Congenital Pulmonary Vein Stenosis: Encouraging Mid-term Outcome

Abstract

Congenital pulmonary vein stenosis (PVS) is a rare entity with limited outcome literature. Multiple interventional approaches have evolved including surgical and catheterization techniques. Our objective is to report our center experience and to compare short-term and mid-term outcomes among these therapeutic modalities. Retrospective study on 23 patients (n = 23) with PVS that required intervention over the last 13 years (2000-2013). Patients were divided into three groups based on type of initial intervention. Of these, 10 (43.5%) had balloon angioplasty, 3 (13.0 %) had surgical dilation, and 10 (43.5%) had surgical marsupialization. Mortality and number of re-interventions were our primary outcomes. Mean age at diagnosis was 10.9 ± 18.4 months. Mean age at initial intervention was 14.5 ± 18.0 months. Mean pre- and post-initial intervention PVS gradients were 9.2 ± 3.4 and 3.4 ± 2.2 mmHg, respectively. Mean survival time and re-intervention-free survival time were 4.8 ± 4.0 and 2.8 ± 3.4 years. No statistical significance was found between the interventions with respect to survival time (p = 0.52) and re-intervention free time (p = 0.78). High initial pre- and post-intervention gradients were significantly associated with re-intervention-free survival (p = 0.01 and p = 0.03, respectively). Patients with bilateral disease have increased mortality (p = 0.01) and decreased 5-year survival (p = 0.009) compared to patients with unilateral disease irrespective of type of intervention. No statistically significant difference in mortality or re-intervention rate was present among these different therapeutic modalities. This study has the longest follow-up so far reported in the current literature (58 months) with overall survival of 78%.

Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study

“…Interestingly, patient-related factors-such as low weight and young age at operation, prematurity, genetic anomalies (including heterotaxy syndrome), and singleventricle anatomy-do not seem to increase the risk of poor outcomes in patients with PPVS, confirming the results of previous series [1][2][3][4][5]. Rather, the preoperative intrinsic severity of PPVS appears to be the most decisive factor determining final outcomes, as shown in the recent literature [1][2][3][4][5] and in our series. Indeed, many anatomical and hemodynamic factors reflecting the severity of the disease at the time of or 1 month after the procedure (bilateral disease, diffuse stenosis with no upstream PV dilatation, preoperative and postoperative severity score, and preoperative and postoperative pulmonary hypertension) tended to be or were significantly associated with a higher risk of death, restenosis, or reoperation in the univariate analysis.…”

“…rate of 47% (11 of 23) at a mean follow-up of 40.2 AE 42.2 months [2], which was confirmed by more recent PPVS studies [1,3,4]. Our multicentric series focusing on patients operated on in the current era (after 2000) confirms such disappointing results, with actuarial freedom from mortality, reoperation, and restenosis of 70% AE 8%, 62% AE 8%, and 47% AE 9%, respectively, at 8 years of follow-up.…”

“…The postoperative severity score and pulmonary hypertension at 1 month after operation were found to be independent risk factors in the multivariate analysis. A greater number of stenotic PVs has been shown to be associated with an increased risk of death in many series, especially when there is bilateral involvement [1,3,5]. Shi and associates [4] demonstrated that PVs with moderate stenosis, compared with those with mild stenosis, developed restenosis, irrespective of the surgical strategy.…”

“…Reported recurrence, reoperation, and mortality rates after treatment of PPVS remain high. Freedom from reoperation or death at 3 to 5 years after operation for PPVS hovers around 50% to 60% in studies focusing on patients with PPVS [1][2][3][4][5] and in studies mixing patients with primary and acquired PVS [6][7][8][9][10][11][12][13]. The concept of a sutureless technique for PVS repair, avoiding suturing of the veins themselves, was introduced almost 20 years ago [14][15][16].…”

Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era

“…[3][4][5][6] This technique was presented as real progress in some early reports, [7][8][9] while appearing disappointing in the more recent ones, [10][11][12][13][14][15][16] especially in the series focusing on patients with congenital PVS. [11][12][13]15,17 The outcomes of this technique in the specific population of PSPVS and risk factors for poor outcomes are still controversial because series are often limited and heterogeneous. We sought to determine current outcomes after surgical repair for PSPVS and prognostic factors in a large multicentric study focusing on PSPVS treated in the current era.…”