Catheterization and Cardiovascular Diagnosis 34:46-47 (1995)

Editorial Comment

FROM IMAGINATION TO REALITY: HOW MUCH CAN INTERVENTIONAL CARDIOLOGISTS DO FOR CONGENITAL CARDIAC ANOMALIES?

Carlos E. Ruiz, MD, PhD, FSCAI, Ranae L. Larsen, MD, and He Ping Zhang, MD Lorna Linda University School of Medicine Lorna Linda, California

During the past decade, nonsurgical cardiovascular catheterization has been rapidly advancing, and new de- vices and interventional techniques have been providing excellent results in the treatment of a number of cardio- vascular diseases which formerly required surgical inter- vention. More recently, these techniques, including bal- loon, laser, stent, and other devices, have been used to treat congenital cardiac malformations, and the initial and short- to intermediate-term results are encouraging. These treatments are used either as a palliative therapy for temporarily relieving symptoms and improving he- modynamic stability prior to the later definitive surgery or as a “definitive” treatment. Rashkind developed an umbrella occluding device to close patent ductus arteri- osus by transvenous percutaneous approach [ 11. Lock and his coworkers modified Rashkind PDA Occluder into a clamshell device to close atrial or ventricular septal defect [2,3]. Grifka et al. [4] and Moore et al. 1.51 used a balloon technique to dilate congenital mitral stenotic valves: Mullins and O’Laughlin and their colleagues used balloon dilatation and/or stent implantation tech- nique(s) to treat hypoplastic pulmonary arteries and other cardiac defects [6-81. Ruiz et al. implanted a stent to the ductus arteriosus to maintain its patency in infants with hypoplastic left heart syndrome [9]. Qureshi et al. [ 101 and Rosenthal et al. [ l l ] used a laser-assisted balloon method to treat pulmonary valve atresia. Hausdorf et al. reported using radiofrequency to perforate muscular atresia of the right ventricular outflow tract followed by balloon dilatation and/or stent implantation [ 121.

In this issue of the journal, Schneider and his cowork- ers report another application of interventional catheter- ization technique in the treatment of congenital cardiac defects [ 131. They successfully combined transcatheter radiofrequency perforation and stent implantation tech- niques as a palliative treatment for fibromuscular pulmo- nary atresia with ventricular septal defect in a 3,060-g baby girl. Pulmonary atresia with ventricular septal de-

fect is not an uncommon form of congenital cardiac anomaly and its treatment remains difficult and chal- lengeable. The clinical manifestations depend on the size of the pulmonary arteries, patency of the ductus arterio- sus, and bronchial collateral vessels. Without interven- tion, 30% of them die during the 1st week of life, and only those with large collateral vessels may survive be- yond infancy [ 141. Surgical correction of pulmonary atresia with ventricular septal defect requires adequate development of the pulmonary vasculature; the proce- dure is performed by a palliative surgical shunt, but often results in postoperative scar formation and distortion of the pulmonary arteries. The importance of the work done by Schneider et al. is to demonstrate that the growth of the pulmonary vascular bed can be achieved by percuta- neous approach to open an atretic outflow tract in an infant with very small body size. Although an implanted stent can support the vessel wall thus improving the blood flow into the pulmonary vascular tree, the pul- monic regurgitation is a concern. However, from surgi- cal experience of reconstruction of right ventricular out- flow tract, such regurgitation is well tolerated and may help in development of the pulmonary vascular bed [ 15- 171. Furthermore, there is a speculation that the pulmo- nary arterial tree may be developed better with an unre- stricted pulsatile flow, such as achieved by stenting, than with a non-pulsatile flow through a shunt.

Interventional catheterization technique is not only a palliative strategy but sometimes also a lifesaving pro- cedure, such as the cases reported by Schneider et al. [ 131 and Ruiz et al. [9]. Without percutaneous interven- tion, the likelihood of survival in these infants was small.

Before entering the catheterization laboratory, two questions must be asked. First, how much can the pro- cedure help the patient with the specific cardiac defect? In other words, what is the expected outcome? Second, what are the potential risks of the procedure compared with the “established treatment” such as surgery?

Another important issue should also be addressed. The catheterization procedures with most new devices per- formed in neonates, infants, and children are technically very demanding and not without risks. At the present time, these interventional procedures are not for every pediatric cardiology center, and we think it prudent to recommend that these procedures be restricted to select, strategically placed referral institutions.

As new endovascular devices continue to develop and

0 1995 Wiley-Liss, Inc.

Nonsurgical Intervention for Heart Defects 47

8. O’Laughlin MP, Slack MC, Grifka RG, Perry SB, Lock JE, Mullins CE: Implantation and intermediate-term follow-up of stents in congenital heart disease. Circulation 88:605-614, 1993.

9. Ruiz CE, Gamra H, Zhang HP, Garcia EJ, Boucek MM: Brief report: stenting of the ductus arteriosus as a bridge to cardiac transplantation in infants with the hypoplastic left-heart syn- drome. N Engl J Med 328:1605-1608, 1993.

10. Qureshi SA, Rosenthal E, Tynan M, Anjos R, Baker EJ: Trans- catheter laser-assisted balloon pulmonary valve dilation in pul- monic valve atresia. Am J Cardiol 67:428-431, 1990.

1 1 . Rosenthal E, Qureshi SA, Kakadekar AP, Anjos R, Baker EJ, Tynan M: Technique of percutaneous laser-assisted valve dilata- tion for valvar atresia in congenital heart disease. Br Heart J

12. Hausdorf G, Schneider M, Lange P: Catheter creation of an open outflow tract in previously atretic right ventricular outflow tract associated with ventricular septal defect. Am J Cardiol 72:354- 356, 1993.

13. Schneider M, Schranz D, Michel-Behnke I, Oelert H: Transcath- eter radiofrequency perforation and stent implantation for pallia- tion of pulmonary atresia in a 3060g infant. Cathet Cardiovasc Diagn 34:42-45, 1995.

14. Samanek M: Children with congenital heart disease: probability of natural survival. Pediatr Cardiol 13:152-158, 1992.

15. Gill CC, Moodie DS, McGoon DC: Staged surgical management of pulmonary atresia with diminutive pulmonary arteries. J Tho- rac Cardiovasc Surg 73:436-442, 1977.

16. Freedom RM, Pongiglione G , Williams WG, Trusler GA, Rowe RD: Palliative right ventricular outflow tract construction for pa- tients with pulmonary atresia, ventricular septal defect, and hy- poplastic pulmonary arteries. J Thorac Cardiovasc Surg 86:24- 36, 1983.

17. Piehler JM, Danielson GK, McGoon DC, Wallance RB, Fulton RE, Mair DD: Management of pulmonary atresia with ventricular outflow construction. J Thorac Cardiovasc Surg 80:552-567, 1980.

69~556-562, 1993.

catheterization techniques continue to improve, we can expect more and more congenital cardiac anomalies to be treated as palliative or even definitive alternative to sur- gical intervention. Combinations of several devices and techniques will continue to be adopted in order to achieve maximal treatment effects. Percutaneous inter- vention will play an increasingly important role conju- gating with surgery in the overall treatment strategy for congenital cardiac defects.

REFERENCES

I . Rashkind WJ, Mullins CE, Hellenbrand WE, Tait MA: Nonsur- gical closure of patent ductus arteriosus: clinical application of the Rashkind PDA Occluder System. Circulation 75583-592, 1987.

2. Rome JJ , Keane JF, Perry SB, Spevak PJ, Lock JE: Double- umbrella closure of atrial defects. Initial clinical applications. Circulation 82:751-758, 1990.

3. Perry SB, Lock JE: Front-loading of double-umbrella devices, a new technique for umbrella delivery for closing cardiovascular defects. Am J Cardiol 70:917-920, 1992.

4. Grifka RG, O’Laughlin MP, Nihill MR, Mullins CE: Double- transeptal, double-balloon valvuloplasty for congenital mitral stenosis. Circulation 85: 123-129, 1992.

5 . Moore P, Adatia I, Spevak PJ, Keane JF, Perry SB, Castaneda AR, Lock JE: Severe congenital mitral stenosis in infants. Circu- lation 89:2099-2106, 1994.

6. Mullins CE, O’Laughlin MP, Vick W 111, Mayer DC, Myers TJ, Kearney DL, Schatz RA, Palmaz JC: Implantation of balloon- expandable intravascular grafts by catheterization in pulmonary arteries and systemic veins. Circulation 77: 188-199, 1988.

7. O’Laughlin MP, Perry SB, Lock JE, Mullins CE: Use of endo- vascular stents in congenital heart disease. Circulation 1 923- 1939, 1991.