Abstract
In the majority of cases, pulmonary atresia with intact ventricular septum (PAiVS) is not restricted on atresia of the pulmonary valve but also affects all structures of the right ventricle. Morphology and size of the right ventricle and anatomy of the right ventricular outflow tract and pulmonary artery can be well displayed by 2D echocardiography. Colour Doppler allows confirmation of patency of the tricuspid valve and absence of antegrade flow in the pulmonary artery, quantification of tricuspid regurgitation and demonstration of collateral lung perfusion via patent ductus arteriosus. In addition colour Doppler is important in the search for ventricular coronary connections. PW Doppler and CW Doppler are employed for estimation of right ventricular pressure by interrogation of tricuspid regurgitation.
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Altun G, Babaoglu K et al (2013) Functional pulmonary atresia in newborn with normal intracardiac anatomy: successful treatment with inhaled nitric oxide and pulmonary vasodilators. Ann Pediatr Cardiol 6(1):83–86
Ashburn DA, Blackstone EH et al (2004) Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 127(4):1000–1008
Bull C, de Leval MR et al (1982) Pulmonary atresia and intact ventricular septum: a revised classification. Circulation 66(2):266–272
Burrows PE, Freedom RM et al (1990) Coronary angiography of pulmonary atresia, hypoplastic right ventricle, and ventriculocoronary communications. AJR Am J Roentgenol 154(4):789–795
Daubeney PE, Blackstone EH et al (1999) Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children. Cardiol Young 9(4):402–410
Daubeney PE, Delany DJ et al (2002) Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol 39(10):1670–1679
Daubeney PE, Wang D et al (2005) Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg 130(4):1071
Ekman Joelsson BM, Sunnegardh J et al (2001) The outcome of children born with pulmonary atresia and intact ventricular septum in Sweden from 1980 to 1999. Scand Cardiovasc J 35(3):192–198
Freedom RM (1989) Pulmonary atresia with intact ventricular septum. Futura Publishing Company, Inc., Mount Kisco, New York
Freedom RM, Anderson RH et al (2005) The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum. Cardiol Young 15(5):447–468
Freedom RM, Jaeggi E et al (2006) The “wall-to-wall” heart in the patient with pulmonary atresia and intact ventricular septum. Cardiol Young 16(1):18–29
Garcia JA, Zellers TM et al (1998) Usefulness of Doppler echocardiography in diagnosing right ventricular coronary arterial communications in patients with pulmonary atresia and intact ventricular septum and comparison with angiography. Am J Cardiol 81(1):103–104
Gardiner HM, Belmar C et al (2008) Morphologic and functional predictors of eventual circulation in the fetus with pulmonary atresia or critical pulmonary stenosis with intact septum. J Am Coll Cardiol 51(13):1299–1308
Hanley FL, Sade RM et al (1993) Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 105(3):406–423, 424–7; discussion 423–4
Kutsche LM, Van Mierop LH (1983) Pulmonary atresia with and without ventricular septal defect: a different etiology and pathogenesis for the atresia in the 2 types? Am J Cardiol 51(6):932–935
Lin MC, Chen CH et al (2010) Functional pulmonary atresia: in a critically ill premature infant. Tex Heart Inst J 37(4):494–495
Lindinger A, Schwedler G 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
Michel-Behnke I, Akintuerk H et al (2004) Stent implantation in the ductus arteriosus for pulmonary blood supply in congenital heart disease. Catheter Cardiovasc Interv 61(2):242–252
Qureshi SA (2006) Catheterization in neonates with pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv 67(6):924–931
Salvin JW, McElhinney DB et al (2006) Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum. Pediatrics 118(2):e415–e420
Sandor GG, Cook AC et al (2002) Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life. Cardiol Young 12(5):436–444
Santos MA, Moll JN et al (1980) Development of the ductus arteriosus in right ventricular outflow tract obstruction. Circulation 62(4):818–822
Satou GM, Perry SB et al (2000) Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum. Am J Cardiol 85(11):1319–1324
Smallhorn JF, Izukawa T et al (1984) Noninvasive recognition of functional pulmonary atresia by echocardiography. Am J Cardiol 54(7):925–926
Trowitzsch E, Colan SD et al (1985) Two-dimensional echocardiographic evaluation of right ventricular size and function in newborns with severe right ventricular outflow tract obstruction. J Am Coll Cardiol 6(2):388–393
Velvis H, Schmidt KG et al (1989) Diagnosis of coronary artery fistula by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging. J Am Coll Cardiol 14(4):968–976
Walsh MA, Lee KJ et al (2007) Radiofrequency perforation of the right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect. Catheter Cardiovasc Interv 69(7):1015–1020
Schumacher G, Hess J, Bühlmeyer K (2008) Klinische Kinderkardiologie. 4. Auflage. Springer Verlag, Heidelberg
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8.1 Electronic Supplementary Material
Video 8.1
Apical four-chamber view in a neonate with PAiVS and severely hypoplastic right ventricle and tricuspid valve (AVI 9691 kb)
Video 8.2
Colour Doppler in the apical four-chamber view (same patient as in Video 8.1) shows diastolic antegrade flow (coded red) confirming patency of the hypoplastic tricuspid valve (AVI 4573 kb)
Video 8.3
Parasternal short-axis view at the level of the aortic valve in a newborn shows patency of the right ventricular outflow tract up to the level of the atretic pulmonary valve (AVI 18118 kb)
Video 8.4
Colour Doppler in the parasternal short-axis view (same patient as in Video 8.3) shows retrograde flow in the main pulmonary artery originating from the patent ductus arteriosus (AVI 6043 kb)
Video 8.5
The colour Doppler sweep from the suprasternal notch in a neonate with PAiVS and well-formed tripartite right ventricle starts with the long-axis view of the aortic arch. Leftward tilt of the transducer reveals a large ductus arteriosus connecting to the pulmonary bifurcation (AVI 10959 kb)
Video 8.6
The suprasternal long-axis view of the aortic arch in a patient with PAiVS and unipartite, hypoplastic right ventricle shows a tortuous ductus arteriosus originating with an acute angle from the undersurface of the arch (AVI 3710 kb)
Video 8.7
Colour Doppler in the parasternal short-axis view of the aortic root shows the left coronary artery in a newborn with PAiVS and hypoplastic right ventricle. Note significant enlargement of the coronary artery at its origin from the aorta. Due to right ventricular coronary artery connections, the left coronary artery exhibits retrograde flow directed towards the aorta in systole (displayed in blue), while there is regular antegrade coronary flow (displayed in red) during diastole (AVI 4755 kb)
Video 8.8
Apical four-chamber view in a newborn with PAiVS and tripartite right ventricle shows dilated right atrium and good size right ventricle and tricuspid valve (AVI 7544 kb)
Video 8.9
Colour Doppler in the apical four-chamber view (same patient as in Video 8.8) reveals moderate regurgitation of a normal-size mitral valve (AVI 1933 kb)
Video 8.10
In this patient with PAiVS and dysplastic tricuspid valve, the right atrium is grossly dilated (AVI 5615 kb)
Video 8.11
Colour Doppler in the apical four-chamber view reveals that dilatation of the right atrium can be attributed to severe tricuspid regurgitation (AVI 1138 kb)
Video 8.12
Colour Doppler in the parasternal long-axis view of the right ventricular outflow shows retrograde flow in the main pulmonary artery directed towards the atretic pulmonary valve (AVI 1477 kb)
Video 8.13
Colour Doppler in a tilted apical four-chamber view of a newborn with PAiVS and moderately hypoplastic right ventricle shows apical in- and outflow due to a ventriculo-coronary communication (AVI 4477 kb)
Video 8.14
In- and outflow phenomena in the right ventricle are also apparent in the tilted parasternal short-axis view of another newborn with PAiVS, hypoplastic right ventricle and ventriculo-coronary communications (AVI 5792 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Pulmonary Atresia with Intact Ventricular Septum (PAiVS). In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_8
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DOI: https://doi.org/10.1007/978-3-319-42919-9_8
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