Total anomalous pulmonary venous connection refers to a spectrum of cardiac anomalies where the pulmonary veins fail to return to the left atrium and the pulmonary venous blood returns through a systemic vein or directly to the right atrium. There is a wide anatomical variety of venous connections and degrees of pulmonary venous obstruction that affect the presentation, surgical repair, and outcomes. In this review, we explore the preoperative physiology, echocardiographic diagnosis, and approach to postoperative complications.
SealeANUemuraHWebberSA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010;122:2718-2726.
2.
Correa-VillasenorAFerenczCBoughmanJANeillCA.Total anomalous pulmonary venous return: familial and environmental factors. The Baltimore-Washington Infant Study Group. Teratology. 1991;44:415-428.
3.
KimHSJeongKChoHJ, et al. Total anomalous pulmonary venous return in siblings. J Cardiovasc Ultrasound. 2014;22:213-219.
4.
BleylSNelsonLOdelbergSJ, et al. A gene for familial total anomalous pulmonary venous return maps to chromosome 4p13-q12. Am J Hum Genet. 1995;56:408-415.
5.
RaisherBDDowtonSBGrantJW.Father and two children with total anomalous pulmonary venous connection. Am J Med Genet. 1991;40:105-106.
6.
LucasRVJrAdamsPJrAndersonRCVarcoRLEdwardsJELesterRG.Total anomalous pulmonary venous connection to the portal venous system: a cause of pulmonary venous obstruction. Am J Roentgenol Radium Ther Nucl Med. 1961;86:561-575.
7.
WebbSKananiMAndersonRHRichardsonMKBrownNA.Development of the human pulmonary vein and its incorporation in the morphologically left atrium. Cardiol Young. 2001;11:632-642.
8.
GevaTVan PraaghS. Anomalies of the pulmonary veins. In: AllenHDriscollMShaddyRFeltesT, eds. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult. 7th ed.Philadelphia, PA: Lippincott Williams & Wilkins; 2008:761-791.
9.
DouglasYLJongbloedMRDeruiterMCGittenberger-de GrootAC.Normal and abnormal development of pulmonary veins: state of the art and correlation with clinical entities. Int J Cardiol. 2011;147:13-24.
10.
RudolphA. Total anomalous pulmonary venous connection. In: Congenital Diseases of the Heart: Clinical-Physiological Considerations. 3rd ed.Chichester, UK: Wiley-Blackwell; 2009:320-344.
11.
SealeANCarvalhoJSGardinerHM, et al. Total anomalous pulmonary venous connection: impact of prenatal diagnosis. Ultrasound Obstet Gynecol. 2012;40:310-318.
12.
BurroughsJTEdwardsJE.Total anomalous pulmonary venous connection. Am Heart J. 1960;59:913-931.
13.
HusainSAMaldonadoERaschD, et al. Total anomalous pulmonary venous connection: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg. 2012;94:825-831; discussion 831-832.
14.
KaramlouTGurofskyRAl SukhniE, et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation. 2007;115:1591-1598.
15.
HorerJNeurayCVogtM, et al. What to expect after repair of total anomalous pulmonary venous connection: data from 193 patients and 2902 patient years. Eur J Cardiothorac Surg. 2013;44:800-807.
16.
BrownVEDe LangeMDyarDAImpastatoLWShiraliGS.Echocardiographic spectrum of supracardiac total anomalous pulmonary venous connection. J Am Soc Echocardiogr. 1998;11:289-293.
17.
TabataTThomasJDKleinAL.Pulmonary venous flow by Doppler echocardiography: revisited 12 years later. J Am Coll Cardiol. 2003;41:1243-1250.
18.
DillmanJRYarramSGHernandezRJ.Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. 2009;192:1272-1285.
19.
ThackerPGSchoolerGRCaplanMJLeeEY.Developmental lung malformations in children: recent advances in imaging techniques, classification system, and imaging findings. J Thorac Imaging. 2015;30:29-43; quiz 44-45.
20.
BurkhardtBEStillerBGrohmannJ.Stenting of the obstructed ductus venosus as emergency and bridging strategy in a very low birth weight infant with infradiaphragmatic total anomalous pulmonary venous connection. Catheter Cardiovasc Interv. 2014;84:820-823.
21.
Michel-BehnkeILuedemannMHagelKJSchranzD.Serial stent implantation to relieve in-stent stenosis in obstructed total anomalous pulmonary venous return. Pediatr Cardiol. 2002;23:221-223.
22.
Lo-A-NjoeSMBlomNABokenkampROttenkampJ.Stenting of the vertical vein in obstructed total anomalous pulmonary venous return as rescue procedure in a neonate. Catheter Cardiovasc Interv. 2006;67:668-670.
23.
KyserJPBengurARSiwikES.Preoperative palliation of newborn obstructed total anomalous pulmonary venous connection by endovascular stent placement. Catheter Cardiovasc Interv. 2006;67:473-476.
24.
RothmanAGalindoAEvansWN.Temporary transumbilical stenting of the ductus venosus in neonates with obstructed infradiaphragmatic total anomalous pulmonary venous return. Pediatr Cardiol. 2011;32:87-90.
25.
HydeJAStumperOBarthMJ, et al. Total anomalous pulmonary venous connection: outcome of surgical correction and management of recurrent venous obstruction. Eur J Cardiothorac Surg. 1999;15:735-740; discussion 740-741.
26.
MoralesDLBraudBEBoothJH, et al. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results. Ann Thorac Surg. 2006;82:1621-1627; discussion 1627-1628.
27.
SadrIMTanPEKieranMWJenkinsKJ.Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol. 2000;86:577-579, A10.
28.
RiedlingerWFJuraszekALJenkinsKJ, et al. Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells. Cardiovasc Pathol. 2006;15:91-99.
29.
SealeANUemuraHWebberSA, et al. Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg. 2013;145:1255-1262.
PengLFLockJENugentAWJenkinsKJMcElhinneyDB.Comparison of conventional and cutting balloon angioplasty for congenital and postoperative pulmonary vein stenosis in infants and young children. Catheter Cardiovasc Interv. 2010;75:1084-1090.
32.
BalasubramanianSMarshallACGauvreauK, et al. Outcomes after stent implantation for the treatment of congenital and postoperative pulmonary vein stenosis in children. Circ Cardiovasc Interv. 2012;5:109-117.
33.
EschJJPorrasDBergersenLJenkinsKJMarshallAC.Systemic embolic complications of pulmonary vein angioplasty in children. Pediatr Cardiol. 2015;36:1357-1362.