Congenital Aortic Stenosis In Children Research Articles

Long-Term Results of Congenital Aortic Stenosis Treatment in the Era of Percutaneous Balloon Valvuloplasty: Up to 33 Years Follow-Up.

Background The goal of this study was to evaluate long-term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children. Methods and Results A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow-up study. The resulting follow-up time reached a median of 18.5 (interquartile range, 12.2-25.1) years. Successful BVPL was defined by residual Doppler gradient <70/40 (systolic/mean) mm Hg. The primary end point was death; secondary end points included any valve reintervention, balloon revalvuloplasty, any aortic valve surgery, and aortic valve replacement, respectively. BVPL effectively reduced the peak and mean gradient both immediately and at the latest follow-up (P<0.001). There was significant procedure-related progression of aortic insufficiency (P<0.001). Higher aortic annulus z score was predictive for severe aortic regurgitation (P<0.05) and lower z score for insufficient gradient reduction (P<0.05). The actuarial probability of survival/survival free from any valve reintervention was 89.9%/59.9%, 85.9%/35.2%, and 82.0%/26.7% at 10, 20, and 30 years after first BVPL, respectively. Left ventricular dysfunction or arterial duct dependency as the indication for BVPL was predictive of both worse survival and survival free from any reintervention (P<0.001). Lower aortic annulus z score and lower balloon-to-annulus ratio were predictive of a need for revalvuloplasty (P<0.001). Conclusions Percutaneous BVPL provides good initial palliation. In patients with hypoplastic annuli and left ventricular or mitral valve comorbidity, the results are less favorable.

Surgical aortic valvuloplasty is a better primary intervention for isolated congenital aortic stenosis in children with bicuspid aortic valve than balloon aortic valvuloplasty

ObjectivesSurgical aortic valvuloplasty (SAV) and balloon aortic valvuloplasty (BAV) are two main treatments for children with isolated congenital aortic stenosis (CAS). We aim to compare the two procedures' midterm outcomes, including valve function, survival, reintervention, and replacement. MethodsFrom January 2004 to January 2021, children with isolated CAS undergoing SAV (n = 40) and BAD (n = 49) at our institution were included in this study. Patients were also categorized into subgroups based on the aortic leaflet number(Tricuspid = 53, Bicuspid = 36) to compare the two procedures’ outcomes. Clinical and echocardiogram data were analyzed to identify risk factors for suboptimal outcomes and reintervention. ResultsPostoperative peak aortic gradient (PAG) and PAG at follow-up in the SAV group were lower compared with the BAV group (p < 0.001, p = 0.001, respectively). There was no difference in moderate or severe AR in the SAV group compared with the BAV group before discharge (5.0% vs 12.2%, p = 0.287) and at the last follow-up (30.0% vs 32.7%, p = 0.822). There were no early death but three late deaths (SAV = 2, BAV = 1). Kaplan-Meier estimated survivals were 86.3% and 97.8% in SAV and BAV groups respectively at 10 years (p = 0.54). There was no significant difference in freedom from reintervention (p = 0.22). For patients with bicuspid aortic valve morphology, SAV achieved higher freedom from reintervention (p = 0.011) and replacement (p = 0.019). Multivariate analysis indicated that residual PAG was a risk factor for reintervention (p = 0.045). ConclusionsSAV and BAV achieved excellent survival and freedom from reintervention in patients with isolated CAS. SAV performed better in PAG reduction and maintenance. For patients with bicuspid AoV morphology, SAV was the preferred choice.

Long-term outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children

ObjectiveWe aimed to assess the long-term outcomes of children with isolated congenital aortic stenosis who underwent primary aortic valve repair. MethodsRecords of all children (n = 111) with isolated congenital aortic stenosis who underwent primary aortic valve repair between 1980 and 2016 were reviewed. An optimal operative outcome consisted of a residual left ventricular outflow tract peak systolic gradient <35 mm Hg and trivial or less aortic insufficiency. ResultsMedian age at surgery was 0.4 years (interquartile range, 1 month-7.9 years) and median weight at surgery was 7.0 kg (interquartile range, 3.7-25.0 kg). Fifty-two patients (46.8%; 52/111) underwent aortic valve repair with the use of patch material. Early mortality was 0.9% (1/111). Late mortality was 0.9% (1/110). Freedom from aortic valve reoperation was 52.1% (95% CI, 38.7-63.8) at 10 years. Freedom from aortic valve replacement was 67.9% (95% CI, 55.4-77.5) at 10 years. An optimal outcome was achieved in 48 patients (43.2%; 48/111). At 10 years, freedom from aortic valve reoperation was 78.2% (95% CI, 63.1-87.8) in patients with an optimal outcome, compared with 39.4% (95% CI, 22.8-55.6) in those with a suboptimal outcome (P = .03). Tricuspid aortic valve was associated with a suboptimal outcome (P = .01). ConclusionsAortic valve repair achieves relief of congenital aortic stenosis with very low early mortality and excellent long-term survival, even in neonates. Although nearly half of the patients required aortic valve reoperation by 10 years, two-thirds of the patients remain free from aortic valve replacement. An optimal outcome was more commonly achieved with bicuspid aortic valves compared with tricuspid aortic valves.

Balloon dilatation versus surgical valvotomy for congenital aortic stenosis: a propensity score matched study.

Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon centre bias with limited relevant, comparative literature. This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches. Retrospective analysis of patients with congenital aortic valve stenosis who underwent balloon valvuloplasty (Queensland Children's Hospital, Brisbane) or surgical valvotomy (Royal Children's Hospital, Melbourne) between 2005 and 2016. Patients were excluded if pre-intervention assessment indicated ineligibility to either group. Propensity score matching was performed based on age, weight, and valve morphology. Sixty-five balloon patients and seventy-seven surgical patients were included. Overall, the groups were well matched with 18 neonates/25 infants in the balloon group and 17 neonates/28 infants in the surgical group. Median age at balloon was 92 days (range 2 days - 18.8 years) compared to 167 days (range 0 days - 18.1 years) for surgery (rank-sum p = 0.08). Mean follow-up was 5.3 years. There was one late balloon death and two early surgical deaths due to left ventricular failure. There was no significant difference in freedom from reintervention at latest follow-up (69% in the balloon group and 70% in the surgical group, p = 1.0). Contemporary analysis of balloon aortic valvuloplasty and surgical aortic valvotomy shows no difference in overall reintervention rates in the medium term. Balloon valvuloplasty performs well across all age groups, achieving delay or avoidance of surgical intervention.

Long-term outcome of percutaneous balloon aortic valvuloplasty for children with congenital aortic valve stenosis

Objective: To assess the efficacy and long-term outcome of percutaneous balloon aortic valvuloplasty (PBAV) for children with congenital aortic stenosis (CAS) and to explore risk factors for significant aortic regurgitation (AR) and reintervention after PBAV during follow up. Methods: This was a retrospective study. Children (≤18 years old) with CAS, who underwent PBAV in Guangdong Provincial Hospital from January 2004 to December 2018, were included in this study. Demographic, preoperative transthoracic echocardiography (TTE) and surgical data were collected. Postoperative complications were closely observed, and the patients were followed up at 1, 6, 12 months after the operation, and then at one year interval thereafter. Endpoint events included significant AR and reintervention. Reintervention was defined as any intervention that needed to be performed on the valve for various reasons, including re-PBAV, surgical valvuloplasty and valve replacement. Significant AR was defined as AR grade≥3 by TTE criteria. The results of the last TTE examination before the end of the study were collected. The Kaplan-Meier curve for long-term AR-free and intervention-free survival was plotted. Cox regression model was used to further analyze the risk factors for significant AR and reintervention after PBAV in CAS patients. Results: A total of 55 patients were enrolled in this study, and the age was 4.6(1.6, 6.5) years, with 37(67.3%) males. The peak systolic valve gradient fell from (80.3±30.6)mmHg to (38.5±18.5)mmHg(P<0.001, 1 mmHg=0.133 kPa). Surgical success rate was 89% (49/55). Acute post-PBAV AR occurred in 18 patients, including 3 patients with RA≥3 grade. In-hospital complications occurred in 6 patients (2 deaths, 2 cases of transient arrhythmia, and 2 cases of femoral artery embolization). Fifty patients accomplished the follow-up and the follow-up time was 6.2(3.4, 8.5) years. Significant AR was found in 20 patients. Significant AR-free survival rate was 53% at 5 years and 19% at 10 years. Reintervention was performed in 11 patients (4 with valvuloplasty and 7 with valve replacement), and the 5-year and 10-year intervention-free survival rates were 87% and 62%, respectively. Multivariate Cox regression analysis showed that acute post-PBAV AR was a risk factor for long-term significant AR (HR=2.398, 95%CI 1.007-5.712, P=0.048). Post-PBAV residual pressure gradient ≥ 35 mmHg (HR=4.747, 95%CI 1.116-19.329, P=0.030)and acute post-PBAV AR (HR=5.104, 95%CI 1.083-24.065, P=0.039)were risk factors for re-intervention. Conclusions: PBAV is safe and effective in the treatment of CAS in children, but attention should be paid on significant AR post procedure. Acute post-PBAV AR is a risk factor for re-intervention and significant AR post PBAV, and high post-PBAV residual pressure gradient is a risk factor for re-intervention.

646 A Ceasefire: Matched Analysis of Balloon vs Surgical Valvotomy for Congenital Aortic Stenosis

Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon center bias with limited relevant, comparative literature. This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches. Retrospective analysis of patients with congenital aortic valve stenosis who underwent balloon valvuloplasty (Queensland Children’s Hospital) or surgical valvotomy (Royal Children’s Hospital, Melbourne) between 2005 and 2016. Patients were excluded if pre-intervention assessment indicated ineligibility to either group. Propensity-score-matching was performed based on age, weight and valve morphology. Sixty-five balloon patients and seventy-nine surgical patients were included. Overall the groups were well matched with 18 neonates / 25 infants in the balloon group and 17 neonates / 28 infants in the surgical group. Median age at balloon was 92 days (range 2 days - 18.8 years) compared to 167 days (range 0 days – 18.1 years) for surgery (Rank-sum p=0.08). Mean follow-up was 5.3 years. There was 1 late balloon death and 2 early surgical deaths due to left ventricular failure. There was no significant difference in freedom from re-intervention (FFI) at latest follow-up (69% in the balloon group and 70% in the surgical group, p=1.0). Contemporary analysis of balloon aortic valvuloplasty and surgical aortic valvotomy shows no difference in overall reintervention rates in the medium-term. Balloon valvuloplasty performs well across all age groups, achieving delay or avoidance of surgical intervention.

Different interventions in treatment of children with congenital aortic stenosis

Objective To evaluate the efficacy and safety of percutaneous balloon aortic valvuloplasty(PBAV) in retrograde, antegrade way or right ventricular pacing in children. Methods This was retrospective clinical study included 32 children who were treated with PBAV for congenital aortic stenosis from January 2008 to June 2017 in Guangdong Cardiovascular Institute.The general clinical data including residual stenosis and aortic stenosis again, and degree of artery injury, aortic regurgitation were particularly assessed. Results A total of 32 patients consisting of 27 boys and 5 girls underwent the procedure, with age of (55.8±52.0) months (ranging from 20 days to 15 years) and body weight (18.2±14.0) kg (ranging from 3.5 kg to 59.0 kg). Two infants accepted left cardiac catheterization through femoral vein and one with interventional indication then accepted PBAV through the patent foramen ovale.The others underwent the retrograde way, including 24 cases with rapid right ventricular pacing.The catheter-measured peak systolic the aortic valve gradient decreased from (81.6±28.0) mmHg(1 mmHg=0.133 kPa) to (41.4±19.0) mmHg immediately after percutaneous interventional treatment, and the difference was statistically significant(t=9.543, P=0.000). The peak systolic valve gradient mea-sured pre-PBAV and on the second day after PBAV measured by Doppler echocardiography decreased from (82.7±23.0) mmHg to (44.6±18.0) mmHg, and the difference was statistically significant(t=11.732, P=0.000). The diameters of the aortic valve were (13.8±3.0) mm(ranging from 6.5 mm to 21.0 mm) and the balloons were (14.1±4.0) mm(ranging from 6 mm to 23 mm). The follow-up period was 1 to 72 months.The peak systolic valve gradient measured by Doppler echocardiography increased from (44.6±18.0) mmHg to (58.6±30.0) mmHg, and the difference was statistically significant(t=-2.549, P=0.016). During the procedure, 5 children(14.7%) accepted surgery for restenosis or regurgitation.Seven had regurgitation, one had femoral artery embolism and one died on the second day after the procedure. Conclusions With the diversification of procedures and the continuous improvement of interventional devices, PBAV can play a very good role in alleviating congenital aortic stenosis in children, and the safety has been continuously improved. Key words: Aortic stenosis; Intervention; Child

Результаты лечения врожденного аортального стеноза у детей первого года жизни

&lt;b&gt;Введение. &lt;/b&gt;В настоящее время рентгенэндоваскулярная баллонная дилатация (РЭБД) широко применяется при лечении врожденного стеноза аортального клапана (АК) у детей как альтернатива операциям с применением искусственного кровообращения. &lt;br /&gt; &lt;br /&gt; &lt;b&gt;Цель. &lt;/b&gt;Оценка непосредственных и отдаленных результатов баллонной дилатации врожденного аортального стеноза у детей 1-го года жизни. &lt;br /&gt; &lt;br /&gt; &lt;b&gt;Материал и методы. &lt;/b&gt;В РНПЦ «Кардиология» с 2005 по 2014 г. и РНПЦ детской хирургии с 2015 по 2017 г. РЭБД врожденного стеноза АК была выполнена у 130 детей 1-го года жизни. В периоде новорожденности были оперированы 92 (70,8%) пациента, при этом 38 (29,2%) детей находились в критическом состоянии. Диаметр клапанного кольца аорты составил в среднем 8,1±0,7 мм. &lt;br /&gt; &lt;br /&gt; &lt;b&gt;Результаты и обсуждение.&lt;/b&gt; На госпитальном этапе летальных исходов не было. Интраоперационные осложнения возникли у 2 (1,5%) пациентов. Пиковый градиент систолического давления на АК снизился с 65,5±23,7 до 29,3±8,6 мм рт. ст., фракция выброса левого желудочка увеличилась с 53,6±1,6 до 65,3±1,7%. Длительность наблюдения составила от 3 мес до 10 лет. В отдаленном периоде 26 (20%) детям были выполнены повторные хирургические вмешательства: повторная баллонная дилатация – 16 (12,3%), реконструктивные операции на АК – 6 (4,6%), операция Росса – 3 (2,3%), протезирование АК механическим протезом – 1 (0,8%). &lt;br /&gt; &lt;b&gt; &lt;br /&gt; Выводы. &lt;/b&gt;РЭБД врожденного аортального стеноза у детей 1-го года жизни может быть как самостоятельным хирургическим вмешательством, так и применяться в качестве 1-го этапа хирургической коррекции, позволяющей выполнить операцию с искусственным кровообращением в более старшем возрасте.

Valvuloplastia aórtica pediátrica: estudio de variables con influencia en los resultados a largo plazo

Introduction and objectiveBalloon aortic valvuloplasty is regarded as a good palliative option for congenital aortic stenosis in children. Our main objective was to review the results of all consecutives balloon aortic valvuloplasties performed in children over a native valve from march 1994 until June 2013, trying to identify those variables associated with need of reintervention during follow-up. MethodsWe studied the echocardiographic, clinical and evolutionary features of 51 pediatric patients who underwent a balloon aortic valvuloplasty. Two groups were recognized: neonatal (n=11) and pediatric (n=40) patients depending on whether valvuloplasty was performed before or after the first month of life. Median follow-up was 5.8 years (0.5-12,3). ResultsThere were 37 boys and 14 girls, and 20 patients had some additional cardiac malformation. The average peak to peak hemodynamic gradient fell from 60±18 a 25±13mmHg. During follow-up, 12 patients needed a second valvuloplasty for reestenosis (23.5%) at 0.61 years (0.3-3.2) and 13 others needed surgery (25%) at 1.62 years (0.4-10.9); 2 cases (4%) had no follow-up. The variables neonatal group, echocardiographic restrictive diastolic pattern, z-score of end-diastolic left ventricle diameter<-2.5, and peak to peak hemodynamic gradient>60mmHg were associated with need of surgery during follow-up (p<0.05). However, we did not find factors associated with repeated valvuloplasty. ConclusionsBalloon aortic valvuloplasty in children is an effective option for palliative treatment of congenital aortic stenosis. In this study the variables previously described were predictors of the need of surgery.

Balloon valvuloplasty as a treatment of congenital aortic stenosis in children and adolescents

Balloon valvuloplasty (BVP) is one of the primary therapies for congenital aortic stenosis in children and adolescents. The aim of this interventional procedure is to gain time before possible surgical therapy (aortic valve replacement) until adulthood. The aim of this study was to evaluate the efficacy, safety and mid-term results oftranscatheter BVP in children and adolescent in our Center. From 2004 to 2011, 50 patients, aged 18 days to 18 years (mean 6.3 years) underwent BVP. Retrospective analysis of the echocardiographic and hemodynamic parameters were performed before and after procedure, especially peak pressure gradient (PG) across the aortic valve, semiquantification of the aortic regurgitation (AR) after the BVP as well as the left ventricle dimensions and functions. The mean peak PG in the whole group decreased from 74.80 +/- 27.72 mm Hg to 27.86 +/- 3.04 mm Hg (p < 0.001) after BVP. In 39 patients (78%), residual PG was lower than 30 mm Hg just after dilation. At the end of follow-up period, 25 patients (50%) had PG above 50 mm Hg, measured by Doppler technique, and four of them underwent re-dilation. Eight patients (16%) had severe AR. During the follow-up period (12-80 months, mean 51 months), six patients (12%) were referred to cardiac surgeons for aortic valve replacement or Ross procedure. This retrospective study analyzes our first experience of BVP as primary therapy of the congenital aortic stenosis. The results confirmed that BVP effectively postponed the need for surgery in children and adolescents toward the adulthood.

Surgery for Congenital Aortic Stenosis in Children With Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is an uncommon genetic disorder of endocardial morphogenesis, which carries a high mortality from heart failure or sudden cardiac death. This condition is often first diagnosed in adults, but it has also been described in children with other cardiac anomalies. We discuss the management of a 10-year-old female with congenital aortic stenosis associated with LVNC.

The influence of valve physiology on outcome following aortic valvotomy for congenital bicuspid valve in children: 30-year results from a single institution

Aortic valvotomy is widely used for the treatment of congenital aortic stenosis in children. We sought to evaluate whether the predominant post-valvotomy physiology, aortic insufficiency (AI) or aortic stenosis (AS) independently affected patient outcome. From 1972-2002, 57 children with congenital aortic stenosis underwent valvotomy. We divided age-matched patients with residual lesions based on their predominant pathology into three groups: Group I (n=14), patients with moderate AI; Group II (n=14), patients with moderate AS, and Group III (n=14), patients with combined AI and AS. Fifteen patients with severe AI or mild residual lesions following valvotomy were excluded from analysis. mean freedom from aortic valve replacement (AVR) was 11.2+/-1.7 years in Group I and 21.5+/-3.9 years in Group II, P=0.05. AVR was required in 11 patients (79%) in Group I vs. only 5 (36%) in Group II, P=0.05. Group III was intermediate, with 9 (64%) requiring AVR. At the time of AVR, patients with aortic stenosis had significantly higher fractional shortening % than those with insufficiency or combined lesions, (Group 1: 38.2+/-7.9 vs. Group II: 46.3+/-5.5 vs. Group III: 39.2+/-3.7, P=0.007). Patients in Group II also had less severely dilated ventricles (mm) than those in the other groups, (Group 1: 50.2+/-12.5 vs. Group II: 39.5+/-8.3 vs. Group III: 49.0+/-8.1, P=0.030). patients with predominant AI following valvotomy are more likely to need AVR sooner than those with residual stenosis without AI. Therefore, cautious use of repeat valvotomy using maneuvers to avoid AI (small balloons), may prolong freedom from aortic valve replacement in those patients with significant residual AS.

Outcomes after balloon dilation of congenital aortic stenosis in children and adolescents

To determine the long-term outcomes and risk factors for, reintervention after balloon dilation of congenital aortic stenosis in children aged 6 months or older. Although balloon dilation of congenital aortic stenosis has become a primary therapeutic strategy, few data are available regarding long-term outcomes. We carried out a retrospective review of 87 children who had undergone balloon dilation of the aortic valve at median age of 6.9 years. The procedure was completed in 98% of the children, with an average reduction in the gradient across the valve of 64 +/- 28%, and without mortality. Of the children, 76 had been followed for a mean of 6.3 +/- 4.2 years. Reintervention on the aortic valve was required in 32 children, with 12 undergoing reintervention within 6 months, with 1 death. Another patient had died over the period of follow-up due to a non-cardiac event. Estimated freedom from reintervention was 86% at 1 year, 67% at 5 years, and 46% at 12 years. Parametric modeling of the hazard function showed a brief early phase of increased risk, superimposed on an ongoing constant risk. The only incremental risk factor for the early phase was a residual gradient immediately subsequent to the procedure greater than 30 mmHg. Incremental risk factors for the constant phase included the presence of symmetric valvar opening, and greater than moderate regurgitation immediately after dilation. Long-term survival was excellent, albeit that the need for further reintervention was high due to the palliative nature of the procedure.

Effect of balloon aortic valvuloplasty of congenital aortic stenosis in children in regression of left ventricular mass

Effect of balloon aortic valvuloplasty of congenital aortic stenosis in children in regression of left ventricular mass

Progression of congenital aortic stenosis in children beyond infancy: assessment using Doppler echocardiography

AbstractCase records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p &lt; 0.002 and p &lt; 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.

Aortic valve repair and replacement after balloon aortic valvuloplasty in children

Little is known about the incidence, indications, and results of surgical repair or replacement of the aortic valve after balloon aortic valvuloplasty (BAV) for congenital aortic stenosis in children. This study was designed to evaluate patterns of failure requiring operation after BAV for congenital aortic stenosis and to review our experience with successful repair, rather than replacement, of selected aortic valves after BAV. From March 1986 to June 1995, 60 patients with congenital aortic stenosis aged 1 day to 27 years (mean +/- standard deviation, 7.3 +/- 6 years) underwent BAV. Twenty-three patients (38%) required operation a mean of 44 +/- 37 months (range, 1 to 110 months) after BAV, because of severe aortic insufficiency in 13 patients and recurrent or residual aortic stenosis in 10 patients. Severe aortic insufficiency was invariably due to avulsion of a cusp from the annulus, with resulting cusp prolapse and insufficiency. Operative intervention consisted of valve replacement in 14 patients and valve repair in 9 patients. Repair techniques included reattachment of an avulsed cusp to the aortic annulus, relief of commissural fusion, and debridement of thickened cusps. Actuarial freedom from surgical intervention after BAV was 88% +/- 4% at 1 year, 70% +/- 6% at 5 years, and 51% +/- 12% at 9 years. The need for aortic valve operation was unrelated to age at the time of BAV, indication for operation (aortic insufficiency versus aortic stenosis), age of operation, or preoperative gradient. All patients survived aortic valve operation; there was one late death at an average follow-up of 27 +/- 20 months (range, 2 to 61 months) after aortic valve operation. Stenosis was well relieved in all patients undergoing valve replacement. The 9 valve repair patients have been followed for 22 +/- 14 months (range, 1 to 47 months). Echocardiographic follow-up of the valve repair patients revealed a mean residual aortic stenosis peak instantaneous gradient of 32 mm Hg and mild aortic insufficiency or less in all patients. Aortic valve operation is required in 5% to 7% of patients yearly after BAV. The need for operation appears to be unrelated to age at the time of BAV; aortic insufficiency predominates over aortic stenosis as an indication for operative intervention. Valve repair can be applied in some patients after BAV with good intermediate-term results and may delay the need for aortic valve replacement.

721-2 Aortic Valve Repair and Replacement After Balloon Aortic Valvuloplasty in Children

Little is known about the incidence, indications and results of surgical repair or replacement of the aortic valve following balloon valvuloplasty for congenital aortic stenosis in children. This study was designed to evaluate patterns of failure requiring surgery after balloon aortic valvuloplasty (BAli.) of congenital AS and to review our experience with successful repair, rather than replacement, of some aortic valves after BAV. From 1986 to 1994, 54 patients with congenital AS aged 1 day-27 years (mean ± sd; 8 ± 6 yrs) underwent BAV. Eighteen patients (33%) required surgery a mean of 33 ± 25 mos (range 1 to 80 mos) following BAV. because of severe AI in 10 patients and recurrent or residual AS in 8 patients. Severe AI was invariably due to avulsion of a cusp from the annulus with resulting cusp prolapse and insufficiency. Actuarial freedom from surgical intervention was 90 ± 4% at 1 year, 79 ± 6% at 3 years, and 64 ± 7% at 6 years. The need for aortic valve surgery was unrelated to age at the time of BAV. Surgical intervention consisted of valve replacement in 11 patients and aortic valve repair in 7 patients. Repair techniques included reattachment of an avulsed cusp to the aortic annulus, relief of commissural fusion, and debridement of thickened cusps. All survived operation and are alive 23 ± mos (range 2–44 mos) following aortic valve operation. LV outflow gradient has been well relieved in all patients undergoing valve replacement. The 7 valve repair patients have been followed 17 ± 10 mos (range 4–33 mos). Echocardiographic follow-up of the valve repair patients revealed mild levels of residual AS and mild (1–2+) AI in all patients. Aortic valve surgery is required in 5–10% of patients yearly following BAli. and appears to be unrelated to age at the time of BAV. AI predominates over AS as an indication for operative intervention. Valve repair can be applied in some patients following BAV. with good intermediate-term results and may delay the need for aortic valve replacement.

Valvotomy for isolated congenital aortic stenosis in children: prognostic factors for outcome.

Early and late results after surgery for isolated congenital valvar aortic stenosis were evaluated in a total of 86 children under 16 years of age (mean 7.4 years). Primary procedure was always conservative. There were 7/86 (8.1%) early deaths. All infants who died after the operation were younger than 4 months of age. Among the clinical variables tested by the univariate analysis only age and duration of cardiopulmonary bypass were significant prognostic factors for early death. There were 6/67 (7.7%) valve-related late deaths. Multivariate analysis could not identify any risk factors for early and late mortality. Actuarial survival was 97% (95% CL 93-101%) after 5 years, 94% (88-100%) after 10 years, 90% (82-98%) after 15 years, and 87% (77-97%) after 20 years. A total of 22/79 (28%) early survivors had a first reoperation and 5 had a second reoperation. Long follow-up interval was the only significant factor for reoperation. Actuarial reoperation-free interval was 91% (85-98%) after 5 years, 70% (58-81%) after 10 years, and 50% (34-64%) after 15 years. Significant factors for poor valve function were long duration of follow-up, endocarditis, and young age at operation. The probability of normal valve function was 91% (84-98%) after 5 years, 67% (55-79%) after 10 years, and 54% (40-68%) after 15 years.

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.

Value of systolic time intervals in assessing severity of congenital aortic stenosis in children.

Simultaneous recordings have been made of electrocardiogram, phonocardiogram, carotid pulse tracing, left ventricular pressure, and aortic pressure in 27 children with aortic valve stenosis and 3 children with membranous subaortic stenosis. Peak systolic pressure difference ranged from 10 to 110 mmHg (1.3 to 14.6 kPa). None of the patients had congestive heart failure and cardiac output was in the normal range in all. Total electromechanical systole, left ventricular ejection time, and pre-ejection time were corrected for heart rate, age, and sex. Mild stenosis (peak systolic pressure difference less than or equal to 50 mmHg (6.7 kPa)) was present in 18, severe stenosis (peak systolic pressure difference greater than 50 mmHg) in 12 patients. The externally measured pre-ejection time and ejection time proved to be nearly equal to the corresponding intervals measured internally; from these data it is concluded that pre-ejection time and ejection time in children with aortic stenosis can be measured reliably by non-invasive methods. Mean values for corrected total electromechanical systole and ejection time were prolonged, but the corrected pre-ejection time did not differ from the normal value. When corrected time intervals were plotted against severity of the aortic stenosis as expressed by the peak systolic pressure difference or the aortic valve orifice index, a wide scatter was found. It is concluded that a normal ejection time is strong evidence against a peak systolic pressure difference of more than 50 mmHg (6.7 kPa) or an aortic valve orifice index less than 0.70 cm2 per m2 BSA. A prolonged ejection time, however, may occur in mild as well as in severe stenosis. Total electromechanical systole and pre-ejection time have no value in predicting the severity of aortic stenosis in children.