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Background Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The optimal management of patients with AAOCA is controversial. We examined our experience with surgical unroofing of AAOCA to determine the midterm effect of surgical repair. Methods From October 1992 through December 2011, 75 patients with AAOCA underwent surgical unroofing. Results Mean age was 39.6 ± 19.6 years; 23 patients (32%) were aged younger than 30 years. Angina, shortness of breath, or syncope was present in 55 patients (72%); 2 (3%) had history of sudden cardiac arrest. Of 40 patients (53%) who had preoperative stress tests, results were abnormal in 20 (50%). Coronary or computed tomography angiography demonstrated an anomalous right coronary artery (RCA) arising from the left sinus in 69 patients (92%) and the left main coronary artery arising from the right sinus in 6 (8%). Two patents (3%) were referred for recurrent anginal symptoms after previous RCA bypass with the right internal mammary artery. Minimally invasive partial upper sternal split was performed in 17 patients (22%). Two patients (3%) needed right internal mammary artery-to-RCA grafting due to flow acceleration at the RCA ostium. There were no early deaths. One late death (1%) occurred related to noncardiac causes. At follow-up (mean, 18 months; maximum, 7 years), all patients remained free of cardiac symptoms. Conclusions Surgical unroofing of AAOCA is associated with low morbidity and mortality. At intermediate follow-up, resolution of symptoms and freedom from sudden death can be expected. The threshold for offering intervention should be low.

Background The vasoactive inotrope score (VIS) is a sum of the total vasopressor dose at a single point in time. Incorporating duration and magnitude of vasopressor requirements during the postcardiac surgical period could improve VIS sensitivity for predicting poor outcome. Methods This is a retrospective review of 244 infants (aged ≤365 days) who underwent cardiopulmonary bypass during congenital cardiac operations from 2002 to 2011. The VIS was calculated hourly for the first 72 hours. Poor outcome was defined as prolonged mechanical ventilation (≥6 days) or intensive care length of stay (≥12 days). First, the association between the maximum VIS (maxVIS) in the first 48 postoperative hours and poor outcome was confirmed for our study population. Next, postoperative intervals and VIS values that were significantly associated with poor outcome were identified and incorporated into a formula, termed the VISindex, which was compared with the traditional maxVIS. Results The VISindex demonstrated improved sensitivity for predicting prolonged mechanical ventilation (VISindex: area under the curve [AUC], 0.85; 95% confidence interval [CI], 0.79 to 0.90; maxVIS: AUC, 0.80; 95% CI, 0.75 to 0.86) and intensive care unit length of stay (VISindex: AUC, 0.84; 95% CI, 0.79 to 0.89; maxVIS: AUC, 0.77; 95% CI, 0.71 to 0.83) after cardiac operations in infants. Conclusions Incorporating magnitude and duration of postoperative vasopressor support into the VIS improves its sensitivity for predicting poor outcome.

A clear consensus regarding the optimal source of pulmonary blood flow in patients with hypoplastic left heart syndrome undergoing the Norwood procedure is lacking.A literature search was undertaken to identify relevant articles from 2005 to 2012 using "Norwood, stage 1 palliation," "Modified Blalock Taussig shunt (MBTS)," "right ventricle-to-pulmonary artery shunt (RV-PAS)" alone or in combination. Three end points were selected: early/stage 1 mortality, interstage mortality, and interstage total/shunt intervention.A total of 20 articles, including 19 observational studies and 1 randomized trial (MBTS, n=1,343; RV-PAS, n=1,028), met the inclusion criteria. Mortality after stage 1 was 22% in the MBTS cohort and 16% in RV-PAS cohort. A pooled analysis showed no difference in early mortality between the two groups (risk ratio [RR], 1.20; 95% confidence interval [CI], 0.99 to 1.45; p=0.07). On pooling data from contemporary series (similar era) of 8 studies (MBTS, n=709; RV-PAS, n=631), to minimize variability in surgical and postoperative management practices, early mortality in both cohorts was comparable (RR, 1.14; 95% CI, 0.89 to 1.45; p=0.29). Interstage mortality was 13.8% and 4.6% in the MBTS and RV-PAS cohorts, respectively, and was significantly lower for RV-PAS (RR, 2.85; 95% CI, 1.65 to 4.89; p0.00002). However, patients with MBTS had fewer shunt interventions (RR, 0.55; 95% CI, 0.44 to 0.68; p0.001; I2=00%).Our pooled analysis demonstrated no survival benefit for the MBTS or RV-PAS in patients undergoing the Norwood procedure. There appears to be an advantage with the RV-PAS with regard to interstage mortality at the cost of an increased rate of shunt intervention.

Background Patients with atriopulmonary Fontan tend to undergo conversion to total cavopulmonary connections secondary to arrhythmias or poor flow dynamics. However, the ideal candidate is unknown. Methods Between December 1994 and May 2011, 70 patients (40 males [57%]) underwent Fontan conversion. Median age was 23 years (range, 4 to 46 years). Excluded were 1.5 ventricle conversions. The most common diagnoses included tricuspid atresia in 34patients (49%) and double-inlet left ventricle in 16 (23%). Atrial tachyarrhythmia was present in 62 patients (89%), 41 (59%) had atrioventricular valve (AVV) regurgitation, and 32 (46%) were in New York Heart Association class III or IV. Atriopulmonary Fontan was the original connection in 58 patients (83%), whereas the Bjork modification was performed in 8 (11%). Results Fontan was performed with an intraatrial conduit in 41 patients, an extracardiac conduit in 18, and a lateral tunnel in 11. Forty-nine patients (70%) underwent concomitant arrhythmia operations. Early death occurred in 10 patients (14%). Multivariate analysis revealed age older than 27 years ( p = 0.009), AVV regurgitation ( p = 0.016), lack of arrhythmia operation ( p = 0.04), and male sex ( p = 0.02) were predictors of perioperative death. Mean follow-up was 5 years (maximum, 17 years). Overall survival at 1, 5, and 10 years was 81%, 70%, and 67%, respectively, and 84% of patients were in New York Heart Association class I or II. Conclusions Proper selection of Fontan conversion candidates is critical. Concomitant arrhythmia operations may be associated with improved survival. Older age and AVV regurgitation increase the risk of poor outcome, and cardiac transplantation may be a better option.

Background Successful tricuspid repair for Ebstein malformation (EM) is challenging. Cone repair has recently emerged as the most anatomic repair technique. There are no data on tricuspid re-repair in EM. We reviewed our experience with tricuspid valve (TV) re-repair using a cone technique. Methods Between June 2007 and October 2012, 134 patients underwent cone repair. Prior TV repair was performed in 20 patients (10 female, 50%). Median age was 15 years (range, 4 to 68 years). Four patients (20%) had prior bidirectional cavopulmonary anastomosis (BDCPA). Preoperative heart failure was present in 8 patients (40%). Recurrent tricuspid regurgitation (TR) was due to incomplete leaflet coaptation with tethered anterior leaflet in all patients; and 10 patients (50%) had diminutive septal leaflet. Prior repair techniques consisted of annuloplasty maneuvers in all patients with no or incomplete surgical delamination in all patients. Results The TV re-repair using cone technique was possible in all patients. Plication of atrialized right ventricle was done in 6 patients (30%). Modifications included annuloplasty band in 16 patients (80%) and TV leaflet augmentation in 8 (40%). The BDCPA was performed in 3 patients (15%) and modified Cox-maze procedure was done in 10 (50%). There were no mortalities or reoperations. Mean follow-up was 7.7 ± 10.7 months; during follow-up, 18 patients had no or mild TR and 2 had moderate TR. Conclusions TV re-repair in EM using the cone technique is feasible, particularly when there has been no surgical leaflet delamination at initial operation. Leaflet augmentation facilitates and annuloplasty band stabilizes a competent TV re-repair. Longer follow-up is required to assess durability.

Background Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 ( p = 0.008) and the presence of significant common AVV regurgitation ( p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV ( p = 0.95). The presence of significant right AVV regurgitation was associated with late death ( p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV ( p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV ( p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation ( p = 0.018) and repair before 1990 ( p = 0.041) were risk factors for late reoperation in both groups. Conclusions Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.

Background The management of the zone of apposition (ZOA) in patients with atrioventricular septal defect (AVSD) and parachute left atrioventricular valve (LAVV) is controversial. Methods Between 1977 and 2010, 28 patients with parachute LAVV associated with AVSD were reviewed. The median age at operation was 10 months (range, 36 days to 14 years). Sixteen (57%) patients had complete AVSD and 12 (43%) had partial AVSD. Thirteen (46%) patients had moderate to severe LAVV regurgitation. Results The ZOA was managed with complete closure in 6 (22%), partial closure in 10 (36%), and no closure in 11 (39%) patients One patient underwent LAVV replacement for dysplastic leaflets. Dismissal echocardiogram demonstrated moderate LAVV regurgitation in 10 (36%) patients; 7 patients had no closure of ZOA, and 3 had partial closure. Mild or moderate LAVV stenosis was present in all 6 patients with complete closure of ZOA and 1 patient with partial closure. Median follow-up was 9 years (maximum, 22 years). Eight patients had progression of LAVV regurgitation through the unsutured ZOA; 6 patients subsequently underwent LAVV replacement. Of the 7 patients who had LAVV stenosis, 1 patient required opening of ZOA 1 month after surgery. The other 6 patients had a decrease in mean gradient. There was 1 late death after the fourth redo LAVV replacement. Conclusions Progression of LAVV regurgitation from the unsutured ZOA was the main indication for reoperation in parachute LAVV with AVSD. The ZOA in parachute LAVV should be partially or completely closed at the time of AVSD repair. Although mild LAVV stenosis appeared to improve with time, life-long surveillance is essential.

Background We developed and tested a clinical simulation program in the principles and conduct of postcardiotomy extracorporeal membrane oxygenation (ECMO) with the aim of improving confidence, proficiency, and crisis management. Methods Twenty-three thoracic surgery residents from unique residency programs participated in an ECMO course involving didactic lectures and hands-on simulation. A current postcardiotomy ECMO circuit was used in a simulation center to give residents training with basic operations and crisis management. Pretraining and posttraining assessments concerning confidence and knowledge were administered. Before and after the training, residents were asked to identify components of the ECMO circuit and manage crisis scenarios, including venous line collapse, arterial hypertension, and arterial desaturation. Results In the hands-on portion, residents had difficulty identifying the gas source and flow rate, centrifugal pump head inlet, and oxygenator outflow line. Timely and accurate ECMO component identification improved significantly after training. The arterial desaturation crisis scenario gave the residents difficulty, with only 22% providing the appropriate treatment recommendations in a timely and accurate fashion. At the end of the simulation training, most residents were able to manage the crises correctly in a timely manner. Posttraining confidence-related scores increased significantly. Most of the residents strongly recommended the course to their peers and reported simulation-based training was helpful in their postcardiotomy ECMO education. Conclusions We developed a simulation-based postcardiotomy ECMO training program that resulted in improved ECMO confidence in thoracic surgery residents. Crisis management in a simulated environment enabled residents to acquire technical and behavioral skills that are important in managing critical ECMO-related problems.

Obstructive hypertrophic cardiomyopathy (HCM) is an important cause of heart failure in children, but there are limited data addressing outcome of myectomy in children. Our objective was to evaluate the early and late results of septal myectomy in pediatric HCM.We reviewed 127 consecutive patients (62% male) who underwent transaortic septal myectomy for obstructive HCM from January 1975 to December 2010 at 21 or less years of age. Mean age at operation was 12.9 ± 5.5 years. Preoperatively, mean maximum instantaneous gradient was 89 mm Hg and 95% had significant systolic anterior motion (SAM) with mitral regurgitation (MR). Implantable cardioverter defibrillator (ICD) and permanent pacemaker prior to surgery was present in 21 patients (17%) and 15 (11.7%), respectively.Transaortic extended left ventricular septal myectomy was performed in all patients with no early deaths. Iatrogenic morbidity included new aortic valve regurgitation requiring repair in 7 (5.5%), mitral regurgitation needing repair in 2 (1.5%), ventricular septal defect in 1 (1%), and heart block requiring permanent pacemaker in 1 (1%). An ICD was implanted postoperatively in 8 during the same hospital admission. Mean MIG decreased from 89 to 6 mm Hg (p0.0001). Postoperatively, residual chordal SAM was present in 23% with mild or no MR; moderate MR was detected in 1 patient. Four patients (3%) died late during the mean follow-up period of 8.3 years (maximum, 37 years); 1 death was sudden. Overall survival was 98.6%, 94.9%, 92.4%, and 92.4% at 5, 10, 15, and 20 years, respectively. Freedom from any cardiac reoperation was 91.2%, 87.8%, 78.7%, and 72.7% at 5, 10, 15, and 20 years, respectively. Repeat septal myectomy was performed in 6 patients (5%). At late follow-up, 95% were in New York Heart Association functional class I or II and 25 patients underwent late ICD placement.Septal myectomy is safe and effective in children with obstructive HCM, but limited exposure may increase risk of aortic or mitral valve injury. Late survival is better than the previously published untreated natural history of HCM. Patient selection and surgical expertise remain critical components of septal myectomy, especially before considering a prophylactic myectomy in a seemingly asymptomatic patient.

Background Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction. Methods Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%). Results All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01). Conclusions The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction.

Background Operating room surgical training has significant limitations. This study hypothesized that some skills could be learned efficiently and safely by using simulation with component task training, deliberate practice, progressive complexity, and experienced coaching to produce safer cardiac surgeons. Methods Training modules included cardiopulmonary bypass, coronary artery bypass grafting, aortic valve replacement, massive air embolism, acute intraoperative aortic dissection, and sudden deterioration in cardiac function. Using deliberate practice, first-year cardiothoracic surgical residents at eight institutions were trained and evaluated on component tasks for each module and later on full cardiac operations. Evaluations were based on five-point Likert-scale tools indexed by module, session, task items, and repetitions. Statistical analyses relied on generalized linear model estimation and corresponding confidence intervals. Results The 27 residents who participated demonstrated improvement with practice repetitions resulting in excellent final scores per module (mean ± two SEs): cardiopulmonary bypass, 4.80 ± 0.12; coronary artery bypass grafting, 4.41 ± 0.19; aortic valve replacement, 4.51 ± 0.20; massive air embolism, 0.68 ± 0.14; acute intraoperative aortic dissection, 4.52 ± 0.17; and sudden deterioration in cardiac function, 4.76 ± 0.16. The transient detrimental effect of time away from training was also evident. Conclusions Overall performance in component tasks and complete cardiac surgical procedures improved during simulation-based training. Simulation-based training imparts skill sets for management of adverse events and can help produce safer surgeons.

BACKGROUND: The Cardiac Surgery Simulation Curriculum was developed at 8 institutions from 2010 to 2013. A total of 27 residents were trained by 18 faculty members. A survey was conducted to gain insight into the initial experience. METHODS: Residents and faculty were sent a 72- and 68-question survey, respectively. In addition to demographic information, participants reported their view of the overall impact of the curriculum. Focused investigation into each of the 6 modules was obtained. Participants evaluated the value of the specific simulators used. Institutional biases regarding implementation of the curriculum were evaluated. RESULTS: Twenty (74%) residents and 14 (78%) faculty responded. The majority (70%) of residents completed this training in their first and second year of traditional-track programs. The modules were well regarded with no respondents having an unfavorable view. Both residents and faculty found low, moderate, and high fidelity simulators to be extremely useful, with particular emphasis on utility of high fidelity components. The vast majority of residents (85%) and faculty (100%) felt more comfortable in the resident skill set and performance in the operating room. Simulation of rare adverse events allowed for development of multidisciplinary teams to address them. At most institutions, the conduct of this curriculum took precedence over clinical obligations (64%). CONCLUSIONS: The Cardiac Surgery Simulation Curriculum was implemented with robust adoption among the investigating centers. Both residents and faculty viewed the modules favorably. Using this curriculum, participants indicated an improvement in resident technical skills and were enthusiastic about training in adverse events and crisis management.

Background In patients with a modified Fontan connection, particularly the right atrial to right ventricular connection, the pulmonary ventricle may enlarge with time. Methods Between January 1990 and December 2006, 10 patients (median age, 24 years) underwent Fontan conversion to a one and one half ventricle repair. Tricuspid atresia was the most common diagnosis (n = 7). The right atrial to right ventricular connection was present in 8 patients; 3 patients had a prior bidirectional cavopulmonary anastomosis. Preoperative median right atrial pressure was 14 mm Hg (range, 12 to 20 mm Hg). Indications for surgery were exercise intolerance, arrhythmias, and conduit obstruction. Echocardiography showed moderate right ventricular hypoplasia, with right atrial to right ventricular regurgitation. The conversion included closure of septal defects, tricuspid valve replacement (n = 8), bidirectional cavopulmonary anastomosis (n = 7), valved right ventricular outflow tract reconstruction (n = 2), and arrhythmia surgery (n = 6). Results There was no early mortality. There was one reoperation for residual ventricular septal defect. Prolonged chest tube drainage occurred in 2 patients. Postoperative right atrial pressure ranged from 8 to 14 mm Hg (median, 10 mm Hg; p = 0.02). Median follow-up was 8 years. There was no late mortality. Nine patients are in New York Heart Association class I or II, and 1 patient has biventricular failure and was awaiting transplantation. There were two late reoperations, 1 for thrombosis of the mechanical tricuspid valve prosthesis and 1 for obstruction of the valved right ventricular outflow tract conduit. Three patients had recurrent atrial arrhythmias requiring cardioversion. Conclusions Fontan conversion to one and one half ventricle repair is feasible in selected patients with a failing Fontan circulation. Operation can be performed with low early mortality. Arrhythmia surgery should be performed routinely. Quality of life is excellent.

Background Pericardial bioprostheses have favorable echocardiographic hemodynamics in the aortic position compared with porcine valves; however, there are few data comparing clinical outcomes. Our objective was to assess the late results of the two valve types. Methods We reviewed 2,979 patients aged 65 years or older undergoing aortic valve replacement with pericardial (n = 1,976) or porcine (n = 1,003) prostheses between January 1993 and December 2007. The most common pericardial prostheses were Carpentier-Edwards Perimount and Mitroflow, and the most common porcine valves were Medtronic Mosaic, Carpentier-Edwards, Hancock modified orifice, and St. Jude Biocor. Follow-up extended to a maximum of 16 years (mean, 5.2 ± 3.5 years). Results Survival at 5, 10 and 12 years was, respectively, 68%, 33%, and 21% overall, was 68%, 30%, and 16% for patients with pericardial bioprosthesis, and was 69%, 38% and 27% for the porcine group. In a multivariate model, long-term survival was reduced in patients with diabetes, renal failure, prior myocardial infarction, congestive heart failure, and older age, but late survival was not higher in the pericardial valve group. Overall freedom from reoperation was 96%, 92%, and 90% at 5, 10, and 12 years, and freedom from explant was 98%, 96%, and 94% during the same period. The reason for explant was structural valve deterioration in 50 patients (2%). Conclusions Despite the better hemodynamic performance documented in prior investigations, pericardial valves do not confer any survival advantage over porcine valves in patients aged 65 years or older undergoing aortic valve replacement.

Background The surgical management of double orifice left atrioventricular valve (DOLAVV) in atrioventricular septal defects (AVSD) can be challenging and has important surgical implications. Methods Between 1961 and 2009, 44 patients with DOLAVV associated with AVSD were reviewed; this constituted 6.7% of cases of AVSD. The median age at operation was 6.6 years (range 2 months–70 years). DOLAVV was associated with partial, intermediate, and complete AVSD in 28 patients (64%), 1 patient (2%), and 15 patients (34%), respectively. Results Forty-one patients (93%) had partial or complete closure of the zone of apposition (ZOA), and in 3 patients (7%), the ZOA was left open. The accessory orifice was found to be regurgitant in 4 patients, and in all 4 patients it was closed. Four patients had partial annuloplasties. Early mortality consisted of 1 death (2.2%). This was a patient in whom the tissue bridge was divided and severe regurgitation resulted. Median follow-up was 10.3 years (maximum, 36 years). Three adult patients required mitral valve replacement for severe mitral regurgitation (MR) at 3, 11, and 20 years, respectively. Two of these patients experienced progression of MR resulting from an unsutured ZOA. There were 3 late deaths (6%). One death occurred after mitral valve replacement and the others died of noncardiac causes. No patient had hemodynamically significant mitral stenosis. Conclusions Repair of DOLAVV in AVSD can be performed with low risk, excellent late survival, and freedom from reoperation. The ZOA is typically closed unless the valve area appears small. In the majority of patients, the accessory orifice is competent and can be left alone. Late repeated repair may be difficult because of leaflet dysplasia.

Background We reviewed our experience with surgical repair of double-chambered right ventricle and long-term outcome. Methods From November 1970 to February 2008, repair of double-chambered right ventricle was performed in 61 patients (31 males). The median age was 13 years (interquartile range, 2 months to 64 years); 10 patients were infants (16%). Mean preoperative right ventricular outflow tract pressure gradient was 67 ± 37 mm Hg. An associated ventricular septal defect was present in 50 patients (82%). Results There were 2 (3%) early deaths due to persistence of low cardiac output postoperatively, despite complete relief of the right ventricular gradient. The overall mean postoperative gradient was 2 ± 4.5 mm Hg. Late follow-up was complete in 92% (mean, 7.4 ± 7.9 years; maximum, 37 years). Late survival was 90% at 10 years. There were 3 late deaths due to heart failure in 2 patients and sudden death in 1 patient, all occurring before 1997. No patients required reoperation for residual or recurrent right ventricular obstruction. Conclusions Surgical correction of double-chambered right ventricle results in excellent functional and hemodynamic long-term results, with complete relief of the right ventricular obstruction. The presence of a double-chambered right ventricle should be considered in anomalies with high or persistent right ventricular outflow tract obstruction.

Congenital mitral valve regurgitation (CMR) is very uncommon in adults and is usually associated with other congenital malformations. Repair of the mitral valve remains the standard of care. Very limited reports are available on minimally invasive surgical approaches in treating CMR. This report represents the first case series of the successful application of robotics in correcting CMR and associated anomalies, including a partial atrioventricular canal defect.

Background Because reoperation is often necessary for bioprostheses, mechanical pulmonary valve replacement (mPVR) may be appropriate for many patients. Mechanical prostheses are durable, but there has been concern concerning valve thrombosis and bleeding complications from warfarin. Methods Between October 1965 and August 2008, 54 patients (33 male, median age 30 years, range 5 to 66) underwent mechanical PVR at our institution (40 patients since 2004). Forty-nine of these 54 patients underwent a total of 110 prior operations (median 2, maximum 5), including 89 prior operations on the right ventricular outflow tract (median 1, maximum 4). Diagnoses included congenital (n = 47) and carcinoid (n = 7) heart disease. Bleeding complications were compared with a 1:2 matched patient cohort (age, gender, and diagnosis) receiving bioprosthetic PVR. Results The most common concomitant procedures were tricuspid valve replacement in 15 patients, aortic root replacement in 14, and aortic valve replacement in 13. At last follow-up in 45 of 51 early survivors (median 2.2 years, maximum 20 years), there was no perivalvular leak, vegetations, pannus formation, or valve thrombosis. Further, no patient required reoperation on mPVR. Major late bleeding complications occurred in 3 of 54 patients in the mPVR group and 4 of 108 in the tissue PVR group. Conclusions Thromboembolic complications are rare with therapeutic international normalized ratios and mechanical PVR provides excellent durability and freedom from reoperation. Tissue PVR does not eliminate bleeding complications. Mechanical PVR should be considered in select patients with multiple prior operations, or when there is another need for warfarin anticoagulation.

Background Pericarditis requiring pericardiectomy is uncommon in the pediatric population. The aim of this study is to characterize our experience with this subset of patients. Methods Between February 1978 and May 2008 pericardiectomy was performed on 27 pediatric patients (25 male). The indication for surgery was inflammatory pericarditis in 16 and constrictive pericarditis in 11. Mean age was 16.7 years (range, 3 to 21 years). Chest pain was the most common presenting complaint. Median duration of symptoms prior to operation was 1 year. Most patients had aggressive pharmacologic treatment prior to operation. Before pericardiectomy, 10 patients were hospitalized for treatment of symptoms, 15 underwent pericardiocentesis, and 3 had a prior partial pericardiectomy. Results Twenty-one patients underwent complete pericardiectomy, 3 a biventricular pericardiectomy, and 3 a completion pericardiectomy. Pathologic histology of all specimens was positive for pericarditis. Pericardial cultures were obtained in 13 cases with bacteria retrieved from only 2 specimens. Median length of stay was 7 days, and the majority had an uneventful postoperative course. The one early mortality was due to acute hepatic failure in a patient with radiation-induced heart disease, 155 days after operation. After median follow-up of 1 year, complete resolution of symptoms was achieved in 89% of patients. Conclusions In properly selected pediatric patients, complete pericardiectomy can be performed with good outcomes. Although the etiology of pericardial irritation is frequently elusive, resolution of symptoms can be expected in most patients. Confronted with medically refractory pericarditis, earlier consideration for pericardiectomy may be warranted.

Background Excellent surgical results have been reported after repair of complete atrioventricular septal defects (CAVSD); however, 5% to 10% require reoperation. We examine causes leading to reoperation and evaluate long-term outcome. Methods Between 1972 and 2007, 50 patients (26 male) underwent reoperation at our institution after initial repair of CAVSD (median interval, 15 months; range, 3 days to 29 years). Median age at first reoperation was 4.5 years (range, 53 days to 38 years). Indications for first reoperation included left atrioventricular valve (LAVV) regurgitation in 41 patients, subaortic stenosis in 5, and LAVV stenosis, residual atrial septal defect (ASD), pulmonary artery (PA) stenosis, and aortic coarctation in 1 each. Results The first reoperation included LAVV repair in 21 patients and replacement in 21, modified Konno procedure in 3, septal myectomy in 2, and PA reconstruction, coarctation repair, and ASD re-repair in 1 each. After LAVV repair (n = 21) 5 patients required a second reoperation, and after LAVV replacement (n = 21) 6 patients required a second reoperation. Overall freedom from further reoperation after the first reoperation was 63%, 48%, and 42% at 5, 10, and 15 years, respectively. There were 2 early deaths (4%) after first reoperation, and none after subsequent reoperations. During late follow-up (median 10.7 years, maximum 30 years), actuarial overall survival was 91%, 91%, and 86% at 5, 10, and 15 years, respectively. Conclusions The most common indication for reoperation after CAVSD repair is LAVV regurgitation. LAVV re-repair offers good durability, and LAVV replacement does not preclude additional reoperations. Long-term survival is very good despite need for multiple reoperations in some.

Background We evaluated endovascular stent-grafting as a new technique in aortic surgery. Methods One hundred ninety-six stent-grafts were implanted in the thoracic aorta in 172 patients. All procedures but one were performed in the operating room by a team of cardiothoracic surgeons; 112 operations (57%) were emergency procedures. Twenty-four procedures (12%) were reoperations for endoleaks. The left subclavian artery origin was covered in 46 cases and the left common carotid artery in 2 cases. Access was by femoral cut-down in 174 procedures, percutaneous femoral approach in 1, and by conduit to the iliac arteries or infrarenal aorta in 17. Surgical reconstruction of damaged access vessels became necessary in 10 cases. Results Thirty-day mortality was 9.7% (19 patients). Paraplegia occurred in 1.0% (2 patients). Primary technical success was 85.2%, secondary 91.8%. Six conversions to open repair were necessary, 3 during the procedures and 3 secondarily before discharge. Actuarial survival was 79% at 1 year, 67% at 3 years, and 55% at 5 years. Conclusions The results are excellent, taking into account the high incidence of emergency procedures and that open surgery is not promising in many patients. The cardiothoracic surgeon can perform the procedure after adequate training in endovascular techniques. Surgical skills are mandatory because of the potential need for extended surgical approach to the access vessels or immediate conversion to open surgery. Therefore, the operating room is the preferred site for this procedure.

The presence of a left aortic arch with an aberrant right subclavian artery is a well-described anatomic variant that rarely causes symptoms. When symptoms of dysphagia lusoria do occur, however, operative intervention may be necessary. The purpose of this report is to describe our preferred surgical approach to treat dysphagia lusoria secondary to an aberrant right subclavian artery.

Background Bridging leaflet division may facilitate repair of atrioventricular septal defects (AVSD). However, the consequences of bridging leaflet division on early valve function and mortality are not well defined. Methods Records of children undergoing AVSD repair between January 1995 and January 2002 were reviewed. Multivariable analysis defined risk factors for moderate or greater atrioventricular valve regurgitation (AVVR) and death/reoperation within 1 year of repair. Results A total of 209 children (median age 5 months, median weight 5 kg) had defects whose repair included the possibility of bridging leaflet division. Bridging leaflets divided were both (n = 119, 58%), one (n = 30, 15%), or none (n = 55, 27%). Freedom from AVVR (moderate or greater) is 84%, 80%, and 78% at 1, 6, and 12 months. Risk factors include technical factors: number of bridging leaflets divided, longer cross-clamp time, and right-sided annuloplasty. Other risk factors include preoperative AVVR (moderate or greater), double-orifice or parachute left AV valve, and younger age. Freedom from death/reoperation for AVVR is 96%, 92%, and 90% at 1, 6, and 12 months. Risk factors are preoperative AVVR (moderate or greater) and parachute left AV valve. Findings at reoperation (n = 15, 7.2%) were cleft dehiscence or tear along cleft closure (n = 10), dehiscence of divided leaflet from septation patch (n = 1), or other (n = 4). Operative mortality (n = 6, 2.9%) included failed reoperations for AVVR (n = 4), dehiscence of divided leaflet from septation patch (n = 1), and sepsis (n = 1). Conclusions Division of bridging leaflets is a risk factor for AVVR (moderate or greater) during the first year after repair. Preservation of bridging leaflet integrity may improve valve competency, decrease the need for future reoperation, and eliminate some causes of operative mortality.

Palliative atrial switch (PAS) procedures that reroute pulmonary and systemic venous drainage and leave a ventricular septal defect (VSD) open have been used in the treatment of deeply cyanotic patients who have severe pulmonary vascular obstructive disease (PVOD). Palliative atrial switch is beneficial for patients with transposition of the great arteries or other complex lesions with VSD who show higher arterial oxygen saturation in the pulmonary artery than in the aorta (transposition hemodynamics/unfavorable streaming). We reviewed the early and late results of PAS (Mustard, n = 25; Senning, n = 3) in patients at two institutions.Between April 1965 and March 2000, PAS was performed in 28 cyanotic patients (18 male, 10 female). Median age was 10 years (range, 1 to 27). Mean preoperative pulmonary arterial pressure was 68 mm Hg (range, 30 to 121 mm Hg). Mean systemic arterial oxygen saturation was 65% (range, 47% to 80%). The majority of patients (95%) were in New York Heart Association (NYHA) functional class III or IV preoperatively.Overall early mortality was 21%; for patients after 1972 (n = 23), the early mortality was 8.7%. Mean follow-up was 8.3 years (maximum 20). Mean postoperative systemic arterial oxygen saturation was increased significantly to 88% (p0.0001). Late survival for early survivors at 5, 10, and 15 years respectively was 84% (59%, 97%), 64% (39%, 88%), and 54% (15%, 72%). The NYHA functional class was significantly improved; 94% of late survivors (n = 17) were in functional class I or II (p = 0.002).The PAS operation significantly improves systemic arterial oxygen saturation and quality of life in selected patients with transposition hemodynamics, VSD, and severe PVOD.

Background We conducted a retrospective study to assess whether providing extracorporeal membrane oxygenation (ECMO) support to elderly patients (aged 70 years or more) who failed separation from cardiopulmonary bypass after cardiac surgery was a viable option. Methods From 2003 to 2013, 45 patients aged 70 years or more underwent 47 runs of ECMO postoperatively. Results There were 31 men (68.9%). The mean age was 76.8 years. Five patients were in cardiogenic shock preoperatively. Forty-four patients required venoarterial ECMO support for cardiogenic shock. Mean duration of support was 103.8 ± 74.3 hours. Twenty-one patients (46.6%) died while on ECMO support. Twenty-four patients (53.3%) were weaned off ECMO initially, and 11 patients were discharged from hospital. Inhospital mortality was 75.6%. Postoperative complications included acute kidney injury in 30 patients (44.4%), pneumonia in 12 (26.7%), and sepsis in 11 (24.4%). There were 30 deaths (88.2%) attributable to cardiac causes. Preoperative atrial fibrillation, chronic kidney injury, lactic acidosis on ECMO support, and persistent coagulopathy were associated with higher mortality. Conclusions Postcardiotomy ECMO support in elderly patients is associated with high postoperative morbidity and mortality. Nevertheless, it often provides the last line of therapy for these critically ill patients and may provide positive outcomes in selected subgroups.

Background Patients with Ebstein malformation (EM) and severe RV dilatation and dysfunction have increased operative risk. Early results with right ventricular unloading utilizing the bidirectional cavopulmonary shunt (BCPS) during repair of EM have been encouraging. We report our experience of the 1.5-ventricle repair strategy for this difficult group of patients. Methods Between July 1999 and January 2013, 62 patients with severe EM underwent BCPS at the time of tricuspid repair. Median age was 21.5 years (range, 9 months to 57 years), 51.6% were male, and 72.5% were children. Severe RV dilatation was present in all patients; severe RV dysfunction was present in 72.5% (n = 45) and moderate to severe RV dysfunction in 22.5% (n = 14). Mean RV systolic pressure was 32.7 ± 0.7 mm Hg and mean PA pressure was 15.6 ± 2.1 mm Hg. Mean preoperative left ventricular ejection fraction (LVEF) was 0.536 ± 0.071; it was less than 40% in 10 patients (16.1%). New York Heart Association class III/IV heart failure was present in 43 patients (69.3%) preoperatively and 20 patients (32.2%) were initially referred for heart transplant evaluation. Prior EM surgery occurred in 35.4% (n = 22; 8 prior valve repair, 8 prior valve replacement, Blalock-Taussig shunt in 4, atrial septal defect (ASD) closure in 2). Results Tricuspid repair was performed in 51.6% (n = 32, 5 had re-repair). Bioprosthetic valve replacement was performed in 48.4% (n = 30, 8 had rereplacement). The BCPS was a planned procedure in 53 patients (85.5%) because of RV dysfunction; BCPS was added after unsuccessful weaning from bypass in 7 (11.2%), and in the early postoperative period due to hemodynamic instability in 2. Concomitant procedures included ASD closure in 48.3%, maze in 38.7%, and mitral valve repair in 6.4%. Postoperative extracorporeal membrane oxygenation support was needed in 8 patients. Delayed chest closure was performed in 25.8%. Early mortality was 1.6% (n = 1). Mean mechanical ventilation time was 69.7 hours. Mean intensive care unit and hospital stays were 5.4 ± 3.5 and 10.7 ± 3.5 days, respectively. Mean follow-up was 3.6 ± 2.6 years (maximum, 12.8 years). Patients (n = 10) with low preoperative LVEF (0.362 ± 0.035) improved to 0.517 ± 0.042 postoperatively ( p = 0.001). There was 1 late death in a patient with cystic fibrosis. Late reintervention was needed in 5 patients (8%). Late follow-up was available in 95% (n = 59); all were acyanotic and 88% were in New York Heart Association functional class I/II. Conclusions Concomitant BCPS is a useful adjunct in repair of advanced EM with severe RV dilatation and dysfunction. Operation can be performed with low early mortality. Intermediate-term survival and quality of life is good to excellent, and transplantation can be delayed or avoided in the majority.

Surgical correction of complex aortic coarctation can be associated with significant risks. Extraanatomic bypass may represent a safer alternative.Between January 1985 and December 2012, 80 consecutive patients with complex coarctation underwent ascending-to-descending aortic bypass through a median sternotomy. Patients were a median age of 42 years (range, 15 to 75 years), and 51 (64%) were males. Recurrent coarctation was present in 52 patients (65%), with 6 (8%) having undergone balloon dilatation. Uncontrolled hypertension was present in 63 patients (79%). The most common concomitant pathology was aortic valve stenosis in 21 patients (26%), subaortic stenosis in 10 (13%), and Shone complex in 4 (5%).There were no early deaths. The most common concomitant procedures were aortic valve replacement, coronary artery bypass grafting, and resection of subaortic stenosis. The mean aortic cross-clamp and cardiopulmonary bypass times were 33 ± 40 and 106 ± 54 minutes, respectively. Morbidity included atrial fibrillation in 17 patients (21%) and reexploration for bleeding in 6 (8%). There was no paraplegia or stroke. Upper extremity blood pressure significantly improved (p0.001). Mean systolic blood pressure decreased from 153 ± 26 mm Hg preoperatively to 123 ± 15 mm Hg postoperatively. Mean follow-up was 7 ± 6 years (maximum, 22 years). Late deaths occurred in 5 patients (6%) and were not graft-related. Three patients (4%) required reoperation for repair of periprosthetic regurgitation in 2 and mitral valve replacement in 1.The ascending-to-descending aortic bypass can be performed with low morbidity and mortality. It is an effective solution to complex aortic coarctation and represents a safe single-stage approach for patients with concomitant cardiac pathology.

Background Cor triatriatum is a rare congenital cardiac defect in which the atrium is divided into 2 chambers by a membrane causing obstruction to the blood flow in either the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dexter) eventually leading to cardiac failure. We sought to review our surgical experience with cor triatriatum sinister. Methods Twenty-five patients underwent surgical correction of cor triatriatum between May 1960 and September 2012. There were 11 males and 14 females with a mean age of 27.4 years (age range, 1 day to 73 years). Results All patients underwent excision of cor triatriatum membrane using cardiopulmonary bypass. Twenty patients (80%) required concomitant cardiac surgical procedures. There was no early mortality. None of the patients had any residual atrial obstruction. Two infants who had concomitant repair of complex congenital anomalies died at 2 and 5 months postoperatively after discharge from hospital. Kaplan-Meier survival at 10 years was 83%. All patients were in New York Heart Association class I or II at a mean follow-up of 12.8 years (maximum 44 years). Conclusions Surgical repair of cor triatriatum provides satisfactory early and long-term survival with low risk for additional intervention. Cor triatriatum with complex congenital anomalies may be associated with adverse outcome.