Editorial Comment

Failure of Cutting BalloonAngioplasty to PreventRestenosis in ChildhoodPulmonary Vein Stenosis

Victor Lucas, MD

Ochsner Clinic Foundation,New Orleans, Louisiana

Pulmonary vein stenosis is usually described as either‘‘congenital,’’ postoperative, or as a complication ofarrhythmia ablation within the pulmonary veins. ‘‘Con-genital’’ pulmonary vein stenosis refers to the unoper-ated patient and is decidedly uncommon, representing0.4% of congenital heart defects [1]. Postoperativestenosis is reported in up to 18 percent of patients afterrepair of total anomalous pulmonary venous connec-tions [2]. Pulmonary vein stenosis after ablation is com-mon particularly with the selective segmental ostialablation approach (18.8%) [3].The nature of pulmonary veno-occlusion is incom-

pletely characterized in all but postablation lesions.Acquired postoperative pulmonary vein stenosis maybe localized to an anastomosis or extend diffusely intothe pulmonary vein branches. Individual small pulmo-nary vein size, small confluence size, and the presenceof heterotaxy syndrome may predict preoperativelystenosis remote from the anastomosis site [4]. Histo-logically, the lesions are characterized by fibrous inti-mal thickening and medial hypertrophy. With congen-ital pulmonary vein stenosis, histologic appearanceand strong diffuse immunoreactivity for smoothmuscle markers indicate that the intimal lesional cellsare myofibroblast-like. Expression of various receptortyrosine kinases and some ligands suggests an autocrineor paracrine role of these proteins in the pathogenesisof the intimal occlusive lesions [5]. Congenital andpostoperative pulmonary vein stenosis typically carrya very poor prognosis. In particular, bilateral pulmo-nary vein involvement almost always leads to earlydeath.Treatment of pulmonary vein stenosis is by surgical

or percutaneous approach or both. With either, preop-erative evaluation typically includes echocardiography,angiography, and axial imaging (CT/MR). Sutureless

pericardial marsupialization is a relatively new surgi-cal technique that may be associated with improvedoutcomes [1]. Use of absorbable polydioxanone suturemay cause less restenosis than nonabsorbable material[6]. Plain old balloon angioplasty is usually ineffectivewith incomplete stenosis relief and nearly universalrestenosis within weeks or months. Bare metal stentswith or without systemic anti-inflammatory treatmentare associated with high risk for restenosis. Currentlyavailable drug eluting stent are too small for pulmo-nary veins. Preliminary experiences suggest that cut-ting balloon angioplasty for pulmonary vein stenosisis safe [7].In the current report, the interesting case of a five

year old boy with ‘‘congenital’’ pulmonary vein stenosisis reviewed, adding to our understanding of the courseof this disease and its treatment. Notably, pathologicexamination postpneumonectomy showed fibroprolifera-tive pulmonary vein obliteration. The authors surmisedreasonably that cutting balloon angioplasty might beeffective acutely in the remaining lung and hoped for alasting improvement. Despite repeated application of thistechnology and addition of systemic anti-inflammatorytreatment severe restenosis remained an ongoingproblem.Clearly there is much more to be learned about the

cause and treatment of pulmonary vein stenosis.Design and implementation of randomized treatmenttrials is hindered by the disease heterogeneity and rar-ity and the lack of consistently effective treatmentapproaches. Although the current surgical and catheterbased interventions along with oral pulmonary vasodi-lators offers hope for extending survival, the unrelent-ing course of this disease continues to make warrantednovel approaches to treatment. Changes in stentdesign including mechanism (self expanding ratherthan balloon expanding), local drug delivery (e.g. siro-limus, paclitaxel), catheter-based thermal angioplasty(cold or hot), and local radiation are exciting modal-ities to consider for the treatment of pulmonary veinstenosis.

Received 30 May 2006; Revision accepted 30 May 2006

DOI 10.1002/ccd.20863

Published online 12 October 2006 in Wiley InterScience (www.

interscience.wiley.com).

' 2006 Wiley-Liss, Inc.

Catheterization and Cardiovascular Interventions 68:767–768 (2006)

REFERENCES

1. Bove E., et al. Management of congenital and acquired pulmo-

nary vein stenosis. Ann Thorac Surg 2006;81:992–996.

2. Tsang VT, et al. Management of pulmonary venous obstruction

after correction of TAPVC: Risk factors for adverse outcome.

Eur J Cardiothorac Surg 2003;24:28–36.

3. Brugada J, et al. Incidence of pulmonary vein stenosis in patients

submitted to atrial fibrillation ablation: A comparison of the selec-

tive segmental ostial ablation vs. the circumferential pulmonary

veins ablation. J Interv Card Electrophysiol 2005;14:21–25.

4. Colan SD, et al. Individual pulmonary vein size and survival in

infants with totally anomalous pulmonary venous connection.

J Am Coll Cardiol 1993;22:201–206.

5. Collins T, et al. Pulmonary vein stenosis: Expression of receptor

tyrosine kinases by lesional cells. Cardiovasc Pathol 2006;15:

91–99.

6. McGough EC, et al. Absorbable polydioxanone suture and results

in total anomalous pulmonary venous connections. Ann Thorac Surg

1995;60:55–59.

7. Rigby ML, et al. Pulmonary vein stenosis: Initial experience with

cutting balloon angioplasty. Heart 2006;92:815–820.

768 Lucas