Cardiac surgery with deep hypothermic circulatory arrest produces less systemic inflammatory response than low-flow cardiopulmonary bypass in newborns.

OBJECTIVE: We sought to compare low-flow cardiopulmonary bypass with deep hypothermic circulatory arrest in respect to the influence on the systemic inflammatory response. METHODS: Twenty-three infants weighing less than 10 kg and scheduled for repair of congenital malformations were enrolled in a randomized, controlled study. Eleven patients underwent cardiac surgery with deep hypothermic circulatory arrest (the DHCA group). Low-flow cardiopulmonary bypass was used in another 12 patients (the LF group). Interleukin 6 and 8 and anaphylatoxin C3a levels were measured 6 times perioperatively. Also, perioperative weight gain and a radiologic soft-tissue index were compared. RESULTS: All patients had an uneventful clinical course. Duration of deep hypothermic circulatory arrest was 40 +/- 4 minutes; the bypass time was significantly shorter in the DHCA group (85 +/- 8 vs 130 +/- 19 minutes). However, the duration of the operation was similar in both groups (245 +/- 30 vs 246 +/- 30 minutes). During cardiopulmonary bypass (rewarming), the concentration of C3a (3751 +/- 388 vs 5761 +/- 1688 ng/mL, mean +/- SEM) was significantly lower in the DHCA group than in the LF group. The interleukin 8 level was significantly lower, and the interleukin 6 level had a tendency to be lower in the DHCA group compared with levels in the LF group. There was less weight gain on the first postoperative day in the DHCA group (65 +/- 61 vs 408 +/- 118 g). The soft-tissue index suggested reduced edema formation in the DHCA group. CONCLUSION: Deep hypothermic circulatory arrest produces less systemic inflammatory response than low-flow cardiopulmonary bypass. In addition, there is an indication of less fluid accumulation postoperatively.

Effect of C1-esterase-inhibitor on capillary leak and inflammatory response syndrome during arterial switch operations in neonates.

OBJECTIVE: To determine if prophylactic administration of C1-esterase-inhibitor would have a beneficial effect on postoperative weight gain and the inflammatory response in neonates undergoing cardiac surgery with cardiopulmonary bypass (CPB). DESIGN: Randomized, double-blinded study. SETTING: University-affiliated heart center. PARTICIPANTS: Twenty-four neonates with transposition of the great arteries. INTERVENTIONS: In group inhibitor (INH) patients (n = 12), 100 IU/kg of C1-esterase-inhibitor (Berinert) was given 30 minutes before CPB. In group placebo (P) patients (n = 12), placebo was administered instead. Interleukin (IL)-6, C3a anaphylatoxin, C1 activity, prekallikrein, Hageman factor, D-dimers, and clinical parameters were measured 6 times perioperatively. MEASUREMENTS AND MAIN RESULTS: All 24 patients had an uneventful clinical course. Mean arterial pressure and pulmonary oxygenation after CPB were superior in group INH patients. The weight gain on postoperative days 1 to 4 was significantly less in group INH patients compared with group P (55 +/- 59 g vs. 340 +/- 121 g, day 1). The concentration of IL-6 (76 +/- 17 pg/mL vs. 262 +/- 95 pg/mL during CPB) was significantly lower in group INH patients compared with group P patients. In contrast, no influence on C3a anaphylatoxin and coagulation factors was found. CONCLUSION: Prophylactic application of C1-esterase-inhibitor in neonates undergoing arterial switch operations produces less inflammatory response compared with placebo. This difference may have contributed to improved clinical parameters, including less weight gain postoperatively.

Performance of allografts and xenografts for right ventricular outflow tract reconstruction.

BACKGROUND: We compared the long-term durability of allografts and xenografts implanted for reconstruction of the right ventricular outflow tract. METHODS: A total of 401 patients were studied from January 1974 to June 2000 (145 xeno- and 256 allografts), follow-up being 98% complete. We analyzed freedom from reoperation and allograft specific factors that may indicate degeneration. RESULTS: The age at implantation was 2 days to 31 years (median 4.0 years). Conduit exchange rate was similar (p = 0.2) for conduit diameters less than 15 mm (41%+/-9% for allografts, 30%+/-6% for xenografts), but significantly different (p = 0.02) for diameters of 15 mm or larger (60%+/-8% for allografts, 30%+/-10% for xenografts). Diagnosis-related 20-year survival analysis showed a significantly (p = 0.01) better survival of patients with tetralogy of Fallot/pulmonary atresia (83%+/-5%) and Rastelli-type surgery (81%+/-8%) compared with patients with truncus arteriosus communis (69%+/-8%). ABO-compatibility, preservation method, and aortic or pulmonary allograft could not be identified as risk factors for allograft longevity. CONCLUSIONS: For smaller diameters (less than 15 mm), allografts exhibit no advantage over xenografts, whereas in larger diameters (15 mm or larger) allografts are the conduit of choice for the right ventricular outflow tract.

Reconstruction of the RVOT with valved biological conduits: 25 years experience with allografts and xenografts.

OBJECTIVE: The reconstruction of the RVOT in congenital heart disease often requires the implantation of a valved conduit. Although allografts are considered the conduit of choice their availability is limited and therefore xenografts are implanted as well. We compared the long-term durability of both grafts in the RVOT over a 25-year period. METHODS: Between January 1974 and August 1999, 505 patients (median age 4.0 years, range 2 days-31 years; median weight 14.5 kg, range 2.2-76.6 kg; median body length 103 cm, range 48-183 cm) with congenital malformations (PA 25.3%, TOF 14.5%, TOF+PA 2.4%, DORV 4.2%, TGA+PS 8.7%, TAC 24.8%, and other 20.2%) received their first valved conduit (174 xenografts: median diameter 14 mm, range 8-27 mm; 331 allografts: median diameter 19 mm, range 8-30 mm). RESULTS: Follow-up is 3017 patient-years. The 10-year survival-probability for all patients. was 66% with a mean reoperation-free interval for conduit-exchange of 13.3 years (mean reoperation-free interval for allografts, 16.0 years; mean reoperation-free interval for xenograft, 10.3 years). One hundred and thirteen patients underwent a conduit-exchange, mostly due to conduit stenosis. Fourteen patients had a second exchange and three patients a third exchange. For patients with conduit diameters <18 mm (n=235: allograft n=116, xenograft n=119; median age 9 months, range 0-27.3 years), the mean reoperation-free interval was 11.2 years (mean interval allograft, 13.1 years; mean interval xenograft, 8.6 years, P=0.03). For conduit diameters >/=18 mm (n=270: allograft n=215, xenograft n=55, median age 7.4 years, range 0-34.3 years) the mean interval from freedom of conduit exchange was 15.1 years (for allografts 14.1 years, for xenografts 12.5 years, P<0.01). Comparing xenografts to allografts, we found no difference in patient survival probability (P=0.62). There was no significant difference between antibiotic (n=198) preserved vs. cryopreserved (n=133) allografts (P=0.06). Blood group compatibility of allografts to recipients had no significant influence on allograft function (P=0.42). The donors allograft origin, whether aortic or pulmonary valve, had also no significant influence on allograft long-term function (P=0.15). CONCLUSION: For the reconstruction of the right ventricular outflow tract (RVOT) allografts show significantly better long-term durability than xenografts regardless of the age at implantation and the diameter.

Mitral-valve replacement in children under 6 years of age.

OBJECTIVE: In patients with congenital mitral-valve disease, reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, mitral-valve replacement (MVR) is the only option. We analyzed, retrospectively, data of 35 patients younger than 6 years of age, who underwent MVR at our institution. METHODS: Between 1974 and 1997, 35 children underwent MVR. The ages ranged from 2.7 months to 5.5 years (mean=1. 9+/-1.7 years) and body weight varied between 3.2 and 16.7 kg (mean=8.2+/-4 kg). The main indication (57%) for valve replacement was severe mitral-valve insufficiency. Eighteen patients (51%) had undergone at least one previous reconstructive operation (mean=1. 46+/-1.86 years) before the MVR. In 29 cases (83%), mechanical prostheses were implanted. Six patients received a bioprosthesis. The size of the prostheses ranged between 14 and 27 mm. RESULTS: The overall hospital mortality was 17.1% (6/35), and decreased from 33 (1974-1985) to 11.5% (1986-1997). Seven children died late. The actuarial survival after 20 years was 51.2+/-13.3%. Eight patients (23%) required 10 reoperations (8.2%/100 patient-years). Freedom from reoperation at 10 years was 50+/-22%. Valve-related complications were thrombo-embolism (n=2; 1.6%/100 patient-years), hemorrhage (n=1; 0.8%/100 patient-years), structural deterioration (n=3; 2.5%/100 patient-years) and non-structural dysfunction (n=3; 2. 5%/100 patient-years). Follow-up is 96% complete, with a total of 122 patient-years (mean=4.2+/-4.7 years). Eighty six percent of the patients are in New York Heart Association (NYHA) class I, 95% have sinus rhythm and 59% do not need medication. All survivors, except for those who received a bioprosthesis, were placed on a regimen of Phenprocoumon (Marcumar((R))), aiming to maintain the International Normalized Ratio (INR) between 2.5 and 3.5. In one third of these children, self-management of oral anticoagulation was performed either by the patients or their parents. CONCLUSIONS: MVR in small children still carries a high risk. In our experience, the long-term results are satisfying. After failed reconstructive surgery, or as a primary procedure, we prefer mechanical prostheses. They are well tolerated and the incidence of anticoagulation-related complications is low.

Repair of interrupted aortic arch: results after more than 20 years.

BACKGROUND: This study focused on the influence of concomitant anomalies, the individual surgical approach, and the probability for reinterventions. METHODS: Between 1975 and 1999, 94 patients with interrupted aortic arch were evaluated for short- and long-term results after surgical treatment. RESULTS: Interrupted aortic arch was associated mainly with a ventricular septal defect (85%) and left ventricular outflow tract obstruction (LVOTO, 13%). Mean follow-up was 6.7 years (median 6.9 years, 628.4 patient years). A single-stage operation was performed in 76 cases. Early mortality for two-stage procedures was 37% and late mortality was 26%, compared with single-stage procedures, with an early mortality of 12% and a late mortality of 20%, respectively. Early mortality in patients with additional LVOTO was 42% and late mortality was 50%. Freedom from reoperation at 5 years was 62%, and at 10 years was 49%. Reinterventions were performed mainly for residual arch stenosis, also with bronchus or tracheal compression, or LVOTO. CONCLUSIONS: Arch continuity and repair of associated anomalies can be achieved with an acceptable overall risk in this often complex entity. Associated anomalies play an important role in the outcome. Single-stage repair with primary anastomosis of the arch should be the surgical goal. The long-term probability for reoperation is high.

Allograft implantation in pediatric cardiac surgery: surgical experience from 1982 to 1994.

Between July 1982 and April 1994, a total of 290 patients (median age 6.5 years, range 1 month to 32.1 years, 69 patients younger than 1 year) underwent repair of their cardiac malformation by insertion of an allograft. The diagnoses were truncus arteriosus communis (n = 78, 27.0%), tetralogy of Fallot (n = 59, 20.0%), pulmonary atresia (n = 72, 25.0%), double outlet right ventricle (n = 15, 5.0%), complex transposition of the great arteries plus pulmonary stenosis (n = 37, 13.0%), and others (n = 29, 10.0%). Either pulmonary (n = 69) or aortic (n = 221) cadaver allografts were implanted. Two hundred twenty-nine of the allografts were antibiotic preserved. Since January 1991 (n = 61), a new cryopreservation procedure was employed for standardized uniform cooling using heat sinks and defined package geometry. Follow-up was complete for 95.2% (n = 276, 1,320 patient-years). Thirty-day mortality was 9.0% (n = 26) and late mortality was 12.1% (n = 35). Kaplan-Meier analysis revealed that patient survival was determined mainly by their underlying cardiac disease. All allografts with valve sizes less than 15.0 mm had to be exchanged within 7 years as these patients had outgrown their conduits. When the allograft was larger than 15.0 mm, exchange was necessary in 20% at 10 years. ABO compatibility and aortic or pulmonary origin of the allograft were not significant influences on allograft survival.(ABSTRACT TRUNCATED AT 250 WORDS)

Bioprosthetic and mechanical valves in the elderly: benefits and risks.

There is controversy over whether elderly patients benefit from the durability of mechanical valves when balanced against the risk of anticoagulation. From 1976 to 1993, 576 patients 65 years old or older underwent isolated valve replacement with mechanical (n = 250) or bioprosthetic valves (n = 326). Total follow-up was 2,222 patient-years. Probability of survival and freedom from thromboembolism and prosthetic valve endocarditis were not different between the two groups. There was a significant difference (p = 0.015) in freedom from anticoagulant-related hemorrhage. Two patients with mechanical prostheses and 7 patients with bioprostheses were reoperated. However, actuarial freedom from reoperation was not different (p = 0.73) in both groups, with no hospital mortality, whereas mortality from thromboembolic events and anticoagulant-related hemorrhage was three times higher in patients with mechanical prostheses as compared with patients with bioprostheses (1.08% versus 0.36% per patient-year). The benefit from the durability of mechanical valves, compared with bioprostheses, is smaller than expected because of the limited number of patients exposed to the onset of bioprosthetic structural deterioration. Elderly patients without absolute indication for anticoagulation should preferentially receive bioprostheses for valvular replacement.

Surgical evaluation of the modified Fontan procedure.

From 1980 to 1990 152 patients underwent Fontan operation at our institution. The following patient groups were identified: 1. patients with tricuspid atresia (n = 82, 54.0%); 2. patients with single ventricle (n = 31, 20.3%); 3. patients with a wide variety of non correctable, complex cardiac malformations (n = 39, 25.7%). In 27.0% of the patients a primary Fontan operation was performed. 45.0% of the patients received a previous shunt to increase pulmonary blood flow and in 29.4% of the patients a pulmonary artery band was placed to reduce pulmonary flow. Overall mortality was not significantly different in patients with previous palliative procedures (19.4%, n = 18) as compared to 17.4% (n = 6) in patients with primary Fontan operation. Risk of death was high in the group with complex cardiac malformations (28.2%, n = 11) and in patients with single ventricle (19.4%, n = 6). Early mortality was considerably less in patients tricuspid atresia (8.5%, n = 7). Postoperatively patients with incorporation of the residual right ventricular chamber and pulmonary valve (Fontan-Bjoerk) showed a significant (p less than 0.05) lower incidence of pleural effusion as compared to patients with other modifications of the Fontan procedure. Actuarial survival rate of all patients is 83.8 +/- 3.1% (mean +/- SEM) at ten years. The modified Fontan procedures are providing an accepted surgical method for patients with otherwise non correctable cardiac malformations.

[Reconstruction of atrioventricular valves in partial and total atrioventricular canal]. / Rekonstruktion der AV-Klappen beim partiellen und totalen AV-Kanal.

Among the anomalies of endocardial cushion defects, the partial (PCAVC) and common (CCAVC) AV canal present a special challenge to the cardiac surgeon. In particular, reconstruction of the AV valve can be difficult in CCAVC because of morphologic variations. Within 17 years, 383 patients with this disease were operated on. Early mortality of PCAVC is below 1%, and of CCAVC below 5%. A total of 90% of the survivors are in NYHA class I. In general, plastic reconstruction of the mitral valve is performed. The risk of reoperation for recurrent mitral incompetence in both groups is between 7% and 10%. Mitral valve replacement is a rare event (1.5%).

Long-term results after right ventricular outflow tract reconstruction with porcine bioprosthetic conduits.

From 1975 to 1990, a total of 110 patients were operated for complex cardiac malformations with impaired pulmonary artery perfusion using porcine valved right heart to pulmonary artery conduits. Twelve- to 30-mm porcine valved conduits (Hancock or Carpentier-Edwards) were implanted at the age of 4 weeks to 28 years (mean 4.3 years). The patients' body weights were 2.9-68 kg (mean 15.3 kg). Early mortality was 5.5% (six patients), late mortality was 12.7% (14 patients), and 90 patients could be included in this long-term follow-up (426 patient-years). So far, 41 of the conduits had to be exchanged 4 months to 15 years (mean 6.5 years) after the first implantation. Forty-nine of the conduits are still in place. At reoperation, 38 patients received an allograft; three patients, reoperated before 1982, had a second xenograft. The main reason for porcine conduit malfunction was degeneration and/or calcification of the valves. In 11 patients, however, with 12- and 14-mm conduits implanted at a mean age of 3.1 years, a reoperation was necessary after a mean time of 6.8 years because these children had "outgrown" the conduit and needed a bigger one. We conclude that even though allografts seem to be the conduit of choice for right ventricular outflow tract reconstruction, our clinical experience shows that porcine valved conduits can be used just as well since most of them function sufficiently well for as long as 5 to 10 years, and early valve failure is relatively rare.

Long-term results after right ventricular outflow tract reconstruction with porcine and allograft conduits.

From 1975 to 1989, a total of 353 patients were operated for complex cardiac malformations with impaired pulmonary artery perfusion using 53 non-valved and 300 valved right heart to pulmonary artery conduits. In 108 patients a porcine conduit (Hancock or Carpentier-Edwards) 12-30 mm was used; the age range was 14 days to 37 years (mean 4.3 years) and body weight 2.9-68 kg (mean 15.3 kg). One hundred and ninety-two patients had an allograft of 9-29 mm implanted at the age of 17 days to 26 years (mean 3.8 years), body weight 2.7-73 kg (mean 14.2 kg). So far, 32 of the porcine valved conduits have had to be exchanged 2.5-10.5 years (mean 6.1 +/- 2.1 years) after the first implantation. In 3 patients, reoperated before 1982, another porcine valved conduit was used. After 1982, 29 exchanges were all performed using allografts. The main reason for xenograft conduit malfunction was degeneration and/or calcification of the valves. Some conduits, however, with rather small sizes of 12 and 14 mm had to be exchanged in spite of still sufficient function because the children had "outgrown" the conduit and needed a bigger one. Out of 192 patients with allografts implanted since 1982, so far 5 patients have had to be reoperated 2 months to 6 years (mean 4.6 +/- 2.8 years) after the first operation. The causes of allograft failure were degeneration and, in one case, infection of the conduit. Even though allografts seem to be the conduit of choice for right ventricular outflow tract reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Special considerations on primary cardiac tumors in infancy and childhood.

Primary cardiac tumors are rare at all ages and are even less common in infants and children. In the vast majority of cases, they are benign tumors that show little tendency to growth. Primary malignant tumors of the heart are exceptionally rare. Rhabdomyomas are the most common type of tumors in infants, followed in frequency by the fibromas and the teratomas. Myxomas are very rare in pediatric patients. Symptoms that derive from the presence of a neoplastic mass are usually due to obstruction or compression. In young symptomatic patients, usually affected by rhabdomyomas, there is little chance of survival without surgery. On the other hand several patients with cardiac neoplasms are totally asymptomatic. The indication for surgery is differenciated according to the severity of the clinical picture. Since 1974, 15 infants with a cardiac tumor have been observed in our hospital. Our recent experience with 5 cases, which are described in this report, confirm our opinion, that the most important fact is the restoration of the best possible heart function. Total resection of a tumor should not always be the main therapeutical aim. Surgical intervention is required for those patients, who develop relevant clinical symptoms. Close observation of the clinical course is mandatory in all patients with a doubtful indication for operation.

[Reconstruction of the right ventricular outflow tract with xenogeneic or allogeneic valve-bearing conduits]. / Die Rekonstruktion der Ausflussbahn des rechten Herzens mit xenogenen oder allogenen klappentragenden Conduits.

From April 1974 to February 1985 131 patients were operated for reconstruction of the right ventricular outflow tract with 147 valve-carrying conduits. 79 were xenogenic (XC) and 68 sterilized and preserved allogenic (AC) conduits between right heart and lung in the following heart defects: DOLV 2, DORV 6, Fallot 24, pulmonary atresia 33, TGA, VSD and pulmonic stenosis 20, single ventricle 7, tricuspid atresia 10 and truncus arteriosus 41. Mean age was 6.6 and 5.2 years of age. The operative mortality was 13.6%. 11 more patients died later--2.0 months to 2.5 years postoperatively. 16 patients were reoperated (change of the conduit). The observation time is now 5 years in the XC-group and 1.4 years in the AC series. Up to now no dysfunction in the AC-group could be seen.

Open mitral commissurotomy. A new plea for an old operation.

From April 1974 through December 1982, 673 patients underwent surgery for isolated mitral valvular disease. Valve replacement was mandatory in 507 patients; of these, 255 received low-profile mechanical (mainly Björk-Shiley) prostheses and 252 bioprostheses. In 166 patients the mitral valve was amenable to reconstructive repair. This analysis is concerned exclusively with a subset of 127 patients, in whom open mitral commissurotomy was carried out (104 females and 23 males with a mean age at the time of operation of 45.5 years). There were 2 postoperative deaths, both due to intractable arrhythmias: one on the 4th postoperative day and one 6 years after surgery, giving a cardiac-related mortality of 1.5%. Thromboembolic complications were observed in 5 patients, none of whom developed permanent neurological deficits. There were 2 non-cardiac-related deaths: one of cancer and one of viral pneumonia. Mitral valve replacement was subsequently required in 2 patients at 2 and 7 years, respectively, after commissurotomy. Surgical intervention is pending for 2 further patients who appear likely to require valve replacement. Based on actuarial curves, the analysis of these results shows that, provided valvular morphology is suitable, open mitral commissurotomy is superior to valve replacement with respect to mortality rate and long-term survival.

Surgical treatment of transposition of the great arteries.

Between 1974 and 1979 171 patients with d-TGA were treated surgically, 109 had correcting operations. 77 patients with simple TGA had hemodynamic correction by atrial inversion techniques. 30 day survival rate was 96%. There were 3 late deaths. One patient had reoperation for superior vena cava obstruction. The methods of diagnosis, indication of surgery and the surgical procedure are described. Results of the Mustard technique (33 patients) technique in infants less than 2 years of age with simple TGA. Mortality was one patient in each group. The incidence of arrhythmia was greater with the senning technique. Better growth potential of the atria with the Senning technique warrants further use of this method.

[Pacemaker therapy in infants (author's transl)]. / Schrittmachertherapie im Kindesalter.

Since April 1974 until January 1978 permanent pacemakers were implanted in 21 children. The patient age at the time of operation averaged 4.5 years; the youngest patient was 3 months old. Indications for pacemaker implantation were: congenital total a-v block (TAVB) (n = 4), sick syndrom (S-S-S) (n = 4), postoperative TAVB (n = 13). Cardiac failure was present in all patients despite optimal medical treatment. Pacemakers were implanted under general anesthesia and intubation. The stimulation electrodes were positioned by the transvenous route in 16 subjects and by direct fixation upon the ventricle and the atrium in 5 patients. 5 children obtained an atrial triggered, 14 patients R-inhibited demand pacemaker and 2 subjects an asynchronous pacemaker. After a mean observation time of 16.4 months mean pacemaker function is normal in 14 patients. 6 children died 1 to 33 months after implantation despite functioning pacemakers because of congestive heart failure. Pacemaker malfunction was observed in 4 patients. The type of malfunction induced: failure of the impulse generator (n = 2), dislodgement of the electrode (n = 2), threshold increase (n = 1). In 5 children generators were changed 9 to 36 months (m = 23 plus or minus 10) after implantation because of battery depletion. The use of the pacemakers in small children is connected with several specific problems: 1. Application of large generators is hazardous because of impending perforation and secondary infection. 2. Until now miniaturization of pacemakers decreases function time and therefore implies frequent surgical intervention. 3. Stretching and dislodgement of transvenous electrodes may occur due to growth of the child. 4. Threshold increase may limit the life-span of myocardial electrodes. 5. Physiological changes in natural frequency requires changes in stimulation rate. 6. To guarantee normal physical activity demand related adaptation of heart rate is necessary. Because of these reasons a pacemaker system for children should have the following criteria: low weight, small wolume, high energy capacity, atrial or programmable stimulation, a thin elastic perhaps coiled electrode.

[Surgical repair of Ebstein's disease (author's transl)]. / Korrektur-Operation bei Morbus Ebstein.

In 1976 4 patients with Ebstein's disease underwent surgical repair. In one patient (22 yrs.) the tricuspid valve leaflets were suitable for reconstructive surgery using Hardys method in addition to De Vegas annuloplasty. In the other three cases (17, 29, 51 yrs. old) excision of the malformed tricuspid valve was necessary, and porcine heterografts of greatest possible diameter were implanted. Three patients improved considerably 9 months postoperatively. The fourth patient, a 51 years old woman, died 2 days postoperatively on cardiac failure. From our own results and based upon reports from the literature we conclude that reconstructive surgery of the tricuspid valve should be preferred to replacement. If valve replacement is necessary, bioprostheses ought to be used. At the age of 15 to 40 years, surgical repair seems to have the best prognosis in patients with Ebstein's malformation.