Interventional catheterization in pediatric congenital and acquired heart disease

Interventional Catheterization in Pediatric Congenital and Acquired Heart Disease STANTON B. PERRY, MD, JOHN F. KEANE, MD, and JAMES E. LOCK, MD

The present status of catheter-directed therapy in pediatric congenital and acquired heart disease is reviewed. Balloon pulmonary valvotomy, established as the procedure of choice for children and adults with isolated pulmonary stenosis, has now been extended to neonates with critical pulmonary stenosis. Balloon mitral and aortic valvotomy are being performed for congenital and acquired stenoses, although indications and optimal techniques remain to be established. Balloon angioplasty of coarctaUon effectively relieves obstruction in native and postoperative aortic arch stenoses, but remains controversial due to late aneurysm formation. Balloon an-

gioplasty of branch pulmonary artery hypoplasia and stenosis, despite a relatively low success rate and significant complications, remains the procedure of choice due to lack of better therapeutic options. Transcatheter closure of congenital and acquired vessels and defects is being performed with increasing frequency. Embolization of aortopulmonary collaterals and shunts using Gianturco coils is safe and effective in selected patients. Although Rashkind umbrellas are most commonly used for closure of a patent ductus arteriosus, their use, especially in closing intracardiac defects, remains investigational. (Am J Cardiol 1988;61:109G-117G)

From its foundation in the ability to create interatrial defects using balloon and blade septostomy,1.2 catheter-directed therapy for pediatric congenital and acquired heart disease has increased dramatically during the last 5 years. The development of balloon dilatation catheters now allows dilatation of stenotic vessels and cardiac valves. A variety of devices, including coils and umbrellas, allows closure of many congenital and acquired vessels and cardiac defects. Whereas some of these techniques and procedures are routine, others remain highly experimental. Balloon pulmonary valvotomy, for example, is now recognized as the procedure of choice for isolated pulmonary stenosis in children and adults, and has now been applied to neonates with critical pulmonary stenosis. On the other hand, catheter closure of intracardiac defects remains highly experimental and will require considerable advances before it becomes accepted therapy.

In this report, we review the present status of percutaneous balloon valvotomy for pulmonary, aortic and mitral stenosis, percutaneous balloon angioplasty of aortic and branch pulmonary artery obstructions, the use of coils and the Rashkind double umbrella to close congenital and acquired defects, as well as a variety of less frequently used interventions. Results from our own and other laboratories, including complications, are discussed in relation to technique and other factors such as patient age and underlying pathology. Given these results and the fact that many of the procedures remain experimental, current indications for these procedures at our institution are discussed.

From the Departments of Pediatrics and Cardiology, Harvard Medical School and The Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts. This study was supported in part by NRSA 5 F32 HL07309.02 B1-2. Dr. Lock is an Established Investigator of the American Heart Association. Address for reprints: James E. Lock, MD, Department of Cardiology, The Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115.

PercutaneousBalloon PulmonaryValvotomy Reports of using blade or balloon catheters to perform cardiac valvotomies appeared as early as 1953.3,4 However, the static balloon technique, reported by Kan et aP in 1982 was the first to be applied widely. Results 5-14 have demonstrated the safety and effectiveness of this technique, and established it as the treatment of choice for children and adults with isolated pulmonary stenosis. We have now performed 73 balloon pulmonary valvotomies in 72 patients. The 72 patients include 2 special subgroups: neonates <1 week old with critical pulmonary stenosis [6 patients), and patients who have

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110G

A SYMPOSIUM: INTERVENTIONAL CARDIOLOGY--1987 O Pre- Dilation

• Post-Dilation

160. 0 -~ "I-

140.

E

120

E

0 000 0

100

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FIGURE 1. Pre- and postdllatatlon transvalvar gradients (ram Hg) In 54 patients with valvar pulmonary stenosls arranged in order of increasing pre-

dilatation gradient.

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0

Balloon Pulmonary Valvotomies

previously undergone surgical valvotomy {9 patients). We routinely use balloon diameters 20 to 40% larger than the pulmonary anulus. Animal studies 1~ and initial results in patients 13 have demonstrated that oversize balloons are safe and yield improved results, reducing the transvalvar gradient by 75 to 80%. When the anulus is >20 mm, overdilatation requires the simultaneous use of 2 balloons. 16.17 In 6 consecutive neonates with critical pulmonary stenosis, 18 the stenotic valve was crossed successfully in 5. After initial dilatation with a low-profile 5- or 6mm balloon, at least 1 larger balloon was used in 4 of the 5 cases. Because of the presence of a patent ductus arteriosus in 3 patients, transvalvar gradients were unreliable indicators of obstruction, but right ventricular pressures decreased from 122 ± 7 to 59 ± 7 mm Hg. The 5 patients dilated successfully are asymptomatic at follow-up of 10.6 ± 11.7 months. Echo/Doppler (n = 4) or repeat catheterization (n = 1} shows a persistent gradient >35 mm Hg in only 1 patient who was the only patient dilated with a balloon/anulus ratio <1 {0.95). Complications have included persistent right bundle branch block in 1 patient and transient obstruction of the iliac vein in 1 case. Of 9 patients who had previously undergone a surgical valvotomy, 7 had been neonates with critical pulmonary stenosis. One patient had undergone a Brock procedure, 4 valvotomy with inflow occlusion, and 4 valvotomy on cardiopulmonary bypass. The mean interval since surgery was 4 years (range 6 months to 18 years}, and the average age at balloon valvotomy was 4.5 years {range 6 months to 19 years}. The transvalvar gradient decreased from 60 ± 18 to 19 ± 10 mm Hg using a balloon anulus ratio of 1.24 ± 0.19, with no significant complications. Of 57 patients older than 1 week who had not undergone previous surgical valvotomy, 54 were successfully dilated with a balloon/anulus ratio of 1.27 ± 0.16. The transvalvar gradient decreased from 74 ± 26 to 15 ± 8 mm Hg and the right ventricular pressure from 101 ± 31 to 50 ± 22 mm Hg, with no significant change in cardiac output. Figure 1 shows gradients before and after dilatation, demonstrating that postdilatation gradients are <30 mm Hg regardless of the gradient before dilatation. In 19 of the last 29 patients, subvalvar gradients of 22 ± 22 mm Hg (range 5 to 80 mm Hg) were

detected after dilatation. Only 4 of 54 patients had subvalvar gradients that were greater than 30 mm Hg (45, 60, 75 and 80 mm Hg). Follow-up is available in 3 of these 4 cases showing that the subvalvar gradient had resolved in each case within 1 year. The gradients are presumably due to reactive infudibular stenosis. 13 Four attempts at pulmonary valvotomy were unsuccessful in 3 patients with severely dysplastic valves. No cases of significant restenosis have been identified. The only significant complication occurred early in the series in a patient in whom complete right bundle branch block developed during an attempt to pass a wedge catheter through the right ventricle before wire placement, with subsequent significant blood loss due to mismatch between pacing catheter and sheath size. Combined with results in reported data, our results demonstrate that balloon pulmonary valvotomy, using oversize balloons, is both safe and effective in relieving pulmonary valve stenosis in patients of all ages, including neonates and patients who have undergone previous surgical valvotomies. We currently attempt balloon pulmonary valvotomy in any patient with a pulmonic gradient >40 mm Hg and in neonates with critical pulmonary stenosis.

Percutaneous Balloon Aortic Valvotomy Balloon aortic valvotomy in a child with congenital aortic stenosis was first reported in 1983,19 and has subsequently been performed in large numbers of patients with both congenital and acquired stenosis. 2°-28 Animal and clinical studies 23,28,27 demonstrate that balloon/anulus ratios >1.0 are more likely to be associated with complications. The mechanism of gradient relief depends on the underlying pathology, 2s,28 but tearing of the leaflet is uncommon. 26 We have performed 75 balloon aortic valvotomies in 71 patients between age 1 day and 39 years (mean 9 + 9 years} with congenital aortic stenosis. 17,28In general, single balloons are used unless the anulus diameter is greater than 22 mm. In 7 patients the gradient reduction was <20%, including 1 neonate with poor left ventricular function and a patent ductus arteriosus (in whom the gradient was an unreliable index of obstruction], 1 in whom the procedure was terminated when the catheter shaft separated from the balloon before dilatation, and in 2 patients in whom the first attempt

May 9, 1988

failed but a second procedure succeeded. In the 68 cases of gradient reductions >20% (mean 61 4- 16%), the transvalvar gradient decreased from 77 4- 27 to 31 4- 19 mm Hg and the valve area increased from 0.55 40.23 to 0.85 4- 0.66 e m 2 / m 2. The percent gradient reduction was unrelated to age, history of prior surgical valvotomy (n = 11), gradient before dilatation or final balloon/anulus ratio (mean 0.96 4- 0.12, range 0.71 to

1.33). Aortic regurgitation averaged grade 0.6 4- 0.6 before dilatation (all patients had grade <2/51 and grade 1.3 41.1 after dilatation. After dilatation, 4 patients had grade 3/5 regurgitation, 1 patient grade 4/5 and 2 patients grade 5/5. In patients in whom regurgitation increased from 0 to 1 grade, the percent reduction in gradient was 53 4- 21%, whereas patients in whom regurgitation increased 2 to 5 grades the percent reduction in gradient was 75 4- 18% (p <0.05}, demonstrating an inverse relation between gradient reduction and increase in regurgitation. The risk of a greater than 1/5 grade increase in regurgitation increased from 11% (6/55} when the balloon/anulus ratio was <1.0 to 30% (6/20) when the ratio was >1.0. With use of 2-dimensional echocardiography, the relation between outcome and valve morphology was studied. Valves were classified according to the number of commissures (uni- or biocommissurall, thickness of the leaflets [compared with the wall of the ascending aorta} and pliancy of the valve (i.e., valves that domed). Whereas differences in outcome between valves of various morphologies were generally small, the percent gradient reduction was greater in thin pliant valves (63 4- 24%, n = 26) than in thick valves (51 + 12%, n = 23} (p <0.01}. Greater than grade 2/5 increases in aortic regurgitation occurred in 2 of 7 unicommissural valves compared with i of 46 bicommissural valves. In 20 of 26 cases in which adequate echocardiograms before and after dilatation were recorded, the mechanism of gradient relief was separation of the commissures; in the remaining patients the mechanism of gradient reduction could not be determined by echo. There were 2 deaths, both from the" group of 11 neonates with critical aortic stenosis. One died of sepsis 8 days after the procedure and another died at surgery to reduce severe regurgitation and perform hypoplastic left heart palliation. Temporary pulse loss occurred in 30% of patients (60% in patients <2 years of age and 15% in patients >2 years} and was permanent in 10%. Transient left bundle branch block occurred in 17% and ventricular arrhythmias requiring cardioversion occurred in 4%. In I patient the catheter shaft separated from the balloon necessitating surgery to remove the balloon (the catheter was subsequently redesigned). Surgical repair of an anterior mitral leaflet tear was required in a patient in whom anterograde dilatation was attempted. Another patient underwent surgical repair of a ruptured femoral artery. During average follow-up of 6 + 6 months there have been no significant changes in transvalvar gradients or the grade of regurgitation. Although direct comparisons are difficult, shortterm results of balloon aortic valvotomy appear corn-

THE AMERICAN JOURNAL OF CARDIOLOGY Volume61

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parable to surgical valvotomy for congenital aortic stenosis. It is likely the current rate of complications can be reduced further as a greater variety of catheters, wires and low-profile balloons become available. Our results demonstrate that outcome is at least partially related to valve morphology, and suggest that it may be possible to optimize the technique in relation to morphology. Recognizing that the procedure remains experimental, we currently perform balloon aortic valvotomy in neonates with critical aortic stenosis and in older patients with a transvalvar gradient >50 mm Hg and regurgitation
Percutaneous Balloon Mitral Valvotomy After the report of Inoue et al, ~9 using a specially designed balloon, Lock et al 3° reported results of percutaneous balloon mitral valvotomy using the single balloon technique in young patients with rheumatic mitral stenosis. The procedure was extended to adults with rheumatic mitral stenosis with and without calcification, 31 and a 2-balloon technique has been described. 31,32 The results demonstrate that the gradient can be reduced approximately 50% and that the valve area can be increased approximately 80%, using a single 25-mm balloon in adult patients. Significant increases in regurgitation are unusual. The major complication of the single balloon technique was creation of a significant atrial septal defect in a small number of patients due to the necessity of pre-dilating the atrial septum to allow passage of the high-profile 25-ram balloon. 31 The use of 2 balloons (each with low profile) has reduced the need to dilate the atrial septum, and has allowed dilatation matched to the mitral anulus with effective diameters >25 mm and improved resuits with postdilatation mitral valve areas >2 c m 2, 32.33 The need to dilate the interatrial septum can also be eliminated by using a transarterial approach, 34 but we have not used this technique. Although the optimal balloon/mitral anulus ratio has not been determined, it appears prudent to use ratios close to 1.0. Short-term follow-up has not shown significant rates of restenosis. 3°'31'35 Given the nature of surgical valvotomy, a significant incidence of late restenasis will probably be seen. At present in our institution, balloon mitral valvotomy is performed in patients with symptomatic rheumatic mitral stenosis who have no more than mild regurgitation and no evidence of atrial thrombi on 2dimensional echocardiography. Although use of balloon mitral valvotomy in congenital mitral stenosis has received relatively little attention, case reports 36.37 of successful dilatations suggest it can be effective in selected patients.

Percutaneous Balloon Angioplasty of Coarctation and Postoperative Aortic Obstructions Percutaneous balloon angioplasty of coarctation was first described in 198238 and has since been used in large numbers of patients with native coarctation and postoperative recoarctation. 39-44A study of experimental coarctation in lambs 45 demonstrated that relief of obstruction occurs by tearing the intima and media, which requires balloon diameters 2.5 to 3 times that of

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A SYMPOSIUM: INTERVENTIONAL CARDIOLOGY--1987

the narrowing. Short- and long-term complications seen in that study, 45 including perforation resulting in death and late aneurysm formation, have now been described in p a t i e n t s . 44'46-5° We have performed 60 balloon dilatation angioplasties in 57 patients with native and postoperative aortic obstructions including coarctation, interrupted aortic arch and hypoplastic left heart syndrome. Details of the technique have been reported27 In patients with coarctation, 43 dilatations have been performed in 42 patients (3 native and 40 postoperative). Procedures are considered successful if the gradient is reduced >50% and the diameter is increased >30%. Based on these criteria, 34 were successful and 9 were unsuccessful (Table I). In 5 of these 9 failures, the gradients before dilatation were <20 mm Hg (Table I), and while no patient had gradient reductions >50%, 2 had increases in the diameter of the coarctation of >30% (67 and 35%}. Although unsuccessful dilatations tended to occur in patients who were significantly older, the most important predictor of failure appears to be a larger pre-dilatation diameter of the coarctation. This makes it difficult to use a balloon large enough to dilate the coarctation without injuring the normal aorta, which is reflected in the significantly smaller balloon/coarctation diameter ratio in unsuccessful compared with successful procedures. In 19 patients, previous results were independent of type of surgical repair of the coarctation (end-to-end, subclavian flap angioplasty or patch angioplasty). 44 An additional 17 balloon angioplasties have been performed in patients with aortic obstructions after surgical repair of interrupted aortic arch {12 in 10 patients) and hypoplastic left heart syndrome (5 in 5 patients] (Table I}. Using the same criteria as with coarctation, 14 of the 17 procedures were successful. The most frequent complication is transient loss of the arterial pulse. 44 The only other significant shortterm complication in our series occurred in a 67-yearold woman with a native coarctation. Dilatation of a 5mm coarctation with a 12-mm balloon resulted in an aortic dissection that was successfully repaired surgically. Although we have had no deaths, mortalities have been r e p o r t e d . 4°,42,46

TABLE I

The major concern regarding dilatation of coarctation is the late development of aneurysms. Aneurysm formation has been reported by several g r o u p s 44,47-5° in both native and postoperative coarctations. Whether the apparent variation in incidence of aneurysms is related to technique, underlying pathology of the coarctation (wedge vs membranous and native vs postoperative) or duration of follow-up remains to be determined. Given the mechanism of dilatation, it seems unlikely that aneurysm formation can be eliminated without significantly compromsing relief of the obstruction. The natural history of these aneurysms and whether the natural history is different in native and postoperative coarctations remains unknown. This information will ultimately establish the indications for balloon angioplasty of coarctation. We currently use balloon angioplasty in patients with native coarctations who are high surgical risks and in patients with postoperative recoarctations or other postoperative aortic obstructions. This policy is based on the fact that surgical results are generally less satisfactory in recoarctations and the speculation that the presence of scar tissue may improve the natural history of any resulting aneurysms. Defining the indications in terms of gradient and coarctation diameter is difficult, particularly given the occasionally poor correlation between the gradient and diameter. However, our results in patients with low gradients and relatively large coarctation diameters suggest that dilatation is unlikely to be successful in these cases.

Percutaneous Balloon Angioplasty of Branch Pulmonary Artery Hypoplasiaand Stenosis Dilatation of branch pulmonary artery stenoses is the intervention most often performed at our institution. Branch pulmonary artery stenosis or hypoplasia may be acquired (e.g., at sites of shunts, bands or conduits) or congenital. Anatomy ranges from single stenotic areas to multiple stenoses to diffuse hypoplasia. Successful dilatation generally results in tearing of the intima and media. 51,52 Details of the procedure have been reported. 17,5a Balloon diameters 2 to 4 times the diameter of the stenotic segment and <2 times the diameter of the "normal" pulmonary artery have been

Results of Balloon Angloplasty in Coarctation and Postoperative Interrupted Aortic A r c h ( I A A ) and HypoplasUc Left Heart

Syndrome ( H L H S ) Gradient (mm Hg) Group Coarctation

Lesion Diameter"

Balloon/Lesion

Age

Pre

Post

% Change

Pre

Post

% Change

Ratio

Initialgradient

5.8-1-5.9 18.1-I-19.9 11.64-8.7

39-I-13 37-1-24 19-t-1

94-8 29-1-15 184-3

78-t-17 12-1-25 6-1-17

4.2-t-2.1 8.4-I-2.3 8.8-1-2.1

7.5-I-4.1 11.4-1-2.9 11.34-3.6

102-t-75 274-23 29-1-28

3.1-t-1.2 1.84-0.4 1.6-1-0.3

-<20 mm Hg IAA (n = 12) HLHS (n = 5)

3.2 4- 3.3 1 4- 0.8

53 4- 17 33 4- 12

18 4- 12 7 4- 6

71 4- 19 78 4- 23

3.5 4- 1.8 3.3 4- 0.6

6.7 4- 2.7 5.6 4- 0.3

104 4- 74 73 4- 37

3.0 4- 1.3 3.0 4- 0.8

Successes(n=34) Failures(n=9)

• Diameter (mm) of the lesion obtained by averaging measurements on 2 orthogonal views. Successes defined as > 5 0 % reduction in gradient and > 3 0 % in-

crease in diameter of the lesion. Gradient < 2 0 mm Hg = group of 5 patients with recoarctation who had pre--dilatation gradients -<20 mm Hg.

May 9, 1988

shown to be relatively safe and necessary to relieve the obstruction. 53,54 Rupture of the balloon has been associated with vessel rupture. 52 In general, the goal of the procedure is to dilate pulmonary artery stenoses and lower right ventricular pressure in patients with intact ventricular septums, or allow closure of the ventricular septum or separation of the circulations in others. However, it is often difficult to assess the results in terms of right ventricular pressure, especially in patients with multiple stenoses who often require 2 or more procedures to optimize outcome. Therefore, a procedure is considered successful if the diameter of the stenosis increases >50% or there is increased blood flow to the affected side, or both. Using these criteria, approximately 55% of 113 procedures from 2 institutions have been successful. 17'54 We have currently performed 117 dilatations (including 61 previously reported ~7 and the success rate for the last 56 dilatations in 40 patients remains 55% (31 of 56). The underlying diagnosis in these 40 patients was Tetralogy of Fallot with pulmonary atresia in 11 (10 of 18 dilatations successful), congenital, isolated peripheral pulmonic stenosis in 10 (8 of 16 successful), Tetralogy of Fallot in 5 (2 of 6 successful), truncus arteriosus (3 of 4 successful), status post Fontan in 4 (4 of 5 successful) and other in 6 {4 of 7 successful). In successful dilatations, using a balloon to lesion diameter ratio of 3.9 4- 1.4 (range 2.4 to 8), the average diameter of the stenosis increased from 3.4 + 1.3 to 7.4 + 2.4 ram. Failures have been attributed to technique and presence of rigid, nondilatable lesions that appear to occur more often in older patients. 54 Long-term follow-up study has not shown a significant incidence of restenosis. Reported complications have included pulmonary artery thrombosis, 55 hemoptysis, nonfatal pulmonary artery rupture, pulmonary edema and aneurysm formation. 54 Of a total of 170 procedures (113 procedures previously reported ~7.54 and the 56 reported here), there have been 3 deaths: 2 due to pulmonary artery rupture and 1 associated with catheter placement in the pulmonary artery. There have been no deaths in the last 80 procedures. The results demonstrate a relatively low success rate compared with the procedures discussed and a T A B L E II

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significant incidence of complications. Despite this, balloon angioplasty remains the procedure of choice in patients with hypoplasia or stenosis of the distal pulmonary arteries given the lack of better therapeutic options.

Coil Embolization of Congenital and Acquired Thoracic Vessels Reports of therapeutic embolization of thoracic vessels appeared as early as 197456 and have described use of bucrylate adhesive, 56 detachable balloons, 57-59 and steel coils 6°-64 to occlude aortopulmonary collaterals, arteriovenous malformations, Blalock-Taussig shunts and a patent ductus arteriosus. The largest series reported attempted embolization of 17 vessels. 64 We have used Gianturco coils 6s to embolize 52 vessels in 38 patients, including 38 aortopulmonary collaterals, 9 Blalock-Taussig shunts, 3 arteries supplying pulmonary sequestrations and 2 venae cavae (Table II]. The technique has been described in detail. 17 In general, coil embolization of a vessel is attempted if there is evidence that patency of the vessel is associated with adverse hemodynamic effects, if the vessel is <1.5 cm in length or has distal stenosis, and if an appropriately sized coil is available. The initial coil is chosen to be 10 to 20% larger than the diameter of the vessel. The underlying diagnosis in the 25 patients with aortopulmonary collaterals was Tetralogy of Fallot, with pulmonary atresia in 19, Tetralogy of Fallot in 1, polysplenia/asplenia syndrome in 2, hypoplastic left heart syndrome in 2 and brochopulmonary dysplasia in 1. Embolization was performed before corrective surgery in 8 patients, before and after corrective surgery in 4 and after corrective surgery in 13 patients. Results are summarized in Table II. An average of 2.1 + 1.2 coils were used per collateral: 1.8 4- 1 coils in collaterals completely occluded and 2.7 4- 1.4 in collaterals with residual flow. During follow-up, I collateral recanalized and 1, which was patent at the end of the procedure, closed. Complications included inadvertent embolization of branch pulmonary arteries in 2 patients. Both coils were left and the patients remain asymptomatic. In I patient, attempts to enter a collateral resulted in perforation of the collateral. The catheter

Results of Coil Embolization of 52 Vessels in 38 Patients Occlusion Vessel

Total

Near-Total

Partial

Failure

Aortopulmonary collaterals Blalock-Taussig shunts Venae cavae Sequestrations t

25 4 2 3

9 3 ---

3 1° ---

-1 ---

38 9 2 3

35 (67%)

12 (23%)

4 (8%)

1 (2%)

52

Total

• The degree of occlusion was determined angiographically: Total = no residual flow; Near-Total = minimal residual flow that failed to opacify distal vessels; Partial = decreased flow that opacified distal vessels; Failure = no change in flow. t Artery supplying pulmonary sequestrations.

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TABLE III Results of Occlusion with Rashkind Patent Ductus Arteriosus Occluder (Double Umbrella) in 4 8 Patients Residual F l o w " Defect

None

Trivial

Mild

Moderate

No C h a n g e

Patent ductus arteriosus

13

7

4

2

2t

SVC-right atrial junction

3

--

1

--

--

4

(Glenn anastomosis) A o r t o p u l m o n a r y collaterals

1

--

1

It

3

Potts anastomosis

1

--

1

--

It

3

---

---

2 1

---

---

2 1

--

--

1

--

--

1

2

--

1

--

1t

4

--

1

1

--

--

2 48

Venae cavae Azygous vein Left v e n t r i o u l a r to aortic conduit Atrial septal d e f e c t Ventricular septal d e f e c t

Total

--

20

8

12

3

5

(42%)

(17%)

(25%)

(5%)

(10%)

28

• Results based on a n g i o g r a m after r e l e a s e of the device: N o n e = c o m p l e t e occlusion; Trivial = residual f l o w that was only seen with close inspection; Mild = residual flow that failed to o p a c i f y distal v e s s e l s or structures; M o d e r a t e = d e c r e a s e d f l o w that o p a c i f i e d distal v e s s e l s or structures; No C h a n g e = d e v i c e e m b o l i z e d to the p u l m o n a r y artery r or n e v e r released~. S V C = s u p e r i o r v e n a cava.

was removed without symptoms or signs of significant hemorrhage. Coil embolization of 9 Blalock-Taussig shunts was attempted using i to 5 coils and results are summarized in Table II. In 4 patients, embolization was performed after corrective surgery and attempted ligation of the shunt, and resulted in complete closure in 1 and neartotal occlusion in 2. In the fourth patient, the first coil that was placed failed to reduce flow significantly. The second coil embolized to the pulmonary artery, was retrieved and the procedure terminated. In 2 patients the shunt was embolized before corrective surgery, resulting in complete closure in I and near-total occlusion in the second. Another patient, with pulmonary atresia/intact ventricular septum, had undergone a shunt and right ventricular outflow tract reconstruction and atrial septal defect had closed spontaneously. Coil occlusion of the shunt, which was complete, eliminated the need for further surgery. In another patient with complex congenital heart disease, placement of the Blalock-Taussig shunt lead to a large pulmonary blood flow, ventricular volume overload and dilatation, and severe atrioventricular valve regurgitation. Attempted partial occlusion of the shunt to reduce volume overload was successful. However, the patient had severe hemolysis presumably due to high flow past the coil. The final patient is discussed in the section on Rashkind umbrellas. The results demonstrate that coil embolizatien is effective and safe, and when performed in appropriate patients, eliminates the need for surgical closure of these vessels.

Rashkind Patent Ductus Arteriosus Occluder (Double Umbrella) In addition to coils and detachable balloons previously discussed, plugs, 66'67 double umbrellas 68-7° and

single umbrellas 71 have all been used to occlude intraand extracardiac defects. 17,66-72 We have used 48 modified Rashkind double umbrellas, which are currently available in 12- and 17mm diameters, to attempt closure of 48 congenital and acquired lesions (Table III). In general, 12-ram umbrellas have been used to close structures less than 4 mm in diameter and 17-mm umbrellas to close structures between 4 and 10 mm in diameter. The procedure has been described in detail. 17 We have attempted closure of a patent ductus arteriosus in 28 patients ranging in age from 3 months to 19 years. The results are shown in Table III. Whereas half had evidence of some residual flow, it was minimal in most cases, and in 23 of 25 patients who had continuous murmurs before occlusion the continuous murmur disappeared after occlusion. In 2 of the first 6 patients, the device embolized to the pulmonary artery and the procedure was terminated. Neither patient was symptomatic and in both cases the device was removed surgically at the time of ductal ligation. In addition to the 2 cases mentioned, the device embolized to the pulmonary artery in 2 other patients. In these 2, the device was retrieved at the catheterization and the ductus closed using a second device. Of 6 patients who have had subsequent surgical ligation of the patent ductus, 2 were performed because of device embolization, 2 were because device occlusion was inadequate and 2 were for other surgical indications. Long-term follow-up is needed to determine how often a ductus with trivial residual flow completely closes and whether the incidence of bacterial endocarditis is reduced in patients with minimal residual flow. At our institution, device closure appears to be the procedure of first choice in patients beyond the neonatal period. The procedure is now routinely performed on an outpatient basis.

We have also used the double umbrella device to occlude a variety of other structures72 {Table III}. Of the 3 atrial defects occluded, 2 were defects in the atrial baffle in Fontan patients who had saturations of 59 and 70 due to right-to-left shunts, and ] was an atrial septal defect in a patient with pulmonary atresia/intact septum who had undergone right ventricular outflow tract reconstruction and a Blalock-Taussig shunt. The shunt was coil-embolized, the outflow tract dilated and the atrial septal defect occluded using an umbrella. Of the ventricular septal defects occluded, 73 1 was a residual defect around the patch in a patient with Tetralogy of Fallot/pulmonary atresia who had undergone repair, and 1 was a small, hemodynamically insignificant membranous ventricular septal defect in a woman who had had 3 episodes of bacterial endocarditis and refused surgery. These atrial and ventricular septal defects were all small {<10 mm} and the currently available double umbrellas are too small to close most defects. Modifications of the Rashkind atrial septal defect occlusion device, 17,71which is a single umbrella with arms that allow the device to be centered in the defect, are being tested.

Miscellaneous Interventions In additon to the procedures previously described, catheter-directed therapy has been applied to a number of other lesions, including retrieval of foreign bodies such as pieces of severed catheters as well as misplaced coils and umbrellas, TM pericardial drainage 17 and the procedures discussed next. In patients with discrete subaortic membranes, short-term results demonstrate that balloon dilatation effectively reduces the obstruction. 7~,76At our institution, the indication that most often prompts surgical resection of a subaortic membrane is prevention of long-term damage to the aortic valve leaflets rather than the presence of severe stenosis. Whether tearing the membrane76 with balloon dilatation will prevent long-term damage to the aortic valve leaflets and decrease the incidence of bacterial endocarditis remains to be determined. It will be also important to compare the incidence of late restenosis after balloon dilatation with that after surgery. Balloon dilatation in patients with fibromuscular rings has been less successful76 and, in our experience, in patients with postoperative stenosis (nondiscrete) has been unsuccessful {n = 2). Of our 11 attempts to dilate biprosthetic valves in the triscuspid {n = 2} and pulmonary {n = 9} position, 3 have been successful (1 tricuspid and 2 pulmonary). These results, combined with those of Lloyd et al, 77 suggest that while balloon dilatation of the bioprosthetic valve is unlikely to be successful, it can provide palliation and postpone conduit or valve replacement in some patients. Balloon dilatation of pulmonary veins and systemic venous channels has been reported. 17,78,79 Balloon dilation of pulmonary vein stenosis, both congenital and acquired, although occasionally associated with shortterm relief of obstruction in our experience with 6 patients, has been uniformly unsuccessful in providing long-term relief. Balloon dilatation of baffle ob-

structions in patients who have undergone Senning or Mustard procedures has been effective in 9 of 11 patients; results appear to be best when the obstruction is at the site of the old atrial septum. In patients with complex congenital heart disease associated with pulmonary stenosis who are potential candidates for the Fontan procedure, dilatation of the pulmonary valve stenosis can increase pulmonary blood flow, partially relieve cyanosis and eliminate the need for aortopulmonary shunts with the attendant risk of pulmonary artery stenosis. We have performed balloon dilatation of the pulmonary valve in 4 such patients, and increased the aortic saturation to 80 to 85% without producing pulmonary artery hypertension. This limited relief of the gradient is presumably due to the relatively small balloons that are used and to the many patients who have associated subpulmonary stenosis with hypoplastic pulmonary anuli. Studies have also demonstrated that balloon dilatation can be used to palliate patients with Tetralogy of Fallot. Recent reports of the use of intravascular stents in animals and humans suggest that they may be useful in a variety of lesions in which current results of balloon dilatation are inadequate or transient, including dilatation of branch pulmonary artery stenoses and systemic and pulmonary veins. 80,81

Conclusions Recent advances have dramatically expanded the scope of catheter-directed therapy available to the pediatric cardiologist. Some procedures, such as balloon valvotomies and device closure of intra- and extracardiac defects, allow definitive therapy, whereas others, such as balloon pulmonary angioplasty and coil embolization of collaterals, allow or simplify surgical repair. Most of the procedures remain investigational either in terms of establishing indications in relation to outcome and complications compared with surgical procedures or in terms of optimizing equipment and technique.

References 1. Rashkind W], Miller WW. Creation of an atrial septal defect without thoracotomy: palliative approach to complete transposition of the great arteries. lAMA 1966;196:991-992, 2. Park SO, Zuberbuhler JR, Neches WH, Lenoa CC, Zoltun RA. A new atrial septostomy technique. Cathet Cardiovasc Diagn 1975;1:195-201. 3. Rubio-Alvarez V, Limon RL, Soni I. Valvulotomias intracardiocas par media de un caterer. Arch Inst Cordial Mex 1953;23:183-192. 4. Semb BKH, Tjonneland S, Stake G. Aabyholm G. Balloon volvulotomy of congenital pulmonary valve stenosis with tricuspid valve insufficiency. Cardiovasc Radial 1979;2:239-241. 5. Kan ]S, White RI Jr, Mitchell SE, Gardner TJ. Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary valve stenosis, N Engl I Med 1982;307:540-542. 6, Lababidi Z, Wu ]. Percutaneous balloon pulmonary volvuloplasty. Am [ Cordial 1963;52:560-562. 7. Kan iS, White RI Jr, Mitchell SE, Anderson JH, Gardner TJ, Percutoneous transluminal balloon valvuloplasty for pulmonary valve stenosis. Circulation 1964;69:554-560. 8. Rocchini AP, Kvelelis DA, Crowley D, Dic, M, Rosenthal A. Percutaneous balloon valvuloplasty for treatment of congenital pulmonary valvular stenosis in children. /ACC 1964;3:1005-1012. 9. Sullivan ID, Robinson PJ, Macartney FJ, Taylor JFN, Rees PG, Bull C, Deanfield IE. Percutaneous balloon valvuloplasty for pulmonary valve s t e n o sis in infants and children. Br Heart J 1985;54:435-441. 10. Tynan M, Baker E], Rohmer J, Jones ODH, Reidy ]F, Joseph MC, Ottenkamp J. Percutaneous balloon pulmonary valvuloplasty. Br Heart [ 1985;53: 520-524.

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11, Miller GAH. Balloon valvuloplasty and angioplasty in congenital heart disease. Br Heart I 1985;54:285-289. 12, Kvelselis DA, Rocchini AP, Snider AR, Rosenthal A, Crowley DC, Dick M. Results of balloon valvuloplasty in the treatment of congenital valvur pulmonary stenosis in children. An 1 Cordial 1985;56:527-532. 13, Radkte W, Keane JF, Fellows KE, Lang P, Lock IE. Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons. IACC 1986;8:909-915. 14, All Khan MA, Yousef SA, Mullins CE. Percutaneous transluminal balloon pulmonary valvuloplasty for the relief of pulmonary valve stenosis with special reference to double-balloon technique. Am kieart I 1986;112:158-166. 15. Ring JC, Kulik T], Burke BA, Lock JE. Morpholagic changes induced by dilation of the pulmonary valve analus with overlarge balloons in normal newborn lambs. Am I Cordial 1984;55:210-214. 16. Yeager S. Letter. [ACC 1987;9:467-468. 17, Lock JE, Keane JF, Fellows KE. Diagnostic and interventional catheterization in congenital heart disease. Boslon: Martinus Nijhoff, 1987. 18, Zeevi B, Keane IF, Fellows KE, Lock JE. Balloon dilation of critical pulmonary stenosis in the firsl week of life. IACC 1988;in press. 19, Lababidi Z. Aortic balloon valvuloplusty. Am Heart I 1983 1983;106:751. 20. Lababidi Z, Wu I, Walls JT. Percurtuneous balloon aortic valvuloplasty: results in 23 patients. Am I Cordial 1984;53:194-197. 21, Lababidi Z, Weinhaus L. Successful balloon valvuloplasty for neonatal critical aortic stenosis. Am Heart I 1986;112:913-916. 22. Choy M, Beekman RH, Rocchini AP, Crowley DC, Snider AR, Dick M, Rosenthal A. Percutaneous balloon valvuloplasty for vulvar aortic stenosis in infants and children. Am I Cordial 1967;59:1010-1013. 23, Helgason H, Keane JF, Fellows KE, Kulik TJ, Lock ]E. Balloon dilation of the aortic valve: studies in normal lambs and in children with aortic stenosis. IACC 1987;9:816-822. 24, Cribier A, Savin T, Berland J, Rocha P, Mechmeche R, Saoudi N, Behar P. Percutaneous transluminal balloon valvuloplasty of adult aortic stenosis: report of 92 cases. IACC 1987;9:381-386. 25. McKay RG, Safian RD, Lock JE, Mandell VS, Thurer RL, Schnitt S], Grossman W. Balloon dilation of calcific aortic stenosis in elderly patients: postmortem, intraoperative, and percutaneous valvuloplasty studies. Circulation 1986:74:119-125. 26. Phillips RR, Gerlis LM, Wilson N, Walker DR. Aortic valve damage caused by operutive balloon dilation of critical aortic valve stenasis. Br Heart l 1987;57:168-170. 27. Waller BF, Girod DA, Dillon ]C. Transverse aortic wall tears in infants afler balloon angioplasty for aortic valve stenosis: relation of aortic wall damage to diameter of inflated angioplasty balloon and aortic lumen in 7 necropsy cases. IACC 1984;4:1235-1241. 28, Sholler GF, Keane IF, Perry SB, Sanders SP, Lock JE. Balloon dilation of aortic stenosis: influences of valve morphology and technique on outcome [abstr], Circulation 1987;76:suppl IV:IV-554. 29. Inoue K, Owaki T, Nakamura T, Kitamura F, Miyamoto N. Clinical application of transvenous mitral commissurotomy by a new balloon catheter. 1 Thorac Cardiovasc Surg 1984;87:394-402. 30. Lock ]E, Khalilullah M, Shrivastava S, Bahl V, Keane ]F. Percutaneous catheter commissurotomy in rheumatic mitral stenosis. N Engl I Med 1985:313:1515-151& 31, McKay RG, Lock IE, Safian RD, Come P, Diver DI, Bairn DS, Berman AD, Warren SE, Mandell VE, Royal kiD, Grossman W. Balloon dilation of mitral stenosis in adult patients: postmortem and percutaneous valvuloplasty studies. IACC 1987;9:723-731. 32. AI Zaibag M, A1 Kasab S, Ribero PA, AI Fagih MR. Percutaneous doubleballoon valvotomy for rheumatic mitral-valve stenosis. Lancet 1986:1:757-761. 33. McKay CR, Kawanishi DT, Rahimtoola SH. Catheterballoon valvuloplasty treatment of milral stenosis in adult patients: initial experience with double balloon technique (abstr]. Circulation 1986:74:II:11-208. 34. Babic UU, Pejcic P, Djurisic Z, Vucinic M, Grujicic SM. Percutaneous transarterial valvuloplasty far mitrol stenosis. Am I Cordial 1986;57:11011104. 35, Diver D], Safian RD, Berman AD, Lock JE, Come PC, Warren SE, Balm DS, Grossman W. Percutaneous balloon mitral valvuloplasty: acute results and long-term follow-up (abstr). IACC 1987;9:14A. 36. Kveselis DA, Rocchini AP, Beekman R, Snider AR, Crowley D, Dick M, Rosenthal A. Balloon angiaplasty for congenital und rheumatic mitral stenasis. Am I Cordial 1986;57:348-350. 37. Alday LE, ]uaneda E. Percutaneous balloon dilatation in congenital mitral slenosis. Br Heart I 1987;57:479-482. 38, Singer MI, Rowen M, Dorsey T]. Tronsluminal aortic balloon angioplasty for coarclation of the aorta in the newborn. Am lleart I 1982;103:131-132. 39. Lock ]E, Bass ]L, Amplatz K, Fuhrman BP, Castaneda-Zuniga W. Balioon dilation angioplasty of aortic coarctation in infants and children. Circulation 1983;68:109-116. 48. Kan IS, White RI, Mitchell SE, Farmlett E l, Donahoo IS, Gardner T]. Treatment of restenosis of coarctation by percutaneous Iransluminal angioplasty, Circulation 1983;68:1087-1094. 41. Cooper RS, Ritter SB, Golinko R]. Balloon dilation angioplasty: nonsurgicol management of coarctation of the aorta. Circulation 1964;70:903-907. 42, de Lezo ]S, Fernandez R, Sancho M, Coneha M. Arizon I, Franco M,

Alemany F, Barcones F, Lopez-Rubio F, Valles F. Percutaneous transluminal angioplasty for aortic isthmic coarctation in infancy. Am ] Cordial 1984; 54:1147-1149. 43. Allen HD, Marx GR, Ovitt TW, Goldberg SJ. Balloon dilation angioplasty for coarctation of the aorta. Am ] Cordial 1986;57:828-832. 44. Saul JP, Keane JF, Fellows KE, Lock ]E. Balloon dilation ungioplasty of postoperative aortic obstructions. Am ] Cardiol 1987;59:943-948. 45. Lock ]E, Niemi T, Burke B, Einzig S, Castaneda-Zuniga W. Transcutaneous angioplasty of experimental aortic coarctation. Circulation 1982;66:12801286. 46. Finley JP, Beaulieu RG, Nanton MA, Roy DL. Balloon catheter dilation of eoarctation of the aorta in young infants. Br Heart [ 1983;50:411-415. 47, Marvin WJ, Mahoney LT, Rose EF. Pathologic sequalae of balloon dilation angioplasty for unoperated confutation of the aorta in children (abstr). IACC 1986;7:117A. 48. Cooper RS, Ritter SB, Rathe WB, Chen CK, Griepp R, Golinko RJ. Angioplasty for coarctation of the aorta: long-term results. Circulation 1987;75:600604. 49. Morrow WR, Vick GW, Nihil MR, Mullins CE, kiedrick T, McNamara DG. Intermediate follow-up of balloon dilution angiaplasty of native courctation (abstr]. [ACC 1987:9:75A. 50. Beekman RH, Rocchini AP. Long-term results of percalaneous balloon angioplasty for native caarctatian. IACC 1987;9:75A. 51. Lock JE, Niemi T, Einzig S, Amplatz K, Burke B, Bass ]L. Transvenous angioplasty of experimental branch pulmonary artery stenosis in newborn lambs. Circulation 1981;64:886-893. 52, Edwards BS, Lucas RV, Lock JE, Edwards ]E. Morphologic changes in the pulmonary arteries after percutaneous balloon angioplasty for pulmonary arterial stenosis. Circulation 1985;71:195-201. 53, Lock JE, Castaneda-Zuniga WR, Fuhrman BP, Bass ]L. Balloon dilation angioplasty of hypoplastic and stenotic pulmonary arteries. Criculation 1983;67:962-967. 54. Ring ]C, Bass ]L, Marvin W, Fuhrman BP, Kulik TJ, Faker IE, Lock JE. Management of congenital stenosis of a branch pulmonary artery with balloon dilation angioplasty. Report of 52 procedures. J Thorac Cardiovasc Surg 1985;99:35-44. 55. Di Sessa TG, Yeatman LA ]r, Williams RG, Lois IF, Friedman WF, Laks H. Thrombosis complicating balloon angioplasty of left pulmonary artery stenosis after Fantan's procedure: successful treatment with intravenous streptokinose. Am I Cordial 1985;55:610-611. 56. Zuberbuhler JR, Anker E, Zoltun R, Burkholder ], Bahnson H. Tissue adhesive closure of aortic-pulmonary communications. Am Heart I 1974;88: 41-46. 57. Barth KH, White RI, Kaufman SL, Terry. RB, Roland ]M. Embolotherapy of pulmonary ateriovenous malformations with detachable balloons. Radiology 1982;142:599-606. 58. Terry PB, White RI It, Barth KH, Kaufman SL, Mitchell SE. Pulmonary arteriovenous malformations: physiologic observations and results of therapeutic balloon embolization. N Engl J Med 1983;308:1197-1200. 59. Florentine M, Wolfe RR, White RI Jr. Balloon embolization to occlude a Blalock-Taussig shunl. ]ACC 1984;3:200-202. 60. Castaneda-Zuniga W, Epstein M, Zollikofer C, Nath PH, Formanek A, Ben-Shachar G, Amplatz K. Embolization of multiple pulmonary artery fistulas. Radiology 1980;134:309-310. 61, Culham JAG, Izukawa T, Burns JE, Freedom RM. Embolizatian of a Blalock-Taussig shunt in a child. A JR 1981:137:413-415. 62, Szarnicki R, Krebber HJ, Wack ]. Wire coil embolizatian of systemicpulmonary artery collaterals following surgical correclion of pulmonary atresin. ] Thorac Cardiovasc Surg 1981;81:124-126. 63. Morag B, Rubinstein ZJ, Smolinsk3., A, Goat DA. Percutaneous closure of a Blalock-Taussig shunt. Cardiavasc Intervent Radial 1984;7:218-220. 64. Fuhrman BP, Bass JL, Castaneda-Zuniga W, Amplatz K, Lock JE. Coil embolization of congenital thoracic vascular anomalies in infants and children. Circulation 1984;70:285-289. 65. Anderson ]H, Wallace S, Gianturco C, Gerson LP. "Mini" Gianturco stainless steel coils for transcatheter vascular occlusion. Radiology 1979; 132:301-303. 66. Porstmann W, Wierny L, Waenke H, Gerstberger G, Romaniuk PA. Catheter closure of patenl ductus arteriosas, 62 cases treated without thoracotomy. Radial Clin North Am 1971;9:203-218. 67. Sato K, Fujino M, Kozuka T, Naito Y, Kitamura S, Nakano S, Ohyama C, Kawashima Y. Transfemoral plug closure of patent ductus arteriosus. Circular/on 1975;51:337-341. 68. Rashkind W 1, Cuaso CC. Transcatheter closure of patent ductus arteriosus. Pediatr Cardiol 1979;1:3-8. 69. Lock JE, Bass ]L, Lund Gunnar, Rysavay IA, Lucas RV. Transcatheter closure of palent ductus arteriosus in piglets. Am ] Cordial 1985;55:826-829. 70, Rashkind W], Mullins CE, Hellenbrand WE, Tait MA. Nonsurgical closure of palent duclus arteriosus: clinical application af the Rashkind PDA occluder system. Circulation 1987;75:583-592. 71_.,Rashkind WJ. Transcatheter treatment of congenital heart disease. Circulalion 1983;67:711-716. 72, Lock ]E, Cockerham IT, Keane IF, Finley JP, Wakely PE, Fellows KE. Transcalheter umbrella closure of congenital henri defecls. Circulation

ty 9, 19•8

1987;75:593-599. 73. Lock ]E, Block PC, McKay RG, Bairn DS, Keane IF. Catheter closure of postinfarction/postoperative ventricular defects: initial experience (abstr]. Circulation 1987;76 suppl IV:IV-28. 74. O'Laughlin MP, Vick GW, Nihill MR, Bricker IT, Mullins CR. Foreign body retrieval: transcatheter removal of embolized patent ductus arteriosus occlusion devices and catheter pieces (abstr}. ]ACC 1987;9:130A. 75. De Lezo IS, Pan M, Sancho M, Herrera N, Arizon l, Franco M, Concha M, Val]es F, Romanos A. Percutaneous transluminal balloon dilation for discrete subaortic stenosis. Am ] Cordial 1986:5&619-621. 76. Lababidi Z, Weinhaus L, Stoeckle H, Walls IT. Transluminal balloon dilation for discrete subaortic stenosis. Am [ Cordial 1987;59:423-425. 77. Lloyd TR, Marvin WJ ]r, Mahoney LT, Lauer RM. Balloon dilation valvuloplosty of bioprosthetic valves in extracardiac conduits. Am Heart [ 1987;

I, . A k .

,.,AN oot~ ,, JAL Ur" L, AHL,. J L ( J ~ f"

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114:268-274. 78. DriscolI O], Hesslein PS, Mullins CE. Congenital stenosis of individual pulmonary veins: clinical spectrum and unsuccessful treatment by transve-

nous balloon dilation. Am ] Cordial 1982;49:1767-1772, 79. Lock IE, Bass ]L, Castaneda-Zuniga W, Fuhrman BP, Rashkind Wl, Lucas RV. Dilation angioplasty of congenital or operative narrowings of venous channels. Circulation 1904;70:457-464. 80. O'Laughlin MP, Vick GW, Mayer D, Schatz RA, Palmax ]C, Fisher D], Mullins CE. Implantation of balloon expandable intravnsculor grafts by catheterization in pulmonary arteries and systemic veins (abstr}. IACC 1987;9: 131A. 81. Sigwart U, Puel I, Mirkovitch V, ]afire F, Kappenberger L. Intravascular stents to prevent occlusion and restenosis after transluminal angioplasty. N F_,ngl I Med 1987;316:701-706.