{\pm}7.9$ vs. $64.8{\pm}16.1$ , p=0.109) without statistical significance. The hernia was located on the left side in 10 patients and the right side in 2 patients. The time interval from ECMO placement to operative repair was about 3~4 days in 5 early cases and around 24 in the remaining cases. There were 3 cases of post operative bleeding requiring re operation and 2 cases of abdominal compartment syndrome requiring abdominal fascia reopening. ECMO catheter reposition was required in 4 cases. Three cases of arterial or venous thrombosis were detected and improved with follow up. Our data suggests that ECMO therapy could save the lives of some neonates with CDH who can not be maintained on other treatment modalities. Protocolized management and accumulation of case experience might be valuable in improving outcomes for neonates with CDH treated with ECMO.】

Background Although it has been inferred that a biatrial maze procedure for atrial fibrillation in left-sided heart lesions may lead to better outcomes compared with a limited left atrial lesion set, it remains controversial whether the biatrial maze procedure is superior to the right atrial maze procedure in right-sided heart lesions. Methods A retrospective review was performed for 56 adults who underwent surgical closure of atrial septal defect and various maze procedures for atrial fibrillation between June 1998 and February 2011. The median age at operation was 59 years (range, 34-79 years). Clinical manifestations of atrial fibrillation were paroxysmal in 8 patients, persistent in 15 patients, and long-standing persistent in 33 patients. A right atrial maze procedure was performed in 23 patients (group 1), and a biatrial maze procedure was performed in 33 patients (group 2). Treatment failure was defined as atrial fibrillation recurrence, development of atrial flutter or other types of atrial tachyarrhythmia, or implantation of a permanent pacemaker. The Cox proportional hazards model was used to identify risk factors for decreased time to treatment failure. Results During the median follow-up period of 49 months (range, 5-149 months), there was no early death and 1 late noncardiac death. On Cox survival model, group 1 showed a significantly decreased time to treatment failure in comparison with group 2 (hazard ratio, 5.11; 95% confidence interval, 1.59-16.44; P = .006). Maintenance of normal sinus rhythm without any episode of atrial fibrillation recurrence at 2 and 5 years postoperatively was 57% and 45% in group 1, respectively, and 82% and 69% in group 2, respectively. Conclusions Left-sided ablation in addition to a right atrial maze procedure leads to better electrophysiologic outcome in atrial fibrillation associated with atrial septal defect.