Biomedical subjects

G K Danielson

Publications and source records attributed to G K Danielson.

At least 73 records · Page 4Linked to original sources

PubMed1995

Outcome of pulmonary and aortic homografts for right ventricular outflow tract reconstruction.

To determine late patient outcome and homograft durability, we reviewed 326 patients who received aortic (n = 230) or pulmonary (n = 118) cryopreserved homografts for right ventricular outflow reconstruction between January 1985 and October 1993. Patient survival, including operative mortality, 5 years after the operation was similar between the two groups (pulmonary homograft 86%, aortic homograft 80%; p = not significant by log-rank test). However, 5-year freedom from homograft failure was significantly better for pulmonary homografts (94% versus 70%, p < 0.01 by log-rank test). Late calcification was evaluated by chest roentgenography and echocardiography. Overall, 20% of aortic homografts became moderately or severely calcified compared with 4% of pulmonary homografts (p < 0.01). Twenty-six percent of aortic homografts in children 4 years old or younger had moderate or severe obstruction associated with calcification, whereas only 11% of aortic homografts in patients over 4 years of age had calcific obstruction (p < 0.01). No late deaths among patients receiving pulmonary homografts were related to graft failure; two late deaths in the aortic homograft group were homograft related. Risk factors for patient mortality and homograft failure (defined as either need for homograft replacement because of homograft failure or as homograft-related death) were identified by the Cox multivariate analysis. Aortic type of homograft was a significant risk factor for homograft failure (p < 0.0001), but type of homograft was not correlated with patient mortality. Age 4 years or younger was a significant risk factor for both mortality (p < 0.01) and homograft failure (p = 0.03) in aortic homograft recipients but not in pulmonary homograft recipients. These results indicate that both aortic and pulmonary homografts provided excellent intermediate-term patient survival after right ventricular outflow tract reconstruction, but pulmonary homografts are more durable than aortic homografts with less calcification and obstruction, especially among children 4 years old or younger.

Adolescent↗

PubMed1995

Late results of systemic atrioventricular valve replacement in corrected transposition.

From December 1964 to October 1993, 40 patients (aged 5 months to 70 years, mean 21.8 years, median 13.6 years) with corrected transposition and systemic atrioventricular valve insufficiency underwent replacement (n = 39) or repair (n = 1) of the systemic atrioventricular valve. Thirty-nine patients had situs solitus and 1 had situs inversus. Associated anomalies included Ebstein's malformation of the systemic atrioventricular valve (n = 22), ventricular septal defect (n = 19), and pulmonary stenosis (n = 14). Preoperatively, 16 patients (40.0%) had complete heart block and 27 patients (67.5%) were in New York Heart Association functional classes III and IV. The early mortality was 10.0% (n = 4) and 8 patients died subsequently. The principal cause of death in all 12 patients was systemic ventricular failure. Overall survival including early mortality was 78.0% at 5 years and 60.7% at 10 years; survival excluding early mortality was 86.7% at 5 years and 67.5% at 10 years. Survivorship correlated with preoperative systemic ventricular ejection fraction of 44% or more (p < 0.001) and later interval of operation (9 deaths in 15 patients before 1981 versus 3 deaths in 25 patients subsequently) (p = 0.06). There were no cases of surgically induced complete heart block. Two patients underwent late reoperations related to the systemic atrioventricular valve prosthesis. Follow-up extended to 26.0 years (median 4.7 years). At last follow-up, 18 of the 28 survivors were in New York Heart Association functional class I, 9 were in class II, and 1 was in class III. We conclude that the results of systemic atrioventricular valve replacement in corrected transposition have improved significantly during the past decade. To preserve systemic ventricular function, we suggest operation be considered at the earliest sign of progressive ventricular dysfunction as assessed by serial clinical evaluation and echocardiography.

Adolescent↗

PubMed1995

The risk of stroke in the early postoperative period following mitral valve replacement.

All patients (285) undergoing mitral valve replacement (MVR) with a Carpentier-Edwards (C-E) bioprosthesis +/- coronary bypass grafts (CABG) were reviewed (109 men and 176 women with a median age of 70 years). Overall, the 5-year survival rate was 58.9%, 62.7% for MVR (199 patients) and 50.1% for MVR+CABG (86 patients). Late survival was adversely affected by the operative time variables of NYHA class IV, older (> or = 70 years) age, low (> or = 56%) ejection fraction (EF), and the additional performance of associated procedures+CABG with MVR (P < or = 0.001). The 5-year freedom from stroke rate was 89.2%, 89.1% for MVR and 90.2% for MVR +/- CABG. Advanced heart class was the only significant variable associated with a greater risk of late stroke (P < or = 0.01). Neither chronic preoperative atrial fibrillation nor operative obliteration of the left atrial appendage increased or decreased the late risk of stroke in patients following MVR. Hazard function for stroke occurring in the first postoperative year (first 48 h excluded to discount intraoperative events) demonstrated the highest rate within the first month (40%), rapidly diminishing thereafter. This pattern was reproduced in the 12-year hazard function in that the rate of stroke occurrence was greatest in the first year (6.7%) following implantation. The mean stroke rate over 12 years was 2.5%. Strokes following MVR +/- CABG are more likely to occur in older and more compromised patients, and the higher early rate is not reflected in the mean rate. A more aggressive approach to early anticoagulation with IV heparin, Coumadin, and possibly antiplatelet therapy is advocated to reduce this complication rate.

Adolescent↗

PubMed1995

Biplane intraoperative transesophageal echocardiography in congenital heart disease.

OBJECTIVE: To evaluate the accuracy, value, and safety of biplane intraoperative transesophageal echocardiography (TEE) in patients with congenital cardiac malformations. DESIGN: We reviewed the results of the first 104 consecutive biplane intraoperative TEE examinations performed during the repair of congenital heart defects at the Mayo Clinic. MATERIAL AND METHODS: TEE results were analyzed for accuracy of diagnosis, effect on the surgical procedure, and associated complications. In a subjective analysis, the relative contributions and advantages of each imaging plane (transverse and longitudinal) were also assessed. RESULTS: Biplane TEE had "significant impact" on intraoperative management in 17 of 104 examinations (16.3%). Preoperative TEE altered the planned procedure in 11 patients (10.6%). Postbypass biplane TEE led to immediate revision of the initial repair in nine patients (8.7%). Patients who underwent modified Fontan operations or subaortic resections had the greatest frequency of significant impact (40% [P = 0.006] and 33% [P = 0.03], respectively). No major complications were associated with TEE. For a complete examination, use of both imaging planes was necessary in all the patients studied. CONCLUSION: Biplane TEE is an accurate, valuable, and safe addition to the perioperative care of patients with congenital heart disease. Although intraoperative TEE is not needed in all operations for congenital heart disease, we recommend that biplane intraoperative TEE be performed routinely during modified Fontan procedures, subaortic resections, and other intracardiac operations for complex congenital cardiac malformations.

Adolescent↗

PubMed1995

Early and late survival after surgical treatment of culture-positive active endocarditis.

OBJECTIVE: To describe a 30-year experience with surgically treated culture-positive active endocarditis. DESIGN: We retrospectively reviewed the microbiologic, clinical, and operative findings and the survival data in 151 patients with culture-positive active endocarditis encountered between 1961 and 1991. RESULTS: The mean age of the 110 male and 41 female patients was 49.8 years. Native valve endocarditis was present in 86 patients, and prosthetic valve endocarditis (PVE) was diagnosed in 65. The aortic valve was involved in 62% of patients, the mitral valve in 25%, and both valves in 10%. The operative mortality was 26%. The most important univariate determinants of mortality were an abscess at operation (P = 0.01) and renal failure (P = 0.03). A trend toward a higher mortality with PVE and staphylococcal infection was noted. For hospital survivors, the 5- and 10-year survival was 71% and 60%, respectively. Univariate determinants of an adverse long-term survival were annular abscess (P = 0.01), renal impairment (P = 0.01), heart failure (P = 0.02), and aortic valve involvement (P = 0.05). On multivariate analysis, the most important adverse determinants of long-term survival were heart failure (P = 0.02), renal impairment (P = 0.02), and PVE (P = 0.03). Thirty patients required a subsequent reoperation; of these, seven required a second and two a third operation. The most common reason for reoperation was periprosthetic regurgitation without infection (N = 19). Four operations were performed for recurrent endocarditis. At 5 and 10 years, the risk of reoperation was 23% and 36%, respectively. CONCLUSION: Although surgical treatment of culture-positive active endocarditis is still associated with substantial mortality, the long-term outcome of hospital survivors is excellent. Subsequent reoperations for periprosthetic leak are common, but recurrent infection is uncommon.

Adolescent↗

PubMed1995

Cardiac echinococcosis: case report of unusual echocardiographic appearance.

An unusual echocardiographic appearance of a cardiac echinococcal lesion confirmed intraoperatively is described in a patient with nonspecific cardiac complaints. Although previous reports have emphasized the echolucent, often multiseptated nature of echinococcal lesions, such characteristics were absent in this case. Thus, in the appropriate clinical setting, echinococcal infection should be included in the differential diagnosis of solid mass lesions of the heart because the surgical approach may need to be altered.

Adult↗

PubMed1995

Late results of pulmonary ventricle to pulmonary artery conduits.

The development of the extracardiac conduit has been one of the greatest advances in cardiac surgery. Conduits have decreased the mortality rate of several standard operations and have made possible the correction of numerous complex congenital cardiac anomalies that previously were uncorrectable. However, the ideal conduit has not yet been developed. We have reviewed the long-term (16 to 29 years) results of our early experience with this technique.

Adolescent↗

PubMed1994

Therapeutic implications of intraoperative pressure measurements after the Fontan operation.

Central venous pressure (CVP) and left atrial pressure (LAP) were monitored continuously for the first 72 hours postoperatively in 32 patients who underwent a Fontan operation in whom preoperative measurements of the pulmonary artery index were available. Integrated mean values were generated for each patient for the following time frames: (1) the first 12 hours after operation, (2) the first 24 hours after operation, (3) postoperative day 2, and (4) postoperative day 3. We found no difference in the CVP, LAP, or transpulmonary gradient, derived as CVP-LAP, measured in the operating room at the completion of the operation versus that measured on the third postoperative day: CVP, 18 +/- 2 mmHg versus 19 +/- 3 mmHg; LAP, 10 +/- 2 mmHg versus 10 +/- 3 mmHg; and transpulmonary gradient, 8 +/- 2 mmHg versus 8 +/- 2 mmHg. The combined incidence of hospital mortality and postoperative takedown associated with the Fontan repair was 12.5%. These findings suggest that a poor hemodynamic result from the Fontan operation can be predicted from intraoperative pressure measurements, because the CVP, LAP, and transpulmonary gradient are unlikely to change significantly in the early postoperative period. Therefore, a decision to take down or fenestrate the repair can reasonably be made in the operating room or the early postoperative period.

Adolescent↗

PubMed1994

Imaging strategies for vascular rings.

Thirty-nine patients have undergone operation for relief of tracheoesophageal compression resulting from vascular rings and related entities at the Mayo Clinic. Nineteen patients had a double aortic arch, 11 patients had a right aortic arch with an aberrant left subclavian artery, 5 patients had a left aortic arch with an aberrant right subclavian artery, 2 patients had a pulmonary artery sling, 1 patient had a right aortic arch with mirror-image branching and a left ligamentum arteriosum, and 1 patient had a left aortic arch, a right descending aorta, and a right ductus arteriosus. Diagnostic examinations included chest radiography, barium esophagography, angiography, and, more recently, transthoracic echocardiography, computed tomography, and magnetic resonance imaging. A comparison among the various diagnostic techniques used in 12 patients during the last 12 years showed that angiography (n = 7), magnetic resonance imaging (n = 5), and computed tomography (n = 3) were the most reliable, as they always accurately delineated the anatomy. However, in the 6 patients who underwent transthoracic echocardiography, 1 of whom was an older child and 2 of whom were adults, the vascular abnormality was described correctly only once; in the other 5 patients, the results were false-negative or the technique failed to visualize the relevant vascular structures sufficiently. Currently, magnetic resonance imaging is our imaging technique of choice for the delineation of the vascular and tracheal anatomy in patients suspected of having a vascular ring.

Adolescent↗

PubMed1994

Diagnostic role of Doppler echocardiography in constrictive pericarditis.

OBJECTIVES: This study was conducted to assess the diagnostic role of Doppler echocardiography in constrictive pericarditis. BACKGROUND: It has been observed that patients with constrictive pericarditis have a characteristic Doppler pattern of respiratory variation in ventricular filling and central venous flow velocities. However, the observation was based on a small number of patients with known diagnosis. METHODS: We reviewed the echocardiographic features of 28 patients (21 men and 7 women; mean age +/- SD 55 +/- 15 years) with suspected constrictive pericarditis who underwent exploratory thoracotomy or pericardiectomy. RESULTS: At operation, constrictive pericarditis was diagnosed in 25 patients, restriction in 1 and normal pericardium in 2. Of the 25 patients with constriction, correct preoperative Doppler diagnosis was made in 22 (88%) and Doppler echocardiography showed restriction in 3. In two patients with a normal pericardium, Doppler features were consistent with constriction in one patient and were normal in the other. In the one patient with restriction, Doppler echocardiography showed restriction. In 19 patients with surgically proved constriction, repeat Doppler study after pericardiectomy showed normal findings in 14 and restriction in 5. Twelve of the 14 patients with normalized Doppler findings became asymptomatic, whereas all 5 with restrictive Doppler features remained symptomatic. CONCLUSIONS: Doppler echocardiography performed simultaneously with respiratory recording is highly sensitive for diagnosing constrictive pericarditis, and it appears to predict functional response to pericardiectomy.

Adult↗

PubMed1994

Enteric protein loss after the Fontan operation.

OBJECTIVE: The current study was undertaken to assess the frequency of excessive enteric protein loss and protein-losing enteropathy in the relatively early period after the Fontan operation. DESIGN: Protein excretion was determined in 26 of 27 consecutive patients who underwent the Fontan procedure between January and June 1990 at the Mayo Clinic. MATERIAL AND METHODS: At two testing intervals during the first 4 months after the Fontan operation, alpha 1-antitrypsin clearance and fecal alpha 1-antitrypsin concentration studies were done. RESULTS: All results were normal for the first postoperative test period (2 to 8 weeks). For the second study period, all 17 patients tested had normal alpha 1-antitrypsin clearances. One of the 17 patients had an appreciably increased fecal alpha 1-antitrypsin concentration and transient protein-losing enteropathy. CONCLUSION: Excessive enteric protein loss and protein-losing enteropathy are relatively uncommon during the first 4 months after the Fontan operation.

Adolescent↗

PubMed1994

Multiple myocardial hamartomas causing ventricular tachycardia in young children: combined surgical modification and medical treatment.

OBJECTIVE: To describe two cases of incessant ventricular tachycardia caused by multiple myocardial hamartomas. MATERIAL AND METHODS: Two infants, 13 and 14 months old, who had had multiple episodes of symptomatic tachycardia were referred to our institution. Incessant ventricular tachycardia was diagnosed. Initially, the patients received pharmacologic therapy. The recurrent tachycardia resulted in notable hemodynamic instability. No structural abnormalities were detected on the echocardiograms. In one patient, an electrophysiologic study revealed that the site of ventricular ectopic beats was in the anterolateral wall of the left ventricle, midway between the apex and the base. In the other patient, a preoperative electrophysiologic study was not undertaken because of the inability to obtain central venous access. RESULTS: Diffuse hamartomas were found throughout the ventricular myocardium in both patients. Surgical resection and cryoablation of the lesions in combination with medical therapy helped control the tachyarrhythmia. At 7 and 17 months postoperatively, the patients were in normal sinus rhythm and were receiving medication. CONCLUSION: In young children who have incessant ventricular tachycardia but no lesion evident on echocardiography, angiography, or other imaging modalities, a myocardial tumor should be suspected, and pharmacologic therapy should be instituted. If the medical regimen fails, surgical intervention should be undertaken, directed at areas localized by inspection and by preoperative and intraoperative electrophysiologic studies.

Anti-Arrhythmia Agents↗

PubMed1994

Long-term outcome of surgical repair of ruptured sinus of Valsalva aneurysm.

BACKGROUND: Because not much is known about the longterm results of surgical treatment of ruptured sinus of Valsalva aneurysm (RSVA), we reviewed our entire 37-year experience with this condition. METHODS AND RESULTS: From August 1956 through September 1993, 31 patients aged 3 to 54 years (median age, 29 years) underwent surgical correction of RSVA. Aneurysms originated in the right (n = 24) and noncoronary (n = 7) sinuses and entered the right ventricle in 21 patients and the right atrium in 10. Coexistence cardiac lesions included ventricular septal defect (VSD) (n = 16, 15 of which were subarterial) and aortic valve insufficiency (n = 13). There was a highly significant correlation between aortic insufficiency and the presence of an associated subarterial VSD (P < .0001). There was no hospital mortality. One patient (3.2%) died of endocarditis 9 years after subsequent aortic valve replacement; overall survival was 95% at 20 years. Two foreign patients were lost to follow-up after 11 and 13 years, respectively. Follow-up in the remaining 28 survivors extended to 37 years (mean, 25.7 years). Five patients (16.1%) underwent reoperation for aortic valve replacement (n = 3), closure of recurrent fistula (n = 1), and closure of both recurrent fistula and recurrent VSD (n = 1); all had their primary operation before 1976. All patients who had reoperation had right sinus of Valsalva-to-right ventricle fistulas, and 4 had an additional subarterial VSD. Risk of reoperation was higher with right ventricle fistulas than with right atrium fistulas, and this approached statistical significance (P = .06). Risk of reoperation in patients with right ventricle fistulas was lower when an aortotomy (with or without right ventriculotomy) was used during repair (1 of 8, 12.5%) versus right ventriculotomy only (4 of 13, 30.8%), although this did not reach statistical significance (P = .10). Need for reoperation was increased with the presence of a subarterial VSD (P = .08) but not with location of fistula or type of repair (direct suture versus patch). Of 9 patients with mild aortic insufficiency at primary operation, two developed late severe aortic insufficiency necessitating aortic valve replacement at 21 and 31 years, respectively. Twenty-five patients are in New York Heart Association class I, and 3 are in class II. CONCLUSIONS: Long-term survival after surgical treatment of RSVA is excellent. The risk for recurrent fistula or VSD is minimal in the current era. Late aortic insufficiency is still a risk, especially in right sinus of Valsalva-to-right ventricle fistula with associated subarterial VSD. Repair of RSVA through an aortotomy with or without cardiotomy permits inspection of the aortic root complex and facilitates aortic valve repair; this approach may reduce the incidence of late aortic insufficiency.

Adult↗

PubMed1994

Traumatic tricuspid valve insufficiency. Experience in thirteen patients.

From 1964 through June 1993, thirteen patients with traumatic tricuspid insufficiency were treated surgically; all were male, and the ages ranged from 17 to 64 years (median 39 years). The condition was associated with blunt chest trauma in all patients: motor vehicle accidents in twelve and an explosion of a tank of compressed air in one. The median duration between trauma and operation was 17 years (range 1 month to 37 years). Preoperatively, six patients were in sinus rhythm and seven were in atrial fibrillation. At operation, the right ventricular function appeared moderately to severely depressed in twelve patients. In twelve patients, the anterior leaflet was flail because of chordal rupture (n = 9), rupture of anterior papillary muscle (n = 3), or tear in the anterior leaflet (n = 1). In one patient, the septal leaflet was missing and in another it was retracted and adherent to the ventricular septum. In five patients the tricuspid valve was repaired and in eight it was replaced. In seven patients in the latter group, the chordae, papillary muscles, and/or tricuspid valve leaflet(s) were found to be in a contracted and atrophic state, precluding repair. No early or late deaths occurred. At follow-up extending to 26 years (median 12 years), 12 patients are in New York Heart Association class I and one patient is in class II. Nine patients were in sinus rhythm and four were in atrial fibrillation. Although our experience indicates that good functional results can still be achieved many years after the onset of traumatic tricuspid valve insufficiency, earlier diagnosis and surgical treatment should increase the feasibility of tricuspid valve insufficiency, earlier diagnosis and surgical treatment should increase the feasibility of tricuspid valve repair, prevent progressive deterioration of right ventricular function, and increase the possibility of maintaining late sinus rhythm in a greater number of patients.

Accidents, Traffic↗

PubMed1994

Aortic dissection as complication of supravalvular aortic stenosis.

A 2-year-old patient with diffuse supravalvular aortic stenosis and the Williams-Beuren syndrome at autopsy was found to have dissection of the ascending aorta in addition to severe coronary artery disease and microfocal myocardial fibrosis. These findings emphasize the need for early surgical intervention in supravalvular aortic stenosis.

Aortic Dissection↗

PubMed1994

Pathology of coronary arteries, myocardium, and great arteries in supravalvular aortic stenosis. Report of five cases with implications for surgical treatment.

Among five patients with supravalvular aortic stenosis in whom autopsy tissues were available, all were male, 1 1/2 to 39 years old (mean 10 years, median 3 years), and the four children had Williams-Beuren syndrome (two familial, two sporadic). Medial thickening and dysplasia (disorganization) characterized the aortic sinotubular junction of three patients with discrete disease and the entire ascending aorta and arch branches of the two with diffuse disease. Medial dysplasia also involved the pulmonary arteries in each case, but less severely than the aorta. Dysplasia of coronary arteries was observed in all five hearts and was more obstructive proximally than distally, in cases with diffuse than discrete aortic disease, and in the adult than in the two children with discrete supravalvular aortic stenosis. All major epicardial arteries were involved, without predilection for any particular vessel. In contrast to the great arteries, coronary artery dysplasia involved all three layers, not just the media. To varying degrees, vessels showed intimal hyperplasia, fibrosis, and disorganization (dysplasia); disruption and loss of the internal elastic membrane, with indistinct intimal-medial junctions; medial hypertrophy and dysplasia; and adventitial fibroelastosis. In severe cases, the microscopic structure resembled that of the ductus arteriosus. Acute intramedial dissections were observed in the ascending aorta and distal right coronary artery in one patient each. Chronic microfocal ischemic fibrosis was identified in the subendocardium and papillary muscles of the left ventricle in four patients, and the adult patient also had an acute myocardial infarction. In summary, these findings emphasize the extraaortic extent of supravalvular aortic stenosis and the development of ischemic heart disease even in childhood. The presence of severe coronary obstruction in the adult with discrete aortic disease suggests that chronic high pulsatile coronary blood pressure may favor the proliferation of dysplastic tissue. Early surgical intervention may minimize the degree of proliferation, as well as allow regression of left ventricular hypertrophy, thereby lessening the risk of myocardial ischemia and aortic dissection.

Adolescent↗

PubMed1994

Supravalvular aortic stenosis. Long-term results of surgical treatment.

To determine long-term outcome after operation for supravalvular aortic stenosis, we reviewed the case histories of 80 patients who had repair of the localized form (group A) (n = 67) or diffuse form (group B) (n = 13) from 1956 to 1992, including 31 patients with the Williams-Beuren syndrome. Ages ranged from 7 months to 54 years (mean = 12.6 years). Forty-six patients had one or more associated cardiovascular anomalies; the most common was aortic valve stenosis (33.8%). Eighteen patients had 22 previous cardiovascular operations, and 28 patients had one or more additional anomalies repaired during their initial procedure at our institution. In group A, the aortic root was enlarged with a teardrop-shaped patch (n = 61) or a pantaloon-shaped patch (n = 6). In group B, patch enlargement of the aorta was confined to the root (n = 4) or extended into the ascending aorta or aortic arch (n = 7); one patient had a graft placed between the ascending and descending thoracic aorta and one patient had a left ventricular-aortic conduit. There were no deaths in group A; two patients in group B in whom patch enlargement was confined to the aortic root died during the operation (2.5%). Follow-up extended to 33.4 years (mean = 14.2 years); there were five late deaths in group A and one in group B. Survival excluding operative mortality was 94% at 10 years and 91% at 20 years. All patients were in functional class I or II. There was no significant difference between patients with a teardrop-shaped or pantaloon-shaped patch in terms of late gradient, survival, or aortic insufficiency. By Cox multivariate model, the only independent predictor of late death for all patients was associated aortic valve disease (p = 0.02), which was also a risk factor for late reoperation (p = 0.02). In group B, overall survival was better in patients who received an extended patch versus aortic root patch only (p = 0.02). We reached the following conclusions: (1) Associated aortic valve disease was strongly correlated with late death and need for reoperation. (2) Both the teardrop-shaped and pantaloon-shaped patch techniques provide excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence. (3) In diffuse supravalvular aortic stenosis, aortic enlargement should be extended into the ascending aorta or beyond as required to relieve the gradient; some patients may require a graft or conduit.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

PubMed1993

Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot.

BACKGROUND: Although corrective surgery for tetralogy of Fallot has been available for more than 30 years, the occurrence of late sudden death in patients in whom surgery was apparently successful remains worrisome. METHODS: We studied long-term survival among 163 patients who survived 30 days after complete repair of tetralogy of Fallot, examining follow-up hospital records and death certificates when relevant. RESULTS: The overall 32-year actuarial survival rate among all patients who survived surgery was 86 percent, as compared with an expected rate of 96 percent in a control population matched for age and sex (P < 0.01). Thirty-year actuarial survival rates were calculated for the patient subgroups. The survival rates among patients less than 5 years old, 5 to 7 years old, and 8 to 11 years old were 90, 93, and 91 percent, respectively--slightly less than the expected rates (P < 0.001, P = 0.06, and P = 0.02). Among patients 12 years old or older at the time of surgery, the survival rate was 76 percent, as compared with an expected rate of 93 percent (P < 0.001). The performance of a palliative Blalock-Taussig shunt procedure before repair, unlike the performance of a Waterston or Potts shunt procedure, was not associated with reduced long-term survival, nor was the need for a trans-annular patch at the time of surgery. Independent predictors of long-term survival were older age at operation (P = 0.02) and a higher ratio of right ventricular to left ventricular systolic pressure after surgery (P = 0.008). Late sudden death from cardiac causes occurred in 10 patients during the 32-year period. CONCLUSIONS: Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.

Analysis of Variance↗