Abstract
An eight-month-old boy with findings of persistent left pulmonary basal infiltrate was diagnosed with congenital unilateral pulmonary vein atresia by bronchoscopy. Cardiac catheterization documented slow left pulmonary venous return to atretic pulmonary veins. Conservative treatment was chosen because the child was asymptomatic and corrective surgery or percutaneous intervention was not technically possible. After a 3-year follow-up, the child still has no documented pulmonary hypertension. Early diagnosis of unilateral pulmonary vein atresia is important to anticipate potential threatening complications like pulmonary hypertension and hemoptysis. Surgical treatment of this entity might be drastic and complex and should be weighed against a conservative alternative and careful follow-up.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Beerman LB, Oh KS, Park SC, et al. (1983) Unilateral pulmonary vein atresia: clinical and radiographic spectrum. Pediatr Cardiol 4:105–112
Cullen S, Deasy PF, Tempany E, Duff DF (1990) Isolated pulmonary vein atresia. Br Heart J 63:350–354
Edwards JE (1960) Congenital stenosis of pulmonary veins. Pathologic and developmental considerations. Lab Invest 9:46–66
vHeyneman LE, Nolan RL, Harrison JK, McAdams HP (2001) Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients. Am J Roentgenol 177:681–685
Kingston HM, Patel RG, Watson GH (1983) Unilateral absence or extreme hypoplasia of pulmonary veins. Br Heart J 49:148–153
Lacour-Gayet F (2006) Surgery for pulmonary venous obstruction after repair of total anomalous pulmonary venous return. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 45–50. Review
Nasrallah AT, Mullins CE, Singer D, Harrison G, McNamara DG (1975) Unilateral pulmonary vein atresia: diagnosis and treatment. Am J Cardiol 36:969–973
Pourmoghadam KK, Moore JW, Khan M, et al. (2003) Congenital unilateral pulmonary venous atresia: definitive diagnosis and treatment. Pediatr Cardiol 24:73–79
Sacco O, Fregonese B, Fregonese L, Gambini C, Pongiglione G, Rossi GA (2002) Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: successful treatment with endovascular stent implantation. Pediatr Pulmonol 34:324–328
Ussia GP, Marasini M, Rimini A, Pongiglione G (2004) Atresia of right pulmonary veins with intact atrial septum and major aorto-pulmonary collateral treated with percutaneous stent implantation and embolization. J Intervent Cardiol 17:183–187
van Son JA, Danielson GK, Puga FJ, Edwards WD, Driscoll DJ (1995) Repair of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg 60:144–150
Wiebe S, Maclusky I, Manson D, Holowka S, Yoo SJ. (2003) Hemoptysis: a rare cause can be related to a bronchial varix due to pulmonary venous obstruction. Pediatr Radiol 33:884–886
Acknowledgment
The authors would like to thank Constance Barazzone, MD, for her support in the follow-up of this patient.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Tissot, C., Corbelli, R., Aggoun, Y. et al. Bronchoscopic Diagnosis of Asymptomatic Unilateral Pulmonary Vein Atresia in an Infant. Pediatr Cardiol 29, 976–979 (2008). https://doi.org/10.1007/s00246-007-9143-6
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-007-9143-6