Congenital pulmonary vein stenosis (PVS) occurs as an isolated lesion or in association with other congenital heart anomalies such as heterotaxy, transposition of the great arteries, and various types of single-ventricle pathology [2]. The obstruction may affect any or all veins, and it can be localized or extended deep into the lung parenchyma [3].

Surgical therapies have evolved to treat PVS, sometimes with satisfactory midterm results [2, 3]. However, for the most extensive form of the disease, neither surgical repair nor transcatheter therapy has resulted in long-term relief of the stenosis [1, 2, 5]. Hence, only lung or heart–lung transplantation can be offered. Unfortunately, this approach is less than optimal due to the high mortality experienced by patients on the waiting list [5].

We present a case of extensive and recurrent congenital PVS despite multiple surgeries. Heart–lung transplantation was performed after a drug-eluting stent was placed in the stenotic pulmonary veins.

Case Report

A baby girl with a neonatal diagnosis of criss-cross heart, double outlet right ventricle, and a noncommitted ventricular septal defect underwent pulmonary artery banding at the age of 2 months. At 12 months, she experienced pulmonary edema due to cor triatriatum associated with obstruction of the four pulmonary veins.

Surgical resection of the intraatrial membrane and sutureless repair of the PVS was performed. The girl’s postoperative course was uneventful, with transcutaneous oxygen saturations (O2sat) of 70–80% and no residual gradient across the pulmonary veins shown by echocardiography. However, worsening of cyanosis developed, with restenosis of the four pulmonary veins, as demonstrated by Doppler-echocardiography. The girl therefore underwent surgery again at the age of 17 months.

Intraoperative examination showed an extensive fibrous reaction in the four pulmonary veins, with retrograde extension deep into the lung. Endocardial resection was performed, but she again experienced restenosis with profound cyanosis, necessitating another reoperation at the age of 23 months. The right PVS was treated by surgical enlargement, with the two pulmonary veins forming a common drainage at the end of the repair. The left pulmonary veins were not identified. Postoperatively, the girl remained unstable with O2sat in the low 70 s and near systemic pulmonary arterial hypertension (PAH). She then was listed for heart–lung transplantation.

After 2 months, the girl presented again with profound cyanosis (40% of O2sat) and low cardiac output. It was then decided to balloon dilate and stent the common drainage of the right pulmonary vein. Because she seemed to have an enormous potential for pulmonary vein neointimal proliferation, drug-eluting stents were used. A Taxus Liberté stent (Boston Scientific, Natick, MA, USA) 5 mm wide and 20 mm long was chosen. With the girl under general anesthesia, right femoral venous access was achieved. The pressure gradient of the common drainage from the right pulmonary vein to the left atrium was 20 mmHg, with severe stenosis shown by angiography (Fig. 1a) and near systemic PAH.

Fig. 1
figure 1

Frontal selective angiogram of the right pulmonary vein collector showing severe stenosis (a), which is successfully relieved after implantation of a 5 × 20-mm Taxus Liberté stent (b)

Full size image

The stent was implanted with pressure gradient abolition and an excellent angiographic result (Fig. 1b). The girl improved with O2sat in the high 70 s and a moderate decrease of PAH to 60% of systemic pressure. She was discharged home receiving aspirin 5 mg/kg, clopidogrel 0.2 mg/kg, and sildenafil 2 mg/kg/day. After 2 months, she had gastrointestinal bleeding, which was treated with antiacid medication. The aspirin was stopped, and she remained stable receiving clopidogrel alone.

The girl underwent heart–lung transplantation 182 days after pulmonary vein stenting at the age of 30 months. Intraoperative examination showed no intimal neoproliferation inside the stent. Postoperatively, phrenic paralysis and superior vena cava stenosis occurred, necessitating reoperation on posttransplantion day 5. She failed to be extubated on posttransplantation day 12 due to right lung pneumonia, which was treated with wide-spectrum antibiotherapy. She evolved toward acute respiratory distress syndrome, and circulatory support was started.

After 5 days, the girl experienced diffuse brain ischemia with bilateral mydriasis. Bronchial aspiration culture showed Legionella pneumophila. Considering her neurologic status and prognosis, it was decided to withdraw circulatory support. She died on posttransplantation day 23.

Discussion

Despite the unfavorable outcome in the reported case, the potential benefits of pulmonary vein drug-eluting stent placement as a bridge to transplantation are compelling. Sutureless surgery of PVS has improved the outcome for patients with congenital or acquired PVS. However, diffuse and extensive PVS, as in the reported case, remains a potential complication and almost uniformly fatal. Pathologic changes resulting in pulmonary vein narrowing include fibrocellular intimal proliferation, thrombus formation, endocardial contraction, and elastic lamina proliferation. Only heart–lung transplantation provides an effective therapy. Nevertheless, half of the patients die while on the waiting list [5].

Our objective in using a drug-eluting stent was to allow a long period of hemodynamic stability until a donor became available. In this setting, the three attempts with surgery were not successful because PVS recurred 4, 3, and 2 months after the operation, respectively.

Other transcatheter therapy approaches seem less than optimal. A high restenosis rate is reported after balloon dilation. Similarly, the use of bare stenting is subject to restenosis due to neointimal proliferation, especially in vessels with a diameter smaller than 10 mm [6, 7]. One of the most recent reports describing the largest series showed restenosis in six of seven PVS stents in four children with congenital PVS [7]. Novel adjunctive therapies such as stenting with oral drug elution have been successful for a few patients [1], but not for the more extensive form of the disease, as in the reported case.

Drug-eluting stent placement is likely to restenose in coronary artery disease. For children with potential concern about systemic drug diffusion, Paclitaxel seems particularly appropriate because it is highly lipophilic and hydrophobic, which may increase vascular absorption in surrounding tissue. No detectable Paclitaxel was found in the bloodstream after Taxus TM stent implantation [4]. Recently, the good tolerance of three similar stents implanted in a neonate with obstructed totally anomalous pulmonary venous return was reported [8]. Finally, premounted drug-eluting coronary stents cannot be considered a definitive therapy for PVS because they require redilation and become restrictive with the patient’s growth.

In conclusion, we describe drug-eluting stent placement as a bridge to heart–lung transplantation in a child with severe and recurrent PVS after surgical failure. More cases and a longer term follow-up assessment are needed to confirm the interest of such therapy.