Postoperative Pulmonary Vein Stenosis Research Articles

Insight into the research history and trends of total anomalous pulmonary venous connection: a bibliometric analysis

BackgroundTotal anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease characterized by the inability of all pulmonary veins to connect to the left atrium. Our previous bibliometric article summarized the characteristics of only the 100 most cited papers in TAPVC research. The purpose of this study was to use comprehensive bibliometric analysis to examine the development history, current status, and future trends in the field of TAPVC.MethodsAll publications on TAPVC published between 2000 and 2023 were collected from the Web of Science Core Collection. The publication and citation data were quantitatively analyzed by publication year, country, institution, author, and journal. Co-authorship and co-occurrence analyses were performed using VOSviewer, and keyword and reference bursts were identified using CiteSpace. Pearson’s test was used to examine the correlations between two continuous variables.ResultsAs of July 20, 2023, we identified 368 publications with 3320 citations. These publications were published in 132 journals and authored by 1835 researchers from 457 institutions in 47 countries. For the number of publications, the top country, top institution, top author, and top journals were the United States (n = 82), Shanghai Jiao Tong University (n = 13), Huiwen Chen (n = 9), and Annals of Thoracic Surgery and Pediatric Cardiology (n = 29 each), respectively. For the number of citations, the top country, top affiliation, top author, and top journal were the United States (n = 1348), University of Toronto (n = 250), Christopher A. Caldarone (n = 315), and Annals of Thoracic Surgery (n = 746), respectively. The number of national publications significantly correlated with GDP (R = 0.887, P < 0.001), research & development (R&D) expenditure (R = 0.375, P = 0.013), population (R = 0.694, P < 0.001), and journals (R = 0.751, P < 0.001). The number of national citations significantly correlated with GDP (R = 0.881, P < 0.001), R&D expenditure (R = 0.446, P = 0.003), population (R = 0.305, P = 0.037), and journals (R = 0.917, P < 0.001). International collaboration in the field of TAPVC was not well developed. The most commonly cited publication discussed era changes in mortality and reoperation rate in TAPVC patients. The most common keywords were “total anomalous pulmonary venous connection” and “congenital heart disease”. The keyword “case report” appeared most recently, with an average occurrence year of 2021.8. The co-occurrence analysis grouped 26 keywords into six themes: surgical repair of TAPVC, postoperative pulmonary vein stenosis, surgical repair of TAPVC patients with heterotaxy, application of echocardiography in diagnosing TAPVC, application of echocardiography in the prenatal diagnosis of TAPVC, and application of the sutureless technique in the surgical repair of TAPVC patients with right atrial isomerism or a single ventricle. Citation burst detection identified 32 references with citation bursts, seven of which had ongoing citation bursts until 2023.ConclusionsThis study conducted a bibliometric analysis to provide a comprehensive overview of TAPVC research. We hope to offer new ideas for promoting development in the field of TAPVC.

Outcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) in children is a challenging condition with poor outcomes. Post-operative stenosis can occur after repair of anomalous pulmonary venous return (APVR) or stenosis within native veins. There is limited data on the outcomes of post-operative PVS. Our objective was to review our experience and assess surgical and transcatheter outcomes. Single-center retrospective study was performed including patients < 18years who developed restenosis after baseline pulmonary vein surgery that required additional intervention(s) from 1/2005 to 1/2020. Non-invasive imaging, catheterization and surgical data were reviewed.We identified 46 patients with post-operative PVS with 11 (23.9%) patient deaths. Median age at index procedure was 7.2months (range 1month-10years), and median follow-up was 10.8months (range 1day-13years). Index procedure was surgical in 36 (78.3%) and transcatheter in 10 (21.7%). Twenty-three (50%) patients developed vein atresia. Mortality was not associated with number of affected veins, vein atresia, or procedure type. Single ventricle physiology, complex congenital heart disease (CCHD), and genetic disorders were associated with mortality. Survival rate was higher in APVR patients (p = 0.03). Patients with three or more interventions had a higher survival rate compared to patients with 1-2 interventions (p = 0.02). Male gender, necrotizing enterocolitis, and diffuse hypoplasia were associated with vein atresia. In post-operative PVS, mortality is associated with CCHD, single ventricle physiology, and genetic disorders. Vein atresia is associated with male gender, necrotizing enterocolitis, and diffuse hypoplasia. Multiple repeated interventions may offer a patient survival benefit; however, larger prospective studies are necessary to elucidate this relationship further.

Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.

Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18years) who had a history of prematurity (< 35weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.

"Half-uncovered technique" to secure larger stents for postoperative pulmonary vein stenosis.

Although larger stent placement could be effective for pulmonary vein stenosis, stents extrusion tends to occur due to caliber narrowing, small landing area, and the slippery nature. We placed stents with diameter ≧8 mm for four stenotic lesions using the "half-uncovered technique". All stents were precisely placed and successfully resolved the stenosis. This technique allows us to avoid extrusion and to perform safe and effective dilatation when placing larger stents for pulmonary vein stenosis.

Total anomalous pulmonary venous drainage repair: the effect of anatomical type and pulmonary vein stenosis on outcomes

BackgroundSurgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ventricle, and heterotaxy syndromes are thought to influence short- and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting.ResultsBetween 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson’s test, Wilcoxson’s test, and generalized linear regression with Poisson distribution were used.Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein stenosis occurred in three patients, and reoperation was done in one patient. Survival free from reoperation was 98%, 98%, and 90% at 1, 24, and 60 months in the absence of pulmonary vein stenosis. However, all three patients who developed pulmonary vein stenosis died, at 0.5, 2.7, and 6.3 months of follow-up, respectively. We were unable to detect a significant effect of anatomical type, preoperative obstruction, associated single ventricle, or heterodoxy syndrome on operative mortality or long-term freedom from death or reoperation. Patients who presented with obstruction and infracardiac or supracardiac TAPVD had longer hospital stay.ConclusionTAPVD repair outcomes are excellent except for patients who develop postoperative pulmonary venous stenosis. Anatomical type, obstructed presentation, associated single ventricle, or heterotaxy are not significant predictors of survival.

Comparison of conventional and primary sutureless surgery for repairing supracardiac total anomalous pulmonary venous drainage

ObjectiveThe efficacy of using a sutureless approach in order to surgically manage postoperative pulmonary vein stenosis following total anomalous pulmonary venous drainage (TAPVD) has been reported, though outcomes of primary treatment of supracardiac TAPVD remain unclear. We retrospectively reviewed our cardiac center experience, and compared the differences in mid-term outcomes for those patients that received conventional surgery and those that underwent sutureless technique for the primary repair of supracardiac TAPVD.MethodsA total of 43 patients (median age, 199 days; range, 35 days to 1572 days) with supracardiac TAPVD underwent surgical treatment at our cardiac center from 2014 to 2018 were studied retrospectively. Primary sutureless repair was conducted in 20 cases (46.5%). The pulmonary vein scores, left ventricular ejection fraction (LVEF), baseline of the included patients, postoperative, and outcomes data were analyzed between the two groups.ResultsThe pulmonary vein scores, indicating the stenosis degree, of two groups were 0.1 ± 0.3 and 0.1 ± 0.3, left ventricular ejection fraction (LVEF) (%) were separately 66.2 ± 12.1 and 67.1 ± 13.6. The average cardiopulmonary bypass time of sutureless techniques group was much longer than conventional group (96.2 ± 32.6 min vs 75.6 ± 28.2 min, P < 0.05), but there was no difference in aortic cross-clamp time between the two groups. Followed up from 0.1 to 4 years, 3 cases died overall, with 1 (5.0%) individual dying from postoperative pulmonary venous obstruction (PVO) in sutureless group, and 2 (8.6%) dying in the conventional group respectively for postoperative infection and post-PVO. There were no differences in the length of stay in the ICU, grades of PVS after surgery, LVEF and reoperation rate between the two groups.ConclusionsThe mortality, post-PVO, follow up results of supracadiac TAPVD showed no differences between sutureless and conventional techniques. Post-PVO supposed to be the main reason for postoperative mortality.

Surgery for scimitar syndrome: the Melbourne experience.

To evaluate the outcomes associated with surgical repair of scimitar syndrome. From 1974 to 2012, 21 patients with scimitar syndrome underwent corrective surgery. Outcomes included mortality, reoperation rate, hospital length of stay and long-term functional status. The mean age of surgery was 5.4 years (2.5 months to 16.7 years). Sixteen (76.2%) patients were symptomatic at presentation. Fifteen (71.4%) patients presented with the infantile form and 6 (28.6%) with the adult form of scimitar syndrome. Operative techniques included baffle repair in 15 (71.4%) patients, reimplantation in 5 (23.8%) patients and pneumonectomy in 1 (4.8%) patient. Six (28.6%) patients were operated in infancy at a mean of 4 months (2.5-9.6 months) of age. There was 1 (4.8%) hospital death and 3 (15%) late deaths. The risk factors for mortality included preoperative pulmonary hypertension (P = 0.006) and surgery during infancy (P = 0.003). The incidence of postoperative pulmonary vein stenosis was 23.8% (n = 5). Three (14.3%) patients underwent reoperation for pulmonary vein stenosis. Neither baffle (P = 0.6) nor reimplantation (P = 0.55) surgical techniques influenced the rate of stenosis. The follow-up was complete in 15 (88%) survivors at a mean of 13.7 years (1.3-38.5 years). All surviving patients were asymptomatic and had a New York Heart Association functional status Class I. Mortality was highest in patients who had preoperative pulmonary hypertension and those who were operated on before 1 year of age. Surgical technique did not influence the occurrence of pulmonary vein stenosis. Survivors were asymptomatic at the long-term follow-up.

Is Restrictive Atrial Septal Defect a Risk in Partial Anomalous Pulmonary Venous Drainage Repair?

The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair may pose a risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and atrial rhythm disturbances. 155 children who underwent repair of right PAPVD between 1990 and 2010 were reviewed. PVS and SVCS were defined by mean gradients on echocardiography: mild=3 to 5 mm Hg; severe=6 mm Hg or higher. Postoperative cardiac rhythms were categorized as sinus, transient nonsinus, and persistent nonsinus rhythms. Outcomes were compared between patients who underwent the creation or superior enlargement of an ASD (group A) and those who did not (group B). There was no early or late death. Freedom from any PVS at 15 years after operation was lower in group A than in group B (76.1% vs 96.5%, p=0.002), and no differences were found in freedom from severe PVS (p=0.103), any SVCS (p=0.419), or severe SVCS (p=0.373). Group A patients had more PVS-related reoperations (p=0.022). Nineteen patients had nonsinus rhythm, and 4 patients experienced first-degree atrioventricular block, but no significant difference was found between the groups. Cox regression revealed the creation or superior enlargement of an ASD as a predictor for postoperative PVS (p=0.032). A case-match analysis confirmed a higher risk of PVS in patients with the creation or superior enlargement of an ASD (p=0.018). Late outcomes after repair of PAPVD are excellent. The subgroup that requires creation or superior enlargement of an ASD in repair of a right PAPVD is at a higher risk of late PVS and a subsequent increase in PVS-related reoperation. The presence of restrictive ASD did not increase SVCS, sinus node, or atrial conduction dysfunction.

Outcome predictors and implications for management of scimitar syndrome

Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

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Outcomes After Stent Implantation for the Treatment of Congenital and Postoperative Pulmonary Vein Stenosis in Children

Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid. We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases. Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

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 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-

Comparison of conventional and cutting balloon angioplasty for congenital and postoperative pulmonary vein stenosis in infants and young children

Pulmonary vein stenosis (PVS) is a rare and often lethal condition in children. The optimal treatment for congenital and postoperative PVS is unknown. We compared outcomes of conventional balloon angioplasty performed for PVS from 1999 to 2003 against cutting balloon angioplasty performed from 2004 to 2007. A total of 100 previously undilated pulmonary veins in 54 patients were studied: 48 veins dilated with conventional balloons and 52 with cutting balloons. Acute results included significantly reduced gradients and increased lumen diameters with both treatments. Acutely, cutting balloon angioplasty and conventional angioplasty yielded similar relative reduction of the PVS gradient (median 78% vs. 63%, P = 0.08) and increase in lumen diameter (median 77% vs. 59%, P = 0.07). There was one procedural death of a critically ill infant, and four cardiac arrests, but no adverse events necessitating surgical intervention. Survival free from reintervention was poor in both groups, and shorter in the cutting balloon group (73% at 1 month, 11% at 6 months, and 4% at 1 year) than in the conventional angioplasty group (77% at 1 month, 35% at 6 months, and 23% at 1 year; P = 0.01). Both conventional and cutting balloon angioplasty were effective at decreasing gradient and increasing lumen size acutely in patients with congenital and postoperative PVS, but reintervention was common with both treatments. Both methods of angioplasty provided limited benefit, and neither was curative for this complex disease.

The Effect of the Cryosurgical Cox-Maze Procedure on Pulmonary Veins Diameter and Left Atrial Size

Objective: The Cox-Maze procedure using cryoablation results in transmural lesions, which follow the lesion pattern of the cut-andsew Cox-Maze procedure. The purpose of our study was to evaluate the effect of the Cox-Maze procedure on left atrial and pulmonary vein size using computed tomography angiogram (CTA). An additional aim was to evaluate pulmonary vein anatomic variability. Methods: Six patients (four women and two men; ages 39 – 63 years, mean age 54.3) underwent chest CTA 1 day before and 38 to 104 days (mean 62.6 days) after the cryosurgical Cox-Maze procedure. Measurements of pulmonary vein ostia and left atrial cranio-caudal, left-to-right and anterior-posterior diameters were derived by consensus. The change in diameters after therapy was compared using the Wilcoxon nonparametric test for paired measurements. Four patients (1 woman and 3 men; age 57–73 years; mean age 59) were evaluated with postoperative CTA alone 296 –530 days (mean 447) after surgery, for the development of postoperative pulmonary vein stenosis. A single patient underwent preoperative CTA, but surgery was not performed. Pulmonary venous anatomy was recorded in all 11 patients. Results: Sinus rhythm was restored in all operated patients. No focal ostial stenosis of the pulmonary veins was observed. The quantitative assessment in the six patients with preoperative and postoperative studies disclosed only slight changes in pulmonary vein diameter with either reduction or dilatation of no more than 20% from baseline (P 0.05). There was a consistent trend toward decrease in left atrial dimensions, which did not reach statistical significance. Six patients (55%) had standard pulmonary venous anatomy and five patients (45%) had at least one variation in their pulmonary vein anatomy. Conclusions: In this study, we found that a very intensive cryoablation protocol around the pulmonary veins did not result in pulmonary vein stenosis. In addition, a relatively high incidence of anatomic variations of the pulmonary veins was documented.

The Effect of the Cryosurgical Cox-Maze Procedure on Pulmonary Veins Diameter and Left Atrial Size

: The Cox-Maze procedure using cryoablation results in transmural lesions, which follow the lesion pattern of the cut-and-sew Cox-Maze procedure. The purpose of our study was to evaluate the effect of the Cox-Maze procedure on left atrial and pulmonary vein size using computed tomography angiogram (CTA). An additional aim was to evaluate pulmonary vein anatomic variability. : Six patients (four women and two men; ages 39-63 years, mean age 54.3) underwent chest CTA 1 day before and 38 to 104 days (mean 62.6 days) after the cryosurgical Cox-Maze procedure. Measurements of pulmonary vein ostia and left atrial cranio-caudal, left-to-right and anterior-posterior diameters were derived by consensus. The change in diameters after therapy was compared using the Wilcoxon nonparametric test for paired measurements. Four patients (1 woman and 3 men; age 57-73 years; mean age 59) were evaluated with postoperative CTA alone 296-530 days (mean 447) after surgery, for the development of postoperative pulmonary vein stenosis. A single patient underwent preoperative CTA, but surgery was not performed. Pulmonary venous anatomy was recorded in all 11 patients. : Sinus rhythm was restored in all operated patients. No focal ostial stenosis of the pulmonary veins was observed. The quantitative assessment in the six patients with preoperative and postoperative studies disclosed only slight changes in pulmonary vein diameter with either reduction or dilatation of no more than 20% from baseline (P > 0.05). There was a consistent trend toward decrease in left atrial dimensions, which did not reach statistical significance. Six patients (55%) had standard pulmonary venous anatomy and five patients (45%) had at least one variation in their pulmonary vein anatomy. : In this study, we found that a very intensive cryoablation protocol around the pulmonary veins did not result in pulmonary vein stenosis. In addition, a relatively high incidence of anatomic variations of the pulmonary veins was documented.

Host Factor Vulnerability and Development of Progressive Intraluminal Pulmonary Vein Stenosis after Congenital Heart Surgery

Objective. The aim of this study is to explore the risk factors for progressive intraluminal pulmonary vein stenosis (PVS) in patients after surgery for congenital heart disease using a case-control design. Design. Using the Children's Hospital Cardiovascular database, all patients who developed progressive intraluminal obstruction of ≥2 pulmonary veins after cardiac surgery between 1989 and 2003 with at least 1 year of follow-up were selected, along with a random sample of control patients undergoing surgery who did not develop PVS. Because initial findings showed heterotaxy syndrome and total anomalous pulmonary venous return (TAPVR) to be strongly associated with PVS, controls were frequency-matched to cases based on these anatomic subtypes. Demographic, neonatal, anatomic, surgical, and postoperative variables were compared for cases vs. controls. Results. Controlling for heterotaxy and TAPVR, younger age at first surgery was identified as being associated with development of PVS (median age 6 days for cases vs. 38 days for controls, P= .01). In subsequent bivariate analyses, adjusting for younger age, blood type B (odds ratio [OR]= 7.6, P= .04) and cardiopulmonary bypass in the first 3 months of life (OR = 3.4, P= .04) were also associated with development of PVS. Other surgical variables indicative of a complex operative course (longer cross-clamp time and total pump time) were more frequent in controls than among cases. Conclusion. Progressive postoperative PVS is associated with anatomic defects, age, and blood type B, suggesting host factor vulnerability. Variables indicative of surgical complexity were not associated with development of progressive intraluminal PVS.

Importance of Totally Anomalous Pulmonary Venous Connection and Postoperative Pulmonary Vein Stenosis in Outcomes of Heterotaxy Syndrome

Historically, outcomes of patients with heterotaxy syndrome and congenital heart disease have been poor. Published series include patients treated over many decades or focus on specific patient/operative subgroups. This study was performed to evaluate midterm outcomes and determine anatomic risk factors for death in patients with all types of heterotaxy syndrome treated after 1985. We reviewed all infants with heterotaxy born between 1985 and 1997, presenting to one institution at <6 months age, followed for > or =5 years. Of 102 study patients (46 with asplenia phenotype, 56 with polysplenia phenotype), 48 (47%) died at a median age of 0.6 months, 12 without intervention. Survivors were followed for a median of 12.8 years (5-21.7 years). Independent predictors of mortality included obstructed totally anomalous pulmonary venous connection (TAPVC) (OR, 7.8; 95% CI, 1.9-32.9; p = 0.005), mild or greater atrioventricular (AV) valve regurgitation at presentation (OR, 3.5; 95% CI, 1.0-12.1; p = 0.03), and common AV canal (OR, 3.1; 95% CI, 1.1-8.5; p = 0.03). Sixteen patients developed pulmonary vein stenosis at a median age of 2 months, with 5 (31%) alive at follow-up. In patients with TAPVC, the mean indexed sum of pulmonary vein diameters was lower among patients who died than in survivors (42.3 +/- 8.3 vs 49.5 +/- 10.1 mm/m2; p = 0.02). Mortality remains high among patients with heterotaxy treated in the modern surgical era, particularly those with obstructed TAPVC. Pulmonary vein stenosis is common after repair of TAPVC in patients with heterotaxy, may be diagnosed beyond infancy, and is associated with poor outcomes.

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.