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Hyperglycemia control is associated with improved outcomes in patients undergoing cardiac surgery. The Surgical Care Improvement Project metric (SCIP-inf-4) was introduced as a performance measure in surgical patients and included hyperglycemia control. Compliance with the SCIP-inf-4 metric remains suboptimal. A novel real-time decision support tool (DST) with guaranteed feedback that is based on the existing electronic medical record system was developed at a tertiary academic center. Implementation of the DST increased the compliance rate with the SCIP-inf-4 from 87.3% to 96.5%. Changes in tested clinical outcomes were not observed with improved metric compliance. This new framework can serve as a backbone for development of quality control processes for other metrics. Further and, ideally, multicenter studies are required to test if implementation of electronic DSTs will translate into improved resource utilization and outcomes for patients.

Background Pulmonary artery (PA) aneurysms are rare and have been reported only in case reports or small series. We reviewed our experience with surgical repair of PA aneurysms. Methods We reviewed all patients with a true PA aneurysm undergoing cardiac operations between 1995 and 2015. We excluded aneurysms and pseudoaneurysms related to right ventricular outflow tract patches or previous conduits. Results There were 38 patients (24 women [63%]; mean age, 46 ± 15 years), and 14 patients (37%) were asymptomatic. The main PA was involved 35 patients (92%). The mean aneurysm diameter was 5.8 ± 1.8 cm. The most common associated pathology was pulmonary valve stenosis/regurgitation in 23 patients (64%). High-pressure (right ventricular systolic pressure >35 mm Hg) aneurysms were present in 23 patients. Operative strategies included reduction arterioplasty in 30 patients (79%) and resection with graft interposition in 8 (21%). The average length of stay was 6.0 ± 2.2 days. There were no early deaths. Late deaths occurred in 3 patients (8%) and were noncardiac related. Late reoperations occurred in 8% and were not related to the PA aneurysm. All high-pressure aneurysms and those larger than 8 cm demonstrated advanced medial necrosis on pathologic examination of the specimens. Conclusions PA aneurysms are a real entity, and surgical repair can be done with low morbidity and mortality. Aneurysmorrhaphy or aneurysmectomy can be performed, depending on the anatomic location. Regardless of the size of the PA, we recommend intervention on high-pressure aneurysms due to the occurrence of advanced medial necrosis.

Sixty years ago, at the Mayo Clinic in Rochester, Minnesota, an ambitious group of pioneers, led by Dr John W. Kirklin and supported by a multidisciplinary team of physicians and technicians embarked on a planned series of surgical cases using a heart-lung machine to allow direct visualization of the inside of the opened human heart to repair otherwise fatal congenital intracardiac defects. Their success sparked the beginning of a new era of open-heart surgery. In this historical article, we discuss the contributions of a few key figures of this revolution and also share the story of the first successful cardiac surgery operation using cardiopulmonary bypass performed at Mayo Clinic.

OBJECTIVES Paravalvular leak (PVL) is a major cause of morbidity and mortality after mitral valve replacement. Risk factors and data on long-term surgical outcomes are lacking. METHODS Between January 1995 and December 2012, 206 [118 males (57%)] patients underwent reoperation due to mitral PVL. Mean age was 64 ± 11 years. Haemolytic anaemia was present in 85 (41%) patients, while 137 (67%) patients were in New York Heart Association Class III or IV. Baseline creatinine was above 1.5 in 91 (44%) patients, while chronic steroids were used in 14 (7%) patients. Active endocarditis was present in 8 (4%) patients. Device occlusion was attempted in 21 (10%) patients. RESULTS PVL was most common at the aortomitral curtain (82 patients, 40%). Repair was possible in 105 (51%) patients. Early mortality occurred in 11 (5%) patients. Mean follow-up was 5 (maximum 19) years. Overall survival at 1, 5 and 15 years was 83%, 62% and 16%, respectively. Death due to heart failure or cardiogenic shock occurred in 39 patients. Recurrence occurred in 43 (21%) patients and reoperation was required in 19 patients. Multivariate analysis revealed advanced New York Heart Association class (P

Excessive bleeding and allogeneic transfusion during adult post-cardiotomy venoarterial extracorporeal membrane oxygenation (ECMO) are potentially harmful and expensive. Balancing the inhibition of clotting and distinguishing surgical from non-surgical bleeding in post-operative period is difficult. The sensitivity of coagulation tests including Thromboelastography® (TEG) to predict chest tube drainage in the early hours of ECMO was examined with the use of receiver-operating characteristics (ROC). The results are useful to incorporate in clinical evidence-based algorithms to guide management decisions. In the eighth hour of ECMO, 26 of the 53 adult patients (49%) studied were identified as non-bleeders (less than 2.0 mL/kg/h). All had experienced various types of cardiac surgical procedures. Fifty-two percent were female and the group was 54 ± 19 (mean ± 1 SD) years old. The coagulation parameter threshold with the maximum sensitivity and specificity to predict non-bleeding at 8 hours on ECMO was the kaolin plus heparinase TEG maximum amplitude (KH-TEG MA) at a significant ROC threshold (t) > 50 mm. The activated partial thromboplastin time (aPTT) t < 49 seconds, KH-TEG alpha-angle t > 51°, and the kaolin activated clotting time (ACT) t < 148 seconds were sensitive predictors of non-bleeders. The whole-blood KH-TEG MA was superior to the plasma-based aPTT or International Normalization Ratio (INR) to predict bleeding in the eighth hour of ECMO. Using coagulation laboratory thresholds that predict non-bleeding can begin a process of identifying patients earlier that are likely to bleed. Awareness of these parameter thresholds may improve care through patient protection from unnecessary transfusion and prolonging the life of the ECMO circuit. An algorithm incorporating the ROC thresholds was created to help recognize surgical bleeding to minimize unnecessary transfusions.

Background The need for pacemaker implantation is a well-described complication of aortic valve replacement. Not so well described is the effect such an event has on long-term outcome. This study reviewed a 21-year experience at the Mayo Clinic (Rochester, Minnesota) with aortic valve replacement to understand the influence of early postoperative pacemaker implantation on long-term mortality rates more clearly. Methods This study retrospectively reviewed the records of 5,842 patients without previous pacemaker implantation who underwent surgical aortic valve replacement from January 1993 through June 2014. The median age of these patients was 73 years (range, 65 to 79 years), the median ejection fraction was 62% (range, 53% to 68%), 3,853 patients were male (66%), and coronary artery bypass graft operation was performed in 2,553 (44%) of the patients studied. Early pacemaker implantation occurred in 146 patients (2.5%) within 30 days of surgical aortic valve replacement. Results The median follow-up of patients was 11.1 years (range, 5.8 to 16.5 years), and all-cause mortality rates were 2.4% at 30 days, 6.4% at 1 year, 23.1% at 5 years, 48.3% at 10 years, and 67.9% at 15 years postoperatively. Early pacemaker implantation was associated with an increased risk of death after multivariable adjustment for baseline patients' characteristics (hazard ratio, 1.49; 95% confidence interval, 1.20, 1.84; p Conclusions Early pacemaker implantation as a complication of surgical aortic valve replacement is associated with an increased risk of long-term death. Valve replacement–related pacemaker implantation rates should be important considerations with respect to new valve replacement paradigms, especially in younger and lower-risk patients.

Cardiac operations in high-risk adult congenital heart disease (ACHD) patients may require mechanical circulatory support (MCS), such as extracorporeal membrane oxygenation (ECMO) or intraaortic balloon pump (IABP), to allow the cardiopulmonary system to recover.We reviewed records for all ACHD patients who required MCS following cardiotomy at our institution from 1/2001 to 12/2013.During the study period, 2264 (mean age 39.1 years, females ∼54.1%) operations were performed in ACHD patients of whom 24 (1.1%) required postoperative MCS (14 males; median age 41 years, range 22-75). Preoperatively the 24 patients had a mean systemic ventricular ejection fraction of 47% (range 10-66%); 72% of these patients were in NYHA class III/IV heart failure. The common underlying diagnoses included pulmonary atresia with intact ventricular septum (20%), tetralogy of Fallot (16%), Ebstein anomaly (12%), cc-TGA (12%), septal defects (12%), and others (28%). Operations performed were valvular operations with/without maze (58.2%), Fontan conversion (21%), coronary bypass grafting with valvular operations (12.5%), and heart transplant (8.3%). Indications for MCS were left-sided (systemic) heart failure (32%), right-sided (subpulmonary) heart failure (24%), biventricular heart failure (36%), persistent arrhythmia (4%), and hypoxemia (4%). Forty-two percent were placed on ECMO only; in the second group, IABP was attempted and subsequently followed by ECMO initiation. The mean duration of MCS was 8.4 days (range 0.8-35.4). Common morbidities included coagulopathy (60%), renal failure (56%), and arrhythmia (48%). Overall, 46% of patients survived to hospital discharge. Deaths were due to either multi organ failure or the underlying cardiac disease; sepsis was the primary cause of death in one patient. Median follow-up for survivors was 41 months (maximum 106 months). NYHA functional class was I/II in all 8 late survivors.Following complex operations in high-risk ACHD patients, MCS may be required. Despite significant morbidity, nearly half of patients survive to hospital discharge.

Background To determine if Fontan conversion (FC) resulted in improvement in exercise capacity (EC), and to determine the role of cardiopulmonary exercise test (CPET) in risk stratification of patients undergoing FC. Methods A retrospective review of patients who underwent CPET prior to FC at Mayo Clinic from 1994 to 2014. The patients who also underwent post-operative CPET were selected for the analysis of improvement in EC defined as 10% increase in baseline peak oxygen consumption (VO 2 ). Results 75 patients CPET prior to FC; mean age 24±6years; 44 males (59%); and 51 (68%) were in NYHA III/IV prior to FC. Pre-operative peak VO 2 was 15.5±3.4ml/kg/min. A comparison of pre- and post-FC CPET data was performed using 42 patients (56%) that underwent CPET after FC. Improvement in EC occurred in 18 of 42 patients (43%). Baseline peak VO 2 >14ml/kg/min was associated with improved EC (hazard ratio [HR] 1.85; P =.02). Improvement in New York Heart Association (NYHA) class occurred in 12 (67%) patients with improved EC vs 2 (8%) without improved EC. Improvement in NYHA class was more likely to occur in patients with improved EC compared to those without improvement EC (odds ratio 4.11, P =.01). There were 10 (13%) perioperative deaths, and baseline peak VO 2 ≤14ml/kg/min was predictive of perioperative mortality (HR 3.74; P Conclusions Baseline peak VO 2 was predictive of perioperative survival, and improvement in EC. Performance on CPET in failing Fontan patients might be a useful clinical parameter in determining appropriate timing of FC.

We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation.Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation.We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n = 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment30 days after operation.We included 996/1052 (95%) patients with no arrhythmia diagnosis prior to Fontan. Overall 10-, 20-, and 30-year freedom from arrhythmias was 71%, 42%, and 24%, respectively. Of 864 patients who survived30 days after Fontan, 304 (35%) had atrial flutter, 161 (19%) had atrial fibrillation, 108 (13%) had atrial tachycardia, 37 (4%) had reentrant supraventricular tachycardia, 40 (5%) had ventricular tachycardia, and 113 (13%) had sinus node dysfunction. Predictors of late arrhythmias included an atriopulmonary Fontan, age at operation (16 years) or atrial arrhythmias postoperatively. During follow-up, 52/1052 (5%) patients had SCD, with 51 having documentation available; 8 patients died suddenly within 30 days and the remaining 43 had an average time to SCD of 6.9 ± 6.7 years (median was 3.8 years). Arrhythmias were documented in 28/43 (65%) patients prior to SCD. Predictors of SCD included atrioventricular valve replacement and post-bypass Fontan pressures20 mm Hg; preoperative sinus rhythm was protective.Arrhythmias and SCD are significant concerns among Fontan patients and specific risk factors may warrant closer follow-up and earlier consideration for therapy.

To report our experience with intravascular and hybrid intra-operative stent placement for baffle obstruction in patients with complete transposition of the great arteries (TGA) after the atrial switch (Mustard/Senning) operation.Venous baffle obstruction is a challenging complication after atrial switch operation in patients with TGA. Traditional treatment options include intravascular stenting or surgery.A retrospective analysis of Mayo Clinic's electronic medical record was completed to identify consecutive pediatric and adult patients with TGA after atrial switch who underwent baffle stent implantation from 1994 to 2015.Overall, 64 patients were referred for cardiac catheterization, in whom 47 (73%) were noted to have hemodynamic and angiographic evidence of baffle obstruction. A total of 20 patients mean age 33 (range: 8-46) years old underwent stent implantation of baffle stenosis at a mean of 33 (range: 7.5-45) years after initial atrial switch operation (Mustard, n = 19; Senning, n = 1). Overall, 27 baffles were stented in 20 patients via the following approaches: intravascular (17); hybrid surgical (3); staged intravascularhybrid (2). Sites of stent placement were: superior vena cava (SVC) (13); inferior vena cava (IVC) (9); pulmonary venous baffle (5). Three patients had stent placement in the SVC and IVC baffles during the same procedure. Procedural adverse events occurred in 2/22 cases (9%) including creation of unintentional baffle leak (n = 1) and stent migration (n = 1). There was no procedure-related mortality. At follow-up (median 2, range 0.02-10 years), significantly improved NYHA class and mean Doppler baffle gradient were demonstrated (P 0.05). Mild baffle re-stenosis (mean Doppler gradient; 2-3 mmHg) occurred in two patients who have not required re-intervention. Trivial baffle leak was noted in four patients. Baffle re-intervention was only occurred in one patient. One patient with pre-procedural Class IV symptoms died 3.5 months after stent implantation.This study is the largest reported experience of intravascular and intra-operative hybrid stent placement for patients with TGA after atrial switch. Transcatheter and minimally invasive hybrid intraoperative surgical stent placement are an effective strategy for relief of systemic and pulmonary venous baffle obstruction. © 2016 Wiley Periodicals, Inc.

To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis.The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness15 mm, ejection fraction40%. Selection guidelines for heart transplant included age60 years, absence of multiple myeloma and significant extra-cardiac organ involvement. Baseline characteristics including age, gender, organ involvement, and New York Heart Association functional class were recorded. Laboratory data, waiting time until heart transplant, and type of treatment of the underlying plasma cell disorder were recorded. Survival from the time of OHT was calculated using Kaplan-Meier survival curves. Survival of patients undergoing OHT for AL was compared to that of non-amyloid patients undergoing OHT during the same time period.Twenty-three patients (median age 53 years) with AL received OHT. There were no deaths in the immediate perioperative period. Twenty patients have died post OHT. For the entire cohort, the median overall survival was 3.5 years (95%CI: 1.2, 8.2 years). The 1-year survival post OHT was 77%, the 2-year survival 65%, and the 5-year survival 43%. The 5-year survival for non-amyloid patients undergoing OHT during the same era was 85%. Progressive amyloidosis contributed to death in twelve patients. Of those without evidence of progressive amyloidosis, the cause of death included complications of autologous hematopoietic stem cell transplantation for 3 patients, post-transplant lymphoproliferative disorder for 2 patients; and for the remaining one death was related to each of the following causes: acute rejection; cardiac vasculopathy; metastatic melanoma; myelodysplastic syndrome; and unknown. Eight patients had rejection at a median of 1.8 mo post OHT (range 0.4 to 4.9 mo); only one patient died of rejection. Median survival of seven patients who achieved a complete hematologic response to either chemotherapy or autologous hematopoietic stem cell transplantation was 10.8 years.Our data demonstrate that long term survival after heart transplant is feasible in AL patients with limited extra-cardiac involvement who achieve complete hematologic response.

Endovascular arch repair technology is driven in large part by the assumption that open arch operations are high-risk. We wanted to evaluate the clinical results of open arch reconstruction in the modern era in a large group practice.From October 2003 to June 2014, 567 patients underwent aortic arch operations: hemiarch repair was performed in 429 patients (75.7%; group A), total arch repair in 129 (22.7%; group B), and patch repair in the remaining 9 (1.6%). The procedure was an emergency in 88 patients (20.5%) in group A and in 41 patients (31%) in group B. Redo sternotomy after a previous aortic operation was performed in 35 patients (8.2%) in group A and in 28 patients (22%) in group B.Permanent neurologic deficits were diagnosed in 12 patients (2.8%) in group A and in 3 patients (2.4%) in group B. No spinal cord injuries occurred. Mortality at 30 days was 4% (17 patients) in group A and 5.4% (7 patients) in group B. Patients in group A were younger than in group B (mean age, 61.3 vs 63.6 years; p = 0.06). Older age (odds ratio, 1.05; 95% confidence interval, 1.01 to 1.09; p = 0.0087) and extracorporeal circulation time (odds ratio, 1.01; 95% confidence interval, 1 to 1.01; p 0.001) were predictors of perioperative 30-day mortality. Age (odds ratio, 1.05; 95% confidence interval, 1.01 to 1.08; p = 0.006) was the only predictor for neurologic dysfunction. Survival at 2, 6, and 8 years was 90%, 80%, and 69%, respectively, for group A, and 85%, 70% and 62%, respectively, for group B.These results set a standard against which endovascular technology needs to be compared.

Left ventricular outflow tract obstruction is a well-known, albeit rare, adverse event that can occur after mitral valve replacement. With the current increase in transcatheter valve interventions, new and unique adverse events may be expected to occur. We present a case of severe left ventricular outflow tract obstruction that developed after transcatheter mitral valve implantation.

Surgery for congenitally corrected transposition of the great arteries (ccTGA) has evolved overtime. Anatomical repair of ccTGA is now performed with quite satisfactory outcomes in children. The double switch operation has become the preferred surgical procedure in selected cases. Nevertheless, a number of patients with ccTGA still require physiological repair due to complex cardiac associations or impaired systemic ventricular function. This chapter outlines the mechanisms of right ventricular failure in ccTGA. Several “old techniques of repair” were abandoned. The introduction of PA banding has reduced the occurrence of tricuspid regurgitation and has allowed, in some cases, anatomical repair after proper LV retraining. The indications for TV repair and replacement, the place of the Fontan operation or heart transplantation are discussed. The decision to proceed with physiologic versus anatomic repair and the timing of operation is individualized.

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 Rheumatoid arthritis (RA) and its treatments are associated with tissue abnormalities, which may influence surgical outcomes of repair for severe mitral valve regurgitation (MR). We examined late survival and durability of mitral valve repair in patients with RA. Methods Thirty-six patients with RA (21 male) underwent mitral valve repair for MR from August 1978 to August 2005. Median age was 70 years (range, 22 to 84). Preoperatively, 27 patients (75%) had New York Heart Association Functional Class III/IV symptoms, and 29 (78%) required immunomodulating medications for RA management. Mechanisms of MR were leaflet prolapse in 26 patients (72%), leaflet malcoaptation in 5 (14%), tethering in 4 (11%), and unknown in 1 (3%). Results All patients underwent mitral valve repair, which included posterior leaflet triangular resection in 11 patients, leaflet plication in 10, and artificial chordae placement in 3. When compared with matched control patients without RA who underwent mitral valve repair, RA patients had decreased survival (27% versus 64%, p = 0.005) and freedom from reoperation (93% versus 98%, p = 0.04) at 8 years. However, RA patients undergoing mitral valve repair had similar survival at 5 years compared with age- and sex-matched comparator patients with RA who did not undergo mitral valve surgery (65% versus 67%, p = nonsignificant). Conclusions Mitral leaflet pathology in RA patients with severe MR is similar to that of non-RA patients with mitral valve disease. Long-term survival after mitral valve repair in RA patients is equivalent to that of the general RA population without mitral valve disease, and the durability of mitral valve repair in these patients is very good.

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.

Objectives This study sought to analyze the presentation, surgical procedures, and outcomes in patients ≥50 years of age with Ebstein anomaly (EA). Background Data on management and surgical outcomes in older patients with EA are limited. Methods Operative and clinical data from patients with EA ≥50 years of age undergoing cardiac surgery at our center between October 1980 and January 2010 were analyzed. Results During the study period, 89 procedures were performed in 81 patients with EA (63% women; mean [range] age 59 [50 to 79] years). Pre-operative symptoms included palpitations (n = 69), edema (n = 30), and previous stroke/transient ischemic attack (n = 21). Seventy-six patients (85%) had functional class III or IV symptoms, and 13 (16%) had previous cardiac surgery. Tricuspid valve surgery was necessary in 87 of the 89 procedures (98%): replacement in 65 (73%) and repair in 22 (25%). Three early deaths occurred (4%). On long-term follow-up (available in 73 of 78 early survivors), 63 patients (89%) had improved functional class and 13 patients died (19%). The 20-year survival was 65% versus 74% for age- and sex-matched controls (p = 0.001). The best predictors of late death were lack of post-operative improvement and older age at surgery. Conclusions Although cardiac surgery in patients with EA ≥50 years of age was often complex, early mortality was low (4%) when surgery was performed at an experienced center. Long-term survival was good, although less than expected. These data suggested that surgery in older patients with EA may have to be performed earlier.

Objective We reviewed our experience with the single-patch, 2-patch, and caval division techniques for repair of a partial anomalous pulmonary venous connection. Methods From 1990 to 2009, 124 patients (65 male patients; 52%) were identified. The single-patch, two-patch, and caval division technique was used in 60 (49%), 24 (19%), and 40 (32%) patients, respectively. The median age was 34.5 years (range, 1–76). Of the 124 patients, 99 (80%) had an atrial septal defect. Results There was no early mortality. Survival at 5, 10, and 15 years was 99%, 95%, and 82%, respectively. One patient (3%) in the caval division group required early reoperation for superior vena caval obstruction. All patients, exept for 3, had regained sinus rhythm at discharge. Late superior vena caval obstruction developed in 3 patients (7.5%) in the caval division group, 3 (5%) in the single-patch group, and 1 (4%) in the 2-patch group ( Р = .5). Two patients underwent reoperation for late pulmonary vein obstruction: 1 (2%) in the single-patch group and 1 (4%) in the 2-patch group ( P = .08). A permanent pacemaker was required in 3 patients (3%). Conclusions Surgical treatment of partial anomalous pulmonary venous connections is associated with excellent outcomes. The overall incidence of late superior vena caval or pulmonary vein stenosis is low. Although not significant, the 2-patch technique might be associated with a greater incidence of sinus node dysfunction and late pulmonary venous stenosis. The late development of superior vena caval obstruction is a concern with all techniques, necessitating close follow-up.

Background Recurrent right-side heart failure after operation for constrictive pericarditis (CP) may be caused by incomplete pericardiectomy, recurrent constriction due to exuberant scar tissue, or diastolic dysfunction. Because the risks and benefits of reoperation are not well defined, we reviewed the outcome of completion pericardiectomy. Methods From 1993 to December 2010, 41 patients underwent redo pericardiectomy. Thirty-eight patients had the initial operation elsewhere, and 3 had first pericardiectomy at our clinic. All patients had the diagnosis of CP at initial operation. We divided patients into two groups according to the interval between the first and second pericardiectomies: group A, 1 year or less, n=20; group B, more than 1 year, n=21. Results The mean age was 57.6 ± 12.7 years, and there were 34 males (83%). Twenty-six patients (63%) were in New York Heart Association class II, 10 (24%) were in class III, and 5 (12%) were in class IV. Etiologies were idiopathic in 20 (49%), prior cardiac surgery in 13 (32%), radiation in 6 (15%), and trauma in 2 (5%). There was no significant difference in patient characteristics between group A and group B. The 30-day and in-hospital mortalities were 7% (n = 3) and 12% (n = 5), respectively. Overall 5-year survival was 49%, and was significantly better in group A than group B (73% versus 29%, p = 0.032). In multivariate analysis, New York Heart Association class III or IV and the interval between operations longer than 1 year were significant risk factors for death ( p = 0.010 and p = 0.027, respectively). Conclusions The significant early mortality of repeat pericardiectomy emphasizes the importance of complete pericardial resection at first operation and accurate diagnosis of recurrent constriction. The poor clinical outcome of late (more than 1 year) reoperation suggests that many of these patients may have unrecognized diastolic dysfunction or recurrent mediastinal scarring as the cause of right-side heart failure rather than incomplete initial pericardiectomy.

The Fontan procedure is now considered the final common pathway for patients with anatomical or functional single ventricle. These patients initially have their systemic and pulmonary circulations in parallel, supported by one functional ventricular chamber. The ultimate goal with this procedure is to separate the two circulations, to prevent mixing of venous and arterial blood, and to provide adequate tissue oxygenation. The objective of this article is to review the Fontan procedure with its various modifications and refinements since its introduction to clinical practice in 1971, by Fontan and Baudet.

Background Evidence suggests that metabolic syndrome (MbS) is associated with early senescence of bioprosthetic aortic valve prostheses. The purpose of this study was to determine whether MbS is also associated with accelerated failure of bioprosthetic valves prostheses in the mitral position. Methods Records of all patients undergoing bioprosthetic mitral valve replacement (MVR) from 1993 to 2000 were reviewed. Results Of 114 patients undergoing bioprosthetic MVR, 48 (42%) had MbS. Mean age was 73 years (vs. 74 years for no MbS). Patients underwent MVR for regurgitation (n = 97; 85%), stenosis (n = 12; 11%), or mixed lesions (n = 4; 4%). Etiology was degenerative (n = 35; 32%), rheumatic (n = 26; 24%), ischemic (n = 30; 28%), calcific (n = 9; 8%), and endocarditis (n = 8; 8%). Mean follow-up was 4.5 years. Overall survival at 5 and 10 years was 56% and 26%, respectively. Survival was similar between groups (p = 0.15). Five patients (2 MbS; 4% vs. 3 no MbS; 5%) required mitral reoperation at a mean of 3.8 years after initial MVR. The risk of prosthetic valve failure was not different between groups (p = 0.66). Despite no initial difference in transmitral gradients, gradients beyond five-year follow-up were greater for those with MbS (6.8 mmHg MbS vs. 4.7 mmHg no MbS, p = 0.007). Independent predictors of gradient progression beyond two years were MbS (p = 0.027) and female gender (p = 0.012). There were no significant differences in valve area, regurgitation, or ejection fraction. Conclusions Although overall survival following bioprosthetic MVR is challenging, MbS did not predict diminished survival or excess reoperative risk compared to non-MbS patients. The trend toward more rapid progression of transprosthetic gradients in MbS patients warrants further investigation.

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.

Dr. Kluin and colleagues question whether the good late survival of patients who had septal myectomy should have been emphasized in our conclusion [(1)][1]. Perhaps, but the fact that survival of patients after transaortic septal myectomy is similar to survival of the general population has been

Alice K. Jacobs, MD, FACC, FAHA, Chair, 2009–2011; Sidney C. Smith, Jr, MD, FACC, FAHA, Immediate Past Chair, 2006–2008[¶¶][1]; Jeffrey L. Anderson, MD, FACC, FAHA, Chair-Elect; Nancy M. Albert, PhD, CCNS, CCRN, FAHA; Christopher E. Buller, MD, FACC[¶¶][1]; Mark A. Creager, MD, FACC, FAHA

ObjectiveRecent reports have shown that robotic mitral valve repair is effective in treating posterior leaflet disease; however, comparison with trans-sternal (open) valvuloplasty for all prolapse categories has not been performed. Moreover, data from the recently published EVEREST II trial infer that adverse event rates after mitral valve repair for degenerative disease are high. We therefore compared early outcomes of robotic versus open mitral valve repair for patients with mitral valve prolapse.MethodsAmong 745 consecutive patients undergoing open or robotic mitral repair for degenerative disease, 95 propensity-matched pairs were identified. Leaflet prolapse categories were similar between groups. Complete mitral valve repair was performed using identical techniques.ResultsMedian crossclamp and bypass times were longer in the robotic group but decreased significantly over time (P

ObjectivesIt is not clear whether clinically silent tricuspid valve regurgitation should be addressed at the time of mitral valve repair for severe mitral regurgitation due to leaflet prolapse. We examined the clinical and echocardiographic outcomes of patients with tricuspid regurgitation who underwent only mitral valve repair.MethodsWe retrospectively analyzed records of patients undergoing mitral valve repair for isolated mitral valve prolapse who had coexistent tricuspid valve regurgitation during an 11-year period at our institution. Echocardiographic data were compared preoperatively, intraoperatively, and postoperatively at less than 1, 1 to 3, 3 to 5, and more than 5 years.ResultsIn 699 patients who underwent mitral valve repair for severe mitral regurgitation, mean age was 60.4 years and 459 (66%) were male. At the time of mitral valve repair, tricuspid valve regurgitation was grade 3 or more in 115 (16%) patients and less than grade 3 in 584 (84%) patients. After mitral valve repair, overall grade of tricuspid valve regurgitation decreased significantly within the first year (P = .01). In patients with grade 3 regurgitation or more, the grade decreased at dismissal and until the third year (P

Ebstein's anomaly (EA) is primarily diagnosed by echocardiography. The purpose of this study was to compare echocardiography and magnetic resonance imaging (MRI) in EA. Data from cardiac MRI and echocardiography were prospectively collected from 16 patients with EA. Imaging data also were compared with intraoperative findings. Information provided by MRI and echocardiography were comparable for left ventricular size and function, tricuspid valve repairability, qualitative assessment of right-sided cavities, and visibility of septal and anterior tricuspid valve leaflets. The posterior tricuspid valve leaflet and tricuspid valve fenestrations were better visualized with MRI; associated heart defects were equally recognized, apart from small shunts that tended to be more readily diagnosed with echocardiography. Quantification of right-cavity size and right ventricular ejection fraction was possible only with cardiac MRI. The degree of tricuspid valve regurgitation was underestimated by echocardiography (2 patients) and by MRI (4 patients) when compared with intraoperative assessment. When evaluating EA, echocardiography and MRI provide complementary data. For visualization of the posterior tricuspid valve leaflet and quantitative assessment of right ventricular size and function, MRI is preferable. For appropriate risk stratification in EA, both MRI and echocardiography should be performed before cardiac surgery.

ObjectivesWe sought to summarize our recent experience with intraoperative monitoring for management of patients undergoing surgical myectomy for hypertrophic obstructive cardiomyopathy with emphasis on dynamic left ventricular outflow tract obstruction. We also analyzed the impact of these data on surgical decision-making and adequacy of septal myectomy.MethodsWe retrospectively analyzed the medical records of 198 patients who underwent transaortic septal myectomy and evaluated baseline and provoked left ventricular outflow tract gradients obtained by Doppler echocardiography and by direct measurement of pressures in the left ventricle and aorta.ResultsAfter induction of anesthesia before myectomy, left ventricular outflow tract obstruction, assessed by direct measurement, was less than the gradient documented by preoperative Doppler echocardiography in 119 patients (60%) (41 ± 31 vs 76 ± 40 mm Hg; P

Objective: The optimal myocardial preservation strategy at re-operation for aortic valve replacement (AVR) after prior coronary artery bypass grafting (CABG) in the presence of a patent internal thoracic artery (ITA) remains undefined. Methods: Patients undergoing AVR after prior CABG at our institution between 1 January 1996 and 31 December 2007 were identified; operative notes and outcomes were reviewed. Results: Of 628 patients with prior CABG undergoing AVR with or without concomitant procedures, 427 patients had a patent ITA. In 390, management of the ITA was detailed in the operative note, including 251 in whom it was clamped and 139 in whom it was left uncontrolled. Groups were demographically similar, although re-operative CABG was more frequent in the clamped group (42% vs 23%, p < 0.001). The operative mortality rate among those undergoing only AVR CABG was 7.7% (19/246), while that for all cases was 10.8% (42/390), and was lower when the ITA was left uncontrolled both for AVR CABG (4.1% vs 10.1%, p = 0.08) and overall (7.2% vs 12.7%, p = 0.09). By multivariate analysis, leaving the ITA uncontrolled appearedprotective (odds ratio (OR) 0.46,p = 0.08). There was no clear trend supportingan optimal perfusion temperature when the ITAwas left uncontrolled. Conclusions: Efforts to control the patent ITA at re-operation for AVR after prior CABG increase risk of injury and may actually increase operative mortality rate compared with leaving this critical graft open and perfusing the heart. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objectives The objective was to evaluate the systemic ventricular ejection fraction (SVEF) at the time of systemic atrioventricular valve (SAVV) replacement as a predictor of SVEF ≥1 year after surgery in patients with congenitally corrected transposition of the great arteries (CCTGA). Background Progressive SAVV regurgitation causes systemic ventricular failure in CCTGA patients, who are commonly referred late for intervention. Survival after surgery is poor when the pre-operative SVEF is l44%. Methods We retrospectively reviewed 46 patients (pre-operative SVEF ≥40% in 27 patients and l40% in 19 patients) with 2 good-sized ventricles, a morphologically right systemic ventricle, and SAVV regurgitation requiring surgery. Median follow-up was not different in patients with a pre-operative SVEF ≥40% (8.8 years) or l40% (7.7 years, p = 0.36). Results Pre-operative SVEF was the only independent predictor of ≥1-year post-operative SVEF (p l 0.0001). The late SVEF was preserved (defined as ≥40%) in 63% of patients who underwent surgery with an SVEF ≥40% compared with 10.5% of patients who underwent surgery with an SVEF l40%. Pre-operative variables associated with late mortality were an SVEF ≤40%, a subpulmonary ventricular systolic pressure ≥50 mm Hg, atrial fibrillation, and New York Heart Association functional class III to IV. Conclusions Post-operative systemic ventricular function after SAVV replacement can be predicted from the pre-operative SVEF. For best results, operation should be considered at an earlier stage, before the SVEF falls below 40% and the subpulmonary ventricular systolic pressure rises above 50 mm Hg.

Although the Fontan procedure has been enormously successful in palliation of single-ventricle patients, many seem to experience progressive failure of the Fontan circulation over time. Ventricular assist devices (VADs) have developed into stable platforms for long-term support of adult patients with heart failure. Given the success of axial flow devices, it was hypothesized that the technology could provide clinical benefit to failing Fontan patients. The aim of this study was to use a computer model to evaluate VAD support in failing Fontan physiology. A computer model of Fontan circulation with heart failure was developed and the HeartMate II (HM II) (Thoratec Corp) axial flow ventricular assist device was connected to this model in a systemic configuration to examine its impact. Cardiac catheterization data from 7 patients (8 catheterization studies) with failing Fontan physiology were applied to this model to evaluate the impact of using the HM II in this manner. When the HM II was used in a systemic configuration at 8000 rpm, there was a 35% decrease in the systemic venous pressure in the Fontan circuit and a 63% decrease in pulmonary capillary wedge pressure with a resultant 41% increase in CI. The model also predicted patient-specific parameters where the VAD may not benefit the patient, such as fixed elevated pulmonary vascular resistance, low systemic ventricular end-diastolic pressure, and high unresponsive systemic vascular resistance. These data suggest a potential benefit from application of axial flow VAD technology in the management of failing Fontan physiology. Clinical correlation will allow for refinement of this model as a predictive tool in discerning which patients may benefit from placement of a VAD and what issues must be addressed prior to implanting the device.

We present a case of a patient with end-stage renal disease and nephrogenic fibrosing dermopathy (NFD) whose echocardiogram demonstrated rare tricuspid valve abnormality with malcoaptation due to tethering and restricted motion of the leaflets causing severe tricuspid regurgitation. The cardiac abnormalities developed 3 years after her initial diagnosis of NFD was made by skin biopsy. The echocardiographic appearance of the tricuspid valve resembled that seen in patients with carcinoid heart disease; however, an evaluation for carcinoid tumor in our patient was negative. Myocardial biopsy performed at the time of right heart catheterization demonstrated trace gadolinium within the lysosomes of one cardiac fibroblast. This report is the first to describe the association between nephrogenic systemic fibrosis and severe valvular heart disease requiring valve replacement. (Echocardiography 2011;28:E46-E49)

Hypertrophic cardiomyopathy (HCM), a heterogeneous genetic heart disease with global distribution, is an important cause of heart failure disability at any age. For 50 years, surgical septal myectomy has been the preferred and primary treatment strategy for most HCM patients with progressive, drug refractory functional limitation due to left ventricular (LV) outflow tract obstruction. With very low surgical mortality at experienced centres, septal myectomy reliably abolishes impedance to LV outflow and heart failure-related symptoms, restores quality of life, and importantly is associated with long-term survival similar to that in the general population. Nevertheless, alternatives to surgical management are necessary for selected HCM patients. For example, after a brief flirtation with dual-chamber pacing 20 years ago, percutaneous alcohol septal ablation has garnered a large measure of enthusiasm and a dedicated following in the interventional cardiology community, achieving benefits for patients, paradoxically, by virtue of producing a transmural myocardial infarct. However, an unintended consequence has been the virtual obliteration of the surgical option for HCM patients in Europe, where several robust myectomy programmes once existed. Therefore, clear differences are now evident internationally regarding management strategies for symptomatic obstructive HCM. The surgical option is now unavailable to many patients based solely on geography, including some who would likely benefit more substantially from surgical myectomy than from catheter-based alcohol ablation. It is our aspiration that this discussion will generate reconsideration and resurgence of interest in surgical septal myectomy as a treatment option for severely symptomatic obstructive HCM patients within Europe.

Objective In natural history studies, increased left ventricular mass and wall thickness are strongly associated with increased late mortality in patients with hypertrophic cardiomyopathy. Our objective was to determine the impact of left ventricular mass and wall thickness on survival after myectomy for hypertrophic obstructive cardiomyopathy. Methods We reviewed the case histories of 796 patients who underwent transaortic septal myectomy for hypertrophic obstructive cardiomyopathy from January 1993 to December 2006. We then selected for study patients who had transthoracic echocardiography within 30 days after myectomy, which included complete measurements of interventricular wall thickness, posterior wall thickness, and left ventricular end diastolic dimension. Late survival was determined for all patients using medical records and surveys. Results Our study group included 416 patients with a mean age of 50 ± 15 years, and 58% were male. Eight percent of patients had coronary artery disease, 17% had a history of arrhythmia, and 17% had a previous or concomitant insertion of an internal cardioverter defibrillator. All patients had successful myectomy, and 17% required an additional mitral valve procedure, most often mitral valve repair. On predismissal echocardiography, the average interventricular wall thickness was 16 ± 5 mm, the posterior wall thickness was 13 ± 3 mm, and the left ventricular end diastolic dimension was 45 ± 6 mm. The indexed left ventricular mass was 135 ± 46 g/m2. Late survival at 1, 5, and 10 years was 99%, 97%, and 85%, which was similar to that of an age- and gender-matched population (P = .453). On multivariate analysis, preoperative and postoperative wall thickness and left ventricular mass were not associated with death; only a history of coronary artery disease (hazard ratio 4.9) was predictive of late mortality. Conclusions Left ventricular mass and wall thickness were not predictors of late survival after myectomy for hypertrophic obstructive cardiomyopathy, and this is in contrast to natural history studies of patients who were not treated surgically. Late survival was similar to that of an age- and gender-matched population.

Congenital mitral valve malformations are rare, but are well known and described entities. Mitral valve malformations involve mitral valve apparatuses (leaflets and annulus) and subvalvar apparatuses (chordae and papillary muscle). Case reports of accessory mitral leaflets were already described, but were usually an appendix of the normal valve. We describe here a case report and present the images of a trileaflet mitral valve sustained by three papillary muscles in a young girl with subaortic stenosis.

BackgroundLeft ventricular hypertrophy is associated with adverse cardiovascular outcomes. It is unclear whether hypertrophy caused by severe chronic mitral regurgitation regresses after mitral valve repair and, if so, which factors promote reverse remodeling and influence its prognostic significance.MethodsBetween March 1995 and December 2005, 2589 patients had mitral valve repair. Five hundred thirty patients (346 of whom were male) underwent isolated repair for leaflet prolapse and had echocardiographic data available from which the left ventricular mass index could be calculated. Concomitant preoperative tricuspid valve regurgitation was more than mild in 95 (18%) patients. Those with preoperative atrial fibrillation and other cardiac pathologies necessitating intracardiac repair were not included.ResultsSignificant regression of left ventricular mass index occurred during the first 3 years (−28 g/m2, P

Objective Patients with complex cyanotic congenital heart disease and a bidirectional cavopulmonary connection who are not candidates for or had failed Fontan operation may experience progressive cyanosis. An axillary arteriovenous anastomosis may be constructed to augment pulmonary blood flow. This report reviews our results with this approach in this complex group of patients. Methods The records of patients with previous cavopulmonary connections who underwent a surgical anastomosis between the axillary artery and the vein for palliation of severe progressive cyanosis were reviewed. Results Eleven patients were identified. The median age at the time of the axillary arteriovenous anastomosis was 19.2 years (7.97–41.75 years). Seven patients were not candidates for the Fontan operation, and 4 patients had failed Fontan surgery. Three of the anastomoses were constructed with a side-to-side technique, and 8 anastomoses were constructed with a short interposition graft. Median fistula size was 5 mm (3–6 mm). There was no operative mortality and 1 late death. Median survival was 2.85 years (0.01–7.22 years). All fistulae were patent at follow-up. Median preoperative arterial oxygen saturation was 84% (80%–86%) and 82% (76%–88%) at follow-up (P = .38). Median preoperative hemoglobin was 18.5 g/dL (11.7–22.6 g/dL) and 19.2 g/dL (14.6–22.6 g/dL) at follow-up (P = .97). Median preoperative systemic ventricular ejection fraction was 51% (27%–60%) and 46.5% (28%–60%) at follow-up (P = 1). Significant functional improvement was seen in only 1 patient. Conclusions In patients with complex cyanotic congenital heart disease who are not candidates for or had failed Fontan operation, palliation with an axillary arteriovenous fistula did not improve cyanosis or polycythemia. Functional outcome and ventricular ejection fraction did not improve or deteriorate.

A 33-year-old woman with an anomalous left coronary artery arising from the pulmonary artery who had undergone Takeuchi repair at age 7 years presented for evaluation. The Takeuchi procedure creates an aortopulmonary window and an intrapulmonary tunnel that baffles the left coronary artery to the aorta. A mediastinal mass was identified as a giant aneurysm of the left coronary artery resulting in compression of the pulmonary artery and left upper pulmonary vein. The patient underwent open repair with patch closure at the aortic entrance of the left coronary Takeuchi repair and resection and evacuation of the aneurysm. A saphenous vein graft to the left anterior descending artery was performed. Postoperative echocardiography demonstrated normal left ventricular function. This is the first reported case of giant aneurysm formation after Takeuchi repair. The reported complications have included the development of pulmonary artery stenosis at the intrapulmonary baffle, baffle leak, decreased left ventricular function, and mitral regurgitation. In conclusion, late complications of the Takeuchi procedure are common, underscoring the importance of lifelong follow-up at a center with experience in treating coronary anomalies.

In patients with congenitally corrected transposition of the great arteries, the main concern has been the long-term performance of the morphologic right ventricle in association with tricuspid valve regurgitation when it remains as the systemic ventricle. Deterioration in ventricular function can occur slowly over many years, even without associated cardiac anomalies or previous surgical interventions. This review summarizes the authors' experience and provides a thorough review of the literature addressing the management of the failing systemic right ventricle as well as the tricuspid valve regurgitation with congenitally corrected transposition. This includes different surgical options, the authors' preferred management algorithm, and the late outcome.