Is there still a place for open surgical valvotomy in the management of aortic stenosis in children? The view from Southampton.

OBJECTIVE: The most appropriate management of aortic stenosis (AS) in children remains controversial. The purpose of this study was to determine the outcome following open valvotomy for AS in children. METHODS: Ninety-seven consecutive, unselected, children (mean age 3.2 +/- 3.6 years, 1 day--15 years) underwent an open valvotomy for critical (n=36) or severe (n=61) AS between 1979 and 2000 in Southampton. Twenty-six were neonates (1--31 days), 27 were infants (1--12 months) and 44 were older children (1--15 years). Mean follow-up was 10 +/- 5.4 years, 1 month--21.9 years. RESULTS: Two neonates died early giving an overall operative mortality of 2.1% (7.7% for the neonates and 0% for infants and older children). The mean aortic gradient was reduced from 76 to 24.5 mmHg (P < 0.0001). Residual or recurrent AS occurred in 17 patients and severe aortic regurgitation in eight patients. Kaplan--Meier 10-year freedom from residual or recurrent AS was 83.1 +/- 4.7% and from severe aortic regurgitation was 95.3 +/- 2.7%. Twenty-five patients required an aortic re-operation or re-intervention, 18 of whom had an aortic valve replacement (AVR) (mean valve size 21.8 +/- 0.9 mm, range 21--25 mm). Ten-year freedom from any aortic re-operation or re-intervention was 78.4 +/- 5.2% and from AVR was 85.1 +/- 4.6%. There were ten late deaths. Overall 10-year survival, including hospital mortality, was 90.2 +/- 3.1% (69.7 +/- 9.7% for the neonates, 92 +/- 5.4% for the infants and 100% for older children, (P < 0.0001). Ten-year survival for children with isolated AS (n = 69) was 100% and for those with associated cardiovascular problems (n = 28) was 67.3 +/- 8.9% (P < 0.0001). All survivors are in New York Heart Association functional class I. CONCLUSIONS: Open valvotomy remains the gold standard in the management of AS in neonates, infants and older children. It is associated with low operative mortality and provides lengthy freedom from recurrent AS and regurgitation. Re-operations are common but if AVR is required, implantation of an adult-sized prosthesis is usually possible. There is a late death-hazard for those with severe associated lesions, but the survival prospects for the patients with isolated AS are excellent.     

Late results of aortic valvotomy for congenital valvar aortic stenosis

Fifty-one patients, aged 1 to 18 years, having aortic valvotomy for congenital valvar aortic stenosis between 1956 and 1986 were followed up. The average age at operation was 11.5 years, with an operative mortality of 3.9%. The aortic valve gradient decreased from a mean preoperative value of 91 mm Hg to 27 mm Hg postoperatively. Current follow-up was 90% and averaged 16.8 years. Late cardiac mortality was 17.6%, with actuarial survival of 93.7% at 10 and 15 years, 81.8% at 20 and 25 years, and 70.9% at 28 years. Nineteen patients required reoperation (39%) at a mean of 17.7 years postoperatively, with a reoperation-free survival of 98% at 10 years. The reoperation rate accelerated in the following decade to 3.3% per year. Ten patients without reoperation were evaluated by continuous-wave Doppler echocardiography. The mean gradient was 21.6 mm Hg, and 90% had mild to moderate aortic insufficiency. This study confirms the efficacy of valvotomy in this age group and suggests that long-term survival and time to reoperation may be longer than previously reported.     

Surgical spectrum of aortic stenosis in children: a thirty-year experience with 257 children

Aortic stenosis accounts for 5 to 6% of infants and children seen for surgical repair of congenital heart disease. The clinical presentation and reported results of operation for aortic stenosis are highly variable. This retrospective review was undertaken to assess our operative mortality and the degree of gradient reduction afforded by each of several surgical techniques used to treat aortic stenosis in children over a 30-year period. Two hundred fifty-seven patients ranging in age from 1 day to 19 years were operated on between 1957 and 1986. The indication for operation included asymptomatic patients with gradients greater than 50 mm Hg to patients in profound cardiogenic shock. The operative mortality for children older than 6 months was 4%, whereas neonates seen with critical aortic stenosis had a 60% mortality. The late mortality was 2%. Eighty percent of surviving patients to date have undergone cardiac catheterization after repair. This shows an overall reduction of 57 mm Hg in the left ventricular-aortic gradient. Patients with supravalvular aortic stenosis and discrete subvalvular aortic stenosis as well as patients undergoing aortic valve replacement showed a reduction in or elimination of associated aortic insufficiency, whereas patients undergoing aortic valvotomy or neonates having valvotomy had a significant increase in demonstrable aortic insufficiency. The incidence of third-degree heart block or cerebral emboli following operation for aortic stenosis was less than 1%. However, the incidence of late bacterial endocarditis following repair was nearly 5%; six of eleven cases occurred in the group with discrete subvalvular aortic stenosis. Twenty-nine (13%) of the 223 long-term survivors have undergone a subsequent procedure for relief of residual or recurrent obstruction; 12 have had insertion of an aortic valve prosthesis, 12 have had insertion of an apicoaortic conduit, and 6 have required repeat aortic valvotomy. These data demonstrate the low operative mortality and excellent hemodynamic benefit of surgical relief of single-level aortic stenosis in children older than neonates. Conduits placed for complex obstructions or operative procedures in neonates have acceptable hemodynamic benefits, but operative mortality remains high.     

Aortic valve debridement by ultrasonic surgical aspirator: a word of caution

Aortic stenosis was relieved in 11 patients by ultrasonic debridement of the valve and annulus, while 102 other patients underwent valve replacement for aortic stenosis during 1988. Debridement was selectively applied based on findings of small annulus size (19 mm or less) and extensive calcification. Additional patient characteristics were mean transvalvular gradient of 78 mm Hg, advanced age, and marked left ventricular hypertrophy. Six patients had no residual gradient and 5 others a mean gradient less than 10 mm Hg. There were no complications related to the debridement process. Intraoperative transesophageal Doppler echocardiography demonstrated improved leaflet mobility and elimination of the gradient in all patients and elimination of associated valvular insufficiency in 2 patients. Follow-up echocardiography demonstrated late onset of new valvular regurgitation in 5 patients that was progressive and required reoperation in 3. Thickened, hardened, and retracted valve leaflets with loss of central coaptation were found in all 3 patients who underwent reoperation. Ultrasonic debridement can effectively relieve aortic stenosis, provide an excellent immediate hemodynamic result, and decrease operative time. However, the early occurrence of aortic insufficiency in a high percentage of patients makes it an unacceptable alternative to valve replacement, and the technique should be abandoned as a treatment for severe calcific aortic stenosis.     

Surgical treatment of subaortic stenosis: a seventeen-year experience

OBJECTIVE: The aim of the study was to analyze the long-term results of subaortic stenosis relief and the risk factors associated with recurrence and reoperation. METHODS: One hundred sixty patients with subaortic stenosis underwent biventricular repair. Before the operation the mean left ventricle-aorta gradient was 80 +/- 35 mm Hg, 57 patients had aortic regurgitation, and 34 were in New York Heart Association functional class III or IV. Median age at repair was 10 years. For discrete subaortic stenosis (n = 120), 39 patients underwent isolated membranectomy, 67 underwent membranectomy with associated septal myotomy, and 14 underwent septal myectomy. Tunnel subaortic stenosis (n = 34) was treated by myotomy in 10 cases, myectomy in 12, septoplasty in 7, Konno procedure in 3, and apical conduit in 2. Aortic valve replacement was performed in 6 cases, mitral valve replacement in 2 cases, and mitral valvuloplasty in 4 cases. RESULTS: There were 5 early (3.1%) and 4 late (4.4%) deaths. Within 3.6 +/- 3.3 years a recurrent gradient greater than 30 mm Hg was found in 42 patients (27%), 20 of whom had 26 reoperations. According to multivariable Cox regression analysis survival was influenced by hypoplastic aortic anulus (P =.01) and mitral stenosis (P =.048); recurrence and reoperation were influenced by coarctation and immediate postoperative left ventricular outflow tract gradients. At a median follow-up of 13.3 years, mean left ventricle-aorta gradient was 20 +/- 13 mm Hg. Relief of the subaortic stenosis improved the degree of aortic regurgitation in 86% of patients with preoperative aortic regurgitation. Actuarial survival and freedom from reoperation rates at 15 years were 94% +/- 1.3% and 85% +/- 6%, respectively. CONCLUSION: Although surgical treatment provides good results, recurrence and reoperation are significantly influenced by previous coarctation repair and by the quality of initial relief of subaortic stenosis.     

Late Results and Reintervention After Aortic Valvotomy for Critical Aortic Stenosis in Neonates and Infants

Background. Many centers have adopted balloon valvuloplasty for treatment of infants with critical aortic stenosis because of historically poor early results and a lack of long-term results with surgical valvotomy. We evaluated our results with open aortic valvotomy over the past decade, specifically examining factors influencing survival and reintervention in the current era.

Methods. From 1986 to 1996, 37 infants in the first 3 months of life underwent open aortic valvotomy for critical aortic stenosis. All patients underwent cardiopulmonary bypass, valvotomy, and valve debridement under direct vision with standard techniques.

Results. Early mortality was 11% (4 of 37, 70% confidence limit 7% to 20%) and all early deaths were in neonates less than 2 weeks of age. Late death occurred in 6 patients a mean of 10 ± 12 months (range, 2 to 36 months) after valvotomy. Actuarial survival, including operative deaths was 92% ± 6% at 1 month, 78% ± 9% at 1 year, and 73.4% ± 10% at 10 years. In a multifactorial regression analysis, the best predictors of death were the presence of endocardial fibroelastosis and small body surface area and the best predictor of the need for late reintervention was preoperative aortic annular size. Thirteen patients required reintervention: repeat operation in 7 patients, balloon valvuloplasty in 3 patients, and both balloon valvuloplasty and reoperation in 3 patients. Actuarial freedom from reintervention postoperatively is 97% ± 3% at 1 month, 73% ± 9% at 1 year, and 55% ± 11% at 10 years. Reintervention was for recurrent left ventricular outflow obstruction in 9 patients and mixed aortic stenosis and aortic insufficiency in 4. Echocardiography 4.3 ± 2.5 years after aortic valvotomy in survivors who have not required reintervention (n = 20) revealed a Doppler peak instantaneous systolic gradient of 37 ± 14 mm Hg and mild or less aortic regurgitation in 16 patients and moderate aortic regurgitation in 4 patients.

Conclusions. Current surgical results with critical aortic stenosis in the neonate and young infant are acceptable in terms of both late survival, reintervention, and functional results in the majority of patients. Newer interventions, such as balloon valvuloplasty, should be carefully evaluated for long-term results and should be compared more appropriately to current surgical results to determine the best treatment modality for the neonate and infant with critical aortic stenosis.     

Surgery for the valvular disease in children

We report the results and long-term follow up in 34 children (17 girls and 17 boys, aged 12 days to 13 years, average age 3.3 years, average body weight 11.7 kg) who underwent valvular surgery in the period between May 1989 and November 1996. Operative mortality was 11.8%. Actuarial survival curves (including hospital mortality) indicate a 68.6% survival rate at 5 years and that 64.7% of patients are free from reoperation at 5 years. For aortic regurgitation two patients applied aortic valvuloplasty and four applied aortic valve replacement. Nine children had aortic stenosis, three of them had balloon valvuloplasty, seven had valvotomy, two had aortic valve replacement. Ten patients were treated for mitral regurgitation. There were nine valvuloplasty and four mitral valve replacement including three times of reoperation. One membranous pulmonary atresia and seven pulmonary stenosis children had valvotomy. There were four cases of tricuspid disease. One had tricuspid valve stenosis with pulmonary stenosis, three had severe tricuspid regurgitation who applied tricuspid valve replacement. Mortality was high in the critical AS, severe MR and TVR groups. Patients who survived the surgery and had no complications showed satisfiable results.     

Aortic valvotomy for congenital valvular aortic stenosis: a 37-year experience

BACKGROUND: The purpose of the study was to analyze the long-term results of aortic valvotomy and the risk factors associated with reoperation and survival. METHODS: From 1960 to 1977, 116 patients with congenital valvular aortic stenosis underwent isolated aortic valvotomy at a mean age of 13.7 +/- 7.8 years with a mean aortic gradient of 78 +/- 33 mm Hg. Fifteen patients had additional aortic regurgitation, and leaflet calcification was present in another 15 patients. RESULTS: Postoperatively the mean aortic gradient decreased to 19.4 +/- 11.3 mm Hg (p < 0.0001). Early mortality was 2.6%. At a mean follow-up of 23.8 years, 26 late deaths (22.4%) occurred among the 113 early survivors. Actuarial 10-, 20-, 30-, and 37-year survival rates were 94.6%, 79.7%, 76.2%, and 72.5%, respectively. According to multivariate Cox regression analysis, survival was influenced by preoperative New York Heart Association class (p = 0.0418), leaflet calcification (p = 0.0339), date of operation (p = 0.0253), and postoperative endocarditis (p < 0.0001). At a mean interval of 18.3 years, 37 patients required reoperation (31.9%) mainly because of recurrent aortic stenosis. The reoperation rate increased significantly 15 years postoperatively from 0.73%/year to 2.31%/ year (p < 0.0001). In a multivariate risk model, reoperation was influenced by older patient age (p = 0.0032) and the presence of leaflet calcification (p = 0.0289). CONCLUSIONS: AORTIC valvotomy is a simple and effective procedure for congenital aortic stenosis with excellent long-term results. However, the rate of reoperation increases 15 years postoperatively, and clinical follow-up should be intensified. Our results suggest that early repair should be performed and that adequate patient selection is the most important determinant of the longterm results.     

Critical aortic stenosis. Survival and management

The factors associated with survival in 40 neonates (age less than 28 days) with critical aortic stenosis undergoing either open (22 patients) or closed (18 patients) transventricular aortic valvotomy were reviewed. Significant adverse correlates with survival included evidence of poor perfusion preoperatively (low pH, greater than Grade 2/6 soft ejection systolic murmur) and marked congestive heart failure (hepatomegaly, cardiomegaly, elevated left atrial pressure). Congenital mitral stenosis (anulus less than 11 mm), a small aortic anulus (less than 6.5 mm), and failure to achieve an adequate aortic orifice (greater than 6 mm), at operation were identified as factors associated with increased mortality. Initial perioperative survival was better with closed aortic valvotomy. However, there was no significant difference in overall operative survival between closed (9/18, 50%) and open (8/22, 36%) aortic valvotomy (p = 0.26). The incidence of early reoperation (less than 1 year of age) was greater in perioperative survivors undergoing closed valvotomy (7/13, 54%) rather than open valvotomy (1/10, 10%) (p less than 0.05). In conclusion, long-term survival among patients with critical neonatal aortic valve stenosis remains disturbingly low (13/40, 32%) and has not significantly improved over the past 20 years.     

Surgery for aortic stenosis in children: a 40-year experience

BACKGROUND: Aortic stenosis (AS) is encountered in approximately 5% of children with heart disease. The indications for surgery and the surgical techniques for AS are well established. This report focuses on the early and long-term outcomes in children with AS over a 40-year period. METHODS: Included in this study were 508 patients ranging in ages from 1 day to 19 years, who were operated on for AS between 1960 and 2002. Eighty-one percent (414 of 508) of the patients had left ventricular outflow tract obstruction (LVOTO) at a single level: 40 supravalvar, 242 valvar (critical AS in 85 neonates and young infants and in 157 older children), and 132 subvalvar. Nineteen percent (94 of 508) of the patients had LVOTO at more than one level. Associated congenital cardiac defects were found in 32% of the patients. RESULTS: The overall hospital mortality rate was 8% (40/508) with neonates with critical AS having the highest mortality (33%). The late mortality was 4% for the entire group. Follow-up was 95% complete. The mean follow-up was 8.5 +/- 7.1 years. In the subgroup with multilevel LVOTO (n = 94), the average intraoperative peak systolic left ventricular-aortic gradient decreased from 80 to 22 mm Hg after repair but increased progressively to 74 +/- 36 mm Hg (p < 0.05) before reintervention was required. One hundred twenty-one patients (24%) underwent 151 reoperations for recurrent or residual LVOTO or aortic regurgitation. Actuarial curves predict a 20-year survival of 88% and 62% freedom from reoperation for all patients with AS. Symptomatic improvement in survivors was excellent (90% New York Heart Association class I). CONCLUSIONS: Surgical relief of LVOTO in infants and children can be accomplished with low mortality and morbidity. Neonates with critical AS have significantly higher mortality and morbidity due to their complex anatomy and their critical presentation that affects outcome. Aortic valvotomy delays valve replacement in a significant percentage of children. The Ross procedure and mechanical aortic valve replacements have had a low mortality and morbidity in our series. Valve replacement will eventually be required in most children presenting with valvar AS and multilevel LVOTO while repair of discrete subaortic stenosis and supravalvar AS may not require reoperation in most patients. Children with LVOTO should have lifetime follow-up.     

Open mitral commissurotomy in the current era: indications,technique, and results

BACKGROUND: The present retrospective study is focused on indications, techniques, and results of open mitral commisurotomy in the current era. METHODS: Of the 1,280 patients undergoing open-heart surgical procedures for rheumatic mitral stenosis between January 1990 and July 2000, 276 (21.6%) patients underwent open mitral commissurotomy. Major indications included presence of left atrial thrombus/clot (n = 82, 29.7%), severe subvalvular disease (n = 110, 39.8%), mitral valve calcification (n = 42, 15.2%), mild mitral regurgitation (n = 28, 10.0%), associated aortic valve disease (n = 55, 19.9%), organic tricuspid valve disease (n = 20, 7.2%), and failure or restenosis after closed or balloon mitral valvuloplasty (n = 55, 19.9%). Age of patients ranged from 7 to 67 years (mean, 30.2 +/- 12 years). The majority (76%) were in New York Heart Association class III or IV, and 6.9% were in congestive heart failure. Atrial fibrillation was present in 134 (48.6%) patients. Mitral valve area ranged from 0.3 to 0.7 cm2 (mean, 0.52 +/- 0.12 cm2). Mid-diastolic gradients across the mitral valve ranged from 8 to 34 mm Hg (mean, 14.5 +/- 6.2 mm Hg), and end-diastolic gradients ranged from 8 to 42 mm Hg (mean, 15.2 +/- 5.7 mm Hg). Open mitral commissurotomy was performed using standard cardiopulmonary bypass. Associated aortic valve procedure was performed in 55 patients, and either tricuspid valvotomy or repair was performed in 28 patients. RESULTS: There were four early deaths. All these patients had associated aortic valve procedure (Ross procedure in 2 and homograft aortic valve replacement in 2). Three patients developed severe mitral regurgitation in early postoperative period (< or = 30 days) and required reoperation. Predischarge echocardiography showed mitral valve area from 1.4 to 3.5 cm2 (mean, 2.6 +/- 0.6cm2) and moderate mitral regurgitation in 4 patients. Follow-up ranged from 1 to 130 months (mean, 64.5 +/- 28.6 months). There was no late death. There were three reoperations for mitral valve failure, and an additional 2 patients developed severe mitral stenosis (mitral valve area < 1.0 cm2). In operative survivors, freedom from mitral valve failure at 10 years was 87.0% +/- 3.5%. In patients with isolated open mitral commissurotomy, the incidence of thromboembolism was 0.5%/patient-year. CONCLUSIONS: Open mitral commissurotomy provides excellent early and long-term results in a selected group of patients.     

Twenty years experience of surgical aortic valvotomy for critical aortic stenosis in early infancy.

OBJECTIVE: To examine early and long-term results of surgical aortic valvotomy in neonates and infants aged less than 3 months. METHODS: A review of all 34 neonates (n=26) and young infants (n=8) aged 1-62 days undergoing primary open valvotomy for aortic valve stenosis between 1983 and 2003 was carried out. Associated major cardiac anomalies were endocardial fibroelastasis (n=8), aortic coarctation (n=3), subvalvular aortic stenosis (n=2), and ventricular septal defect (n=1). Risk factors for early mortality were estimated. Current information was available for 31 patients for a follow-up of 115+/-67 months. Kaplan-Meier method was used to estimate freedom from reintervention. RESULTS: Two neonates died early: operative mortality of 6% (2/34). Risk factors for early mortality were associated endocardial fibroelastosis, monocuspid aortic valve and impaired left ventricular function. No patient died late. Seven patients needed reintervention for re-aortic stenosis (n=5) or aortic insufficiency (n=2), i.e., re-valvotomy (n=3), valve replacement (n=2), Ross procedure (n=1), and balloon valvuloplasty (n=1). Freedom from reintervention was 85.1+/-6.9%, 78.0+/-9.35%, and 53.5+/-15.9% at 5, 10, and 15 years, respectively. CONCLUSIONS: Primary surgical aortic valvotomy in early infancy carries a low early and late mortality, a low occurrence of significant aortic regurgitation and a low early recurrence of aortic stenosis. In great majority of cases, reintervention can be delayed to allow implantation of an adult-sized prosthesis, when required.     

Surgical management of congenital aortic stenosis in children and young adults

The surgical management of congenital aortic stenosis in 33 patients (age 2-20, mean 11 years) is described. The stenosis was valvular in 19 cases, subvalvular in eight and supravalvular in six. The primary procedures in valvular stenosis were valvotomy (12 cases), valve replacement (6) and exploration (1). Subvalvular stenosis was treated with resection of muscle (4), membrane (2) or both (2), and all six supravalvular lesions with patch aortoplasty. One death occurred during primary operation. Follow-up averaged 11.5 years. Of the 12 valvotomized patients, five required valve replacement after on average 10.6 years because of restenosis. Aortic regurgitation developed in six of the seven surviving patients treated for subvalvular stenosis, requiring reoperation in four. No mortality was associated with reoperation. All patients without valve replacement were reinvestigated. Of the seven with valvotomy, four had restenosis and three valves were in good condition. In supravalvular stenosis the gradients were low. Valvotomy and subvalvular resection can give effective palliation of aortic stenosis until the patient is old enough for definitive repair.     

Aortic valvotomy in the treatment of congenital aortic stenosis

Forty patients aged 1.5-23 (mean 11) years underwent open aortic valvotomy for congenital aortic stenosis. Mean preoperative peak aortic gradient for the group was 88 (SD +/- 24) mmHg. Follow-up data was available on all patients for a total of 269 patient-years (means 6.6 year). There was no early mortality, and only one (2.5%) late death. During the follow-up period 3 patients (7.5%) required aortic valve replacement. Actuarial survival curves predict a 7 year survival of 95.7 (SE +/- 8.4%) for all patients, and a freedom from reoperation rate at 6 years of 8.7 (SE +/- 9.3)%. Symptomatic results in the survivors were excellent (97.5% NYHA Class I). Data from this series compares favourably with the literature which is reviewed for a total of 1136 patients undergoing open aortic valvotomy.     

Surgical aortic valvotomy in infancy: impact of leaflet morphology on long-term outcomes

BACKGROUND: Surgical valvotomy for critical aortic stenosis in children enables relatively accurate commissurotomies to be fashioned, resulting in the formation of two or three leaflets. We hypothesized that outcomes after surgery may be best in patients in whom three leaflets are produced. METHODS: A retrospective review of infants undergoing primary surgical valvotomy at our institution during a 12-year period was carried out. Patients who had additional intracardiac defects were excluded. Clinical and echocardiographic follow-up data were analyzed. RESULTS: Fifty-four patients fulfilled the study criteria. Median age at surgery was 3 weeks (range, 0 to 51 weeks). Commissurotomy resulted in bileaflet anatomy in 41 patients (group A) and trileaflet anatomy in 13 patients (group B). Operative mortality was 5% in group A and 0% in group B (p = 1.0). In group A, 18 patients required one or more aortic valve reinterventions, including valve replacement in 8 patients. In group B, there was only one reintervention (repeat valvotomy). Kaplan-Meier analysis showed that at 10 years, comparisons of group A versus group B were as follows: actuarial survival, 85% versus 100% (p = 0.15); freedom from reintervention, 33% versus 92% (p = 0.01); freedom from aortic reoperation, 45% versus 92% (p = 0.04); and freedom from aortic valve replacement, 57% versus 100% (p = 0.07). CONCLUSIONS: Long-term outcomes after aortic valvotomy are significantly better in infants in whom surgery results in trileaflet rather than bileaflet anatomy. Preoperative evaluation of valve morphology may enable selection of a group of patients in whom results of surgery are excellent.     

Successful repair of critical aortic stenosis with coarctation on the first day of life

Successful open aortic valvotomy and end-to-end anastomosis were performed to the patient with critical aortic stenosis and CoA on the first day of life. A fetus was diagnosed as aortic stenosis and coarctation by fetal echocardiography at 29 weeks of gestation. The mother was transferred to our hospital at the onset of labor and delivered vaginally at 40 weeks of gestation. Soon after the birth, the newborn, birth weight 2630 gram, female, underwent echocardiography by pediatric cardiologists which demonstrated the aortic orifice of 5.1 mm in diameter and thickened cusps with poor mobility. Transaortic pressure gradient measured by Doppler echocardiography was 111 mmHg and the left ventricular wall motion was hyperdynamic without any signs of endocardial fibroelastosis. Prostaglandin E₁ administration was started to maintain systemic circulation and the hemodynamic status has been stable before surgery. On her first day of life, the operation was performed using cardiopulmonary bypass with moderate hypothermia. Isolated cerebral and myocardial perfusion technique was applied during the repair of coarctation of the aorta. The open aortic valvotomy with resection of myoxomatous nodules on leaflet edges effectively released pressure gradient across the aortic valve without regurgitation. Post-operative course was uneventful and she discharged at 28th day after surgery. We conclude early diagnosis including fetal echocardiography and early repair would lead the better surgical outcome to the neonates with critical aortic stenosis.     

Unexpected durability of Smeloff-Cutter aortic ball valve prosthesis

We report a case in which replacement of a Smeloff-Cutter aortic ball prosthesis was required 28 years after initial implantation. A 57-year-old woman underwent aortic valve replacement with a 21-mm Smeloff-Cutter ball prosthesis and open mitral commissurotomy for aortic stenosis, aortic regurgitation, and mitral stenosis in 1973. Severe aortic regurgitation occurred in April 2001, and aortic valve reoperation combined with mitral valve replacement was successfully performed. The patient's aortic ball valve was nearly intact with perivalvular leakage probably causing the aortic regurgitation. Our experience documents longer durability for the Smeloff-Cutter prosthesis than has been reported to date.     

Neonatal aortic stenosis

Aortic stenosis in the neonate has been associated in the past with a high operative mortality. As a result, in the current era of percutaneous balloon dilatation, the optimal mode of therapy remains controversial. An approach of stabilization with cardiopulmonary bypass, followed by relief of left ventricular outflow tract obstruction, was used at three institutions, and the results are presented. During the period 1983 to 1989, 40 neonates with isolated aortic stenosis and patent ductus arteriosus or coarctation of the aorta, or both, underwent operative therapy. Ages ranged from 1 to 30 days, median of 12 days, including 17 patients in the first week of life. There were 30 boys and 10 girls; weights ranged from 2.5 to 5.5 kg with a mean of 3.6 kg. Perioperative conditions included congestive heart failure in 38 and mitral regurgitation in 16; left ventricular-aortic gradients ranged from 15 to 130 mm Hg, with a mean of 67 mm Hg. There were 30 open valvotomies and 10 transventricular dilatations. The hospital survival rate was 87.5% (35/40) with no significant difference between the methods of valvotomy (9/10 in the transventricular dilatation group, 90%; 26/30 in the open valvotomy group, 87%). Although multiple methods of perfusion and valvotomy were used, the single unifying factor of cardiopulmonary bypass stabilization was present in all 40 patients. No significant difference in survival was noted between institutions, methods of cardiopulmonary bypass, cardiopulmonary bypass times, crossclamp times, or method of valvotomy. There have been five reoperations, with one late death in a patient requiring mitral valve replacement and an apical-aortic conduit. One sudden death occurred; autopsy revealed endocardial fibroelastosis. Results demonstrate that in the three institutions using the methods described, a high operative and late survival rate is possible. The results of this technique, against which percutaneous dilatation should be compared, are standard in the current era.     

Surgical management of congenital aortic valve stenosis in neonates and infants

From 1985 to 1989, 4 neonates or infants (3 males and 1 female) underwent open valvotomy for severe aortic valve stenosis. In all patients, preoperative echocardiograms showed abnormal findings of endocardial fibroelastosis and/or poor performance of left ventricle. All patients underwent valvotomy using cardiopulmonary bypass. There was one early death from left ventricular failure. All other patients survived and the pressure gradient through aortic valve was reduced from 50-100 mmHg to 25-50 mmHg postoperatively. We conclude that infants with severe aortic valve stenosis should undergo open valvotomy before the manifestation of endocardial fibroelastosis and/or left ventricular muscle damage, and that open aortic valvotomy using cardiopulmonary bypass is most suitable method for severe aortic valve stenosis in infants.     

Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children

BACKGROUND: We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. METHODS: Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 +/- 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 +/- 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. RESULTS: Mean pressure gradient decreased significantly more in the surgical group (from 64.8 +/- 30.8 mm Hg to 12.8 +/- 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 +/- 21.4 mm Hg to 21.5 +/- 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV (p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty (p < 0.04). CONCLUSIONS: Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.