Comprehensive echocardiographic assessment of the hemodynamic parameters of 285 tricuspid valve bioprostheses early after implantation.

BACKGROUND: Doppler-derived hemodynamic data for normal tricuspid valve bioprostheses are limited. METHODS: A comprehensive retrospective Doppler echocardiographic assessment of 285 normal Carpentier-Edwards Duraflex, Medtronic Mosaic, St. Jude Medical Biocor, Carpentier-Edwards Perimount, and Medtronic Hancock II tricuspid valve bioprostheses was performed early after implantation. All the important Doppler-derived hemodynamic variables reported to date for mitral valve prostheses were used. Mean values for hemodynamic variables were obtained by averaging measurements of five and nine consecutive cardiac cycles. RESULTS: No clinically significant difference was found in the mean values obtained for the Doppler parameters when measurements were averaged from five or nine consecutive cardiac cycles. The mean value for the mean gradient was 5.2 mm Hg. Regardless of valve type and body surface area, pressure half-time was <200 msec for all 76 patients in whom it could be measured. Mean gradient <9 mm Hg, E velocity <2.1 m/sec, time-velocity integral of the tricuspid valve prosthesis <66 cm, and ratio of the time-velocity integral of the tricuspid valve prosthesis to the time-velocity integral of the left ventricular outflow tract <3.3 were recorded in 254 of the 285 patients (89%). CONCLUSIONS: This study establishes parameters for Doppler-derived hemodynamic data for various types of normal tricuspid valve bioprostheses. These threshold values (mean + 2 standard deviations) are specific, but not sensitive, for identifying tricuspid valve bioprosthesis dysfunction. Prostheses with hemodynamic values that are higher than these threshold values are likely dysfunctional, but in select cases, tricuspid valve bioprosthesis dysfunction may be present even when hemodynamic values are lower than these thresholds.

Comparison of the outcome of porcine bioprosthetic versus mechanical prosthetic replacement of the tricuspid valve in the Ebstein anomaly.

Our objective was to determine the relative merits of using a bioprosthetic porcine valve (BPV) versus a mechanical valve (MechV) when tricuspid valve (TV) replacement is required in patients with Ebstein anomaly. From 1972 to 2006, 333 patients received a BPV and 45 received a MechV. Patient records were reviewed, vital status ascertained, and all patients not known to be deceased were mailed a medical questionnaire or contacted by telephone. Early mortality was not statistically higher for patients who had a MechV (11%) than for those who had a BPV (5%) inserted in the TV position (p = 0.173). The only independent preoperative predictor of operative mortality was moderate to severe left ventricular dysfunction (odds ratio 3.1, p = 0.03); 20-year survival was better in patients who had a BPV (75%) than for those who had a MechV (43%, p = 0.003). On multivariate analysis, after adjusting for ablation of accessory pathways, sinus rhythm at dismissal, and concomitant repair of pulmonary valve stenosis, a BPV remained a predictor of late survival (hazard ratio 0.42, p = 0.004). Survival free of reoperation on the TV at 20 years postoperatively was similar for patients who had a MechV (49%) compared with those who had a BPV (42%) inserted (p = 0.941). A greater percentage of patients who had a MechV reported endocarditis (12% vs 2%), bleeding requiring hospitalization (6% vs 3%), and thrombosis (12% vs 6%); however, none of these differences were statistically significant. In conclusion, a BPV in the tricuspid position was an independent predictor of improved survival. This may be related to the higher incidence of bleeding and thrombotic complications in the patients with MechVs or may be related to differences between the 2 groups. A BPV may offer superior late survival when compared with a MechV when TV replacement is required in patients with Ebstein anomaly, but patient selection must be individualized.

Effect of operation for Ebstein anomaly on left ventricular function.

Our objective was to examine the outcomes of patients with left ventricular (LV) dysfunction who underwent operation for Ebstein anomaly. From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly underwent operation at Mayo Clinic. LV function was determined by echocardiography. Of the 495 patients with preoperative echocardiographic assessment of LV function, 50 had moderate or severe LV systolic dysfunction. In patients with LV dysfunction, the tricuspid valve (TV) was repaired in 12 patients and replaced in 36 patients; 1 patient had a 1.5 ventricle repair, and 1 patient had cardiac transplantation. There were 5 early deaths (10%). LV function improved in all but 4 patients after operation. In no patient did LV function worsen after operation. The 1-, 5-, and 10-year survival was 86%, 77%, and 67%, respectively. On univariate analysis, absence of sinus rhythm at dismissal (p = 0.003) was associated with greater overall mortality. For the entire cohort of 539 patients, LV dysfunction was independently predictive of late mortality (hazard ratio 3.76, p <0.001). At late follow-up (mean 6.9 years), 86% of patients were in New York Heart Association class I or II. In conclusion, LV systolic dysfunction occurs infrequently in patients with Ebstein anomaly and is a risk factor for increased late mortality. Although early mortality is greater in patients with LV dysfunction, the late results are favorable. Decreasing LV function should be an indication to promptly restore TV competence rather than a contraindication to TV operation.

Functional status after operation for Ebstein anomaly: the Mayo Clinic experience.

OBJECTIVES: The objective of this study was to review the long-term functional outcome of patients with Ebstein anomaly who had cardiac operation at our institution. BACKGROUND: Ebstein anomaly is a spectrum of tricuspid valvular and right ventricular dysplasia. Many patients will require operation in an attempt to improve quality of life. METHODS: From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly underwent 604 cardiac operations at the Mayo Clinic in Rochester, Minnesota. Patient records were reviewed, and all patients known to still be alive were mailed a medical questionnaire or contacted by telephone. RESULTS: At the initial operation at our institution, the mean age of the patients was 24 years (range 8 days to 79 years) and 53% were female patients. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively. Surveys were returned by 285 of 448 (64%) patients known to be alive at the time of this study. Two hundred thirty-seven (83%) patients were in New York Heart Association functional class I or II, and 34% were taking no cardiac medication. One hundred three patients (36%) reported an incident of atrial fibrillation or flutter, 5 patients (2%) reported having had endocarditis, and 1 patient (<1%) reported having a stroke. There were 275 pregnancies among 82 women. The recurrence of congenital heart disease was reported in 9 of 232 (3.9%) liveborn children. CONCLUSIONS: Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein anomaly. Atrial arrhythmias are common both before and after surgery. Many patients have had one or more successful pregnancies with a low-recurrence risk of congenital heart disease.

The outcomes of operations for 539 patients with Ebstein anomaly.

OBJECTIVE: Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution. METHODS: Patient records were reviewed, and all patients were mailed a medical questionnaire or contacted by means of telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded. RESULTS: From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations. The mean age at the time of the initial operation at our institution was 24 years (range, 8 days-79 years). Three hundred seventeen of the patients were female. One hundred forty-three (26.5%) patients had a prior invasive cardiac procedure before coming to Mayo Clinic. At the time of the first operation at Mayo Clinic, 182 patients had tricuspid valve repair, and 337 had tricuspid valve replacement. The 30-day mortality was 5.9% for the entire cohort (2.7% after 2001). Late survival was 84.7% at 10 years and 71.2% at 20 years. In a multivariate analysis of overall mortality for the patients' first operation at Mayo Clinic, increased hematocrit values, pulmonary valve stenosis, tricuspid valve replacement, absence of ablation of an accessory pathway, miscellaneous arrhythmia procedure, branch pulmonary artery enlargement, need for mechanical support postoperatively, emergency chest opening in the intensive care unit, and absence of sinus rhythm at dismissal were all predictive of mortality. When only preoperative characteristics were included, increased hematocrit values, mitral valve regurgitation requiring surgical intervention, prior cardiac procedure, and moderate-to-severe to severe reduction in right ventricular systolic function were associated with mortality. Preoperative sinus rhythm and an accessory pathway were associated with survival. Patients rated their health as excellent or good (New York Heart Association class I or II) in 83% of surveys returned. CONCLUSION: Ebstein anomaly can be surgically treated with low perioperative mortality. Both tricuspid valve repair and tricuspid valve replacement are associated with good long-term survival. Risk factors for poorer outcome included right, and/or left ventricular systolic dysfunction; increased hemoglobin/hematocrit values; male sex; right ventricular outflow tract obstruction; or hypoplastic pulmonary arteries.

Left-sided partial anomalous pulmonary venous connections.

BACKGROUND: Left-sided partial anomalous pulmonary venous connection (PAPVC) is a congenital defect where pulmonary veins from the left lung drain into the right atrium. If left untreated, PAPVC may result in severe right ventricular failure and pulmonary vascular disease. We sought to determine the effectiveness and long-term outcome after surgical correction of this anomaly. METHODS: From 1954 to 2006, 376 patients were treated at our institution for PAPVC. Of these, 27 (7.1%) patients underwent surgical repair for left-sided PAPVC. Thirteen patients were treated with a minimally invasive approach and 14 were treated with median sternotomy and cardiopulmonary bypass (CPB). RESULTS: Mean age at time of operation was 33 +/- 18 years. Eleven patients had complete left-sided PAPVCs; patients with one anomalous vein had a significantly higher right ventricular systolic pressure (RVSP) than those with two (31 vs 42 mm Hg, p = 0.003). There was a trend toward early surgical treatment in patients with two anomalous veins (27.5 vs 34.6 years, p = 0.14). Patients who underwent off-pump correction were younger (p = 0.04), presented later in our surgical experience (p = 0.004), and had more tricuspid regurgitation (TR) (p = 0.04). Repair of left-sided PAPVC was performed by left thoracotomy without CPB in 13 patients. There were no early or late deaths. Three patients were rehospitalized for cardiovascular reasons during the follow-up period. All patients had right-sided chamber reduction at follow-up, decreased RVSP (p = 0.02), and decreased TR (p = 0.04). Patients who presented with higher grades of TR showed evidence of pulmonary vascular disease at follow-up (p = 0.017). CONCLUSIONS: Repair of PAPVC can be completed successfully with low morbidity. Patients with left-sided PAPVC, right-sided chamber enlargement, evidence of TR, or clinical symptoms should undergo surgical repair.

Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus.

OBJECTIVE: To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with partial fenestrations of the coronary sinus, a rare congenital disorder. BACKGROUND: Partial fenestrations of the walls that usually separate the coronary sinus from the left atrium, also known as partial unroofing of the coronary sinus, may result in increased flow of blood to the lungs, cyanosis, or bidirectional shunting. The diagnosis is important, but difficult to confirm. METHODS: We reviewed retrospectively the clinical, echocardiographic, operative, and follow-up data on 25 patients with partial fenestration of the coronary sinus. The patients had a median age of 8 years, and underwent cardiovascular surgery at Mayo Clinic between 1958 and 2003. RESULTS: The initial diagnosis of a fenestration of the coronary sinus was made by the surgeon at repair of other congenital cardiac anomalies, by cardiac catheterization, or by echocardiography. In 14 patients, fenestration was missed during previous cardiovascular surgery. The most common associated cardiac lesions were atrial septal defects within the oval fossa, persistent left or right superior caval veins, and pulmonary or tricuspid atresia. In 7 patients, the symptoms were at least partially attributable to the fenestration, and included dyspnea, cerebral abscess, transient ischaemic attacks, and cyanosis. The fenestration was addressed surgically in 23 patients, and consisted of closure of the mouth of the coronary sinus, creation of an intra-atrial baffle, or direct repair of the fenestration. Death occurred in 1 patient due to complications unrelated to the repair. In the 24 early survivors, who have been followed up for a median of 85 months, 1 patient has died after a third reoperation for complex congenital cardiac disease. CONCLUSIONS: Fenestrations of the coronary sinus are often missed in the preoperative evaluation of congenitally malformed hearts. When associated with right heart hypoplasia, atrial septal defect, or persistent superior caval vein, fenestrations of the coronary sinus should be considered as a possible additional finding. Once the diagnosis has been made, repair is straightforward.

Surgery insight: Septal myectomy for obstructive hypertrophic cardiomyopathy--the Mayo Clinic experience.

Septal myectomy has been the gold standard treatment for the relief left ventricular outflow tract obstruction and cardiac symptoms in both adults and children with obstructive hypertrophic cardiomyopathy. In almost all circumstances, abnormalities of the mitral valve and subvalvar mitral apparatus can be managed without the need for mitral valve replacement, and other cardiac lesions can be repaired simultaneously. In the current era, the operative mortality for isolated septal myectomy at an experienced center is low in both children and adults (approximately 1%). Excellent late results with myectomy are gratifying: 90% of patients improve by at least one NYHA class, and improvements persist in most individuals on late follow-up. Late survival in patients with obstructive hypertrophic cardiomyopathy who undergo myectomy exceeds that of patients who do not receive surgical treatment and, in addition, myectomy may be associated with reduced long-term risk of sudden cardiac death. These results should serve as a basis for comparison with newer nonsurgical treatment regimens.

Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle.

OBJECTIVE: Repair of Ebstein anomaly and impaired right ventricular function pose challenges for the cardiac surgeon. The bidirectional cavopulmonary shunt may improve early outcomes. We reviewed our experience with the 1.5-ventricle repair in this patient population. METHODS: Between July 1999 and March 2006, 169 patients underwent operations to repair Ebstein anomaly. Fourteen patients had a bidirectional cavopulmonary shunt constructed. The median age at operation was 6 years (17 months-57.8 years). All of the patients had severe Ebstein anomaly with dilated right-sided chambers and/or right ventricular dysfunction. The mean left ventricular ejection fraction was 54.5% (range 35%-72%). Three patients were initially referred for heart transplantation, and the bidirectional cavopulmonary shunt allowed a conventional repair. RESULTS: Procedures included bidirectional cavopulmonary shunting (14), tricuspid valve replacement (11), tricuspid valve repair (2), and right ventricular resection (3). Shunting was planned preoperatively in 9 patients; the indication in 5 other patients was hemodynamic instability after separation from cardiopulmonary bypass. One patient died of multiple organ failure. Median follow-up in 10 patients was 18 months (3 months-6.5 years). The preoperative left ventricular ejection fraction of less than 50% improved in 3 patients to greater than 50% postoperatively. CONCLUSIONS: The 1.5-ventricle repair can be utilized in patients with severe Ebstein anomaly and impaired right ventricular function who are at high risk for surgical treatment. We believe the bidirectional cavopulmonary shunt may be considered as a planned procedure, as an intraoperative salvage maneuver, or as an alternative to cardiac transplantation in selected patients.

Surgical management of Ebstein's anomaly.

Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle. Surgical repair of Ebstein's anomaly improves functional class and exercise tolerance, eliminates right-to-left intracardiac shunting (if present), and reduces the incidence of supraventricular tachyarrhythmias. As a result, quality of life and survival are improved. Because of the variable degree of malformation present, repair is predicated on favorable anatomic factors, most importantly the arrangement of the anterior leaflet of the tricuspid valve. When anatomic derangements threaten a durable tricuspid valve repair, valve replacement with protection of the conduction tissue and right coronary artery should be performed. The vast majority of patients can undergo a biventricular repair. The application of the bidirectional cavopulmonary anastomosis is reserved for patients with poor right ventricular function. Freedom from reoperation after tricuspid valve repair is similar when compared with valve replacement. In the current era, overall early mortality after surgical repair in children and adults has fallen to less than 3% in experienced centers. Surgical treatment of the symptomatic neonate remains a significant challenge, with approaches that include either a biventricular or single ventricle algorithm.

Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients.

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.

Long-term results of the Konno procedure for complex left ventricular outflow tract obstruction.

OBJECTIVE: This study was undertaken to determine long-term clinical and echocardiographic outcomes after the Konno procedure. METHODS: Fifty-three patients who underwent the Konno procedure between January 1, 1980, and January 1, 2004, were reviewed. RESULTS: Mean age at operation was 19 years (range, 1-65 years). Indications were as follows: complex subaortic or tunnel stenosis in 22 (41%), multilevel left ventricular outflow tract obstruction in 20 (38%), and aortic valve stenosis or hypoplasia in 11 (21%). Before the Konno procedure, 66 operations were performed in 41 (77%) patients. Thirty-three (62%) patients had greater than New York Heart Association class I symptoms preoperatively. A mechanical aortic valve was implanted in 40 (75%), a homograft in 10 (19%), and a xenograft prosthesis in 3 (6%). Mortality at 30 days was 8% (n = 4). Survival at 10 years was 86%. Risk factors for overall mortality were New York Heart Association class (hazard ratio 2.22, P = .04) and longer bypass time (hazard ratio 1.93/hour, P = .04). The cumulative probability of aortic valve reoperation was 19% at 5 years and 39% at 10 years, occurring in 15 patients at a median of 3.8 years. The average left ventricular outflow tract mean gradients were 19 mm Hg at 1 year (n = 9), 13 mm Hg at 1 to 3 years (n = 9), and 13 mm Hg at 3 to 5 years (n = 5). Pulmonary regurgitation was detected in 6 patients. Pulmonary valve replacement was performed in 3 (6%). At the date of last contact, all patients for whom data was available were in New York Heart Association functional class I or II. CONCLUSION: The Konno procedure is effective, allowing both long-term reduction of left ventricular outflow tract obstruction and improvement in functional class. Prosthetic aortic valve and native pulmonary valve complications may necessitate reoperation.

Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease.

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.

Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience.

BACKGROUND: The purpose of this study was to examine early and late outcome of tricuspid valve repair for Ebstein's anomaly in young children. METHODS: Between October 1974 and November 2003, 52 children (25 boys) underwent tricuspid valve repair and annuloplasty for Ebstein's anomaly. Mean age was 7.1 +/- 3.9 years (range, 5 months to 12 years). Concomitant procedures included atrial septal defect closure (n = 46), division of accessory conduction pathways (n = 4), ventricular septal defect closure (n = 3), and other (n = 7). RESULTS: Early mortality was 5.8% (3 of 52 patients; no mortality since 1984, n = 31). Risk factors were age younger than 2.5 years (p = 0.03) and weight less than 10.7 kg (p = 0.03). Morbidity included transient atrial (n = 11) and ventricular arrhythmias (n = 5), and early reoperation in 3 patients. There was no need for a permanent pacemaker. Mean follow-up was 12.2 +/- 7.4 years (maximum, 24.3 years). Actuarial survival at 5, 10, and 15 years was 92.3% +/- 3.7%, 89.9% +/- 4.3%, and 89.9% +/- 4.3%, respectively. Freedom from all reoperations at 5, 10, and 15 years was 91.0% +/- 4.3%, 76.9% +/- 6.8%, and 61.4% +/- 8.8%, respectively. Moderate (grade II) or more tricuspid regurgitation on dismissal echocardiogram was the only risk factor for reoperation (p = 0.04). Tricuspid stenosis did not occur in any patient. At late follow-up, 89% of patients were in New York Heart Association class I or II. CONCLUSIONS: Ebstein's anomaly in young children can now be repaired with low mortality and good tricuspid valve durability. Tricuspid regurgitation at the completion of operation should be mild or less to minimize need for reoperation. Tricuspid valve repair and annuloplasty did not result in stenosis despite somatic growth. Most patients enjoy an excellent quality of life.

Septal myectomy for obstructive hypertrophic cardiomyopathy in pediatric patients: early and late results.

BACKGROUND: Symptomatic pediatric patients with obstructive hypertrophic cardiomyopathy (HCM) have a higher death rate (6% annually) compared with adults. Transaortic left ventricular septal myectomy provides excellent outcomes for adults with obstructive HCM. We sought to assess the effect of septal myectomy on late survival and outcome in pediatric patients with obstructive HCM. METHODS: From 1975 to 2003, 56 pediatric patients underwent septal myectomy for obstructive HCM. Mean age at diagnosis was 6.3 +/- 5.4 years. Ages at operation ranged from 2 months to 20 years (mean 11 +/- 5.6 years). Concomitant procedures included mitral valve repair (n = 7), closure of atrial septal defect (n = 3), and other (n = 5). RESULTS: After myectomy, mean left ventricular outflow tract (LVOT) gradient decreased from 103 +/- 34 to 16 +/- 12 mm Hg and mean degree of mitral regurgitation decreased from 2.0 +/- 1.0 to 1.0 +/- 0.3 (both p < 0.0001). There were no early deaths. Four patients underwent elective cardioverter defibrillator implantation and 1 patient received a permanent pacemaker. Follow-up ranged up to 29 years (mean, 8.6 +/- 6.2). Cardiac reoperations were required in 8 patients: heart transplantation (n = 2), repeat myectomy (n = 2), mitral valve repair-replacement (n = 2), Konno-Rastan procedure (n = 1), and aortic valve replacement (n = 1). Age 14 years or less at operation was the only predictor of late reoperation (p = 0.017). Two patients died late; one suddenly without residual LVOT obstruction and one from chronic rejection after heart transplantation. Ninety-six percent of surviving patients were in New York Heart Association functional class I or II. Survival estimates at 5 and 10 years were 97% and 93%, respectively. CONCLUSIONS: Septal myectomy is safe and effective in symptomatic pediatric patients with obstructive HCM. Late survivorship compares very favorably with the natural history of this disease.

Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.

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.

Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.

OBJECTIVES: This study sought to determine the impact of surgical myectomy on long-term survival in hypertrophic cardiomyopathy (HCM). BACKGROUND: Left ventricular (LV) outflow tract obstruction in HCM increases the likelihood of heart failure and cardiovascular death. Although surgical myectomy is the primary treatment for amelioration of outflow obstruction and advanced drug-refractory heart failure symptoms, its impact on long-term survival remains unresolved. METHODS: Total and HCM-related mortality were compared in three subgroups comprised of 1,337 consecutive HCM patients evaluated from 1983 to 2001: 1) surgical myectomy (n = 289); 2) LV outflow obstruction without operation (n = 228); and 3) nonobstructive (n = 820). Mean follow-up duration was 6 +/- 6 years. RESULTS: Including two operative deaths (procedural mortality, 0.8%), 1-, 5-, and 10-year overall survival after myectomy was 98%, 96%, and 83%, respectively, and did not differ from that of the general U.S. population matched for age and gender (p = 0.2) nor from patients with nonobstructive HCM (p = 0.8). Compared to nonoperated obstructive HCM patients, myectomy patients experienced superior survival free from all-cause mortality (98%, 96%, and 83% vs. 90%, 79%, and 61%, respectively; p < 0.001), HCM-related mortality (99%, 98%, and 95% vs. 94%, 89%, and 73%, respectively; p < 0.001), and sudden cardiac death (100%, 99%, and 99% vs. 97%, 93%, and 89%, respectively; p = 0.003). Multivariate analysis showed myectomy to have a strong, independent association with survival (hazard ratio 0.43; p < 0.001). CONCLUSIONS: Surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation. In this retrospective study, septal myectomy seems to reduce mortality risk in severely symptomatic patients with obstructive HCM.

Mechanisms for recurrent left ventricular outflow tract obstruction after septal myectomy for obstructive hypertrophic cardiomyopathy.

BACKGROUND: Left ventricular septal myectomy provides excellent outcomes for symptomatic patients with severe obstructive hypertrophic cardiomyopathy. However, in a few patients, left ventricular outflow tract obstruction may recur and require repeat myectomy. We reviewed this subset of patients to assess the mechanisms of recurrence. METHODS: From 1975 to July 2003, 610 septal myectomies were performed; 13 of these were repeat myectomies after classic myectomies performed at our institution (n = 6) or elsewhere (n = 7). Ages ranged from 4 to 70 years (mean, 32 +/- 22). The interval between initial myectomy and repeat myectomy ranged from 13 months to 11 years (mean, 5.0 +/- 3.7 years). RESULTS: Mechanisms for obstruction included limited myectomy at the initial myectomy (n = 11), septal hypertrophy at the midventricular level (n = 8), and anomalous papillary muscles (n = 3). Mean intraoperative pressure gradients decreased from 82 +/- 24 to 6.2 +/- 4.4 mm Hg. No mitral valve replacement was performed, and there were no early deaths. Mean follow-up was 5.8 +/- 5.8 years. There was one late death. All surviving patients were free from recurrence of outflow tract obstruction and were in the New York Heart Association functional class I or II. CONCLUSIONS: Mechanisms for recurrent obstruction included limited myectomy at the initial operation, midventricular obstruction, anomalies of papillary muscles, and ventricular remodeling, especially in pediatric patients. Repeat myectomy can be performed with excellent outcomes. Need for reoperation may be reduced with current surgical approaches that include a more extended resection of the midventricular septum, relief of papillary muscle anomalies, and routine use of intraoperative transesophageal echocardiography.