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.

[1]  K. Jenkins,et al.  Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells. , 2006, Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology.

[2]  W. Williams,et al.  Conventional and sutureless techniques for management of the pulmonary veins: Evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies. , 2005, The Journal of thoracic and cardiovascular surgery.

[3]  W. Johnson,et al.  Progressive pulmonary venous obstruction and pulmonary interstitial fibrosis associated with tetralogy of Fallot , 1994, Pediatric Cardiology.

[4]  K. Jenkins,et al.  Mechanism of pulmonary vein stenosis in infants with normally connected veins. , 2000, The American journal of cardiology.

[5]  Qingbo Xu,et al.  Mechanical stress-initiated signal transductions in vascular smooth muscle cells. , 2000, Cellular signalling.

[6]  D. Ivy,et al.  Congenital pulmonary venous stenosis presenting as persistent pulmonary hypertension of the newborn , 1999, Pediatric pulmonology.

[7]  R. Shaddy,et al.  Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. , 1999, The Annals of thoracic surgery.

[8]  W. Williams,et al.  Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage. , 1998, The Annals of thoracic surgery.

[9]  J. Smallhorn,et al.  A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. , 1998, The Journal of thoracic and cardiovascular surgery.

[10]  F. Lacour-Gayet,et al.  [Pulmonary vein stenosis. Description of a sutureless surgical procedure using the pericardium in situ]. , 1996, Archives des maladies du coeur et des vaisseaux.

[11]  T. Doyle,et al.  Pulmonary vein stenosis. , 1995, Human pathology.

[12]  L. Minich,et al.  Absorbable polydioxanone suture and results in total anomalous pulmonary venous connection. , 1995, The Annals of thoracic surgery.

[13]  S. Colan,et al.  Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection. , 1993, Journal of the American College of Cardiology.

[14]  R. Anderson,et al.  Morphologic features of stenosis of the pulmonary veins. , 1988, The American journal of cardiology.

[15]  J. Kirklin,et al.  Congenital pulmonary vein stenosis. , 1984, The American journal of cardiology.

[16]  P. Hesslein,et al.  Congenital stenosis of individual pulmonary veins: clinical spectrum and unsuccessful treatment by transvenous balloon dilation. , 1982, The American journal of cardiology.

[17]  M. Freed,et al.  Stenosis of individual pulmonary veins. Review of the literature and report of a surgical case. , 1974, The Journal of thoracic and cardiovascular surgery.