Intermediate-term follow-up results of pulmonary balloon valvuloplasty in children

Twenty-two patients (10 boys, 12 girls) with pulmonary valve stenosis whose mean age was 6.7 +/- 4.1 years (range 1 to 14 years) at time of the procedure underwent balloon dilatation angioplasty. All patients had isolated pulmonary valve stenosis with no associated cardiac anomalies, and their pulmonary valvular gradients were greater than 50 mmHg. Diagnostic catheterization and balloon valvuloplasty were performed during the same procedure. The mean peak systolic pressure gradient before balloon dilation of 95.8 +/- 29.5 mmHg (54-163 mmHg) was reduced to 30.2 +/- 15.8 mmHg (7-64 mmHg) after balloon dilation (p < 0.001). A linear relation was found between the predilation pressure gradient and the pressure gradient drop (r = 0,86; SE: 28.94) (y = 0.89x - 12.11). Following a successful valvuloplasty, the mean peak systolic pressure in the right ventricle decreased from 119.0 +/- 30.4 mmHg (71-184 mmHg) to 55.2 +/- 16.9 mmHg (29-97 mmHg) (p < 0.001). Continuous wave Doppler was used for follow-up. Pressure gradients were estimated in 14 patients between one and 29 months after the dilation 120 +/- 7.1 months). The mean follow-up gradient was 19.0 +/- 6.0 mmHg (11-32 mmHg). No important complication was noted in the immediate course or throughout the follow-up period, but four patients (28.6%) had minimal pulmonary insufficiency. In conclusion, these data confirm that balloon dilation in valvular pulmonary stenosis is safe and effective, and suggest that stenosis does not recur.     

Doppler estimation of pressure gradient in pulmonary stenosis: Maximal instantaneous vs peak-to-peak,vs mean catheter gradient

Summary We undertook a study to identify the hemodynamic significance of a Doppler-derived gradient across a stenotic pulmonary valve. Furthermore, we attempted to define the optimal plane for velocity data acquisition. A total of 17 children with valvar pulmonary stenosis were evaluated using Doppler echocardiography. Flow-velocity profiles were obtained from both the parasternal and subxiphoid windows. Ten of 17 patients were studied before and after balloon valvotomy. Therefore, 27 different transvalvar gradients were assessed by Doppler and these data were compared with the catheter-derived maximal instantaneous, peak-to-peak, and mean pressure gradients. The maximal Doppler gradient correlated well with the catheter-derived peak-to-peak pressure gradient (r=0.95) and catheter maximal instantaneous pressure gradient (r=0.95). Although these correlation coefficients were similar, the Doppler maximal gradient consistently overestimated the peak-to-peak catheter gradient by as much as 25%–40%. Such an overestimation was not observed when we compared the maximal Doppler gradient with the catheter-derived maximal instantaneous gradient. Moreover, the regression line of the latter comparison closely approximated the line of identity. The correlation coefficient between Doppler mean and catheter mean gradients was only 0.91. Doppler velocities were best derived when multiple transducer positions were employed to interrogate pulmonary artery velocity.     

Successful percutaneous balloon valvuloplasty of the aortic valve in an infant

Summary Percutaneous balloon aortic valvuloplasty was attempted in an eight-month-old infant with severe aortic valve stenosis. The procedure resulted in a fall in the resting transvalvular systolic pressure gradient from 106 mmHg to 40 mmHg and no aortic regurgitation. Clinical and Doppler echocardiography findings suggest persistent improved status 3 1/2 months after the procedure.     

Brock transventricular pulmonary valvotomy in patients with pulmonary stenosis: Long-term results

Summary As no long-term results of a blind opening up of the pulmonary valve either by balloon valvoplasty or closed Brock valvotomy have been published, we examined the outcome of 12 patients with valvar pulmonary stenosis 17±5 (11–22) years after surgery. The Brock valvotomy had been carried out at a mean age of 3±2.8 (0.2–8.5) years. The mean pressure gradient across the right ventricular outflow tract had been 116±45 (75–97) mmHg at the catheter study or 106±43 (40–160) mmHg as measured intraoperatively. The right ventricular pressure after the Brock procedure was measured in the operating room in five patients as 46±15 (30–60) mmHg. Seven patients had been recatheterized at a mean age of 9.5±2 (7–12.7) years; at that time the gradient across the pulmonary valve had been 20±14 (10–37) mmHg. At a mean age of 21.7±3 (15–26) years these and five further patients were reexamined by echo Doppler. This time the pressure gradient across the pulmonary valve was 13±6 (7–20) mmHg. Moderate pulmonary incompetence was present in four and mild incompetence in eight patients; two had mild tricuspid insufficiency. All except one patient, who had suffered a cerebrovascular accident before surgery, were in NYHA functional class 1 and pursuing a profession. From these data we conclude that the blind opening-up of the pulmonary valve achieves excellent long-term palliation.     

Medical management of three asymptomatic infants with severe valvar Aortic stenosis

Summary We report on the clinical course and serial hemodynamic studies of three patients with severe valvar aortic stenosis diagnosed in the neonatal period. None of the children were symptomatic in the first year of life. In each case, a conservative initial management approach was adopted. Between the time of initial study (mean age, 1.8 months) and the follow-up at 12–27 months of age (mean, 14.3 months), mean left ventricular systolic pressure decreased from 151 to 125 mmHg, the mean peak systolic pressure gradient across the aortic valve decreased from 61 to 33 mmHg, and the mean calculated aortic valve area index increased from 0.24 to 0.60 cm²/m². One patient was operated on for symptoms that appeared at 14 months of age. The patient followed longest is now 5 years old, is growing well, has a normal electrocardiogram and an echo-predicted left ventricular systolic pressure of 128 mmHg. This experience suggests that not all asymptomatic neonates with severe valvar aortic stenosis require surgical intervention early in life. In some, the aortic valve orifice may increase in size with somatic growth and obviate the need for surgery in infancy or early childhood.     

Echocardiographic Follow-Up of Congenital Aortic Valvular Stenosis

We investigated the morphology of the stenotic aortic valve, the progression of the stenosis, and the onset and progression of aortic regurgitation (AR) in patients with congenital aortic valvular stenosis (AVS). The medical records of 278 patients with AVS were reviewed, with the patients with concomitant lesions besides AR excluded. Very mild aortic stenosis was defined as a transvalvular Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 4.9 ± 4.3 years (range, 3 days to 15 years), and 203 (73%) were male. The number of the cusps was determined with two-dimensional echocardiography in 266 patients (95%): unicuspid in 3 patients (1%), bicuspid in 127 patients (48%), and tricuspid in 136 patients (51%). A total of 192 of all patients were followed for 2 months to 14.6 years (mean 4.2 ± 3.3 years) with medical treatment alone. Among 72 patients with very mild stenosis at initial echocardiographic examination, 20% had mild, 3% moderate, and 1% severe stenosis after a mean period of 3.7 years. In 70 patients with mild stenosis at initial echocardiographic examination, 28% had moderate and 9% severe stenosis after a mean period of 5 years. Among 44 patients with moderate stenosis at initial echocardiographic examination, 36% had severe stenosis after a mean period of 3.7 years. Among 192 patients, 40% had AR (3% trivial, 28% mild, and 9% moderate) at initial echocardiographic examination. After a mean period of 4.2 years, 58% of the patients had AR (13 % trivial, 25% mild, 16% moderate, and 4% severe). There was not statistically significant difference between catheterization peak systolic gradients (47 ± 16 mmHg) and Doppler estimated mean gradients (45 ± 9 mmHg) (p = 0.53), whereas Doppler PSIGs (74.9 ± 15.7 mmHg) were higher than catheterization peak systolic gradients (p < 0.0001) in 25 patients who were studied in the catheterization lab. Patients with very mild stenosis may be followed with a noninvasive approach every 1 or 2 years, and an annual follow-up is suggested for patients with mild stenosis. Nearly one-third of patients with moderate stenosis at initial echocardiographic examination had severe stenosis after a mean period of 3.7 years. Therefore, we recommend, that patients with moderate stenosis undergo noninvasive evaluation every 6 months. Doppler estimated mean gradient is very useful in predicting the need for intervention in children with AVS.     

Significance of the Doppler-derived gradient across a residual aortic coarctation

Summary Few data exist which address the significance of the Doppler gradient across a residual narrowing in older children who have had a coarctation repaired. Therefore, we evaluated 11 patients with repaired aortic coarctation with and without residual obstruction by Doppler echocardiography. The Doppler-derived transcoarctation pressure gradient correlated poorly with catheter-measured peak-to-peak and catheter maximal instantaneous gradients when only the maximal velocity across the repair was utilized in the simplified Bernoulli equation, [r=0.73, standard of error of the estimate (SEE)=5.0 mmHg andr=0.56, SEE=7.6 mmHg, respectively]. However, when the precoarctation velocity was included in the simplified Bernoulli equation, the correlation between Doppler-derived and catheter-measured gradients became excellent. The maximal Doppler gradient correlated well with catheter peak-to-peak gradient (r=0.95, SEE=2.2 mmHg) and catheter maximal instantaneous gradient (r=0.94, SEE=3.2 mmHg). However, the maximal Doppler gradient slightly overestimated the catheter peak-to-peak gradient and underestimated the catheter maximal instantaneous gradient. The Doppler mean gradient showed excellent correlation with the catheter mean gradient (r=0.97, SEE=0.85 mmHg). Precoarctation velocities were generally twofold greater than published normals due to a spatial acceleration phenomenon. All subjects had residual hypoplasia of the transverse aorta such that its transverse diameter was 29% less than, and its crosssectional area was 50% less than, the ascending and descending aorta. Thus, it is apparent that proximal velocities must be accounted for in the modified Bernoulli equation in order to achieve an accurate gradient determination, and that this gradient is representative of a value that is between the maximal instantaneous and peak-to-peak catheter gradient. Flow acceleration within the transverse aorta may be related to significant reductions in luminal radius and crosssectional area.     

Serial Exercise Performance in Children with Surgically Corrected Congenital Aortic Stenosis

We examined serial exercise test performance in children with congenital aortic stenosis (AS) treated surgically compared to that of nonoperated children with mild to moderate AS. Maximal treadmill exercise data were assessed in 21 children 5.5 ± 3.8 years after aortic valve (AO) surgery. Patients had undergone the Ross procedure (n = 6) or previous aortic valvotomy, balloon valvuloplasty, or neonatal aortic valvotomy (n = 15). Follow-up treadmill tests were conducted 3.7 ± 2.8 years later. Data were compared to those of 19 nonoperated AS patients (mean gradient by echocardiogram <50 mmHg). These patients were exercised 3.6 ± 3.2 years apart. Endurance time, heart rate, systemic blood pressure, and electrocardiogram were compared as repeated measures between tests and to age- and sex-matched normative data. Postsurgical children with AS had normal endurance times despite low peak heart rates on the initial test, and they maintained endurance over time. Nonoperated children with mean AO gradients <50 mmHg also had normal endurance times on the initial test but increased endurance over 3.6 years. Children with operated and nonoperated AS were able to reach or exceed normal endurance times, which may make it difficult to achieve compliance to imposed activity restrictions in this population.     

Medium-term results of percutaneous transluminal balloon aortic valvuloplasty with Inoue balloon catheter for congenital aortic stenosis

Although percutaneous transluminal balloon aortic valvuloplasty (PTAV) has been performed for congenital aortic stenosis in infants and children for several years, its efficacy and the associated aortic regurgitation (AR) have not been widely discussed. Percutaneous transluminal balloon aortic valvuloplasty using an Inoue balloon catheter was performed for congenital aortic stenosis in 12 patients (4–16 years old) in this study. The systolic aortic valve pressure gradient ranged from 42 to 111 mmHg before PTAV and became < 50 mmHg immediately after PTAV in 10 cases (83%). Eight of these 10 patients had no increase in the gradient during subsequent observation for a period of 9–40 months. Aortic regurgitation increased immediately after PTAV in nine cases (75%). It increased from grade 1 to grade 2 in eight cases and from grade 1 to grade 3 in one patient; no significant enlargement of the left ventricular end-diastolic diameter and no significant change in the left ventricular end-diastolic pressure (LVEDP) or the cardiac index was observed during follow-up in these patients. There was a correlation between the diameter of the balloon and efficacy; an appropriate diameter was considered to be about 90% of the aortic annular diameter. Changes in the hemodynamic parameter after PTAV with an Inoue balloon were small in most patients and this procedure is considered to be a treatment that should be attempted prior to surgery for congenital aortic stenosis.     

自体心包修复儿童主动脉瓣狭窄9例病例系列报告

目的：总结并分析以自体心包修复儿童先天性主动脉瓣狭窄的手术方法和早期预后。方法：纳入2013年7月至2015年6月在复旦大学附属儿科医院行自体心包主动脉瓣成形术的先天性主动脉瓣狭窄患儿,收集患儿的一般资料,围手术期情况,并发症,术前、术中、术后和随访时的超声心动图资料。结果：符合本文纳入标准的9例患儿进入本文分析,男6例,女3例,年龄4月龄至9岁。术前超声提示,三叶式和二叶式主动脉瓣分别为3例和6例;重度狭窄6例,中度狭窄1例,重度狭窄伴中度返流2例。9例均以自体心包再造或扩大主动脉瓣瓣叶。术后即刻与术前超声心动图比较：主动脉瓣最大跨瓣压差［（31.6±9.4）mm Hg vs（73.0±22.2）mm Hg, P=0.000］和主动脉瓣平均跨瓣压差［（15.8±18.3）mmHg vs （35.8±18.3）mmHg, P= 0.004］均下降。术后随访24～48（32±8）月,无死亡和再干预病例,未见严重不良事件,未见主动脉瓣重度狭窄或重度反流、升主动脉狭窄或扩张、主动脉瓣瓣环狭窄与扩张、瓣膜脱垂或瓣膜赘生物病例;3例患儿分别在随访18、24和12个月时出现瓣叶活动僵硬,瓣叶开放不完全。术后左室后壁厚径均呈下降趋势;末次随访时,8例患儿左室后壁厚径Z值（Z-LVPWd）均下降至正常水平（＜2）。结论：以自体心包修复主动脉瓣可改善先天性主动脉瓣狭窄患儿的血流动力学,手术风险低,术后早期疗效可,避免或延缓儿童主动脉瓣置换术,减少施行ROSS手术机会。     

Aortic Stenosis: The Spectrum of Practice

There is significant variation in practice patterns in managing congenital aortic valve stenosis. Review of medical literature reveals no significant information regarding the current practice methods in the treatment of a simple lesion such as aortic stenosis (AS). Therefore, this survey-based study was conducted in an attempt to better understand the uniformity or heterogeneity of practice in treating AS. A questionnaire was prepared to evaluate the style of management of AS. This survey was designed to assess the practice of follow-up visitations, type and frequency of investigative studies, pharmacological therapy, and exercise recommendations. Questions about therapeutic intervention included those of timing and type of intervention. Questionnaires were sent to all academic pediatric cardiology programs in the United States (48 program) and selected international programs from Europe, Asia, and Australasia (19 program). The total number of surveys sent out was 67, and the total number of respondents was 25 (37%), 15 (31%) from the United States and 9 (53%) from outside the United States. The definition of moderate AS varied among respondents. The range provided for mild AS was identified as that with a peak-to-peak pressure gradient of < 25–30 mmHg, peak instantaneous Doppler gradient of < 36–50 mmHg, or mean Doppler gradient of < 25–40 mmHg. On the other hand, severe AS was defined as that with a peak-to-peak gradient of > 50–60 mmHg, peak instantaneous Doppler gradient of > 64–80 mmHg, or mean Doppler gradient of > 45–64 mmHg. In assessing follow-up patterns, 84% of respondents recommended seeing patients with mild AS annually, the longest time of follow-up listed in the questionnaire, whereas 20% suggested follow-up every 6 months. There was no consensus among survey centers regarding follow-up of patients with moderate AS. For severe AS, 16% recommend immediate intervention, 16% arrange follow-up every 6 months, and 56 and 28% recommend follow-up in 3 and 1 month(s), respectively. In making the decision to proceed with biventricular versus univentricular repair in patients with AS in the neonatal period, many factors were considered. Ninety-two percent of respondents rely on mitral valve z score, 84% on aortic valve z score, 52% on left ventricle length, 48% on the presence of antegrade ascending aorta flow, and only 32% considered significant endocardial fibroelastosis as a factor. Rhodes score was used by 20% of respondents in decision making regarding the approach to management of this subset of AS. This study shows that there is consensus in the management of mild and severe forms of AS. As expected, disagreement is present in the definition, evaluation, and therapy of moderate aortic valve stenosis. There is a tendency for catheter intervention except in the presence of dysplastic aortic valve or moderate to severe aortic regurgitation. There is also disagreement regarding methods used to determine biventricular versus univentricular repair of a borderline hypoplastic left heart.     

Persistent truncus arteriosus operated during infancy: Long-term follow-up

Summary Between July 1974 and October 1988 19 consecutive infants (mean age 2.7 months, range 5 days to 11.7 months) underwent surgical correction for persistent truncus arteriosus by one surgeon (J.L.M.). Continuity between the right ventricle and pulmonary arteries was achieved with an antibiotic-sterilized aortic homograft (diameter 13–18 mm) together with patch closure of the ventricular septal defect. There were 3 early postoperative deaths (16%): 1 patient had severe aortic regurgitation, the other 2 had preoperative cardiac arrests. Of the latter, 1 had suffered severe cerebral damage, and the other developed recurrent pulmonary hypertensive crises and low cardiac output. The 16 survivors have been followed for 3.1–17.3 years (mean 7.8 years). Four patients required subsequent replacement of the homograft for stenosis, aortic valve replacement for regurgitation, or both (3.0, 4.0, 8.5, and 12.0 years postoperatively). Of the 16 survivors, 15 are in NYHA class I. Of the 13 patients who have not had aortic valve surgery, 9 have no evidence of stenosis or regurgitation. In the 14 children with the original homograft the median of the residual peak gradient across the right ventricular outflow tract is 15 mmHg (range 10–40 mmHg), and no patient has severe homograft regurgitation at follow-up. Repair of persistent truncus arteriosus has been achieved with low early mortality and no late mortality, which reflects excellent long-term function of the homograft. Furthermore, if truncal valve function is good at presentation, patients are unlikely to require aortic valve surgery.     

Long-term Results of Balloon Pulmonary Valvuloplasty in Children with Congenital Pulmonary Valve Stenosis

Objective

Immediate, short and midterm outcome of balloon pulmonary valvuloplasty are well known, but there is limited information on long term results. We report long term results of 2–13. 5 years follow up of balloon pulmonary valvuloplasty in children.

Methods

From June 1998 to January 2012 sixty consecutive patients (33 females, 27 males) with moderate to severe valvar pulmonary stenosis (right ventricular to pulmonary artery pressure gradient greater than 50 mmHg) were considered for balloon valvuloplasty. The gradient was measured pre and immediately post–valvuloplasty at catheterization, and then by echocardiography at follow up. Follow up studies were performed 2–13.5 years (mean±SD; 7.1±2.5 years, median: 5.5 years) after procedure, by Doppler echocardiography in all patients and catheterization and angiography in two patients.

Findings

Balloon pulmonary valvuloplasty BPV was successful in 53 of 60 (88.3%) patients whereas surgical valvotomy was necessary in 6 to 60 (10%). There was one immediate death due to perforation of the right ventricular outflow tract. Pulmonary valve systolic pressure gradient decreased from 83.3±32.1 to 19.3±14.2 mmHg immediately after BPV and to 12.3±6.6 mmHg at late follow up (P<0.001). Pulmonary insufficiency was noted in 20 (38%) patient at short–term, but it was demonstrated in 17 (32%) at late follow up. A second valvuloplasty was performed in two (3.8%) patients presenting with re-stenosis.

Conclusion

The short, intermediate and long-term outcomes of pulmonary balloon valvuloplasty in children are excellent. Therefore it can be considered as the treatment of choice for children with pulmonary valve stenosis.     

Doppler evaluation of femoral arteries in children after aortic balloon valvuloplasty or coarctation balloon angioplasty

Summary To assess long-term femoral artery complications after aortic balloon valvuloplasty or coarctation balloon angioplasty, we examined 19 children who were 3 weeks to 21 years old (mean 7.6 years) at the time of catheterization. Two-dimensional and Doppler echocardiographic examinations of the common, superficial, and deep femoral arteries were performed at an average of 2.0 years after balloon dilatation. Pulsatility index (PI) was calculated as the maximum velocity minus the minimum velocity divided by the mean velocity. No patient was suspected clinically of having peripheral arterial disease prior to the echocardiographic examination. Fourteen patients had normal femoral arteries. Of these, 10 had normal two-dimensional and Doppler echocardiographic examinations of both femoral arteries. These patients had triphasic flow patterns (forward in systole, reverse in early diastole, forward in middiastole) and Pls of 3.7–41.6 (mean 9.5). Four of the 14 normal patients had abnormal pulsed Doppler examinations showing continuous forward flow and low Pls (1.7–3.5) reflecting residual coarctation (10–30 mmHg gradients). Five patients had abnormal femoral arteries. Of these, two had no visible obstruction by two-dimensional echocardiography and color-flow imaging but had abnormal pulsed Doppler patterns (continuous forward flow and low Pls of 2.5 and 2.9) only on the side of the balloon catheter insertion. Three of the five abnormal patients had visible obstructions by two-dimensional echocardiography and color-flow imaging and had abnormal pulsed Doppler patterns (continuous forward flow and low Pls from 1.1–3.6). One of these three had bilateral occlusions of the common femoral arteries with multiple collateral vessels; the second had occlusion of the right common femoral artery; and the third had 1–2-cm long severely narrowed segments in both common femoral arteries. All three children were <1 year old at the time of catheterization and had balloon catheter insertions in the affected arteries.Echocardiography provides a useful technique for detection of clinically unsuspected but significant arterial obstruction after balloon dilatation. Thus, echocardiographic examinations of the femoral arteries should be performed on all children after balloon dilatation, particularly those <1 year old at the time of the procedure.     

Transcarotid Balloon Valvuloplasty with Continuous Transesophageal Echocardiographic Guidance for Neonatal Critical Aortic Valve Stenosis: An Alternative to Surgical Palliation

Neonatal critical aortic valve stenosis is a life-threatening malformation if untreated. Before the late 1980s, the preferred treatment was surgical valvotomy; however, operative mortality was high. Early reports of transcatheter balloon dilation were encouraging, although femoral artery damage and aortic valve insufficiency were procedural limitations. With new balloon catheter technology, transumbilical, transvenous, and transcarotid approaches have been advocated, although a comparison with recent surgical results has not been performed. We compared all neonates who presented to our institution since 1985 with the diagnosis of critical aortic stenosis. Ten patients underwent surgical transventricular valvotomy and 13 patients underwent balloon valvuloplasty via a right carotid cutdown with continuous transesophageal echocardiographic guidance. Prior to intervention, all patients had either left ventricular dysfunction, an aortic valve gradient >100 mmHg, significant mitral valve insufficiency, and/or ductal dependent systemic blood flow. All patients had successful relief of aortic valve obstruction with normalization of left ventricular function and successful discontinuation of prostaglandin E₁. Use of continuous transesophageal echocardiographic guidance resulted in fluoroscopic exposure of only 12 ± 8 minutes. At the latest follow-up, a similar proportion of patients has required additional aortic valve procedures (38% vs 25%) and overall mortality (20% vs 15%) is similar. In the transcarotid group, 9 of 13 patients (69%) have a normal appearing right carotid artery by Duplex imaging, and no neurologic events have been reported. Balloon aortic valvuloplasty via a right transcarotid approach is safe, simplifies crossing the valve, and is effective for the initial palliation of neonatal critical aortic stenosis. The use of transesophageal echocardiographic guidance reduces fluoroscopy exposure, enables accurate assessment of hemodynamics without catheter manipulation or angiography, and avoids femoral artery injury.     

Retrograde transfemoral catheterization of the left ventricle in children with aortic valve stenosis

Summary In 19 consecutive children with aortic valve stenosis, the left ventricle was entered retrogradely with a Gensini catheter guided by a tip-deflector guidewire which could produce any desirable degree of curvature at its tip. In all patients, the aortic valve was retrogradely traversed for measurement of the pressure gradient (mean gradient=53 mmHg, mean valve area=0.36 cm²), and left ventriculography. No complications were encountered. This technique is safe, simple, and effective in retrograde catheterization of the left ventricle in children with aortic stenosis.     

Balloon valvuloplasty for congenital heart disease: Immediate and long-term results of multi-institutional study

BACKGROUND AND OBJECTIVES: Several studies have been reported in Japan. However, the reports consist of small series at individual institutions. We evaluated the immediate to long-term results of balloon valvuloplasty (BVP) of congenital pulmonary and aortic stenosis at multi-institutions in Japan. METHODS AND RESULTS: Immediate and follow-up data were obtained from eight institutions in Japan. In our series of 172 cases of pulmonary valvuloplasty excluding critical pulmonary stenosis, the mean pressure gradient decreased immediately after BVP from 61+/-27 mmHg to 28+/-20 mmHg and the reduced gradient continued at follow-up in most cases. The BVP for critical pulmonary stenosis could be accomplished in 35 of 39 patients. The mean right ventricular systolic pressure decreased from 102+/-29 mmHg to 62+/-23 mmHg. One of them required the surgical operation for perforation of the right ventricular outflow tract. In BVP for congenital aortic valvular stenosis of 77 cases excluding critical aortic stenosis, the mean pressure gradient decreased immediately after BVP from 68+/-24 mmHg to 34+/-23 mmHg. Thirty-one cases (55%) were free from any interventions in long-term follow-up. The BVP for critical aortic stenosis was performed in 29 neonates. The overall mortality rate was 34% and 24% of the patients required repeat intervention. The remaining 42% was free from any interventions. CONCLUSIONS: Balloon valvuloplasty for congenital pulmonary valvular stenosis is a safe and effective procedure and the initial treatment of choice. In spite of an occasional major complication, BVP for critical pulmonary stenosis is effective in many infants. Balloon aortic valvuloplasty is palliative. However, this procedure has the efficacy in deferring the surgical intervention. Balloon valvuloplasty for neonatal critical aortic stenosis is a useful method to recover from serious conditions.     

Percutaneous Balloon Valvuloplasty of Both Pulmonary and Aortic Valves in a Neonate with Pulmonary Atresia and Critical Aortic Stenosis

We report a case of hydrops fetalis originating from critical aortic stenosis and pulmonary atresia with intact ventricular septum that was diagnosed in utero by echocardiography. We performed a percutaneous balloon valvuloplasty of the bilateral semilunar valves on the 2nd day after birth. We used a stiff-ended guidewire to perforate the pulmonary valve. After valvuloplasty, the aortic valve pressure gradient decreased from 55 to 25 mmHg. The procedure was successful, and the patient’s heart condition improved.     

Percutaneous transvenous balloon mitral valvuloplasty: mid-term results in adolescents

Six patients with mitral valve stenosis underwent percutaneous balloon mitral valvuloplasty (PBMV) in our department between November 1992 and December 1997. Five patients had rheumatic mitral valve stenosis and one had congenital mitral valve stenosis and Eisenmenger's syndrome with patent ductus arteriosus (PDA). Functional status before PBMV was class IV in two patients, class III in two patients, and class II-III in two patients, as classified by the New York Heart Association (NYHA). The mean diastolic pressure gradient across the mitral valve measured during heart catheterization before and immediately after PBMV was 18.8 +/- 10.42 and 9.4 +/- 7.7 mmHg, respectively (p < 0.01). The patients were followed for a mean period of 36.6 +/- 8.5 months (range 12 to 72 months) after the procedure. During follow-up, post PBMV mean diastolic transmitral gradient measured by color Doppler echocardiography decreased from 19.3 +/- 11.16 to 7.43 +/- 7.3 mmHg (p < 0.01) and the mitral valve area increased from 1.09 +/- 0.7 to 3.1 +/- 0.9 cm2 (p < 0.002). Functional capacity showed improvement to NYHA class I in four patients, to class II-III in the patient with congenital mitral valve stenosis and Eisenmenger's syndrome with PDA and to class II in one patient with severe mitral valve calcification in whom restenosis occurred three years after PBMV. Percutaneous balloon mitral valvuloplasty PBMV can achieve very good short- and mid-term results in relieving symptomatic rheumatic mitral valve stenosis.     

Left ventricular function and myocardial mass after aortic valvotomy in infancy

Summary Eighteen of 25 survivors of aortic valvotomy in infancy were reinvestigated by cross-sectional echocardiography a mean of 7.5 (2.3–13.4) years after surgery. They had been operated at a median age of 38 (5–330) days. At the follow-up examination the gradient across the aortic valve was 41±19 (15–85) mmHg and the ejection fraction was 0.73±0.10 (0.48–0.84). Left ventricular (LV) end-diastolic volume was 66±17 (33–191) ml/m². LV mass was 96±36 (44–204) g/m² and the LV mass volume index (LVMVI) (mass divided by end-diastolic volume) was 1.43±0.4 (0.9–2.28). Eleven of 18 patients had an abnormally high mass volume index compared with 95 age-matched controls with structurally normal hearts. The correlation between the residual pressure gradient across the aortic valve and mass volume index yielded anr value of 0.75 (p<0.0004). One patient had been reoperated and underwent resection of a subaortic stenosis 4 years after the initial operation. Four patients with a resting gradient of more than 50 mmHg and one with grade 4 aortic regurgitation are scheduled for further surgical treatment.We conclude that, although LV function was normal in most patients who underwent aortic valvotomy in infancy, LV mass remains elevated in a significant number of patients, who may remain at risk of developing subendocardial ischemia.