Interventional catheterization in infantile congenital aortic valve diseases

The authors present their experience of percutaneous aortic valvuloplasty in 10 neonates and 12 children with critical aortic stenosis. In the neonate group the left ventricular aortic gradient dropped from 49 +/- 20 mmHg to 15 +/- 16 mmHg. This improvement persisted in 57 p. cent of the cases after 2 years. In the older group (mean age 6 years) the gradient was reduced from 73 +/- 22 mmHg to 30 +/- 17 mmHg with maintained results in 78 p. cent after 2 years. Aortic incompetence occurred in the majority of cases but did not seem to be more important than after surgical commissurotomy. In critical stenosis, balloon aortic valvuloplasty seems to be a valuable alternative to surgery as a palliative measure before prosthetic valve replacement.     

Balloon valvuloplasty of the aortic valve in children: a surgical view

The paper by Fratz et al. describes the follow-up of 188 children who had balloon aortic valvuloplasty (AoVP) between 1986 and 2004. Moderate or severe aortic regurgitation occurred more often in the 68 neonates--in whom there was significant early mortality--than in the 120 older children; however, AoVP produced a satisfactory reduction in aortic valve gradient in both groups. Reintervention-free status declined in both groups over time and, at 10 years follow-up, 59% of the neonates and 70% of the older group were intervention free. These results show that AoVP postpones the need for aortic valve surgery and has the advantage of being less invasive than initial surgical valvotomy. AoVP is, however, associated with an increased risk of aortic regurgitation and, in neonates, early subsequent aortic valve surgery is likely to be needed.     

Current treatments for congenital aortic stenosis

PURPOSE OF REVIEW: Congenital valvar aortic stenosis is a challenging disease that often requires repeated palliative procedures. Stenosis can range from mild and asymptomatic, not requiring intervention, to severe, as seen in hypoplastic left heart syndrome. New advances such as fetal balloon valvuloplasty, improvements in the Ross technique, and long-term studies of trans-catheter balloon valvuloplasty and surgical valvotomy warrant a review of the outcomes and optimal timing of the various interventions. RECENT FINDINGS: Fetal balloon valvuloplasty has shown promise. Despite some mortality and morbidity, some fetuses are showing significant growth in left ventricular structures, allowing biventricular repair. In neonates and infants with congenital aortic stenosis, excellent initial results are obtained with trans-catheter balloon valvuloplasty, although stenosis resistant to further balloon dilation or regurgitation may develop, necessitating surgical intervention. Midterm results from the Ross procedure are encouraging, demonstrating low rates of mortality, aortic insufficiency and re-intervention. Stenosis of the pulmonary allograft may be inevitable, and recent long-term follow-up suggests an increase in aortic insufficiency. SUMMARY: While availability of fetal balloon valvuloplasty is limited, it has promise for promoting in-utero left ventricle growth and improving function. The optimal procedure for infants and neonates is trans-catheter balloon valvuloplasty. For older patients, the Ross procedure is the repair of choice, although more long-term studies are needed to assess the natural course of the autograft. Outcomes should improve with advances in pulmonary allografts.     

Measurement of effective aortic valve area using three-dimensional echocardiography in children undergoing aortic balloon valvuloplasty for aortic stenosis

Aims: Pressure gradient is used for timing of balloon aortic valvuloplasty for aortic stenosis (AS) in children, but does not correlate well with outcome and is limited if ventricular function is poor. In adults, effective orifice area (EOA) is used to assess AS severity, but EOA by continuity equation or 2D echo is unreliable in children. Three‐dimensional echocardiography (3DE) may reliably assess EOA but has not been studied in children. We assessed measurement of aortic valve EOA by 3DE in children with AS before and after balloon aortic valvuloplasty and compared results with change in aortic valve gradient. Methods: 3DE was performed at time of catheterization before and after balloon aortic valvuloplasty. Using 3DE multiplanar review mode, valve annulus diameter, area, and EOA were measured and compared with change in aortic gradient and degree of aortic insufficiency. Results: Twenty‐four 3DE studies in 12 children (mean age 4.4 ± 5.0 years) were analyzed. EOA was measurable in all. Catheter peak gradient decreased from 45 ± 10 to 26 ± 17 mmHg (P = 0.0018). 3DE EOA increased after balloon aortic valvuloplasty (0.59 ± 0.52 cm² vs 0.80 ± 0.70 cm²; P = 0.03), without change in valve diameter. EOA change correlated with change in peak (r = 0.77; P = 0.005) and mean (r = 0.60; P = 0.03) aortic valve gradient post balloon aortic valvuloplasty. Conclusion: 3DE facilitates EOA measurement in pediatric AS and correlates with change in aortic valve gradient after balloon valvuloplasty. (Echocardiography 2012;29:484‐491)     

Neonatal isolated critical aortic valve stenosis: balloon valvuloplasty or surgical valvotomy

BACKGROUND: Open surgical valvotomy and transcatheter balloon valvuloplasty are recognised treatments for neonatal critical aortic stenosis. METHODS: A retrospective analysis was undertaken of all newborns with critical aortic valve stenosis between 1990 and 2000 presenting to a tertiary centre and who required intervention. The initial catheter and surgical intervention was generally based on the preference of the attending cardiologist and the anatomy of the aortic valve and in consultation with the cardiothoracic surgeon. The two groups were therefore not strictly comparable. Twelve were subjected to balloon valvuloplasty and thirteen to surgical valvotomy at a median age of 11 days (2-42 days) and 3.5 days (1-19 days) respectively. There was no significant difference in the timing of the procedure, weight of the infant, aortic annulus or left ventricular dimensions in either group. RESULTS: There was one unrelated hospital death in the balloon group compared to two in the surgical group both of whom had endocardial fibroelastosis. Mild to moderate aortic regurgitation was seen after both procedures. Four patients in the balloon valvuloplasty group, developed femoral artery thrombosis and two had cardiac perforation that resolved with non operative management. The mean Doppler gradient was reduced from 44+/-14 mmHg to 13.4+/-5 mmHg (p<0.01) in the valvuloplasty group compared to a reduction from 42+/-15 mmHg to 27+/-8 mmHg (p<0.05) in the surgical group. Five patients in the balloon group required re-intervention within 3 weeks to 21 months after the initial procedure. Two patients in the surgical group required a pulmonary autograft and Konno Procedure 3 and 5 years following surgical valvotomy. CONCLUSION: Both aortic valvuloplasty and valvotomy offered effective short and medium term palliation. Balloon valvuloplasty patients had a higher re-intervention rate but shorter hospital and intensive care stay, reduced immediate morbidity and were associated with less severe aortic regurgitation.     

Percutaneous balloon valvuloplasty for aortic stenosis: improved quality of life for elderly patients

From September 1986 to September 1987 percutaneous balloon valvuloplasty was performed in 20 patients with critical aortic stenosis. The mean age was 74, range 54 to 90 years. Two patients were NYHA class II, 10 patients were functional class III and eight were class IV. After percutaneous balloon valvuloplasty, using 15, 18 or 20 mm diameter balloon catheters via a 14F vascular sheath from the femoral artery, one patient was class I, 11 class II and seven were class III. One patient, who had only minimal reduction of aortic valve gradient from 120 to 100 mmHg, remained in class IV. The systolic gradient was reduced by 50% from 70 +/- 26 (+/- SD) to 35 +/- 22 mmHg (P less than 0.001) after valvuloplasty and the aortic valve area increased by 43% from 0.51 +/- 0.12 to 0.73 +/- 0.25 cm2 (P less than 0.001). There were no procedural or 30 day deaths nor any embolic events. Percutaneous balloon valvuloplasty is a relatively safe and effective treatment for the older adult with symptomatic aortic stenosis.     

Balloon Aortic Valvuloplasty in the First Year of Life

Between February 1988 and September 1993 balloon aortic valvuloplasty was attempted in 33 consecutive patients in the first year of life: 20 patients (61%) were younger than J month. Major associated anomalies such as mitral stenosis, coarctation, and hypoplastic left ventricle were found in 11 cases (33%). The balloon dilation of the aortic valve was accomplished through the right carotid cut-down approach in neonates and patients with body weight < 5 kg, through a percutaneous femoral approach in the others; the procedure was completed in all. The peak systolic gradient across the aortic valve measured at catheterization fell from 80 ± 33 mmHg (range 25–165) before the dilation to 27 ± 17 mmHg (range 0–65), afterwards (P < 0.0001). The left ventricular ejection fraction increased from 44%± 26% to 61%± 17%, 24–48 hours after the procedure (P < 0.0001). Aortic insufficiency developed in 17 cases, being moderate in 2, mild in 6, and trivial in 9. Seven patients (21%), all in the first month of life, died within 30 days from the valvuloplasty; major associated anomalies were present in six; the death was due to a procedure related complication in one. No mortality was observed among the patients undergoing valvuloplasty beyond the first month of life. On follow-up (6 months to 6 years) aortic restenosis occurred in 3 cases; 1 was treated by surgical valvotomy, 2 by repeat balloon valvotomy; in another 2 cases, a subvalvular aortic obstruction developed and was relieved by surgical resection. There was no late mortality. Thus, balloon valvuloplasty appears to be an effective palliation for critical aortic stenosis in infancy. Early mortality is mainly related to associated anomalies.     

Balloon aortic valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry

Data from 204 children and infants who underwent aortic balloon valvuloplasty between 1982 and 1986, reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry, were reviewed. Valvuloplasty was successful in 192 of 204 children, reducing the peak systolic left ventricular ejection gradient from 77 +/- 2 to 30 +/- 1 mm Hg, p less than 0.001. The same degree of aortic stenosis gradients reduction was noted in both the 38 children under 1 year of age and in the 166 children over 1 year of age. Significant complications included death, aortic regurgitation and femoral artery thrombosis or damage. The incidence of these complications correlated with the age of the child, the ratio of valvuloplasty balloon size/anulus size, or both. The data suggest that percutaneous balloon valvuloplasty provides effective acute relief of valvar aortic stenosis in both infants and children. However, long-term follow-up data are necessary before balloon valvuloplasty can be established as a treatment of choice for congenital valvar aortic stenosis.     

Gradient reduction, aortic valve regurgitation and prolapse after balloon aortic valvuloplasty in 32 consecutive patients with congenital aortic stenosis

From 1986 to 1988, balloon aortic valvuloplasty was performed in 32 patients with congenital valvular aortic stenosis. The patients ranged in age from 2 days to 28 years (mean +/- SD 8.3 +/- 5.9). One balloon was used in 17 patients and two balloons were used in 15 patients. Immediately after valvuloplasty, peak systolic pressure gradient across the aortic valve decreased significantly from 77 +/- 27 to 23 +/- 16 mm Hg (p less than 0.01), a 70% reduction in gradient. At early follow-up study (4.1 +/- 3.3 months after valvuloplasty), there was a 48 +/- 20.5% reduction in gradient compared with that before valvuloplasty, and at late follow-up evaluation (19.2 +/- 5.6 months), a reduction in gradient of 40 +/- 29% persisted. Echocardiography showed evidence of significantly increased aortic regurgitation in 10 patients (31%) and aortic valve prolapse in 7 patients (22%). There was no correlation between the balloon/anulus ratio and the subsequent development of aortic regurgitation or prolapse. In fact, no patient who showed a significant increase in aortic regurgitation had had a balloon/anulus ratio greater than 100%. It is concluded that balloon aortic valvuloplasty effectively reduces peak systolic pressure gradient across the aortic valve in patients with congenital aortic stenosis. However, subsequent aortic regurgitation and prolapse occur in a significant number of patients, even if appropriate technique and a balloon size no greater than that of the aortic anulus are used.     

Valvuloplasty of congenital aortic stenosis

While percutaneous transluminal valvuloplasty has largely replaced open commissurotomy for congenital pulmonary valve stenosis, the experience with valvuloplasty for congenital aortic stenosis is limited. Between August, 1984 and June, 1987, a total of 27 valvuloplasties were carried out in 25 patients with congenital aortic stenosis; nine infants and 16 patients ranging in age from four to 25 years (mean age 13 +/- 6 years). The indication for valvuloplasty was established on the basis of congestive heart failure or severe stenosis in infants and in the presence of a pressure gradient in excess of 60 mm Hg in those older than one year of age. In eight infants, valvuloplasty reduced the transvalvular pressure gradient from 73 +/- 5 to 36 +/- 9 mm Hg (p less than 0.01; Figure 1). In the 16 patients older than one year of age, the pressure gradient was reduced from 93 +/- 25 to 49 +/- 15 mm Hg (p less than 0.01; Figure 2). The size of the balloon chosen was based on two-dimensional echocardiographic measurements of the aortic ring diameter from inner edge to inner edge. In infants, coronary artery dilating catheters and balloon catheters with an inflated diameter of 4.2 to 8 mm were employed; in children, the balloon diameter selected was 1 to 3 mm less than that of the valve ring diameter, in 15 cases a single-balloon catheter and in one a trefoil catheter. After puncture of the femoral artery and retrograde advancement of a guide-wire into the left ventricle, the balloon catheter was positioned via the guide-wire across the aortic valve.(ABSTRACT TRUNCATED AT 250 WORDS)     

Carotid artery approach for balloon dilation of aortic valve stenosis in the neonate: a preliminary report

Balloon valvuloplasty in neonates with severe aortic valve stenosis is limited by difficulties in catheter manipulation around the arch and across the valve and by the risk of femoral artery complications. A right common carotid artery cutdown was utilized for balloon aortic valvuloplasty in five neonates 1 to 20 days of age, weighing 3.1 to 3.9 kg. Standard balloon valvuloplasty was performed through a 6F sheath inserted in the right carotid artery. The arteriotomy was repaired at the end of the procedure. Mean left ventricular systolic pressure was reduced from 142 to 97 mm Hg, with a decrease in mean peak systolic pressure gradient from 76 to 33 mm Hg. Only one patient developed mild aortic regurgitation. One patient with a hypoplastic left ventricle died, and one patient required open valvotomy. All four survivors have a normal carotid pulse and no neurologic sequelae. Two of these patients required repeat balloon dilation to treat residual aortic valve stenosis at 8 and 10 months of age, respectively. Balloon valvuloplasty using a carotid artery approach is feasible and was safe in five neonates with severe aortic valve stenosis.     

经皮球囊扩张术治疗儿童先天性主动脉瓣狭窄

目的探讨儿童先天性主动脉瓣狭窄（aortic stenosis,AS）球囊扩张的治疗效果。方法选择21例单纯性AS患儿,其中1例合并动脉导管未闭（PDA）,3例因导管难以进入左室,2例左心导管测跨主动脉瓣压力阶差（AP）小于50mmHg（1mmHg=0．133kPa）而未扩张,其余16例采用与瓣环直径之比为0．98±0．04（0．92～1．10）的球囊扩张治疗,合并的PDA予Amplatzer伞片封堵治疗。结果即刻AP下降至原AP50％以上者13例,占81．25％;下降至原AP40％～50％者2例。随访6个月～5年,3例患儿AP回复至50mmHg以上,分别予第二次扩张或外科手术后好转。无一例轻度以上的主动脉瓣反流。结论AS经皮球囊扩张术结果表明,球囊扩张对单纯性AS具有一定疗效,由于其非开胸、方便且较安全地缓解左心室流出道梗阻,从而可替代或延缓外科开胸瓣膜切开术,为AS的治疗提供了另外一种选择。     

Management of pediatric patients with isolated valvar aortic stenosis by balloon aortic valvuloplasty

Moderate to severe aortic stenosis in children requires an initial procedure to improve the stenosis and often additional procedures for recurrent stenosis or aortic insufficiency before adulthood. The purpose of this study was to evaluate children who underwent balloon valvuloplasty and were followed with a specific management plan. Twenty-two children with aortic stenosis underwent balloon valvuloplasty and were followed on a regular basis. Repeat valvuloplasty was performed if indicated. The initial gradient was reduced from 63 ± 9 mmHg to 28 ± 8 mmHg (P<0.001). There were no deaths and only one major complication, which had no sequelae. Average follow-up was 61 ± 23 months. Three patients required valve replacement 39–76 months after valvuloplasty for progressive insufficiency. Seven patients underwent successful repeat valvuloplasty. The overall probability of survival without surgical intervention was 75% at 100 months. Balloon valvuloplasty is an effective intermediate palliation for aortic stenosis and is an acceptable alternative to surgical valvotomy. Repeat valvuloplasty is successful without additional risk. In a subgroup of patients, aortic insufficiency is progressive and will require surgical intervention. © 1996 Wiley-Liss, Inc.     

Patient age and results of balloon aortic valvuloplasty: the Mansfield Scientific Registry experience. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators

Patients enrolled in the Mansfield Scientific Aortic Valvuloplasty Registry were followed up a mean of 7 months after balloon aortic valvuloplasty. Results were compared for patients less than 70, 70 to 79 and greater than or equal to 80 years of age at time of valvuloplasty. As assessed by aortic valve area indexed to body surface area, stenosis was more severe in the older patients and the incidence of congestive heart failure was also greater in those aged greater than or equal to 80 years. The results of valvuloplasty were comparable in all three age groups, and indexed final valve area was not significantly different among the groups. In-hospital mortality ranged from 4.2% to 9.4%, but this and other complications were not significantly different among the groups. Total 7 month mortality was 23%. As performed in this registry study, balloon aortic valvuloplasty produced similar results in older and younger patients, despite initially more severe disease in the older patients.     

Double vs single balloon technique for aortic balloon valvuloplasty

Percutaneous aortic valvuloplasty using a single dilating balloon has been associated with significant but modest reduction in transvalvular pressure gradient and increase in valve area. The balloon diameter is usually 20 mm or smaller to avoid disruption of aortic root structure and to permit forward blood flow during inflation. To evaluate the safety and efficacy of valvuloplasty using a combination of balloons with larger maximum inflated diameters, we compared results of aortic valvuloplasty in 21 patients using either the single or double balloon technique. Mean maximum inflated balloon diameter was 19.4 mm +/- 1.4 for the single balloon technique, while the mean sum of diameters for the simultaneous double balloon technique was 36.3 mm +/- 3.9. The mean age, aortic annulus diameter, and predilatation aortic valve area were not different among groups. Mean aortic transvalvular gradient reduction and mean aortic valve area increase were greater for the double balloon technique. The procedure was well tolerated with no major complications. No change in the degree of aortic regurgitation was noted. The double balloon technique for aortic valvuloplasty is safe and more effective at improving aortic valve area and transvalvular gradient than the conventional single balloon technique.     

Anterograde balloon valvuloplasty for the treatment of neonatal critical valvar aortic stenosis.

We report our experience with anterograde balloon valvuloplasty in 17 neonates treated between November 1996 and June 2001 for critical aortic stenosis. Patients with hypoplastic left heart syndrome were excluded. Anterograde balloon valvoplasty of the aortic valve was possible in all 17 patients. The mean peak systolic gradient prior to cardiac catheterization was 73 mm Hg (range, 30-117 mm Hg) and decreased to 37 mm Hg (range, 21-60 mm Hg) after the dilation. Aortic regurgitation after balloon valvoplasty was absent or mild in 14/17 patients, moderate in 2 patients, and severe in 1 patient. There was no mortality or echocardiographic evidence for aortic cusp perforation or mitral regurgitation associated with the procedure. Redilation was necessary in 3/17 patients. Two patients are awaiting elective Ross operation. One patient with endocardial fibroelastosis died at 11 months of age. Anterograde balloon valvoplasty can be safely and effectively performed to palliate neonates with critical aortic valve stenosis.     

Intermediate-term effectiveness of balloon valvuloplasty for congenital aortic stenosis. A prospective follow-up study.

BACKGROUND. Percutaneous balloon valvuloplasty has proven to be acutely effective in the treatment of congenital valvar aortic stenosis; however, the intermediate- and long-term effectiveness of the procedure remain to be documented. METHODS AND RESULTS. To assess the intermediate-term effectiveness of balloon valvuloplasty, repeat catheterization was performed in 27 of 30 children 1.7 +/- 0.1 years after balloon valvuloplasty for congenital aortic stenosis (AS). In 33 children the peak AS gradient was reduced acutely by 55% from 77 +/- 4 to 35 +/- 3 mm Hg (p less than 0.001), and left ventricular systolic pressure was reduced from 176 +/- 4 to 138 +/- 4 mm Hg (p less than 0.001). Despite a technically adequate valvuloplasty procedure, three patients had inadequate relief of obstruction and required complex surgical intervention. Twenty-seven of the 30 patients available for late reevaluation (90%) enrolled in the follow-up study. The peak AS gradient remained significantly reduced compared with that present before valvuloplasty (29 +/- 3 versus 77 +/- 4 mm Hg, p less than 0.001). Furthermore, there was no difference in peak AS gradient at follow-up compared with that immediately after valvuloplasty. The greatest increase in gradient at reevaluation was 14 mm Hg. Twenty of 27 patients (74%) had no change in the degree of aortic insufficiency at follow-up compared with that present before valvuloplasty. At follow-up, 16 patients had no aortic insufficiency at all, and only two had moderate-to-severe (3-4+) insufficiency. Femoral artery injury was documented in four patients, three of whom were under 12 months of age at valvuloplasty. CONCLUSIONS. Balloon aortic valvuloplasty provides safe and effective intermediate-term gradient relief without early restenosis in children and adolescents with congenital AS.     

Balloon valvuloplasty for critical aortic stenosis in the newborn: influence of new catheter technology

Between 1986 and July 1990, balloon valvuloplasty was attempted in eight newborns (less than 28 days of age) with isolated critical aortic valve stenosis. Balloon valvuloplasty could not be successfully accomplished in any of the three infants presenting before 1989. Since March 1989, when improved catheter technology became available, all five neonates presenting with critical aortic stenosis were treated successfully by balloon valvuloplasty. A transumbilical approach was utilized in all four infants in whom umbilical artery access could be obtained. One newborn who was 25 days of age underwent transfemoral balloon valvuloplasty. Balloon valvuloplasty was immediately successful in all five newborns, as evidenced by a decrease in valve gradient and improvement in left ventricular function and cardiac output. Peak systolic gradient was reduced by 64% from 69 +/- 8 to 25 +/- 3 mm Hg (p = 0.005). Left ventricular systolic pressure decreased from 128 +/- 9 to 95 +/- 9 mm Hg (p = 0.02) and left ventricular end-diastolic pressure decreased from 20 +/- 2 to 11 +/- 1 mm Hg (p = 0.02). Moderate (2+) aortic regurgitation was documented in two infants after valvuloplasty. The time from first catheter insertion to valve dilation averaged 57 +/- 14 min (range 26 to 94) and the median length of the hospital stay was 4 days. With the use of recently available catheters, the transumbilical technique of balloon valvuloplasty can be performed quickly, safely and effectively in the newborn with critical aortic stenosis. It does not require general anesthesia, cardiopulmonary bypass or a left ventricular apical incision and it preserves the femoral arteries for future transcatheter intervention should significant aortic stenosis recur.