Long-term Outcomes After Surgical Intervention for Congenital Supravalvar Aortic Stenosis in Children.

BACKGROUND: Primary supravalvar aortic stenosis (SVAS) is a rare congenital cardiovascular condition that can coexist with Williams-Beuren syndrome, coronary artery involvement, aortic coarctation, and pulmonary artery stenosis. SVAS repair can be achieved with low perioperative mortality, but long-term survival remains less well understood. We used the Pediatric Cardiac Care Consortium, a multicenter United States-based registry for pediatric cardiac operations, to assess long-term outcomes after SVAS repair. METHODS: We used Kaplan-Meier plots and Cox proportional hazards regression to examine factors associated with postdischarge deaths. These included sex, age-group, weight z-score, coexisting conditions (Williams-Beuren syndrome, coronary artery involvement, coarctation, and pulmonary artery stenosis), surgical techniques, and era, defined as early (1982-1995) or late (1996-2003). Survival was assessed by matching with the National Death Index through 2021. RESULTS: Of 333 patients who met inclusion criteria, 313 (94.0%) survived to discharge and 188 (60.1%) had identifiers for National Death Index matching. Over a median follow-up of 25.2 years (interquartile range, 21.1-29.4 years), 17 deaths occurred. The 30-year survival after discharge from SVAS repair was 88.7% (95% CI, 82.9%-94.8%). Infantile surgery and non-Williams-Beuren syndrome were associated with decreased 30-year survival. From the various repairs, the 2-sinus technique had better outcomes compared with all other types, except the 3-sinus technique (nonsignificant difference). Adjusted analysis revealed infantile age and type of repair as associated with postdischarge probability of death. CONCLUSIONS: These data demonstrate favorable long-term outcomes after SVAS repair, except for the infantile group that was associated with more diffuse arteriopathy. As techniques continue to evolve, future studies are warranted to investigate their long-term outcomes.

Surgical Techniques in Management of Supravalvular Aortic Stenosis in Children.

BACKGROUND: Multiple techniques exist for the repair of supravalvular aortic stenosis (SVAS), but given the lesion's rarity, analyses comparing the efficacy of each repair have been limited. METHODS: A retrospective review of all children at a single institution who underwent repair of SVAS from June 1995 to May 2019 was performed. Anatomic and physiologic measurements across time points were compared between 2 predominant surgical techniques. Time-to-event outcomes were compared using the log-rank test. RESULTS: SVAS was repaired in 89 patients, by using a single-patch in 31 (35%) and the Doty repair in 58 (65%). Median age at operation was 2.5 years (interquartile range [IQR], 1.0 to 6.8 years), with median follow-up of 5.8 years (IQR, 1.8 to 10.7 years). Reoperation was required in 8 (9%) patients at a median of 1.5 years postoperatively (IQR, 0.3 to 4.8 years). There was 1 death after multiple reinterventions. The change from the preoperative to the postoperative sinotubular junction z-score was greater for patients after Doty repair (median change +2.5; IQR, 1.5, 4.1) than for patients after single-patch repair (median change +0.8; IQR, -0.1, 2.1; P = .001). Freedom from reoperation was longer for patients after Doty repair than after the single-patch technique (P = .008). CONCLUSIONS: The Doty repair provides longer freedom from reoperation after supravalvular aortic stenosis repair compared with a single-patch technique, likely through a greater increase in the sinotubular junction at the time of initial operation.

Long-term Surgical Prognosis of Primary Supravalvular Aortic Stenosis Repair.

BACKGROUND: Supravalvular aortic stenosis (SVAS) represents a heterogeneous group, including Williams syndrome, familial elastin arteriopathy, sporadic cases, and others. This study sought to evaluate long-term outcomes of SVAS repair. METHODS: A total of 87 patients underwent surgical repair of congenital SVAS at Boston Children's Hospital in Boston, Massachusetts, between 1997 and 2017. A total of 41 patients had Williams syndrome, and 46 did not. Of the 46 patients who did not have Williams syndrome, 23 had sporadic SVAS, and 13 had familial elastin arteriopathy. Demographic data and outcomes were reviewed and analyzed from medical records. RESULTS: The median age at operation was 2.9 years. Mean z score of the sinotubular junction was -3.29 ± 1.42 and of the aortic root was -0.09 ± 1.19. A total of 26% (n = 22) patients had coronary ostium stenosis, and 41% (n = 9) of them required patch plasty. Survival rates at 5, 10, and 20 years were all 94.3%. Freedom from left ventricular outflow tract reoperation at 5, 10, and 20 years was 78.5%, 70.3%, and 70.3%, respectively. Freedom from aortic arch reintervention at 5, 10, and 20 years was 98.6%, 94.3%, 89.3%, respectively. In risk factors analysis, age younger than 1 year, z scores of the aortic valve and aortic root, and concomitant right ventricular outflow tract surgical repair were predictive of the need for reoperation and reintervention for left or right ventricular outflow tract obstruction. CONCLUSIONS: Excellent long-term survival rates can be achieved with surgical repair of SVAS. Age younger than 1 year, small aortic valve and aortic root z scores, and concomitant right ventricular outflow tract surgical repair were predictors of reoperation and reintervention.

Early and mid-term outcomes after surgical repair of congenital supravalvular aortic stenosis with the Doty technique.

OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of the Doty surgical approach in pediatric patients with congenital supravalvular aortic stenosis (SVAS) by examining early and mid-term outcomes. METHODS: Surgical repair using the Doty technique was performed in a total of 10 pediatric patients with SVAS between January 2005 and July 2015 at this clinic. These patients were evaluated retrospectively. Demographic characteristics, echocardiographic findings, and clinical outcomes were analyzed. RESULTS: The mean patient age was 4.8±3.9 years. Williams-Beuren syndrome was diagnosed in 4 of the patients. Early mortality was observed in 1 patient with diffuse SVAS. At the final follow-up (mean follow-up: 3.7±1.2 years; range: 6-61 months), echocardiograms revealed a mean pressure gradient of 14±4.2 mmHg. Two patients displayed minimal aortic insufficiency during the follow-up period. No reoperation or reintervention was required. CONCLUSION: The Doty technique is an anatomically and technically effective surgical approach to treating SVAS.

Surgical Correction of Supravalvar Aortic Stenosis: 52 Years' Experience.

OBJECTIVES: Supravalvar aortic stenosis (SVAS) is a rare congenital anomaly. The "single-patch technique," "'two sinus augmentation with an inverted Y-patch" (both nonsymmetrical corrections), "three-patch technique," and the "slide aortoplasty" (both symmetrical corrections) are the techniques implemented by the majority of surgeons for the correction of SVAS. In the few studies that compared these techniques, no technique was shown to be superior over another. The aim of the present study is to review the 52-year experience with the surgical correction of SVAS in two of four congenital cardiothoracic surgical centers in the Netherlands. METHODS: We retrospectively reviewed all patient files of those who underwent an operation to correct their SVAS, between 1962 and 2014 in our centers. Patients were divided according to their operating technique. These groups were compared using the end points freedom from reoperation and mortality. RESULTS: A total of 49 patients were included, 23 (46.9%) patients in the nonsymmetrical group and 26 (53.1%) patients in the symmetrical group. Survival after 20 years in the nonsymmetrical group was 80% (standard error [SE]: 0.091) and in the symmetrical group was 85% (SE: 0.085; P = .163). Freedom from reoperation after 20 years in the nonsymmetrical group was 88% (SE: 0.079) and in the symmetrical group was 71% (SE: 0.107; P = 0.313). CONCLUSION: In this patient group, there is no significant difference in survival and freedom from reoperation between the different surgical techniques for SVAS repair. Compared to the survival in the general population, the survival of SVAS patients is remarkably low. Apparently, SVAS is not a benign disease and probably patients should be followed more closely for the rest of their lives.

Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.

OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.

[Reintervention with percutaneous balloon angioplasty in patients with congenital heart disease with left-sided obstructions]. / Reintervención con angioplastía percutánea con balón en enfermos con cardiopatías congénitas con obstrucciones izquierdas.

BACKGROUND: Left-sided cardiac obstructions represent 15% of congenital heart disease (CHD). The treatment in adults is surgical; however, balloon dilation by interventional catheterization can alleviate the symptoms in pediatric patients to allow them to reach the target height. The aim was to determine the survival and the factors associated with reintervention in patients with CHD with left-sided obstruction treated with balloon angioplasty. METHODS: A cohort study was conducted in patients aged 4 to 17 years with left-sided heart obstruction (valvular stenosis [VS], supravalvular aortic stenosis [SAS], coarctation of the aorta [CA]) successfully treated with balloon angioplasty. The follow-up was of 10 years and the outcome variable was the restenosis with reintervention criteria. Pediatric stage at the time of the procedure, nutritional status, residual gradient, and presence of genetic syndromes were considered prognostic variables. For statistical analysis, measures of central tendency and dispersion were used. Chi squared was employed in qualitative variables and Kruskal-Wallis in quantitative variables. RESULTS: We had a total of 110 patients: 40% had CA, 35% VS, and 25% SAS. 39% required reintervention: 80% in SAS, 35% in CA, and 14% in VS. CONCLUSION: The intervention balloon is a stopgap measure that allows patients with left-sided obstructions to reach the target height.

Introducción: las obstrucciones izquierdas representan 15% de las cardiopatías congénitas (CC). El tratamiento en la edad adulta es quirúrgico; no obstante, la dilatación con balón puede paliar los síntomas en edad pediátrica para permitir que alcancen la talla diana. El objetivo fue determinar la sobrevida y los factores asociados a la reintervención en enfermos con CC con obstrucción izquierda tratados con plastía con balón. Métodos: se realizó un estudio de cohorte en pacientes entre 4 y 17 años con cardiopatías de obstrucción izquierda (estenosis valvular, supravalvular y coartación aórtica) tratados con angioplastía con balón exitosa. El seguimiento fue de 10 años y la variable de desenlace fue la reestenosis con criterios para reintervención. Se consideraron variables pronósticas la etapa pediátrica al momento del procedimiento, el estado nutricional, el gradiente residual y la presencia de síndromes genéticos. Se usaron medidas de tendencia central y dispersión. Se usó chi cuadrada en variables cualitativas y Kruskal-Wallis en variables cuantitativas. Resultados: fueron 110 pacientes; 40% presentó coartación aórtica, 35% estenosis valvular y 25% estenosis supravalvular aórtica. El 39% requirió reintervención y la frecuencia fue la siguiente: 80% en estenosis supravalvular, 35% en coartación aórtica y 14% en estenosis valvular aórtica. Conclusión: la intervención con balón es una medida paliativa que permite a los enfermos con obstrucciones izquierdas alcanzar la talla diana.

Surgical repair of supravalvular aortic stenosis in children with williams syndrome: a 30-year experience.

BACKGROUND: Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. METHODS: Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). RESULTS: A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. CONCLUSIONS: Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up.

Late outcomes for surgical repair of supravalvar aortic stenosis.

BACKGROUND: We reviewed our experience with the surgical management of supravalvar aortic stenosis (SVAS) to determine long-term outcomes and factors related to late reoperation. METHODS: Between August 1956 and May 2009, 78 patients (50 males) underwent surgical correction of SVAS. Median age was 10.4 years (range, 16 days to 55.2 years). Mean preoperative gradient was 57.2±21.9 mm Hg with a mean peak gradient of 99.5±34.8 mm Hg. Supravalvar aortic stenosis was discrete in 51 patients (64%) and diffuse in 27 patients (35%). Aortic valve stenosis was present in 22 patients (29%). Williams-Beuren syndrome was present in 32 patients (41%). RESULTS: Surgery was either a diamond-shaped patch in 67 patients (85.9%) or a pantaloons-shaped patch in 11 patients (14.1%). Aortic valve intervention was required in 20 patients (25.64%). Mean gradient immediately after repair was 25±25 mm Hg, with 13 patients (16.7%) having a residual gradient. A high residual gradient was more likely in the diffuse group (odds ratio, 3.73; 95% confidence interval, 1.07 to 12.98). There were 2 (2.6%) early deaths, both with diffuse SVAS. Median follow-up was 19.8 years; maximum was 48.5 years. The mean gradient across the left ventricular outflow tract at late follow-up was 8.8 mm Hg (95% confidence interval, 3.7 to 14.01). Overall survival was estimated at 90%±7%, 84%±9%, and 8%2±10% at 5, 10, and 20 years, respectively. Predictors of mortality were age younger than 2 years (p=0.021), diffuse SVAS (p=0.045), aortic valve stenosis (p=0.032), and high postoperative gradient (p=0.023). Presence of Williams-Beuren syndrome did not affect survival (p=0.305). Freedom from late reoperation was 97%±4%, 93%±7%, and 86%±10% at 5, 10, and 20 years, respectively. Significant aortic valve disease (p<0.001) and diffuse SVAS (p=0.009) were risk factors for late reoperation. CONCLUSIONS: Surgical repair for SVAS can be performed with a single-patch technique with good long-term outcome. Late mortality and need for reoperation are more likely with diffuse SVAS or the presence of aortic valve stenosis.

Pulmonary stenosis is a predictor of unfavorable outcome after surgery for supravalvular aortic stenosis.

We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100 %) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65 %) were diagnosed with Williams-Beuren-Syndrome, six (17 %) had a diffuse form of SVAS and 10 (39 %) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p = 0.30) or diffuse form of SVAS (p = 0.13). Patients with PS were operated at younger age (p = 0.028). Median follow-up time was 14.6 years. Overall mortality was 11.5 %. One patient with preoperatively severely decreased LV-function died 27 days postoperatively. Two late deaths occurred 7 and 10 years after the initial operation. Reoperations were required in 4 patients (15 %), 4-19 years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p = 0.005) and for the combined reoperation/death end-point (p = 0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.

Reoperation after supravalvular aortic stenosis repair.

BACKGROUND: Supravalvular aortic stenosis (SVAS) is the rarest type of left ventricular outflow tract obstruction. We reviewed our experience with this anomaly and analyzed risk factors for death or reoperation. METHODS: Between 1984 and 2009, 49 patients had surgery for SVAS. A single-patch technique was used in 3, two-sinus enlargement in 39, and three-sinus enlargement in 7. Variables evaluated included age at surgery (<2 versus >2 years old), presence of pulmonary artery stenosis, type of SVAS (focal versus diffuse), presence of valvular aortic stenosis, and era of surgery. RESULTS: The only early death occurred in a patient who experienced cardiac arrest during anesthesia induction and could not be separated from bypass after surgery. There were 2 late deaths at 3 and 11 years after SVAS repair, both related to treatment for pulmonary artery stenosis. Actuarial survival at 5, 10, and 20 years was 95%, 95%, and 90%, respectively. Sixteen patients required 23 reoperations: for pulmonary artery stenosis (n = 10), distal aortic stenosis (n = 9), aortic valve stenosis (n = 4), and coronary artery stenosis (n = 1). Actuarial reoperation-free survivals at 5, 10, and 20 years were 73%, 58%, and 52%, respectively. Coexistent pulmonary artery stenosis, young age at surgery, and diffuse type SVAS were predictors of lower freedom from death or reoperation by both univariate and multivariate analyses. CONCLUSIONS: Survival after surgical repair of SVAS is excellent. However, reoperation is frequent, especially when the patients also have pulmonary artery stenosis, diffuse type SVAS, and initial surgery at a young age.

Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.

BACKGROUND: Surgical techniques for repair of supravalvular aortic stenosis (SVAS) include McGoon's one-patch, Doty's two-patch, and Brom's three-patch method. In this review we evaluated mid-term clinical outcomes of these techniques at our institution. METHODS: Our cardiac surgery database identified patients with SVAS repair from 1990 to 2008. Follow-up records, reintervention and reoperation data, and most recent echocardiograms were obtained. RESULTS: From 1990 to 2008, 20 patients (70% male) underwent surgery for SVAS. Mean age was 3.6 +/- 5.6 years. In chronological sequence, 8 patients had single-patch aortoplasty, 4 had the Doty procedure, and 8 received Brom's symmetric three-patch aortoplasty. Of the Brom patients, 6 had Williams syndrome. Aortic cross-clamp times were 40.1 +/- 13.6 minutes (one-patch), 60.3 +/- 38.8 minutes (Doty), and 104 +/- 20.5 minutes (Brom). Perioperative mortality was 5.0% (1 patient in one-patch group). Mean postoperative length of stay was 10 +/- 10.6 days. Follow-up data were available for all survivors (mean follow-up, 6.3 +/- 6.0 years; range, 6 months to 16 years). There were no late deaths. Follow-up echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 33 +/- 18.0 mm Hg (one-patch), 10 +/- 1 mm Hg (Doty), and 18 +/- 12 mm Hg (Brom). All patients in the Doty and Brom groups had less than moderate aortic insufficiency. Reoperations were required in 5 of 8 one-patch patients (62%) for residual aortic stenosis (n = 3), aortic insufficiency (n = 1), and subvalvar stenosis (n = 1). No Doty or Brom patient has required aortic reoperations, which was nearly statistically associated with freedom from reoperation (p = 0.06). Subvalvar stenosis was the only risk factor associated with reoperation (p = 0.0028). CONCLUSIONS: Despite a longer cross-clamp time, SVAS repair by Doty or Brom aortoplasty restores normal hemodynamics and reduces the need for reoperation when compared with the classic one-patch technique. Our current preference for SVAS repair is the Brom three-patch symmetric aortoplasty.

Predictors of survival after aortic valve replacement in patients with low-flow and high-gradient aortic stenosis.

AIMS: To identify predictors of survival following aortic valve replacement (AVR) in patients with low-flow and high-gradient aortic stenosis (AS). METHODS AND RESULTS: Eighty-six patients (aged 71 +/- 10 years) with severe AS [aortic valve mean pressure gradient >40 mmHg or valve area <1.0 cm(2)] and left ventricular (LV) dysfunction [ejection fraction (EF) <50%] underwent AVR. Cox proportional hazards were used to identify independent clinical and echocardiographic predictors of mortality. Operative (30-day) mortality was 10%. Peri-operative mortality was associated with lower mean LVEF, higher mitral E:A ratio, peak systolic pulmonary artery pressure (PSPAP), and serum creatinine (by 12%, 2.3, 28 mmHg, and 74 mmol/L, respectively, all P < 0.001), NYHA class III-IV (100 vs. 65%), concomitant CABG (89 vs. 55%), urgent surgery (78 vs. 35%), and longer bypass-time (by 28 min, all P < 0.05). Mortality at 4 years was 17%. Univariate predictors [hazard ratio (HR)] of 4-year mortality were: lower EF (HR 0.68 per % increase, P < 0.001), presence of restrictive LV filling (HR: 3.52, P < 0.001), raised PSPAP (HR: 1.07, P < 0.001), and CABG (HR: 4.93, P = 0.037). However, only low EF (<40%, HR 0.74, P = 0.030), the presence of restrictive filling (HR 1.77, P = 0.033), and raised PSPAP (>45 mmHg, HR 2.71, P = 0.010) remained as independent predictors after multivariate analysis. CONCLUSION: The severity of pre-operative systolic and diastolic LV dysfunction is the major predictor of mortality following AVR for low-flow and high-gradient AS.

Twenty-year surgical experience with congenital supravalvar aortic stenosis.

BACKGROUND: Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome and other elastin gene deletions. Our objectives were to review outcomes of congenital SVAS repair and to compare prosthetic patch repair techniques to all-autologous slide aortoplasty. METHODS: Congenital SVAS repairs from 1988 to 2008 were retrospectively reviewed. Peak instantaneous gradients were estimated by Doppler interrogation. Variables were compared by either Student's t test or Fisher's exact test. Risk factors were analyzed by chi(2) test. Survival was estimated by the Kaplan-Meier method. RESULTS: Of 25 primary SVAS repairs, there were 10 all-autologous slide aortoplasties and 15 prosthetic patch aortoplasties. The prosthetic patch group included the Doty technique (n = 9), patch-augmented slide aortoplasty (n = 3), modified Brom technique (n = 1), interposition graft (n = 1), and two-sinus patch with transverse arch augmentation (n = 1). There was 1 early and 1 late death. Cumulative survival for all patients was 96% at 5 and 10 years. Event-free survival did not differ between groups (p = 0.481). There were 2 late reoperations (both were prosthetic patch patients with bicuspid aortic valve: 1 with recurrent aortic valve stenosis and 1 with aortic insufficiency). Bicuspid aortic valve was the only risk factor for reoperation (p = 0.003). Three patients weighing less than 10 kg with diffuse disease underwent attempted slide aortoplasty: 2 required patch augmentation and 1 had a recurrent gradient in less than 1 year postoperatively. CONCLUSIONS: Outcomes after SVAS repair were good by any technique. No advantage to all-autologous slide aortoplasty was apparent at current follow-up. Based on our experience, slide aortoplasty is not recommended for small patients with diffuse disease.

Congenital supravalvular aortic stenosis: defining surgical and nonsurgical outcomes.

BACKGROUND: Supravalvular aortic stenosis is a rare stenotic lesion of the left ventricular outflow tract (LVOT). We characterized the natural history of the disease and the effect of surgical intervention. METHODS: Ninety-five children diagnosed with supravalvular aortic stenosis between 1976 and 2006 were studied. Procedural and repeated echocardiography reports were analyzed. RESULTS: Stenosis morphology (localized, 82%; diffuse, 18%) was independent of Williams syndrome (n = 59, 62%). The risk of open operation (n = 47) was 46% +/- 6% at 10 years. Increased risk of operation was associated with higher baseline LVOT peak gradients (p < 0.001), smaller minimum LVOT z scores (p < 0.01; thresholds > 50 mm Hg and < -3, respectively), and the absence of Williams syndrome (p = 0.01). Patients who did not undergo operations had gradually reducing LVOT gradients and enlarging ascending aorta z scores over time. Persistently small minimum LVOT z scores and higher gradients were associated with children who required an operation. Operation resulted in persistent relief of LVOT obstruction and accelerated increases in ascending aorta dimensions. Overall survival was 94% +/- 3% and 85% +/- 7% at 10 and 15 years and was similar for surgical and nonsurgical groups. No independent risk factors for death were identified on univariate or multivariable analysis. CONCLUSIONS: Many children-particularly those with Williams syndrome-show regression of stenosis without intervention. Children who undergo operation have high LVOT gradients and smaller LVOT z scores that do not improve over time. Surgical intervention alters the natural history: LVOT obstruction is relieved and does not recur, and ascending aortic dimensions progressively enlarge towards normal values.

Efeitos da inibição prolongada da enzima de conversão da angiotensina sobre as características morfológicas e funcionais da hipertrofia ventricular esquerda em ratos com sobrecarga pressórica persistente / Effects of the prolonged inhibition of the angiotensin-converting enzyme on the morphological and functional characteristics of left ventricular hypertrophy in rats with persistent pressure overload

OBJETIVO: Avaliar os efeitos do lisinopril (L) sobre as taxas de mortes (M), insuficiência cardíaca (ICC), características da remodelação miocárdica, geométrica e funcional do ventrículo esquerdo (VE), em ratos com estenose aórtica supravalvar (EAS). MÉTODOS: Ratos foram submetidos a EAS ou cirurgia simulada (GC:n=10). Randomizados após 6 semanas para receber L (GL:n=30) ou nenhum tratamento (GE:n=73) sendo avaliados 6s e 21s por estudos ecocardiográfico, hemodinâmico e morfológico concomitantes. RESULTADOS: As taxas de M (GE: 53,9 por cento vs GL: 16,7 por cento e ICC GE: 44,8 por cento vs GL: 20 por cento p<0,05). No final do experimento, os valores da pressão sistólica do VE dos grupos GE e GL foram equivalentes e significantemente mais elevados do que no grupo GC; (p<0,05) não diferindo dos observados 6 semanas após os procedimentos cirúrgicos. Os valores da pressão diastólica do VE no grupo GE foram maiores do que os do grupo GL (p<0,05) sendo ambos maiores do que os do grupo GC (4 ± 2 mmHg, p<0,05). O mesmo comportamento foi observado com as variáveis: razão E/A; índice de massa, área seccional dos miócitos e conteúdo de hidroxiprolina do VE. A porcentagem de encurtamento do VE foi semelhante nos grupos GC e GL (p>0,05) sendo ambos maiores que os verificados no grupo GE. Comportamento semelhante foram obtidos com os valores da primeira derivada positiva e negativa da pressão do VE. CONCLUSAO: Em ratos com EAS o L reduziu as taxas de M e ICC e exerceu efeitos benéficos sobre a remodelação e a função do VE.

Acquired supravalvar aortic stenosis following heart transplantation: report of 2 cases.

We report 2 cases of acquired supravalvar aortic stenosis (SVAS) complicating orthotopic cardiac transplantation that contributed to the post-operative death of both patients. In the first patient the stenosis resulted from kinking of a too-long graft aortic component that was rendered into a fixed deformity by healed mediastinitis and constrictive pericarditis. Supra-aortic stenosis in the second patient occurred at a site of multiple aortic cannulations that was surrounded by dense fibrosis. Acquired SVAS contributed to the death of the first patient (who had graft arteriopathy) and was the proximate cause of death in the second patient. Specific echocardiography of the ascending aorta, which is not routinely performed, may detect SAVS.

Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis.

OBJECTIVE: Stenosis of the left main coronary artery is a recognized complicating feature of supravalvular aortic stenosis. We have retrospectively identified three anatomic subtypes of left main coronary obstruction in patients with supravalvular aortic stenosis, each necessitating a distinct surgical approach. METHODS: From 1991 to 1998, 9 patients underwent surgical repair of supravalvular aortic stenosis and left main coronary stenosis. Five patients (group 1) had obstruction from near-circumferential thickening of the left main ostium, 2 patients (group 2) had restricted coronary flow due to fusion of an aortic valve leaflet to the supravalvular ridge, and 2 patients (group 3) had diffuse narrowing of the left main coronary artery. Group 1 patients were treated with patch aortoplasty encompassing the left main ostium and supravalvular aortic stenosis. Group 2 patients were treated with excision of the fused leaflet from the aortic wall and patch aortoplasty. Group 3 patients were treated with bypass grafting and aortoplasty. RESULTS: Surgical strategy was determined by coronary angiography and intraoperative assessment of coronary anatomy. There was 1 early death. All surviving patients underwent echocardiography with or without postoperative catheterization. The mean postoperative supravalvular gradient for 7 patients was 8 mm Hg (range 2-15 mm Hg). One patient required reoperation for a residual aortic gradient as a result of aortic arch involvement. No evidence of left main coronary artery stenosis was seen in groups 1 and 2; bypass grafts were patent in group 3 patients at a mean follow-up of 54.8 months. CONCLUSION: Three subtypes of left main coronary stenosis with supravalvular aortic stenosis are described. Each anatomic type mandates an individual surgical approach. Favorable surgical outcomes are achievable with each category.