Post-repair Pulmonary Vein Stenosis Research Articles

Single-institution outcomes of surgical repair of infracardiac total anomalous pulmonary venous connection

ObjectiveThis contemporary study sought to describe the outcomes of patients undergoing biventricular repair of infracardiac total anomalous pulmonary venous connection. MethodsA retrospective study was performed on patients with infracardiac total anomalous pulmonary venous connection who underwent sutureless technique or conventional repair between 2006 and 2018. Risk factors for survival and post-repair pulmonary vein stenosis (PVS) were assessed with Cox regression model. Time-to-event analysis was conducted using Kaplan-Meier estimates. ResultsThis study included 82 consecutive patients with the median age of 21 days (interquartile range, 9-40 days). The median follow-up was 29 months (interquartile range, 12.5-59 months) and was available in 95% of the survivors at the end of the study period in 2019. Overall, 8 deaths (8.5%) occurred in the conventional repair group. There was a trend of higher mortality in the conventional repair group, although it did not reach a statistical difference (P = .2). Postrepair PVS occurred at a median of 2 months (interquartile range, 1.2-3.6 months) postoperatively and all occurred in the conventional repair group. Time-to-event analysis with the event of postrepair PVS showed significantly higher freedom from restenosis in the sutureless technique group (P = .0004). Adjusted hazard ratios from time-dependent Cox model described the association between postrepair PVS and pulmonary venous confluence of antler configuration (hazard ratio, 2.14; 95% confidence interval, 1.03-5.47; P = .002) and the use of sutureless technique (hazard ratio, 0.72; 95% confidence interval, 0.39-0.97; P = .003). ConclusionsSutureless technique is associated with a lower risk of postrepair PVS in patients with infracardiac total anomalous pulmonary venous connection. pulmonary venous confluence configuration of antler appearance appears to be associated with restenosis and mortality.

Computed tomography-based surgical strategy for total anomalous pulmonary venous connection.

We used computed tomographic angiography (CTA)-based surgical planning to clarify the anatomical indications of sutureless repair technique for total anomalous pulmonary venous connection. The mid-term impact of the current surgical strategies was evaluated. One hundred twelve patients underwent repair for total anomalous pulmonary venous connection. The study period was divided into era 1 (1996-2010, n = 56) and era 2 (2011-2018, n = 56). Patients with single ventricular heart (SVH) were included. In era 2, the indications for primary sutureless repair and branch pulmonary vein incision were based on CTA findings. For patients with biventricular heart, the 5-year survival was 69% and 97% in eras 1 and 2, respectively (P = 0.0024). For patients with SVH, the 5-year survival was 21% and 70% in eras 1 and 2, respectively (P = 0.0007). During the follow-up period, the evidence of post-repair pulmonary vein stenosis (PVS) was observed in 12 patients with biventricular heart [era 1, 8 patients (23%); era 2, 4 patients (13%)], and 14 patients with SVH [era 1, 6 patients (60%); era 2, 8 patients (36%)]. Using multivariable analysis, preoperative CTA was associated with improved survival in both biventricular heart and SVH and associated with post-repair PVS-free survival in SVH. Since 2011, 12 patients with post-repair PVS underwent multiple reintervention with 1 recorded death (5-year survival: 88%). CTA-based surgical strategy for total anomalous pulmonary venous connection provided significant survival benefit. Although post-repair PVS could occur in era 2, aggressive reintervention appeared to be associated with improved survival and vein patency.

Surgical Approaches to Total Anomalous Pulmonary Venous Connection.

Total anomalous pulmonary venous connection (TAPVC) constitutes a spectrum of congenital lesions whereby the pulmonary veins remain connected to systemic venous vessels or aberrantly connect to the right atrium. Definitive management requires surgical intervention and, in patients with obstruction to pulmonary venous flow, urgent operation is required. Use of temporizing catheter-based interventions allow for optimization in hemodynamically unstable neonates. Overall, survival has significantly improved over the past decades through better perioperative management and evolution of surgical approaches to minimize post-repair pulmonary vein stenosis, which persists as a major determinant of long-term outcomes.

Primary Sutureless Repair and Anterior Translocation of the Atrial Septum for Cardiac Total Anomalous Pulmonary Venous Connection

The incidence of pulmonary vein stenosis after correction of a cardiac total anomalous pulmonary venous connection in neonates may be underestimated because of the small population of patients; however, it is associated with significant mortality and morbidity. This report describes a primary sutureless repair and anterior translocation of the atrial septum to create a large left atrial cavity and avoid post-repair pulmonary vein stenosis.

Early Outcomes of Primary Sutureless Repair of the Pulmonary Veins

The "sutureless" repair technique has improved outcomes for post-repair pulmonary vein (PV) stenosis. The purpose of this study is to determine the early outcomes of primary sutureless repair of pulmonary venoocclusive disease in infants with congenital PV stenosis-hypoplasia or PVs at high risk for progressive stenosis. This is a retrospective review of infants who had primary sutureless repair of the PVs from October 2002 to April 2010. Twenty-five infants had primary sutureless repair of the PVs. Eighteen infants had total anomalous pulmonary venous return; 14 with obstruction, 10 with heterotaxy syndrome, and 9 with univentricular anatomy. Seven infants had congenital PV stenosis. There were 24 perioperative survivors (96%; 95% confidence interval [CI], 75% to 99%) and 2 late deaths from extracardiac causes. Follow-up was available on 21 out of 22 survivors at a median duration of 34 months (range, 9 to 100 months). Persistence-recurrence of PV stenosis occurred in 3 veins (3%) of 2 infants (8%). On follow-up echocardiography, right ventricular systolic pressure was normal in 13 out of 14 infants with a biventricular heart and 60% of systemic blood pressure in 1 infant. Kaplan-Meier 1-year cumulative survival was 88% (95% CI, 66% to 96%). Kaplan-Meier cumulative disease-free survival was 96% (95% CI, 75% to 99%) at 30 days and 84% (95% CI, 58% to 95%) at 1 year. By Cox proportional hazards, age, univentricular anatomy, and atrial isomerism-heterotaxy syndrome were not associated with an increased risk of death or persistence-recurrence. One-year disease-free survival was lower in infants with prematurity (p=0.0055) and low birth weight (p=0.0011). Primary sutureless repair is a feasible, safe, and relatively effective method of addressing congenital PV stenosis and (or) high-risk PVs, particularly in infants with single ventricle anatomy and (or) heterotaxy syndrome.

Primary sutureless repair for “simple” total anomalous pulmonary venous connection: Midterm results in a single institution

We have previously reported the use of an atriopericardial or "sutureless" repair for surgical management of postoperative pulmonary vein stenosis. The potential of avoiding geometric distortion of pulmonary venous suture lines and preventing post-repair pulmonary vein stenosis encouraged us to extend the use of this technique for primary "simple" total anomalous pulmonary venous connection repair. Between January 1997 and July 2009, 57 consecutive patients (median age, 15 days; median weight, 3.4 kg) underwent sutureless or conventional total anomalous pulmonary venous connection repair. Types of total anomalous pulmonary venous connection included supracardiac in 31 patients (54%), cardiac in 15 patients (26%), and infracardiac in 11 patients (19%). Median follow-up time was 2.9 years. Preoperative mean pulmonary vein score, a composite measure of stenosis in all 4 pulmonary veins, was 0.3/0-12, and vertical vein obstruction was found in 35 patients (61.4%). A primary sutureless repair was carried out in 21 patients (36.8%; supracardiac, n=12; cardiac, n=4; infracardiac, n=5). The sutureless repair group had proportionally greater high-risk infracardiac total anomalous pulmonary venous connection (24% vs 16%, P=.05). Primary outcomes of death or reoperation for pulmonary vein stenosis and postoperative pulmonary vein scores (0.2 ± 0.7 vs 0.7 ± 1.7, P=.26) were not different between the techniques. The sutureless repair group had proportionally more infracardiac total anomalous pulmonary venous connection and a higher rate of decline in postoperative right ventricular systolic pressure. Despite increased preoperative risk, no difference was observed in primary outcomes of death and reoperation in the conventional repair group.

Surgical Management of Post-repair Pulmonary Vein Stenosis

Obstructed pulmonary venous drainage - either in association with total anomalous pulmonary venous drainage, congenital stenosis, or post-repair stenosis - is associated with poor outcome. Post-repair stenosis typically involves fibrotic scar tissue extending from the site of anastomosis. "Sutureless" repair techniques avoid direct left atrial-pulmonary vein suture lines by instead reconstituting atrial tissue to posterior pericardium. Hence, the repair leaves widely decompressed pulmonary veins in the posterior mediastinum draining directly into the left atrium as a 'controlled bleed.' In our experience, late outcomes are significantly more favorable with sutureless repair techniques versus conventional pulmonary vein surgery. Therefore, after these encouraging results, we have now extended the application of this repair strategy to all scenarios of pulmonary vein surgery, including primary repair of unobstructed total anomalous pulmonary venous connection. The sutureless repair is versatile and facile. In particular, complex geometry of multiple decompressed veins can be easily accommodated by wide left atrial-pericardial suture lines. Common pitfalls can be avoided by mobilizing and protecting the phrenic pedicle and preserving the integrity of the areolar connective tissue and pleuro-parietal membrane. Overall, sutureless repair of anomalous or stenotic pulmonary veins appears safe and effective. Furthermore, in patients known to be at high risk of recurrent stenosis, sutureless techniques appear to offer improved freedom from recurrent stenosis.

Surgical Repair of Post-Repair Pulmonary Vein Stenosis Using “Sutureless” Techniques

m e n p Surgical interventions on the pulmonary veins pose technical challenges at the time of repair, primarily the cretion of an unobstructed anastomosis between the pulmoary veins and the left atrium. Among survivors, the ncidence of postoperative pulmonary vein stenosis remains a roubling clinical reality with significant potential for moridity and mortality. Potential etiologies of late pulmonary ein stenosis may include direct trauma of the pulmonary eins at the time of repair, and turbulence arising from geoetric distortion of the pulmonary vein anastomosis due to mperfections in surgical technique. For example, the diided edges of the pulmonary vein confluence at the time of epair of total anomalous pulmonary venous drainage may be ave a complex morphology and, therefore, some distortion f these edges can be created at the time of primary anasto-

Conventional and Sutureless Techniques for Management of the Pulmonary Veins: Evolution of Indications From Postrepair Pulmonary Vein Stenosis to Primary Pulmonary Vein Anomalies

Conventional and Sutureless Techniques for Management of the Pulmonary Veins: Evolution of Indications From Postrepair Pulmonary Vein Stenosis to Primary Pulmonary Vein Anomalies

Conventional and sutureless techniques for management of the pulmonary veins: Evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies

We have previously reported a limited but favorable experience with a novel sutureless technique for surgical management of postoperative pulmonary vein stenosis occurring after repair of total anomalous pulmonary venous drainage. Because this technique requires integrity of the retrocardiac space for hemostasis, extension of the technique to the primary repair of pulmonary vein anomalies requires evaluation. This analysis reviews our experience with the sutureless technique in patients with postrepair pulmonary vein stenosis, as well as our extension of the technique into primary repair of pulmonary vein anomalies. Retrospective univariable-multivariable analysis of all pulmonary vein stenosis procedures and sutureless pulmonary vein procedures over a 20-year period was performed. Cox proportional hazards modeling was used to identify variables associated with freedom from reoperation or death. Sixty patients underwent 73 procedures, with pulmonary vein stenosis present in 65 procedures. The sutureless technique was used in 40 procedures. Freedom from reoperation or death at 5 years after the initial procedure was 49%. Unadjusted freedom from reoperation or death was greater with the sutureless technique for patients with postrepair pulmonary vein stenosis ( P = .04). By using multivariable analysis, a higher pulmonary vein stenosis score was associated with greater risk of reoperation or death. After adjustment, the sutureless repair was associated with a nonsignificant trend toward greater freedom from reoperation or death ( P = .12). Despite the absence of retrocardiac adhesions, operative mortality was not increased with the sutureless technique ( P = .64). Techniques to control bleeding (intrapleural hilar reapproximation) and improve exposure (inferior vena cava division) were identified. The sutureless technique for postrepair pulmonary vein stenosis is associated with encouraging midterm results. Extension of the indications for the technique to primary repair appears safe with the development of simple intraoperative maneuvers.

Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage

Background. Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy. Methods. Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques. Results. Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes ( p < 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died ( p < 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years’ follow-up. Stenting was uniformly unsuccessful. Conclusions. In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression.