Balloon Pulmonary Valvuloplasty: Results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry Paul Stanger, MD, Steven C. Cassidy, MD, Donald A. Girod, MD, Jean S. Kan, MD, Zuhdi Lababidi, MD, and Stephen R. Shapiro, MD
Eight hundred twenty-two balloon pulmonary valvuloplasties were reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Before and after systolic outflow gradients were recorded in 764 valvuloplasties, and the gradient decreased from 71 f 33 to 28 f 21 mm Hg. The sites of residual obstructions could be ascertained in 166 patients. In these, the total systolic outflow gradients decreased from 66 f 41 mm Hg to 33 f 27 mm Hg. Of this total residual gradient, 16 f 15 mm Hg was transvalvar and 18 f 24 mm Hg was infundibular. The degree to which infundibular obstruction subsequently resolved was not determined in this study. The procedure was less effective in reducing outflow gradients in patients with dysplastic valves with or without Noonan’s syndrome. There were 5 major complications (0.6%), including 2 deaths (0.2%), a cardiac perforation with tamponade (0.1%) and 2 tricuspid insufficiencies (0.2%). There were 11 minor compliitions (1.3%) and 21 hrcidents (2.6%). The incidence of major complications, minor compliitions and incidents was inversely related to age; it was substantially higher in infants and, in particular, neonates. Balloon pulmonary valvuloplasty is a safe and effective method of lowering pulmonary outflow gradients in infants, children and adults. Small transvalvar and varied infundibular gradients commonly are present at the end of the procedure. Assessing the full effect of the procedure requires intermediate-term follow-up and assessing the duration of relief requires long-term follow-up. (Am J Cardiol1990;65:775-763)
From the University of California, San Francisco, California, University of Indiana, Indianapolis, Indiana, Johns Hopkins University Hospital, Baltimore, Maryland, University of Missouri, Columbia, Missouri, and the Children’s Hospital National Medical Center, Washington, DC. Manuscript received November 22,1989, and accepted November 28. Address for reprints: Paul Stanger, MD, Pediatric Cardiology, Box 0544, University of California, San Francisco, San Francisco, California 94143.
B
alloon pulmonary valvuloplasty (BPV) is the most common interventional procedure performed at cardiac catheterization in children. During the 6-year study period of this report, BPV accounted for nearly half of all interventional procedures performed by members of the Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry. Although the procedure has gained wide acceptance and is considered to be the preferred method of treatment for valvar pulmonary stenosis, this is the first large-scale study of the efficacy and safety of the procedure. METHODS Patient population: Between January 1, 198 1, and December 3 1, 1986, pediatric cardiologists attempted 822 BPVs at the 26 member institutions of the VACA Registry. During the early years of this study, pediatric cardiologists were the only cardiologists performing balloon valvuloplasties; consequently, 35 adults over age 21 are included in the series. The registry was formed in June 1984, after which prospective records were kept of all procedures; retrospective data were obtained for earlier procedures. Data were gathered on all patients in whom pulmonary valvuloplasty was attempted, including those in whom the procedure was abandoned for technical reasons. Data collection: Pediatric cardiologists completed data collection forms, and a central data gathering system collated the data. The requested data included date of birth, date of procedure, whether the valve was dysplastic, whether the patient had Noonan’s syndrome, the diameter of the pulmonary valve anulus and the diameter(s) of the dilation balloon(s). In addition, the following information was requested for the before and after valvuloplasty states: right pulmonary arterial pressures, right ventricular pressures, systemic arterial pressures and cardiac output. Pressure differences between the right ventricle and pulmonary artery before and after valvuloplasty were available in 784 cases. Midway into the study, it was evident that many patients with severe valvar pulmonic stenosis had apparently excellent valvar responses to balloon valvuloplasty, but showed significant and often increased infundibular constriction after dilation. Subsequently, right ventricular outflow pressures were also requested; however, such data were obtained in only 196 of the 784 patients. All complications and valvuloplasty failures were to be reported. It was the consensus of the investigators that mild degrees of pulmonary insufficiency could not THE AMERICAN
JOURNAL
OF CARDIOLOGY
MARCH
15. 1990
775
TABLE I Results of Balloon Pulmonary Valvuloplasty All Cases (n = 784) Pre-BPV total gradient Post-BPV total gradient lnfundibular Transvalvar
(mm Hg) (mm Hg)
71 f33 28f21 -
Cases with Outflow Measurements
(n = 196) 85f41 33 f 27 17 f 24 16f 15
be adequately assessed;consequently, only data regarding moderate or severe degrees of pulmonary insufficiency were sought. RESULTS During the 6-year period, 822 balloon valvuloplasties were performed or attempted in 8 11 patients at the 26 participating pediatric cardiology centers. The number of studies performed at each institution varied marked80 -I
10 0
FtGURE 1. Nmber ot balloon pulmonary VW pedemedate!acbeftbe26member-efttle VACA Registry betwem 1961 and 1966.
ly, ranging from 1 to 77 (Figure 1). The age of the patients ranged from 1 day to 76 years; the age distribution is shown in Figure 2. Within the pediatric age range, the distribution is heavily skewed to the younger age groups, with 35% of the patients under age 2 years, 55% under age 5 years and 77% under age 10 years. Figure 3 shows the number of procedures performed each year. The number increased rapidly during the first 3 years but then leveled off. Hemodynamic results: There were 784 cases in which sufficient data were available to compare the preand immediate postvalvuloplasty pressure differences between the right ventricle and pulmonary artery. The results of all 784 cases are plotted in Figure 4. After BPV, most patients show a major reduction in the total pressure difference with 199 (25.4%) showing a residual gradient of -< 15 mm Hg. Many, however, continued to have large total pressure differences that could have been due to residual valvar obstruction, infundibular constriction or both. An analysis of the level of residual obstructions was possible in 196 procedures in which postvalvuloplasty right ventricular outflow tract pressures were also available; the results are plotted in Figure 5. It is apparent from Figure 5A that the total pressure difference between the right ventricle and pulmonary artery was reduced considerably in most patients but that many had substantial residual differences and that they correlated slightly with pre-BPV pressure differences. Figure 5B demonstrates that the majority of residual pressure differences were subvalvar and that the magnitude of subvalvar pressure difference correlated slightly with initial severity. Figure 5C shows that the postvalvuloplasty transvalvar pressure difference averaged 16.5 mm Hg and showed virtually no correlation with initial severity. Sixteen patients (8%) had postBPV transvalvar gradients >30 mm Hg. Although 196 patients represent only 25% of the total patients, the results shown in Figures 4 and 5A are very comparable and suggest that similar results were obtained in the entire group. The average gradients before and after BPV for both groups are listed in Table I. In an effort to determine whether the efficacy of BPV was age related, we plotted the results in the 196 procedures with outflow pressures by age group (Figure
250 225
212
175 %5 $ 150 -on aE ” 3 125 2c a-100
FIGURE 2. Age dim eftbepubnoMry VW m -tNng 822ft4vdms.Tbenumbe.rofprecadures mpmsented by each bar is indkated atthetopofeachfw.Thehorizontal scale is extendsd in the eadier years.
75 50 25 0 4
16 7-12 Months
l-2 I
776
THE AMERICAN
JOURNAL
Z-4
S9 10-14 181920-2930-3940-495.~~.~~-~ Years Age OF CARDIOLOGY
VOLUME
Unknown
65
TABLE
II
Results
of Balloon
Pulmonary
Valvuloplasty
Dysplastic Non-Noonan’s (n = 34) RV-PA gradient (mm Hg) Before BPV After BPV RV pressure (mm Hg) Before BPV After BPV Values are mean f standard dewatlon * p
Eight hundred twenty-two balloon pulmonary valvuloplasties were reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Before and after systolic outflow gradients were recorded in 764 valvuloplasties, and the gradient decreased from 71 f 33 to 28 f 21 mm Hg. The sites of residual obstructions could be ascertained in 166 patients. In these, the total systolic outflow gradients decreased from 66 f 41 mm Hg to 33 f 27 mm Hg. Of this total residual gradient, 16 f 15 mm Hg was transvalvar and 18 f 24 mm Hg was infundibular. The degree to which infundibular obstruction subsequently resolved was not determined in this study. The procedure was less effective in reducing outflow gradients in patients with dysplastic valves with or without Noonan’s syndrome. There were 5 major complications (0.6%), including 2 deaths (0.2%), a cardiac perforation with tamponade (0.1%) and 2 tricuspid insufficiencies (0.2%). There were 11 minor compliitions (1.3%) and 21 hrcidents (2.6%). The incidence of major complications, minor compliitions and incidents was inversely related to age; it was substantially higher in infants and, in particular, neonates. Balloon pulmonary valvuloplasty is a safe and effective method of lowering pulmonary outflow gradients in infants, children and adults. Small transvalvar and varied infundibular gradients commonly are present at the end of the procedure. Assessing the full effect of the procedure requires intermediate-term follow-up and assessing the duration of relief requires long-term follow-up. (Am J Cardiol1990;65:775-763)
From the University of California, San Francisco, California, University of Indiana, Indianapolis, Indiana, Johns Hopkins University Hospital, Baltimore, Maryland, University of Missouri, Columbia, Missouri, and the Children’s Hospital National Medical Center, Washington, DC. Manuscript received November 22,1989, and accepted November 28. Address for reprints: Paul Stanger, MD, Pediatric Cardiology, Box 0544, University of California, San Francisco, San Francisco, California 94143.
B
alloon pulmonary valvuloplasty (BPV) is the most common interventional procedure performed at cardiac catheterization in children. During the 6-year study period of this report, BPV accounted for nearly half of all interventional procedures performed by members of the Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry. Although the procedure has gained wide acceptance and is considered to be the preferred method of treatment for valvar pulmonary stenosis, this is the first large-scale study of the efficacy and safety of the procedure. METHODS Patient population: Between January 1, 198 1, and December 3 1, 1986, pediatric cardiologists attempted 822 BPVs at the 26 member institutions of the VACA Registry. During the early years of this study, pediatric cardiologists were the only cardiologists performing balloon valvuloplasties; consequently, 35 adults over age 21 are included in the series. The registry was formed in June 1984, after which prospective records were kept of all procedures; retrospective data were obtained for earlier procedures. Data were gathered on all patients in whom pulmonary valvuloplasty was attempted, including those in whom the procedure was abandoned for technical reasons. Data collection: Pediatric cardiologists completed data collection forms, and a central data gathering system collated the data. The requested data included date of birth, date of procedure, whether the valve was dysplastic, whether the patient had Noonan’s syndrome, the diameter of the pulmonary valve anulus and the diameter(s) of the dilation balloon(s). In addition, the following information was requested for the before and after valvuloplasty states: right pulmonary arterial pressures, right ventricular pressures, systemic arterial pressures and cardiac output. Pressure differences between the right ventricle and pulmonary artery before and after valvuloplasty were available in 784 cases. Midway into the study, it was evident that many patients with severe valvar pulmonic stenosis had apparently excellent valvar responses to balloon valvuloplasty, but showed significant and often increased infundibular constriction after dilation. Subsequently, right ventricular outflow pressures were also requested; however, such data were obtained in only 196 of the 784 patients. All complications and valvuloplasty failures were to be reported. It was the consensus of the investigators that mild degrees of pulmonary insufficiency could not THE AMERICAN
JOURNAL
OF CARDIOLOGY
MARCH
15. 1990
775
TABLE I Results of Balloon Pulmonary Valvuloplasty All Cases (n = 784) Pre-BPV total gradient Post-BPV total gradient lnfundibular Transvalvar
(mm Hg) (mm Hg)
71 f33 28f21 -
Cases with Outflow Measurements
(n = 196) 85f41 33 f 27 17 f 24 16f 15
be adequately assessed;consequently, only data regarding moderate or severe degrees of pulmonary insufficiency were sought. RESULTS During the 6-year period, 822 balloon valvuloplasties were performed or attempted in 8 11 patients at the 26 participating pediatric cardiology centers. The number of studies performed at each institution varied marked80 -I
10 0
FtGURE 1. Nmber ot balloon pulmonary VW pedemedate!acbeftbe26member-efttle VACA Registry betwem 1961 and 1966.
ly, ranging from 1 to 77 (Figure 1). The age of the patients ranged from 1 day to 76 years; the age distribution is shown in Figure 2. Within the pediatric age range, the distribution is heavily skewed to the younger age groups, with 35% of the patients under age 2 years, 55% under age 5 years and 77% under age 10 years. Figure 3 shows the number of procedures performed each year. The number increased rapidly during the first 3 years but then leveled off. Hemodynamic results: There were 784 cases in which sufficient data were available to compare the preand immediate postvalvuloplasty pressure differences between the right ventricle and pulmonary artery. The results of all 784 cases are plotted in Figure 4. After BPV, most patients show a major reduction in the total pressure difference with 199 (25.4%) showing a residual gradient of -< 15 mm Hg. Many, however, continued to have large total pressure differences that could have been due to residual valvar obstruction, infundibular constriction or both. An analysis of the level of residual obstructions was possible in 196 procedures in which postvalvuloplasty right ventricular outflow tract pressures were also available; the results are plotted in Figure 5. It is apparent from Figure 5A that the total pressure difference between the right ventricle and pulmonary artery was reduced considerably in most patients but that many had substantial residual differences and that they correlated slightly with pre-BPV pressure differences. Figure 5B demonstrates that the majority of residual pressure differences were subvalvar and that the magnitude of subvalvar pressure difference correlated slightly with initial severity. Figure 5C shows that the postvalvuloplasty transvalvar pressure difference averaged 16.5 mm Hg and showed virtually no correlation with initial severity. Sixteen patients (8%) had postBPV transvalvar gradients >30 mm Hg. Although 196 patients represent only 25% of the total patients, the results shown in Figures 4 and 5A are very comparable and suggest that similar results were obtained in the entire group. The average gradients before and after BPV for both groups are listed in Table I. In an effort to determine whether the efficacy of BPV was age related, we plotted the results in the 196 procedures with outflow pressures by age group (Figure
250 225
212
175 %5 $ 150 -on aE ” 3 125 2c a-100
FIGURE 2. Age dim eftbepubnoMry VW m -tNng 822ft4vdms.Tbenumbe.rofprecadures mpmsented by each bar is indkated atthetopofeachfw.Thehorizontal scale is extendsd in the eadier years.
75 50 25 0 4
16 7-12 Months
l-2 I
776
THE AMERICAN
JOURNAL
Z-4
S9 10-14 181920-2930-3940-495.~~.~~-~ Years Age OF CARDIOLOGY
VOLUME
Unknown
65
TABLE
II
Results
of Balloon
Pulmonary
Valvuloplasty
Dysplastic Non-Noonan’s (n = 34) RV-PA gradient (mm Hg) Before BPV After BPV RV pressure (mm Hg) Before BPV After BPV Values are mean f standard dewatlon * p
