BACKGROUND:Congenital heart disease (CHD) that causes right atrial dilatation is commonly associated with atrial flutter and/or fibrillation (AFl/F). To reduce late recurrence of AFl/F in patients undergoing repair of CHD, we utilized a concomitant right-sided maze procedure.METHODS:From 1993 to 2003, 99 patients with CHD and associated AFl/F underwent a concomitant right-sided maze procedure at the time of CHD repair. Ages ranged from 9 to 72 years (median, 43 years). Atrial flutter and/or fibrillation was paroxysmal in 81 and chronic in 18; duration ranged from less than 1 month to 39.5 years (median, 2.9 years). Primary cardiac diagnoses were Ebstein anomaly (n = 47), other congenital tricuspid regurgitation (n = 19), univentricular heart (n = 11), isolated atrial septal defect (ASD, n = 8), tetralogy of Fallot (n = 8), and other (n = 6).RESULTS:Other concomitant procedures included tricuspid valve repair or replacement (n = 70), ASD closure (n = 39), and pulmonary valve procedures (n = 18). There were 6 early deaths. At hospital dismissal, 83 patients were free of AFl/F and 63 were in sinus rhythm. Follow-up in 87 of the 93 early survivors extended up to 8 years (mean, 2.7 years). There were 4 late deaths, all from noncardiac causes. Of the 83 known late survivors, 77 (93%) were free of AFl/F. Eighty-two of the 83 survivors were in New York Heart Association class I or II.CONCLUSIONS:In patients with AFl/F associated with CHD, a concomitant right-sided maze procedure at the time of intracardiac repair is effective in reducing late recurrent AFl/F. Most patients enjoy an excellent quality of life.

OBJECTIVE:To analyze the effect of adjuvant perfusion techniques of the distal aorta on the outcome of traumatic thoracic aortic transections.PATIENTS AND METHODS:From 1973 to 2004, 72 patients (mean age, 39 years) with thoracic aortic transections arrived alive at the emergency department. Nineteen patients arrived in extremis and underwent emergency operations, 42 patients were stable and underwent diagnostic evaluation before surgery (4 patients experienced aortic rupture during evaluation), and 11 patients presented more than 24 hours after the accident. Sixteen patients died before aortic repair could be performed. Operative repair was possible in 53 patients (46 stable and 7 in extremis). Interposition graft was performed in 47 patients, and primary repair was performed in 6 patients. Morbidity, mortality, and paraplegia rate were analyzed.RESULTS:Patients in extremis had a mortality rate of 84% (16 of 19), stable patients had a mortality rate of 11% (4 of 38), patients who experienced rupture during evaluation had a mortality rate of 100% (4 of 4), and patients who underwent delayed operation had a mortality rate of 0% (0 of 11). The paraplegia rate with and without adjuvant distal aortic perfusion techniques was 2% (1 of 41 patients) and 33% (4 of 12 patients), respectively (P=.007). Mortality and paraplegia rates were 4% and 4% for partial bypass (n=24), 42% and 33% for the clamp and sew technique (n=12), 0% and 0% for Gott shunt (n=10), and 29% and 0% for full cardiopulmonary bypass (n=7), respectively.CONCLUSIONS:Although thoracic aortic transections remain a highly lethal injury, hemodynamically stable patients have a low operative mortality. Spinal cord injury is decreased by the use of adjuvant perfusion techniques that maintain distal aortic perfusion during cross-clamping of the aorta.

OBJECTIVES:We sought to evaluate the early and late results of the modified Fontan operation for patients with heterotaxy syndrome, assess variables traditionally known to correlate with poor outcome, and assess current health status of survivors after the Fontan procedure.BACKGROUND:The natural history of unoperated cardiac lesions in heterotaxy is known to be poor. Therefore, the Fontan operation has been proposed to improve survival.METHODS:Patients with heterotaxy were identified from the Mayo Clinic Fontan database. Hospital and outpatient records were abstracted for preoperative, operative, and postoperative clinical and hemodynamic data. All patients not known to be deceased were sent health status questionnaires.RESULTS:A total of 142 patients with heterotaxy syndrome had a modified Fontan operation. Asplenia was present in 76 patients (54%). Median age at operation was 9 years (range 2 to 35 years). Median follow-up was 4 years (range 0 to 23 years). There were a total of 61 deaths (43%), with 32 (23%) within 30 days of operation or before hospital discharge. The 5-, 10-, and 15-year survival was 64%, 57%, and 53%, respectively. In the modern era (1995 to 2004) early mortality was 10%. Of the 81 survivors, questionnaires were available from 41 (51%). Eighty percent reported having no or mild symptoms. However, 19 (46%) had arrhythmias, 5 (12%) had a thromboembolic event, and 1 (2%) developed protein-losing enteropathy.CONCLUSIONS:Early survival has improved for heterotaxy patients after the Fontan operation; however, late morbidity and mortality remain substantial. Better strategies for long-term treatment of this high-risk group need to be identified.

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OBJECTIVE:The contemporary risk of reoperative aortic valve replacement is ill-defined. We therefore compared the recent early results of reoperative and primary aortic valve replacement in our institution.METHODS:Between January 1993 and January 2001, a total of 162 patients underwent reoperative aortic valve replacement with or without coronary artery bypass grafting, and 2290 underwent primary aortic valve replacement with or without coronary artery bypass grafting. The reoperative and primary groups were similar with regard to gender (37% female in both), preoperative New York Heart Association functional class (2.8 +/- 1 vs 2.8 +/- 1), and ejection fraction (58% +/- 15% vs 57% +/- 15%). Patients undergoing reoperative aortic valve replacement were younger than those undergoing primary aortic valve replacement (64 +/- 15 years vs 70 +/- 13 years, P < .001). Previous prostheses were xenografts in 77 patients (48%), homografts and autografts in 25 (15%), and mechanical prostheses in 60 (37%). Mean time to reoperation was 9.7 +/- 6.8 years.RESULTS:Early mortality for reoperative aortic valve replacement (8/162, 5%) was not statistically different from that for primary aortic valve replacement (71/2290, 3%, P = .20). Endocarditis was more common in the reoperative group (22% vs 3%, P < .001); when endocarditis was excluded from the analysis, early mortality was 3% in both groups. Multivariate predictors for early mortality were prosthetic valve endocarditis ( P < .001, odds ratio 9.8), advanced preoperative functional class ( P < .001, odds ratio 2.0), peripheral vascular disease ( P = .008, odds ratio 2.0), preserved left ventricular ejection fraction ( P = .004, odds ratio 0.98), and male gender ( P = .009, odds ratio 0.49). After adjustment for these factors, there was no difference in early mortality between the groups ( P = .095).CONCLUSION:The risk of reoperative aortic valve replacement is similar to that for primary aortic valve replacement. These data support the expanded use of bioprosthetic valves in younger patients.

BACKGROUND AND AIM OF THE STUDY:Surgical aortic root reconstruction techniques are standard therapy to avoid catastrophic vascular events in patients with Marfan syndrome with a dilated and/or dissected aortic root. The study aim was to evaluate the long-term results of aortic root reconstruction.METHODS:Eighty-three patients (54 males, 29 females; mean age 37+/-17 years) fulfilling strict Ghent criteria for Marfan syndrome underwent aortic root surgery between 1971 and 2001. Of these patients, 65 (78%) underwent a composite valve conduit repair and 18 (22%) a valve-sparing aortic root reconstruction. Six patients (7%) suffered from an acute type A dissection, and 16 (19%) a chronic type A dissection.RESULTS:In-hospital and 30-day mortality was 3.6% (n = 3). Morbidity included stroke (1.2%; n = 1), perioperative myocardial infarction (1.2%; n = 1) and reoperation for bleeding (10%; n = 8). Of 21 late deaths, the cause was cardiac in nine cases. Actuarial survival at 5, 10, 15 and 20 years was 84% (95% CI 76-93%), 73% (CI 61-86%), 59% (CI 45-77%) and 43% (CI 26-72%), respectively. Multivariate predictors for late death were postoperative dysrhythmias and need for inotropes (p < or =0.01). Freedom from reoperation at 5, 10, 15 and 20 years was 86% (CI 78-95%), 69% (CI 56-85%), 53% (CI 38-74%) and 48% (CI 23-71%), respectively. Multivariate predictors for reoperation were preoperative mitral valve prolapse and an initial valve-sparing aortic procedure (p < or =0.05). In the composite valve conduit patients, freedom from thromboembolism was 88% (CI 76-100%), and from endocarditis was 99% (CI 93-100%) at 15 years.CONCLUSION:Composite valve conduit replacement of the aortic root in patients with Marfan syndrome offers a durable result, with low mortality and long-term complication rates. Reoperation was most commonly required for cardiac and vascular disease unrelated to the initial operation and in patients undergoing a valve-sparing aortic root procedure.

Objectives: Freedom from anticoagulation is the principal advantage of bioprosthesis; however, the American Heart Association/American College of Cardiology and the American College of Chest Physicians guidelines recommend early anticoagulation with heparin, followed by warfarin for 3 months after bioprosthetic aortic valve replacement. We examined neurologic events within 90 days of bioprosthetic aortic valve replacement at our institution.Methods: Between 1993 and 2000, 1151 patients underwent bioprosthetic aortic valve replacement with (641) or without (510) associated coronary artery bypass. By surgeon preference, 624 had early postoperative anticoagulation (AC+) and 527 did not (AC-). In the AC- group, 410 patients (78%) received antiplatelet therapy. Groups were similar with respect to gender (female, 36% AC+ vs 40% AC-, P = .21), hypertension (64% AC+ vs 61%, P =.27), and prior stroke (7.6% AC+ vs 8.5% AC-, P = .54). The AC+ group was slightly younger than the AC- group (median, 76 years vs 78 years, P = .006).Results: Operative mortality was 4.1% with 43 (3.7%) cerebrovascular events within 90 days. Excluding 18 deficits apparent upon emergence from anesthesia, we found that postoperative cerebrovascular accident occurred in 2.4% of AC+ and 1.9% AC- patients. By multivariable analysis, the only predictor of operative mortality was hypertension (P < .0001). Postoperative cerebrovascular accident was unrelated to warfarin use (P = .32). The incidence of mediastinal bleeding requiring reexploration was similar (5.0% vs 7.4%), as were other bleeding complications in the first 90 days (1.1% vs 0.8%). No variables were predictive of bleeding by multivariate analysis.Conclusions: Although these data do not address the role of antiplatelet agents, early anticoagulation with warfarin after bioprosthetic aortic valve replacement did not appear to protect against neurologic events.

The purpose of this study was to quantify and determine predictors of long-term survival and functional outcome in patients with double-inlet left ventricle (DILV) after the Fontan operation. The Fontan operation has become the procedure of choice for DILV. Early survival has improved, but mortality and morbidity persist. Record review and follow-up questionnaires were used to ascertain the status of 225 patients with DILV who had Fontan operations from 1974 to 2001 at the Mayo Clinic. The median age at operation was 9 years. The median follow-up period was 12 years (range 3 months to 25 years). There were 22 deaths (9.3%) <30 days after the operation. Early mortality decreased to 3% (2 of 70 patients) after 1989. Overall late survival was 78% (159 of 203 patients). Actuarial survival for the 203 early operative survivors at 5, 10, 15, and 20 years was 91%, 80%, 73%, and 69%, respectively. Forty-nine percent (99 of 203) had additional surgical procedures after the Fontan operation. Other frequent late events were atrial flutter or fibrillation (57%), protein-losing enteropathy (9%), and thromboembolic events (6%). Current health status was described as good or excellent by 84% of patients, fair by 18%, and poor by 12%. In conclusion, the Fontan operation for DILV is now performed with a low operative mortality rate. Long-term survival has improved, and most patients have good functional status.

Background— Sinus venosus atrial septal defect (SVASD) differs from secundum atrial septal defect by its atrial septal location and its association with anomalous pulmonary venous connection (APVC). Data on long-term outcome after surgical repair are limited. Methods and Results— We reviewed outcomes of 115 patients (mean age±SD 34±23 years) with SVASD who had repair from 1972 through 1996. APVC was present in 112 patients (97%). Early mortality was 0.9%. Complete follow-up was obtained for 108 patients (95%) at 144±99 months. Symptomatic improvement was noted in 83 patients (77%), and deterioration was noted in 17 patients (16%). At follow-up, 7 (6%) of 108 patients had sinus node dysfunction, a permanent pacemaker, or both, and 15 (14%) of 108 patients had atrial fibrillation. Older age at repair was predictive of postoperative atrial fibrillation ( P =0.033). No reoperations were required during follow-up. Survival was not different from expected for an age- and sex-matched population. Clinical improvement was more common with older age at surgery ( P =0.014). Older age at repair ( P =0.008) and preoperative New York Heart Association class III or IV ( P =0.038) were independent predictors of late mortality. Conclusions— Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of age at surgery. Postoperative atrial fibrillation appears to be related to older age at operation. SVASD repair achieves survival similar to that of a matched population and should be considered whenever repair may impact survival or symptoms.

Background. Advances in tissue prosthetic valve design and manufacturing have stimulated renewed interest in the use of biological valves in younger patients. This approach, however, risks reoperation. We therefore reviewed our recent experience with repeat mitral valve replacement to better define its contemporary risks.Methods. Using a computerized database, we identified and compared 106 patients undergoing repeat mitral valve replacement with 562 control patients undergoing primary mitral valve replacement between January 1993 and December 2000 at our institution.Results. There were no significant differences between repeat and primary surgery groups with respect to age (mean 66 +/- 12 vs 64 +/- 13 years), gender distribution (women 65% vs 64%), preoperative functional class, ejection fraction, or active endocarditis (6.6% vs 3.4%). The indication for reoperation in the repeat group was structural dysfunction in 49 patients (46%), paravalvular leak in 21 patients (20%), nonstructural dysfunction in 11 patients (10%), and progression of other native valve disease in 8 patients (8%). Prior prostheses were mechanical in 46 patients (43%). Mean time to reoperation was 11.5 +/- 7.1 years. There were 5 deaths out of 106 patients in the repeat group (4.7%) and there were 23 deaths out of 562 patients in the control group (4.1%) (p = NS). Multivariate analysis identified prior myocardial infarction (p = 0.014, odds ratio 2.9) and nonelective surgical status (p = 0.004, odds ratio 2.3) as significant predictors of operative mortality.Conclusions. The risk of repeat mitral valve replacement was low suggesting that there should be less reluctance to recommend patients choose a bioprosthesis over a mechanical prosthesis. Given the expected durability of current designs, bioprosthetic use may be explored in younger patients without subjecting those individuals to excessive risk. (C) 2004 by The Society of Thoracic Surgeons.

OBJECTIVE:To assess outcome of valve repair in patients with aortic valve regurgitation with emphasis on incidence and risk of reoperation.METHODS:We retrospectively reviewed 160 consecutive patients (127 men) who underwent aortic valve repair between 1986 and 2001. Ages ranged from 14 to 84 years (mean 55 +/- 17 years). Patients were categorized according to the main etiology of valve disease; 63 patients (39%) had annular dilation leading to central leakage, 54 (34%) had bicuspid valve, 34 (21%) with tricuspid valve had cusp prolapse, and 9 (6%) had cusp perforation. Repair methods included commissural plication (n = 154, 96%), partial cusp resection with plication (n = 47, 29%), resuspension or cusp shortening (n = 44, 28%), and closure of cusp perforation (n = 10, 6%).RESULTS:There was 1 early death (0.6%). Two patients required re-repair of the aortic valve during initial hospitalization. During a mean follow-up of 4.2 years, there were 16 late deaths. Overall, 16 of 159 hospital survivors had late reoperation on the aortic valve (mean interval 2.8 years) without early mortality. Risks of reoperation on the aortic valve were 9%, 11%, and 15% at 3, 5, and 7 years, respectively.CONCLUSIONS:Aortic valve repair can be performed with low risk and excellent freedom from valve-related morbidity and mortality. Late recurrence of aortic valve regurgitation led to reoperation in 8.8% of patients, but mortality associated with subsequent procedures is low. Aortic valve repair appears to be a good option for selected patients, particularly young patients who wish to avoid chronic anticoagulation with warfarin.

OBJECTIVES:Infants and children undergoing cardiopulmonary bypass for repair of congenital heart defects are at substantial risk for excessive bleeding, contributing greatly to morbidity and mortality. Aprotinin significantly reduces bleeding and transfusion requirements in adults but is of indeterminate value for pediatric patients. The aim of this study was to determine plasma aprotinin concentrations in these patients with a functional aprotinin assay. METHODS:Thirty patients less than 16 years of age scheduled for cardiac surgery with aprotinin were enrolled. Aprotinin was administered as a 25,000 KIU/kg bolus, 35,000 KIU/kg cardiopulmonary bypass prime, and 12,500 KIU.kg(-1).h(-1) continuous infusion. Blood samples for aprotinin concentrations (kallikrein-inhibiting units/milliliter) were obtained before aprotinin; 5 minutes post-bolus; 5 minutes after cardiopulmonary bypass initiation; 30 and 60 minutes on cardiopulmonary bypass; on discontinuation of aprotinin; 1 hour after aprotinin discontinuation; and 4 hours after permanent separation from cardiopulmonary bypass. For analysis, patients were grouped according to weight (<10 kg, 10-20 kg, >20 kg). Differences between weight groups were assessed using an exact test for categoric variables and 1-way analysis of variance for continuous variables. RESULTS:Aprotinin concentrations differed significantly across weight groups. Five minutes after aprotinin bolus and initiation of cardiopulmonary bypass, there was significant correlation between weight and aprotinin concentration (r =.57, P =.003; r =.69, P =.001, respectively). CONCLUSION:A functional assay reveals significant variability in aprotinin concentration for pediatric patients using current weight-based aprotinin dosing. Additional investigation is necessary to determine target aprotinin concentration dosing regimens to provide better efficacy.

Background— This study evaluated long-term results of aortic root replacement and valve-preserving aortic root reconstruction for patients with aneurysms involving the aortic root. Methods and Results— Two-hundred three patients aged 53±16 years (mean±SD; 153 male, 50 female) underwent elective or urgent aortic root surgery from 1971 to 2000 for an aortic root aneurysm: 149 patients underwent a composite valve conduit reconstruction, and 54 patients underwent valve-preserving aortic root reconstruction. Fifty patients had Marfan syndrome. In-hospital and 30-day mortality was 4.0% (8/203) overall: for a composite valve conduit procedure, the corresponding value was 4.0% (6/149) and for valve-preserving procedure, 3.7% (2/54) ( P =NS). Morbidity included 3 strokes (1%), 10 perioperative myocardial infarctions (5%), and 8 reoperations for bleeding (4%). Actuarial survival at 5, 10, 15, and 20 years was 93% (95% confidence interval [CI] = 88% to 97%), 79% (95% CI = 71% to 87%), 67% (95% CI = 57% to 79%), and 52% (95% CI = 36% to 69%), respectively. Freedom from reoperation was 72% (95% CI = 54% to 86%) at 20 years. Complications with anticoagulation occurred in 29 patients; with valve thrombosis, in 2; and with hemorrhage, in 27 (4 life threatening and 23 minor). Freedom from thromboembolism was 91% (95% CI = 77% to 98%) at 20 years. Freedom from endocarditis was 99% (95% CI = 92% to 100%) at 20 years. Multivariate analysis revealed preoperative mitral valve regurgitation (+3 to 4) and older age to be significant predictors of late death ( P ≤0.005), and Marfan syndrome, initial valve-preserving aortic root reconstruction, and need for a concomitant procedure at initial operation to be significant predictors of the need for reoperation ( P ≤0.01). Conclusions— Aortic root replacement for aortic root aneurysms can be done with low morbidity and mortality. Composite valve conduit reconstruction resulted in a durable result. There were few serious complications related to the need for long-term anticoagulation or a prosthetic valve. Reoperation was most commonly required because of failure of the aortic valve when a valve-preserving aortic root reconstruction was performed or for other cardiac or aortic disease elsewhere.

We report a case of unguarded mitral valve orifice in a newborn boy that was associated with mirror-image cardiac sidedness, ambiguous abdominal sidedness, discordant atrioventricular connection, double-outlet right ventricle, and pulmonary atresia.