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.

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.

Constrictive pericarditis in 26 patients with histologically normal pericardial thickness.

BACKGROUND: Traditionally, increased pericardial thickness has been considered an essential diagnostic feature of constrictive pericarditis. Although constriction with a normal-thickness pericardium has been demonstrated clinically by noninvasive imaging, the details of clinicopathological correlates have not been described. METHODS AND RESULTS: A total of 143 patients with proven constriction underwent pericardiectomy at Mayo Clinic between 1993 and 1999. Their baseline characteristics, operative data, and pathological specimens were reviewed retrospectively. The pericardium was of normal thickness (< or =2 mm) in 26 patients (18%; group 1) and was thickened (>2 mm) in 117 (82%; group 2). The most common causes of constriction in group 1 included previous cardiac surgery, chest irradiation, previous infarction, and idiopathic disease. There was little difference in symptoms and findings on physical examination between the 2 groups. Microscopically, no patient had an entirely normal pericardium. Histopathological abnormalities in group 1 were mild and focal, including fibrosis, inflammation, calcification, fibrin deposition, and focal noncaseating granulomas. Pericardiectomy was equally effective in relieving symptoms regardless of the presence or absence of increased thickness. CONCLUSIONS: Pericardial thickness was not increased in 18% of patients with surgically proven constrictive pericarditis, although the histopathological appearance was focally abnormal in all cases. When clinical, echocardiographic, or invasive hemodynamic features indicate constriction in patients with heart failure, pericardiectomy should not be denied on the basis of normal thickness as demonstrated by noninvasive imaging.

Surgery for aneurysms of the aortic root: a 30-year experience.

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< or =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< or =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.

Transient constrictive pericarditis: causes and natural history.

OBJECTIVES: This study was designed to elucidate the causes and natural history of transient constrictive pericarditis (CP). BACKGROUND: In some patients with acute CP, the symptoms and constrictive physiologic features resolve with medical therapy alone, a phenomenon that has been labeled "transient constrictive pericarditis." No large studies have examined the causes or natural history of transient CP. METHOD: Review of the Mayo Clinic echocardiogram database identified 212 patients who had echocardiographic findings of CP from 1988 through 1999. Demographic, clinical, and echocardiographic findings were identified in all patients. In 36 of these patients, follow-up echocardiograms showed resolution of the constrictive hemodynamics without pericardiectomy. RESULTS: The average age of the patients was 49 +/- 21 years, and 72% were men. The causes for the CP were diverse, the most common being prior cardiovascular surgery (25%). In a subset of 22 patients who were followed serially during the course of their illness, resolution of the constrictive physiologic features occurred at an average of 8.3 weeks after diagnosis. CONCLUSIONS: A subset of patients with CP experience resolution of the disorder without requiring pericardiectomy.

Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries.

OBJECTIVES: The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries. BACKGROUND: The presentation of this disorder and the outcome in unoperated adults have not been well defined. METHODS: All unoperated patients > or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome. RESULTS: Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age > or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade > or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index > or =2/4). In 16 (53%), SAVV regurgitation > or =3/4 and ventricular dysfunction had been documented for >6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001). CONCLUSIONS: Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.

Long-term results of the Fontan operation for double-inlet left ventricle.

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.

Surgical management of Ebstein's anomaly in the adult.

Ebstein's anomaly is a rare cardiac malformation that affects the tricuspid valve, right ventricle, and right atrioventricular junction. These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Diagnosis is made by echocardiography. Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures. Repair of Ebstein's anomaly eliminates right-to-left intracardiac shunting, improves exercise tolerance and functional class, and reduces supraventricular arrhythmias. In addition, quality of life and longevity are improved.

Septal myectomy for obstructive hypertrophic cardiomyopathy.

Septal myectomy effectively relieves left ventricular outflow tract obstruction (LVOTO) and cardiac symptoms in both adults and children with obstructive hypertrophic cardiomyopathy (HCM). Abnormal attachments of the papillary muscles and chordae and other cardiac lesions can be repaired at the same time. Early mortality for isolated septal myectomy in both children and adults is low (0% to 2.5%). Median echocardiographic LVOT gradients at rest on late follow-up have been as low as 0 to 5 mm Hg. Symptomatic improvements after myectomy occurs in the majority; 90% of patients improve by at least one functional class, and most remain improved on late follow-up. Late survivorship compares very favorably with the natural history of nonoperated patients with symptomatic obstructive HCM. These results serve as a basis for comparison with newer nonsurgical alternatives.

Spectrum of reoperations after repair of aortic coarctation: importance of an individualized approach because of coexistent cardiovascular disease.

OBJECTIVE: To determine the indications for and spectrum of late reoperations in adults who had previously undergone coarctation repair. PATIENTS AND METHODS: We reviewed clinical, cardiac catheterization, and echocardiographic data and criteria for reoperation, surgical procedures, and outcome in 43 patients who underwent 54 reoperations between 1972 and 1996. RESULTS: Of the reoperations for recoarctation or associated cardiovascular disease (or both), 20% were performed in asymptomatic patients and 80% in symptomatic patients. Associated cardiovascular disease included bicuspid aortic valve in 36 patients (84%), aortic arch hypoplasia in 12 (28%), true or false aortic aneurysm in 6 (14%), mitral valve disease in 6 (14%), and subvalvular aortic stenosis in 5 (12%). Surgical procedures included 22 recoarctation repairs and 32 other cardiovascular interventions. Simultaneous repair of recoarctation and associated cardiovascular disease was performed as a single-stage repair in 5 reoperations through a median sternotomy using an extra-anatomic, ascending-to-descending aortic bypass, with no complications. One patient died (surgical mortality, 1.9%) of preexisting severe pulmonary vascular obstructive disease. CONCLUSIONS: After coarctation repair, associated cardiovascular diseases are the most common cause for reoperation. An individualized surgical approach is important and may range from valve replacement or recoarctation surgery to extra-anatomic bypass combined with other cardiovascular procedures, enabling simultaneous repair of recoarctation and associated lesions. Despite complex surgical techniques and multiple reoperations, morbidity and mortality were low in our series.

Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome.

OBJECTIVE: This study was undertaken to review the role of electrophysiology testing and to determine the early and late results of medical and surgical management of supraventricular tachyarrhythmias in Ebstein anomaly. METHODS: We reviewed 130 patients between 1990 and 2001 with Ebstein anomaly and history of tachyarrhythmia with a median age of 25 years (mean age 27.5 years); 106 underwent electrophysiologic testing and 24 had documented atrial flutter or fibrillation. We excluded 21 patients: negative results of testing (n = 18), ventricular tachycardia (n = 2), and junctional tachycardia (n = 1). The remaining 109 patients had more than one mechanism: accessory pathway-mediated tachycardia (n = 49), atrioventricular nodal reentrant tachycardia (n = 10), and atrial flutter/fibrillation (n = 70). RESULTS: Eighty-three patients underwent at least one arrhythmia procedure combined with surgery for Ebstein anomaly. Early mortality was 4.8%. Forty-one patients underwent surgical ablation of an accessory pathway without recurrent accessory pathway-mediated tachycardia at a mean follow-up of 48 months. Seven patients underwent surgical perinodal cryoablation for atrioventricular nodal reentrant tachycardia without recurrence at a mean follow-up of 57 months. Forty-eight patients underwent surgical intervention for atrial flutter or fibrillation (right-sided maze procedure, n = 38, and cryoablation of the atrial isthmus, n = 10). Freedom from recurrent atrial flutter or fibrillation was 75% at a mean follow-up of 34 months. CONCLUSION: Concomitant arrhythmia procedures can be performed without increase in early mortality and should be added to Ebstein repairs for all patients who have supraventricular tachyarrhythmias. Surgical procedures for accessory pathway-mediated tachycardia and atrioventricular nodal reentrant tachycardia give excellent (100%) freedom from recurrence of those arrhythmias. Surgical intervention for atrial flutter/fibrillation yields freedom from late recurrence in 75% of cases.

Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae.

OBJECTIVES: Transaortic left ventricular septal myectomy yields excellent results for most severely symptomatic patients with hypertrophic obstructive cardiomyopathy. However, associated anomalies of the mitral subvalvular apparatus may prevent complete relief of obstruction, and mitral valve replacement has been advocated. We reviewed our results of procedures designed to relieve obstruction with preservation of the mitral valve. METHODS: Among 291 patients undergoing septal myectomy from 1975 to 2002, 56 (ages 2-77 years) had anomalous mitral subvalvular apparatus including anomalous chordae (n = 28) and papillary muscles with direct insertion into mitral leaflets (n = 13) or fusion to septum (n = 31) or free wall (n = 12); 82% of patients were in New York Heart Association class III or IV. Operation included resection of anomalous chordae (28 patients), relief of papillary muscle fusion (36 patients), and extended septal myectomy, wider at the apex than the base. RESULTS: There were no early deaths and no patients required mitral valve replacement. Mean peak pressure gradients decreased from 70 +/- 28 to 4.9 +/- 8.4 mm Hg and mean mitral regurgitation grade decreased from 2.3 to 1.0 (P <.001). Mean follow-up was 2.8 +/- 2.6 years. Freedom from reoperation at 4 years was 95%. There were 3 late noncardiac deaths; 98% of patients were in New York Heart Association class I or II. CONCLUSIONS: Hypertrophic obstructive cardiomyopathy associated with anomalous mitral papillary muscles or chordae can be successfully treated without mitral valve replacement by surgical relief of the anomalies and an extended septal myectomy; early mortality is low, obstruction and mitral regurgitation are significantly reduced, and late results are excellent.

The modified Fontan procedure: early and late results in 132 adult patients.

OBJECTIVE: The modified Fontan procedure, usually performed in children, is used for the treatment of anomalies with a single functional ventricle. We reviewed our experience with the modified Fontan procedure performed in the adult patient. METHODS: Between October 1973 and May 2001, the modified Fontan procedure was performed on 132 adult patients (74 men, 58 women). Median age was 23 years (range, 18 to 53 years). Diagnoses included tricuspid atresia in 34 patients (26%), double-inlet left ventricle in 48 (36%), and complex lesions in 50 (38%). The majority of patients (89%) had at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt in 85 patients and Glenn anastomosis in 31. RESULTS: Operations included an atriopulmonary connection in 74 patients, lateral tunnel in 27, intra-atrial conduit in 14, right atrium-to-right ventricle in 9, extra-cardiac conduit in 3, and other in 5. Overall early mortality was 8.3%. Mortality was 6.5% for operations performed after 1980. This is comparable to the mortality of the modified Fontan procedure performed in children during the same time interval at our institution. All 7 of the early deaths since 1980 occurred in the complex lesion group. Morbidity included prolonged pleural effusion in 36 patients, atrial arrhythmias in 25, reoperation for bleeding in 13, permanent pacemaker in 8, and stroke in 2. Mean follow-up was 9.1 years with a maximum of 21.2 years. Actuarial survival for early survivors was 89% (84,95), 75% (67,84), and 68% (58,79) at 5, 10, and 15 years, respectively. Freedom from late reoperation was 89% (83,95), 85% (78,93), and 80% (70,91) at 5, 10 and 15 years, respectively. The majority (90%) of present survivors were New York Heart Association class I or II at follow-up. CONCLUSIONS: In properly selected adult patients with functional single ventricle, the modified Fontan procedure can be performed with early mortality similar to younger patients. Early mortality is more likely with complex lesions. The majority of late survivors have a good quality of life.

Is repair of aortic valve regurgitation a safe alternative to valve replacement?

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.

Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries.

OBJECTIVE: We sought to determine the results of surgical treatment of patients with tetralogy of Fallot and pulmonary atresia with or without major aortopulmonary collateral arteries, to clarify variables affecting early and late mortality, and to expose late, nonfatal events affecting surgical patients. METHODS: The records of 495 patients operated on from 1977 to 1999 were reviewed. Patients were separated into those who did not undergo complete repair (group A) and those who did (group B). RESULTS: Group A consisted of 160 patients. Eighty-one (51%) had palliative procedures, 45 (28%) had preliminary surgical stages (unifocalization and right ventricular outflow tract reconstruction) as initial operations, and 34 (21%) had all surgical stages but were rejected for complete repair. Early and late mortality were 16.3% (n = 26) and 23.1% (n = 31), respectively. Mean follow-up was 72.3 months. The presence of major aortopulmonary collateral arteries was a risk factor for late mortality (P =.0182). Group B consisted of 335 patients. Mean age at complete repair was 11.3 years (SD, 9.2). One hundred three (30%) patients had single-stage complete repair, whereas 232 (69%) had staged reconstruction. Twenty-two (6.6%) patients underwent reopening of the ventricular septal defect for high right ventricular pressure. Early and late mortality were 4.5% (n = 15). Risk factors were a peak right ventricular/left ventricular pressure ratio of greater than 0.7 and reopening of the ventricular septal defect (P < or = .05). Late mortality was 16% (n = 51). Mean follow-up was 11.4 years (SD, 7.5). Risk factors included male sex, nonconfluent central pulmonary arteries, reopening of the ventricular septal defect, and postrepair conduit exchange (n = 137). Ten- and 20-year results were an actuarial survival of 86% and 75% and freedom from reoperation of 55% and 29%, respectively. CONCLUSIONS: Surgical repair of patients with simple or complex forms of tetralogy of Fallot with pulmonary atresia can be achieved with low early mortality. Late mortality and need for reoperation, especially conduit replacement, continue to affect the long-term well-being of these patients.

Intraoperative transesophageal echocardiography during surgery for congenital heart defects.

OBJECTIVE: This study was undertaken to further define the impact of intraoperative transesophageal echocardiography during surgery for congenital heart disease and to determine appropriate indications. METHODS: The impact of transesophageal echocardiography on patient care was assessed in 1002 patients who underwent this procedure during surgery for congenital heart defects. It had major impact when new information altered the planned procedure or led to a revision of the initial repair. The safety of intraoperative transesophageal echocardiography was evaluated by review of the prospective data sheets and the medical record. A simple relative cost analysis was also performed. RESULTS: Patient median age was 9.9 years (range 2 days to 85 years). Transesophageal echocardiography had prebypass or postbypass major impact in 13.8% of cases (n = 138/1002). Major impact was more frequent during reoperations (P <.03). Procedures that benefited most from the additional information were valve repairs (aortic or atrioventricular) and complex outflow tract reconstructions. Partial anomalous pulmonary venous connection, tricuspid valve repair (other than of Ebstein anomaly), simple atrioventricular discordance, aortic arch anomalies, and secundum atrial septal defects had major impact rates less than 5%. No major complications occurred. Minor complications occurred in 1% of patients and were most often observed in infants smaller than 4 kg. Routine use of transesophageal echocardiography for all patients with congenital heart defects proved cost-effective. CONCLUSIONS: On the combined basis of the observed rates of major impact, the minimal complications, and the relative cost advantage, we believe that routine use of transesophageal echocardiography during most intracardiac repairs of congenital heart defects is justified, particularly for patients undergoing repeat operations for congenital cardiac malformations.

Surgical pathology of subaortic septal myectomy associated with hypertrophic cardiomyopathy. A study of 204 cases (1996-2000).

BACKGROUND: No large surgical series have qualitatively examined all histopathologic features of hypertrophic cardiomyopathy (HCM). METHODS: Medical records and microscopic slides were reviewed from 204 patients undergoing septal myectomy for HCM at Mayo Clinic Rochester. RESULTS: The 108 females and 96 males (1-86 years old; mean, 48) included 133 patients <60 years old (Group 1) and 71 patients > or =60 years (Group 2). Clinical features more prevalent in Group 2 than in Group 1 included female gender (68% vs. 45%; P=.003), aortic valve disease (31% vs. 12%; P=.01) and severe coronary atherosclerosis (43% vs. 9%; P=.001). Overall, microscopic abnormalities included myocyte hypertrophy (100%), endocardial (96%) and myocardial (93%) fibrosis, myocyte disarray (79%) and vacuolization (60%), endocardial inflammation (48%), arterial thickening (46%), dilated venules (28%), arterial dysplasia (16%), left bundle branch tissue (12%), infarction (2%), endocardial (1%) and myocardial (<1%) calcium, and amyloid (1%; senile type in all three). Lesions more prevalent in Group 1 than Group 2 were vacuolization (68% vs. 45%; P=.002), disarray (87% vs. 65%; P=.0003) and dilated venules (33% vs. 18%; P=.02). In contrast, lesions more frequent in Group 2 than in Group 1 included left bundle branch (20% vs. 8%; P=.02) and amyloid and endocardial calcium (4% vs. 0%; P=.04, each). CONCLUSIONS: Among patients undergoing septal myectomy for HCM, 53% were women and 35% were >/=60 years old. The most common microscopic features were hypertrophy, disarray, fibrosis, inflammation and vascular alterations. Disarray cannot be used as a morphologic hallmark for HCM in small surgical myectomy specimens because it was absent in 21% of the patients. Because amyloid unexpectedly affected three elderly patients, routine amyloid staining is recommended for patients >/=65 years old.

Surgical pathology of subaortic septal myectomy not associated with hypertrophic cardiomyopathy: a study of 98 cases (1996-2000).

BACKGROUND: No series have described the surgical pathology of subaortic septal myectomy in patients with conditions other than hypertrophic cardiomyopathy (HCM). METHODS: Medical records and microscopic slides were reviewed from 98 non-HCM patients undergoing septal myectomy at Mayo Clinic Rochester from 1996 to 2000. Concurrently, 204 other patients had myectomy for HCM. RESULTS: The study group (65 women, 33 men) ranged in age from 1.5 to 92 years (mean, 61). Seventy underwent surgery for aortic stenosis (Group 1), 25 for congenital subaortic stenosis (Group 2) and 3 for other conditions (Group 3). Group 1 patients were older than Group 2 patients (72 vs. 26 years; P<.0001). Microscopic evaluation showed myocyte hypertrophy (97%), vacuolization (35%), left bundle branch tissue (26%) (33% in Group 1 vs. 8% in Group 2; P=.02) and disarray (19%); interstitial fibrosis (92%), inflammation (10%) and amyloidosis (7%, all prealbumin type, all Group 1, >80 years old); arterial thickening (18%) and dysplasia (12%), and dilated venules (6%); and endocardial fibrosis (74%) (64% in Group 1 vs. 100% in Group 2; P=.0001) and chronic inflammation (17%). CONCLUSIONS: Of 302 patients undergoing subaortic septal myectomy, 32% had conditions other than HCM. Myocyte disarray was present in 19% of patients without HCM (and was absent in 21% of HCM patients in a companion study). Thus, disarray alone cannot be used reliably to include or exclude a diagnosis of HCM in small surgical specimens. Because amyloid was found unexpectedly in seven elderly patients, we recommend routine amyloid staining on surgical myectomy tissue from patients >/=65 years old.

Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits.

BACKGROUND: Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. METHODS: Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 +/- 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. RESULTS: Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% +/- 1.5% and 59.5% +/- 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time (p < or = 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio > or = 0.72 (p < or = 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% +/- 2.0% and 31.9% +/- 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size (p < or = 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. CONCLUSIONS: Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation.