Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions.
SmallhornJFFreedomRM.Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection. J Am Coll Cardiol. 1986;8:1413-1420.
2.
MichielonGDi DonatoRMPasquiniL, et al. Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions. Eur J Cardiothorac Surg. 2002;22:184-191.
3.
GoswamiKCShrivastavaSSaxenaADevV.Echocardiographic diagnosis of total anomalous pulmonary venous connection. Am Heart J. 1993;126:433-440.
4.
PadalinoMACavalliGDe FranceschiM, et al. Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience. J Card Surg. 2014;29:678-685.
5.
Hancock FriesenCLZurakowskiDThiagarajanRR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg. 2005;79:596-606.
6.
HinesMHHammonJW.Anatomy of total anomalous pulmonary venous connection. Oper Tech Thorac Cardiovasc Surg. 2001;6:2-7.
7.
DelisleGAndoMCalderAL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J. 1976;91:99-122.
8.
LucasRVJrWoolfreyBFAndersonrc LesterRGEdwardsJE.Atresia of the common pulmonary vein. Pediatrics. 1962;29:729-739.
9.
NeillCA.Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics. 1956;18:880-887.
10.
CraigJMDarlingRCRothneyWB.Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest. 1957;6:44-64.
11.
SinzobahamvyaNArenzCBrecherAMBlaschczokHCUrbanAE.Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants. Eur J Cardiothorac Surg. 1996;10:433-438.
12.
SealeANUemuraHWebberSA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010;122:2718-2726.
13.
HaworthSGReidL.Structural study of pulmonary circulation and of heart in total anomalous pulmonary venous return in early infancy. Br Heart J. 1977;39:80-92.
14.
BharatiSLevM.Congenital anomalies of the pulmonary veins. Cardiovasc Clin. 1973;5:23-41.
15.
ChowdhuryUKAiranBMalhotraA, et al. Mixed total anomalous pulmonary venous connection: anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg. 2008;135:106-116.
16.
SealeANCarvalhoJSGardinerHM, et al. Total anomalous pulmonary venous connection: impact of prenatal diagnosis. Ultrasound Obstet Gynecol. 2012;40:310-318.
17.
AllanLDSharlandGK.The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus. Heart. 2001;85:433-437.
18.
BoveKEGeiserEAMeyerRA.The left ventricle in anomalous pulmonary venous return. Morphometric analysis of 36 fatal cases in infancy. Arch Pathol. 1975;99:522-528.
19.
KongDPanCDongLJinHShuX.Rare variant of mixed total anomalous pulmonary venous drainage (TAPVD) in a 55-year-old patient. Eur Heart J Cardiovasc Imaging. 2015;16:227.
20.
Romero-CardenasAVargas-BarronJRylaarsdamM, et al. Total anomalous pulmonary venous return: diagnosis by transesophageal echocardiography. Am Heart J. 1991;121(6 pt 1):1831-1834.
21.
PaquetMGutgesellH.Echocardiographic features of total anomalous pulmonary venous connection. Circulation. 1975;51:599-605.
22.
GrahamTPJarmakaniJMCanentRV.Left heart volume characteristics with a right ventricular volume overload. Total anomalous pulmonary venous connection and large atrial septal defect. Circulation. 1972;45:389-396.
23.
WukaschDCDeutschMReulGJHallmanGLCooleyDA.Total anomalous pulmonary venous return. Review of 125 patients treated surgically. Ann Thorac Surg. 1975;19:622-633.
24.
FreedomRMOlleyPMCoceaniFRoweRD.The prostaglandin challenge. Test to unmask obstructed total anomalous pulmonary venous connections in asplenia syndrome. Br Heart J. 1978;40:91-94.
25.
YapSHAnaniaNAlbolirasETLilienLD.Reversed differential cyanosis in the newborn: a clinical finding in the supracardiac total anomalous pulmonary venous connection. Pediatr Cardiol. 2009;30:359-362.
26.
HaworthSG.Total anomalous pulmonary venous return. Prenatal damage to pulmonary vascular bed and extrapulmonary veins. Br Heart J. 1982;48:513-524.
27.
ShenQPaMHuXWangJ.Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection. Pediatr Radiol. 2013;43:827-835.
28.
KimTHKimYMSuhCH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol. 2000;175:1381-1386.
29.
LincolnCRRigbyMLMercantiC, et al. Surgical risk factors in total anomalous pulmonary venous connection. Am J Cardiol. 1988;61:608-611.
30.
FrommeltPCSheridanDCDeatsmanS, et al. Unobstructive total anomalous pulmonary venous return: impact of early elective repair on the need for prolonged mechanical ventilatory support. Pediatr Cardiol. 2010;31:1191-1197.
31.
YanagawaBAlghamdiAADragulescuA, et al. Primary sutureless repair for “simple” total anomalous pulmonary venous connection: midterm results in a single institution. J Thorac Cardiovasc Surg. 2011;141:1346-1354.
32.
CopeJTBanksDMcDanielNLShockeyKSNolanSPKronIL.Is vertical vein ligation necessary in repair of total anomalous pulmonary venous connection?Ann Thorac Surg. 1997;64:23-29.
33.
ShahMJShahSShankargowdaSKrishnanUCherianKM.L→R shunt: a serious consequence of TAPVC repair without ligation of vertical vein. Ann Thorac Surg. 2000;70:971-973.
34.
KumarRNDharmapuramAKRaoIM, et al. The fate of the unligated vertical vein after surgical correction of total anomalous pulmonary venous connection in early infancy. J Thorac Cardiovasc Surg. 2001;122:615-617.
35.
AtzAMWesselDL.Inhaled nitric oxide in the neonate with cardiac disease. Semin Perinatol. 1997;21:441-455.
36.
BandoKVijayPTurrentineMW, et al. Dilutional and modified ultrafiltration reduces pulmonary hypertension after operations for congenital heart disease: a prospective randomized study. J Thorac Cardiovasc Surg. 1998;115:517-525.
37.
BerdatPAEichenbergerEEbellJ, et al. Elimination of proinflammatory cytokines in pediatric cardiac surgery: analysis of ultrafiltration method and filter type. J Thorac Cardiovasc Surg. 2004;127:1688-1696.
38.
PapadopoulosNBakhtiaryFGrunVWeberCFStrasserCMoritzA.The effect of normovolemic modified ultrafiltration on inflammatory mediators, endotoxins, terminal complement complexes and clinical outcome in high-risk cardiac surgery patients. Perfusion. 2013;28:306-314.
39.
RussellIAZwassMSFinemanJR, et al. The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease. Anesth Analg. 1998;87:46-51.
40.
LindbergLOlssonAKJögiPJonmarkerC.How common is severe pulmonary hypertension after pediatric cardiac surgery?J Thorac Cardiovasc Surg. 2002;123:1155-1163.
41.
KleinMDShaheenKWWhittleseyGCPinskyWWArciniegasE.Extracorporeal membrane oxygenation for the circulatory support of children after repair of congenital heart disease. J Thorac Cardiovasc Surg. 1990;100:498-505.
42.
ZhaoKWangHWangZ, et al. Early- and intermediate-term results of surgical correction in 122 patients with total anomalous pulmonary venous connection and biventricular physiology. J Cardiothorac Surg. 2015;10:172.
43.
DuffJPJoffeARVatanpourS, et al. Neurocognitive outcomes at kindergarten entry after surgical repair of total anomalous pulmonary venous connection in early infancy. Pediatr Cardiol. 2015;36:350-357.
44.
St LouisJDHarveyBAMenkJS, et al. Repair of “simple” total anomalous pulmonary venous connection: a review from the Pediatric Cardiac Care Consortium. Ann Thorac Surg. 2012;94:133-137.
45.
KhanMSBryantR3rdKimSH, et al. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. 2015;99:2134-2139.
46.
MarcondesLDFGalatiJCJonesBO, et al. Abnormal left ventricular diastolic function at late follow-up after repair of total anomalous pulmonary venous drainage: the impact of altered ventricular loading in utero. J Thorac Cardiovasc Surg. 2014;148:238-244.
47.
SealeANUemuraHWebberSA, et al. Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg. 2013;145:1255-1262.
