Pediatric Interventions

One-Step Treatment of Patent Ductus Arteriosus andPulmonary Artery Stenosis by Cardiac Catheterization

Giuseppe Santoro,* MD, Maurizio Cappelli Bigazzi, MD, Maria Teresa Palladino, MD,Marianna Carrozza, MD, Maria Giovanna Russo, MD, and Raffaele Calabro, MD

Coexistence of pulmonary artery (PA) stenosis and patent ductus arteriosus (PDA) maylead to overestimating the relative importance of these malformations. This article re-ports on a patient with a trivial PDA and severe PA stenosis erroneously diagnosed asmoderate PDA. He was successfully submitted to simultaneous PA stenting and PDAembolization. Cathet Cardiovasc Intervent 2003;59:271–275. © 2003 Wiley-Liss, Inc.

Key words: pulmonary artery stenosis; patent ductus arteriosus; stent; coil embolization

INTRODUCTION

The association between patent ductus arteriosus(PDA) and pulmonary artery (PA) stenosis is well known[1,2]. Indeed, pulmonary artery stenosis is supposed to becaused by invasion of ductal tissue into the pulmonaryartery wall, with resulting retraction and coarctation afterthe postnatal ductal closure. However, in the presence ofPDA, a coexistent pulmonary artery stenosis may beoverlooked, with unfavorable outcome after transcatheterductal occlusion. Here we report on a child sent totranscatheter closure of a moderate ductus arteriosus whoshowed instead a severe left pulmonary artery stenosisassociated to a trivial PDA. This patient successfullyunderwent pulmonary artery stenting followed by PDAcoil embolization during the same procedure. To ourknowledge, no previous reports of this combination ofcatheter-based interventions during a single procedurehave been reported in literature.

CASE REPORT

A 7-year-old boy was referred to our institution forcardiac evaluation of a systolic murmur. Clinical exam-ination was negligible, except for a harsh 2/6 systolicmurmur at the left subclavian area. Electrocardiogramand chest X-ray were normal. At echocardiography, theleft chamber dimensions were in the upper quartile of thenormal values for body surface area. Color Doppleranalysis from the short-axis parasternal view showed aleft-to-right shunt due to an apparently moderate-sized

PDA (Fig. 1), with a transductal peak pressure gradientof about 40 mm Hg. Based on these clinical and echo-cardiographic findings, it was indicated percutaneousPDA embolization. At cardiac catheterization, a conicalPDA was imaged. It was smaller than expected (minimaldiameter, 1.1 mm) and resulted in a trivial left-to-rightshunt (QP/QS 1.1; Fig. 2A). However, hemodynamicdata showed a moderate right ventricular systolic over-load that, angiographycally, resulted from a long, severe,segmental hypoplasia of the left pulmonary artery, be-ginning distal from the PDA implantation site. The ste-notic segment was 12 mm in length and showed a min-imal diameter of 2.5 mm (Fig. 2B), resulting in a peak-to-peak pressure gradient of 30 mm Hg and an RV/LVpressure ratio of 0.6. Thus, it was planned to relieve firstthe pulmonary artery stenosis by stent implantation, re-lying on the PDA-occluding power of a successful stentdeployment. Thus, a 17 mm Jomed stent was dilated to 8mm (Fig. 3), completely abolishing the pulmonary pres-sure gradient and significantly reducing the right ventric-ular pressure overload (final RV/LV pressure ratio 0.38).

Cardiac Catheterization Laboratory, Division of Pediatric Cardi-ology, A.O. “Monaldi,” II University of Naples, Naples, Italy

*Correspondence to: Dr. Giuseppe Santoro, Via Vito Lembo, 14,84131, Salerno, Italy. E-mail: santoropino@tin.it

Received 16 July 2002; Revision accepted 18 December 2002

DOI 10.1002/ccd.10507Published online in Wiley InterScience (www.interscience.wiley.com).

Catheterization and Cardiovascular Interventions 59:271–275 (2003)

© 2003 Wiley-Liss, Inc.

However, stent implantation did not cause the expectedclosure of the PDA. Instead, it appeared larger thanbefore (Fig. 4A), presumably due to local geometricchanges caused by the stent deployment. Thus, the PDAwas successfully occluded with a 3PDA-5 Cook detach-able coil (Fig. 4B).

DISCUSSION

Isolated peripheral pulmonary artery stenosis occurs in2%–3% of all patients with congenital heart disease [1],resulting in a frequently asymptomatic right ventricularpressure overload. Pulmonary branch stenosis is oftenfound in association with a PDA or ligamentum arterio-sus, thus supporting the pathogenetic hypothesis of anarterial coarctation due to the extension of contractileductal tissue within the pulmonary artery wall [1,3,4].

However, ductal patency and pulmonary artery stenosis,mainly in the form of long segmental hypoplasia, maynot have any cause-effect relationship [2]. In the pres-ence of PDA, clinical and echocardiographic diagnosis ofleft PA stenosis may be challenging and the true PDAsize and/or hemodynamic impact of the vascular stenosismay be difficult to assess. In this case, overlooking aneven mild PA stenosis might result in a significant in-crease of the vascular obstruction once the PDA-occlud-ing device is implanted. Although the association be-tween PDA and pulmonary artery stenosis is notinfrequent, the review of the English literature did notshow any report of their simultaneous treatment by in-terventional catheterization. In this malformation com-plex, the sequence of interventions could be the PDAembolization followed by the pulmonary artery angio-plasty/stenting or vice versa. In the former option, the

Fig. 1. Echocardiographic color Doppler analysis in parasternal short-axis view (ductus arte-riosus cut) showing a significant left-to-right shunt due to a supposed moderate PDA (arrow).Ao, aorta.

272 Santoro et al.

Fig. 2. A: Aortic angiography in lateral view showing a small-sized conical PDA (arrow) thatfaintly opacifies the pulmonary artery. B: Pulmonary angiography in four-chamber view showinga severe stenosis of the left pulmonary artery (asterisk) due to a long segmental hypoplasia justdistal the PDA implantation site.

Fig. 3. Pulmonary angiography in right oblique view (A) and four-chamber view (B) afterstenting of the left pulmonary artery with a 17 mm long Jostent dilated to 8 mm.

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positioning of the occluding device may prevent thedilating balloon to be advanced into the stenotic pulmo-nary artery. Again, the long sheath for stent deploymentmight dislodge the PDA-occluding device. Conversely, itmight be difficult to negotiate the PDA from either thevenous or arterial side after stenting of the left pulmonaryartery. Another potential option for a simultaneous treat-ment of PDA and pulmonary artery stenosis could be toimplant a covered stent, long enough to cover the PDAinsertion site, at the site of pulmonary artery stenosis.Our attempt of treating both malformations by dilatingthe left pulmonary artery with an uncovered stent provedunsuccessful. Indeed, after stent deployment, the ductalsize even increased, similar to a previously reported caseof aortic coarctation and PDA treated by aortic angio-plasty and PDA coil occlusion [5]. Also, in this case, theductal morphology and size changed soon after the co-arctation angioplasty, presumably due to geometric mod-ifications of the PDA implantation site. It could be hy-

pothesized that even a controlled tear of the aortic orpulmonary artery wall, resulting from angioplasty/stent-ing, might extend to the ductal insertion region, so mod-ifying the PDA morphology or size. Thus, in our opinion,when PDA is associated with aortic coarctation or pul-monary artery stenosis, it seems advisable to performangioplasty/stenting first, before proceeding to the PDAclosure, choosing the type and size of the occludingdevice only after a second angiographic evaluation.

In conclusion, the association of PDA and pulmonaryartery stenosis should always be ruled out, mainly in caseof a discrepancy between echocardiographic and angio-graphic appearance of the ductal shunt. Dilating or stent-ing the PDA closely related regions might result in asignificant change of the local geometry, so increasingthe PDA size and/or its hemodynamic relevance. There-fore, in this case, any vascular dilating procedure shouldbe followed by a new angiographic and hemodynamicevaluation of the ductus arteriosus, in order to choose the

Fig. 4. A: Aortic angiography after pulmonary artery stent im-plantation showing a significant increase of the PDA size (max-imal diameter, 2 mm), presumably due to geometric changesdue to pulmonary artery stenting. B: Aortic angiography after

the successful PDA closure with a Cook detachable coil (3PDA-5). The distance between the pulmonary end of the PDA and thesite of pulmonary stenosis highlights the spatial relationshipbetween these malformations.

274 Santoro et al.

best PDA-occluding device size and type for the individ-ual patient.

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