Abstract
Total anomalous pulmonary venous connection (TAPVC) may occur as an isolated lesion or in association with complex congenital heart disease. 2D echocardiography in patients with isolated TAPVC reveals significant enlargement of the right atrium, right ventricle and pulmonary arteries. Colour Doppler is a valuable tool to identify the pulmonary venous confluence and its connection to the right atrium or systemic veins. Possible sites of anomalous pulmonary venous connection, which should be checked by colour Doppler interrogation, include the right atrium, the coronary sinus, the right and left superior vena cava, the azygos vein, the innominate vein, the inferior vena cava, the portal vein system and the hepatic veins. Echocardiographic detection of TAPVC in the context of complex congenital heart disease requires a systematic sequential approach that starts with clarification of venous connections. Frequently these patients also have anomalies of visceral and atrial situs summarized as heterotaxy syndromes. Partial anomalous pulmonary venous connection (PAPVC) can be associated with various cardiovascular malformations or it may occur as an isolated lesion. Anomalous drainage of the right upper pulmonary vein is frequently encountered in patients with sinus venosus ASD, while PAPVC of the right lower pulmonary vein is an integral part of the Scimitar syndrome.
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References
Alpert JS et al (1977) Anomalous pulmonary venous return with intact atrial septum: diagnosis and pathophysiology. Circulation 56(5):870–875
Amoretti F et al (2005) The levoatriocardinal vein. Pediatr Cardiol 26(4):494–495
Apitz C et al (2008) Systematic evaluation of the proximal cardiac veins for echocardiographic diagnosis of total anomalous pulmonary venous connection. Ultraschall Med 29(5):525–530
Bechtold SM et al (2004) Partial anomalous pulmonary vein connection: an underestimated cardiovascular defect in Ullrich-Turner syndrome. Eur J Pediatr 163(3):158–162
Bernstein HS et al (1995) The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection. J Am Coll Cardiol 26(4):995–1001
Brown DW (2009) Pulmonary venous anomalies. In: Lai WW, Mertens LL, Cohen MS, Geva T (eds) Echocardiography in pediatric and congenital heart disease. Blackwell Publishing Ltd, Chichester
Brown VE et al (1998) Echocardiographic spectrum of supracardiac total anomalous pulmonary venous connection. J Am Soc Echocardiogr 11(3):289–293
Caldarone CA et al (1998) Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg 66(5):1521–1526
Caspi J et al (2001) The beneficial hemodynamic effects of selective patent vertical vein following repair of obstructed total anomalous pulmonary venous drainage in infants. Eur J Cardiothorac Surg 20(4):830–834
Cooper MJ et al (1984) Study of the infradiaphragmatic total anomalous pulmonary venous connection with cross-sectional and pulsed Doppler echocardiography. Circulation 70(3):412–416
Frye RL et al (1968) Partial anomalous pulmonary venous connection without atrial septal defect. Am J Cardiol 22(2):242–250
Gao YA et al (1993) Scimitar syndrome in infancy. J Am Coll Cardiol 22(3):873–882
Gilljam T et al (2000) Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 36(3):908–916
Hancock K et al (2005) First clinical experience with a new non-indwelling voice prosthesis (Provox NID) for voice rehabilitation after total laryngectomy. Acta Otolaryngol 125(9):981–990
Hashmi A et al (1998) Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 31(5):1120–1126
Hawker RE et al (1972) Common pulmonary vein atresia. Premortem diagnosis in two infants. Circulation 46(2):368–374
Haworth SG (1982) Total anomalous pulmonary venous return. Prenatal damage to pulmonary vascular bed and extrapulmonary veins. Br Heart J 48(6):513–524
Haworth SG et al (1983) Pulmonary hypertension in scimitar syndrome in infancy. Br Heart J 50(2):182–189
Ho VB et al (2004) Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. Circulation 110(12):1694–1700
Huhta JC et al (1982) Two dimensional echocardiographic diagnosis of situs. Br Heart J 48(2):97–108
Jenkins KJ et al (1993) Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection. J Am Coll Cardiol 22(1):201–206
Kawashima Y et al (1977) Tree-shaped pulmonary veins in infracardiac total anomalous pulmonary venous drainage. Ann Thorac Surg 23(5):436–441
Lam J et al (1984) 2D echocardiographic diagnosis of total anomalous pulmonary venous connection of the infradiaphragmatic type. Eur Heart J 5(10):842–845
Lindinger A et al (2010) Prevalence of congenital heart defects in newborns in Germany: results of the first registration year of the PAN Study (July 2006 to June 2007). Klin Padiatr 222(5):321–326
Musewe NN et al (1992) Anomalies of pulmonary venous connections including cor triatriatum and stenosis of individual pulmonary veins. In: Freedom RM, Benson LN, Smallhorn JF (eds) Neonatal heart disease. Springer, London/Berlin/Heidelberg
Najm HK et al (1996) Scimitar syndrome: twenty years’ experience and results of repair. J Thorac Cardiovasc Surg 112(5):1161–1168; discussion 1168–1169
Palmisano JM et al (1992) Unsuspected congenital heart disease in neonates receiving extracorporeal life support: a review of ninety-five cases from the Extracorporeal Life Support Organization Registry. J Pediatr 121(1):115–117
Rubino M et al (1995) Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg 110(3):641–650
Sahn DJ et al (1979) Cross-sectional echocardiographic diagnosis of the sites of total anomalous pulmonary venous drainage. Circulation 60(6):1317–1325
Schuhmacher G et al (2008) Klinische Kinderkardiologie. Springer Medizin Verlag, Berlin/Heidelberg
Schwedler G et al (2011) Frequency and spectrum of congenital heart defects among live births in Germany : a study of the Competence Network for Congenital Heart Defects. Clin Res Cardiol Off J Ger Card Soc 100(12):1111–1117
Sinzobahamvya N et al (1996) Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants. Eur J Cardiothorac Surg 10(6):433–438
Smallhorn JF, Freedom RM (1986) Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection. J Am Coll Cardiol 8(6):1413–1420
Smallhorn JF et al (1981) Assessment of total anomalous pulmonary venous connection by two-dimensional echocardiography. Br Heart J 46(6):613–623
Snider AR et al (1982) Evaluation of infradiaphragmatic total anomalous pulmonary venous connection with two-dimensional echocardiography. Circulation 66(5):1129–1132
Sreeram N, Walsh K (1992) Diagnosis of total anomalous pulmonary venous drainage by Doppler color flow imaging. J Am Coll Cardiol 19(7):1577–1582
Ucar T et al (2008) Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections. Int J Cardiovasc Imaging 24(2):229–235
Volkl TM et al (2005) Cardiovascular anomalies in children and young adults with Ullrich-Turner syndrome the Erlangen experience. Clin Cardiol 28(2):88–92
Webb S et al (2001) Development of the human pulmonary vein and its incorporation in the morphologically left atrium. Cardiol Young 11(6):632–642
Webber SA et al (1992) Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis. Pediatr Cardiol 13(2):112–115
Wong ML et al (1995) Echocardiographic evaluation of partial anomalous pulmonary venous drainage. J Am Coll Cardiol 26(2):503–507
Ziemer G, Haverich A (2010) Herzchirurgie. 3. Auflage. Springer Verlag, Heidelberg
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17.1 Electronic Supplementary Material
Video 17.1
The apical four-chamber view in this newborn with TAPVC shows significant enlargement of the right ventricle and squeezing of the left ventricle. Note significant bulging of the atrial septum from right to left (AVI 7095 kb)
Video 17.2
Colour Doppler in the apical four-chamber view (same patient as in Video 17.1) shows moderate tricuspid regurgitation (AVI 1396 kb)
Video 17.3
Enlargement of the right ventricle is also apparent in the parasternal long-axis view of another newborn with TAPVC. Aortic valve and mitral valve are morphologically normal (AVI 19379 kb)
Video 17.4
Severe enlargement of the right ventricle is evident in the parasternal short-axis view of this newborn with TAPVC (AVI 6707 kb)
Video 17.6
Colour Doppler in the posterior plane of the apical four-chamber view (same patient as in Video 17.5) shows the enlarged coronary sinus with significantly increased flow from the pulmonary veins draining to the right atrium (AVI 1329 kb)
Video 17.5
Colour Doppler in the subcostal coronal view in this newborn shows drainage of the pulmonary veins to the coronary sinus resulting in significant increase in flow directed towards the transducer in this structure. Right to left shunting from the right to left atrium is visualized cranial to the coronary sinus (AVI 8677 kb)
Video 17.7
This clip shows a sweep from the parasternal long-axis view of the left ventricular outflow to the right ventricular inflow in a newborn with TAPVC to the coronary sinus. In the long-axis view of the left ventricular outflow, the pulmonary venous confluence is displayed posterior to the left atrium. Tilting of the transducer towards the right hip displays the right ventricular inflow showing the connection of the venous confluence to the enlarged coronary sinus and its drainage into the right atrium (AVI 32488 kb)
Video 17.8
Colour Doppler in the suprasternal coronal view of a newborn with supracardiac type of TAPVC shows the left-sided ascending vein draining the pulmonary veins to the significantly dilated innominate vein and superior vena cava (AVI 24392 kb)
Video 17.9
The slightly more posterior plane (same patient as in Video 17.8) displays cephalad flow in the left-sided ascending vein (AVI 10794 kb)
Video 17.10
The sweep in a high left parasternal short-axis view (same patient as in Videos 17.8 and 17.9) starts with the confluence of the pulmonary veins behind the pulmonary artery confluence. Cranial tilt of the transducer displays the left-sided ascending vein with cranial flow directed towards the innominate vein (AVI 36236 kb)
Video 17.11
Colour Doppler in the posterior plane of the subcostal coronal view in a neonate with infracardiac type TAPVC shows the pulmonary venous confluence behind the left atrium with flow directed towards the transducer (AVI 26699 kb)
Video 17.12
Colour Doppler in the subcostal short-axis view (same patient as in Video 17.11) displays the descending vein anterior to the aorta. Flow is directed towards the transducer both in the descending vein and in the descending aorta (AVI 1667 kb)
Video 17.13
The colour Doppler sweep in another newborn with infracardiac type TAPVC starts with the subcostal coronal view of the atria. The pulmonary veins come together behind the left atrium. Posterior and inferior tilt of the transducer reveals the left-sided descending vein draining below the diaphragm to reach the liver (AVI 27301 kb)
Video 17.14
The sweep in the longitudinal view of the upper abdomen in this newborn with infracardiac type TAPVC starts on the right displaying drainage of the inferior vena cava and the hepatic veins to the right atrium. Leftward tilt of the transducer displays a large vessel entering the liver, which represents the descending vein (AVI 79432 kb)
Video 17.15
Colour Doppler in the longitudinal view of the upper abdomen (same patient as in Video 17.14) starts on the left displaying the large descending vein with venous flow directed away from the heart. Rightward tilt of the transducer shows the intrahepatic connection of the descending vein to the portal vein and finally its drainage via the ductus venosus to the inferior vena cava (AVI 38545 kb)
Video 17.16
The colour Doppler sweep in the suprasternal coronal view of an infant with PAPVC at the beginning displays the innominate vein. Leftward angulation and some posterior tilt of the transducer reveal an ascending vein draining the left upper pulmonary vein with cranially directed flow to the innominate vein (AVI 49741 kb)
Video 17.17
The high right parasternal long-axis view in an infant with PAPVC of the right upper pulmonary vein to the superior vena cava shows a large vessel connecting to the posterior wall of the SVC cranial to the right pulmonary artery which is displayed in cross section (AVI 8302 kb)
Video 17.18
Colour Doppler in the high right parasternal long-axis view (same patient as in Video 17.17) confirms venous inflow into the superior vena cava (AVI 1610 kb)
Video 17.19
The posteriorly orientated suprasternal coronal view (same patient as in Videos 17.17 and 17.18) displays the innominate vein, the superior vena cava and anomalous connection of the right upper pulmonary vein to its posterior wall (AVI 9325 kb)
Video 17.20
Inflow from the right upper pulmonary vein to the superior vena cava is well displayed by colour Doppler (same patient as in Videos 17.17, 17.18 and 17.19) (AVI 1072 kb)
Video 17.21
Colour Doppler in the subcostal coronal view of a newborn with left isomerism shows drainage of the right pulmonary veins (RPV) into the right-sided atrium, while the left pulmonary veins (LPV) drain into the left-sided atrium (AVI 4642 kb)
Video 17.22
Due to hypoplasia of the right lung, the high parasternal short-axis view of a neonate with scimitar syndrome shows discrepancy in size of right and left pulmonary artery (AVI 3856 kb)
Video 17.23
Colour Doppler shows laminar flow in both pulmonary arteries (same patient as in Video 17.22) (AVI 4153 kb)
Video 17.24
Dextrocardia due to hypoplasia of the right lung is evident in the subcostal coronal view (same patient as in Videos 17.22 and 17.23) (AVI 3708 kb)
Video 17.25
A posterior subcostal coronal view of the right atrium (same patient as in Videos 17.22, 17.23 and 17.24) shows hepatic veins draining inferiorly as well as a left lower pulmonary vein draining into the lower right atrium (AVI 4266 kb)
Video 17.26
Colour Doppler confirms venous inflow from the right lower pulmonary vein which is directed towards the transducer (same patient as in Videos 17.22, 17.23, 17.24 and 17.25) (AVI 872 kb)
Video 17.27
Colour Doppler in the longitudinal view of the upper abdomen reveals anomalous drainage of a small right lower pulmonary vein into the inferior vena cava (AVI 2425 kb)
Video 17.28
This sweep in the longitudinal view of the upper abdomen in an infant with scimitar syndrome starts with the longitudinal section of the abdominal aorta. Close to the celiac artery originates an arterial vessel that is followed by lateral tilt of the transducer to the right. This abnormal systemic passes the inferior vena cava and takes a cranial course to the right lung (see also Fig. 17.12d) (AVI 13625 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Anomalous Pulmonary Venous Connections. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_17
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