Abstract
Pulmonary atresia with ventricular septal defect is a rare cyanotic congenital heart disease with decreased pulmonary
blood flow and with morphological variability. Right-to-left shunt and absence of direct flow from the right ventricle
to the pulmonary artery in this disease lead to a decrease in arterial oxygen saturation. Surgical treatment depends on
presence or absence of native pulmonary arteries, main aortopulmonary collateral arteries, clinical condition of
patient, accompanying congenital heart disease or diseases other organs. There are several surgical options: primary
complete repair of pulmonary atresia with ventricular septal defect or multistage surgical approach.
References
- Burakovskiy V.I., Bockeria L.A. (Eds.) Cardiovascularsurgery. Moscow; 1996 (in Russ.)
- Fyler D.C. Nadas' pediatric cardiology. Philadelphia;
1992.
- Samánek M., Vorísková M. Congenital heart disease
among 815,569 children born between 1980 and 1990 and
their 15-year survival: a prospective Bohemia survival
study. Pediatr. Cardiol. 1999; 20 (6): 411–7. DOI: 10.1007/s002469900502
- Devis G., Fulton R., Ritter D. Congenital pulmonary
atresia with ventricular septal defect: angiografic and
surgical correlates. Radiology. 1978; 128: 133–43.
DOI: 10.1148/128.1.133
- Van Praagh R., Van Praagh S. The anatomy of common
aorticopulmonary trunk (truncus arteriosus communis)
and its embryologic implications. Am. J. Cardiol. 1965;
16: 406–25. DOI: 10.1016/0002-9149(65)90732-0
- Soto B., Pacifico A.D., Luna R.F., Bargeron L.M. Jr.
A radiographic study of congenital pulmonary atresia
with ventricular septal defect. Am. J. Roentgenol. 1977;
129 (6): 1027–37. DOI: 10.2214/ajr.129.6.1027
- Somerville J. Management of pulmonary atresia. Br.
Heart J. 1970; 32: 641. DOI: 10.1136/hrt.32.5.641
- McGoon D.C., Danielson G.K., Puga F.J., Ritter D.G.,
Mair D.D., Ilstrup D.M. Late results after extracardiac
conduit repair for congenital cardiac defects. Am. J.
Cardiol. 1982; 49 (7): 1741–9. DOI: 10.1016/0002-9149(82)90254-5
- Barbero-Marcial M. Classification of pulmonary atresia
with ventricular septal defect. Ann. Thorac. Surg. 2001;
72: 316. DOI: 10.1016/s0003-4975(01)02487-0
- Podzolkov V.P., Kokshenev I.V., Gadzhiev A.A. Pulmo-nary atresia with ventricular septal defect. Moscow; 2003(in Russ.).
- Tchervenkov C.I., Roy N. Congenital heart surgery
nomenclature and database project: pulmonary atresiaventricular
septal defect. Ann. Thorac. Surg. 2000; 69:
S97–105. DOI: 10.1016/s0003-4975(99)01285-0
- Bharati S., Paul M.H., Idriss F.S., Potkin R.T., Lev M.
The surgical anatomy of pulmonary atresia with ventricular
septal defect: pseudotruncus. J. Thorac. Cardiovasc.
Surg. 1975; 69 (5): 713–21.
- Gorbachevskiy S.V., Plakhova V.V., Bockeria L.A. Thecapabilities of echocardiography in assessement of mor-phofunctional condition of heart and prognostication ofnatural course of high pulmonary hypertension. RussianJournal of Thoracic and Cardiovascular Surgery.2002; 3:22–30 (in Russ.).
- Bespalova E.D., Ivanitskiy A.V., Demidov B.S., Netali-eva G.S. The capabilities of prenatal echocardiography inanomalies of conotrunkus. Sonoace international.2001; 8:65–73 (in Russ.).
- Alekyan B.G., Pursanov M.G., Tumanyan M.R., Kim A.I.,Krupyanko S.M., Agasyan A.G. Endovascular treatmentof critical valve stenosis of the pulmonary artery in new-borns. Bakoulev Journal for Cardiovascular Diseases.2010; 11 (6, appl.): 164 (in Russ.).
- Choi B.W., Park Y.H., Choi J.Y., Choi B.I., Kim M.J.,
Ryu S.J. et al. Using electron beam CT to evaluate
conotruncal anomalies in pediatric and adult patients.
Am. J. Roentgenol. 2001; 177 (5): 1045–9. DOI: 10.2214/ajr.177.5.1771045
- Sugiyama H., Naito H., Tsukano S., Yoshibayashi M.,
Echigo S., Kamiya T. Evaluation by contrast-enhanced
electron beam computed tomography of myocardial perfusion
and tissue characteristics in congenital aortic
stenosis. Circ. J. 2003; 67: 998–2002. DOI: 10.1253/circj.67.998
- Goo H.W., Park I.S., Ko J.K., Kim Y.H., Seo D.M.,
Park J.J. Computed tomography for the diagnosis of congenital
heart disease in pediatric and adult patients.
Int. J. Cardiovasc. Imaging. 2005; 21 (2–3): 347–65.
DOI: 10.1007/s10554-004-4015-0
- Eichenberger A.C., Von Schulthess G.K. Magnetic resonance
imaging of the heart and the great vessels: morphology,
function, and perfusion. Curr. Opin. Radiol.
1992; 4 (4): 41–7.
- Tsai-Goodman B., Geva T., Odegard K.C., Sena L.M.,
Powell A.J. Clinical role, accuracy, and technical aspects
of cardiovascular magnetic resonance imaging in infants.
Am. J. Cardiol. 2004; 94 (1): 69–74. DOI: 10.1016/j.amjcard.2004.03.033
- Lombardi M., Bartolozzi C. (Eds.) MRI of the heart and
vessels. Milan: Springer-Verlag; 2004. DOI: 10.1007/b139059
- Alfieri O., Blackstone E.H., Kirklin J.W., Pacifico A.D.,
Bargeron L.M. Surgical treatment of tetralogy of Fallot
with pulmonary atresia. J. Thorac. Cardiovasc. Surg. 1978;
76: 321–35.
- Piehler J.M., Danielson G.K., McGoon D.C., Wallace R.B.,
Fulton R.E., Mair D.D. Management of pulmonary
atresia with ventricular septal defect and hypoplastic pulmonary
arteries by right ventricular outflow construction.
J. Thorac. Cardiovasc. Surg. 1980; 80 (4): 552–67.
- Nakata S., Imai Y., Takanashi Y., Kurosawa H., Tezuka K.,
Nakazawa M. et al. A new method for the quantitative
standardization of cross-sectional area of the pulmonary
arteries in congenital heart diseases with decreased pulmonary
blood flow. J. Thorac. Cardiovasc. Surg. 1984; 88:
610–9.
- Lofland G.K. The management of pulmonary atresia,
ventricular septal defect, and multiple aorta pulmonary
collateral arteries by definitive single stage repair in early
infancy. Eur. J. Cardiothorac. Surg. 2000; 18: 480–6.
DOI: 10.1016/s1010-7940(00)00547-9
- Marshall A.C., Love B.A., Lang P., Jonas R.A., Del
Nido P.J., Mayer J.E., Lock J.E. Staged repair of tetralogy
of Fallot and diminutive pulmonary arteries with a fenestrated
ventricular septal defect patch. J. Thorac.
Cardiovasc. Surg. 2003. 126: 1427–33. DOI: 10.1016/s0022-5223(03)01182-6
- Ishikawa S., Takahashi T., Sato Y. Growth of the pulmonary
arteries after systemic-pulmonary shunt. Ann.
Thorac. Cardiovasc. Surg. 2001; 7 (6): 337–40.
- Kirklin J.W., Bargeron L.M.J., Pacifico A.D. The
enlargement of small pulmonary arteries by preliminary
palliative operations. Circulation. 1977; 56: 612–7.
DOI: 10.1161/01.cir.56.4.612
- Gale A.W., Arciniegas E., Green E.W., Blackstone E.H.,
Kircklin J.W. Growth of the pulmonary annulus and pulmonary
arteries after the Blalock–Taussig shunt.
J. Thorac. Cardiovasc. Surg. 1979; 77: 459–65.
- Amato J.J., Cotronero J.V., Galdieri R.J. The central
shunt: aortopulmonary Gore-Tex shunt. Sem. Thorac.
Cardiovasc. Surg. 1990; 2 (l): 34–45.
- Waterson K.G., Wilkinson J.L., Karl T.R., Mee R.B.B.
Very small pulmonary arteries: central end-to-side shunt.
Ann. Thorac. Surg. 1991; 52: l132–7. DOI: 10.1016/0003-4975(91)91294-6
- Alkhulaifi A.M., Lacour-Gayet F., Serraf A., Belli E.,
Planché C. Systemic pulmonary shunts in neonates: early
clinical outcome and choice of surgical approach. Ann.
Thorac. Surg. 2000; 69 (5): 1499–504. DOI: 10.1016/s0003-4975(00)01078-x
- Podzolkov V.P., Gadzhiev A.A., Alekyan B.G. The firstexperience of multistage surgical treatment of pulmonaryatresia with ventricular septal defect. Russian Journal of Tho-racic and Cardiovascular Surgery.1997; 2: 28–9 (in Russ).
- Petrosyan Y.S., Alekyan B.G., Sobolev A.V. Ballon
angioplasty for branch pulmonary arterial stenosis.
Pediatric Cardiac Surgery. Paris; 1993: 79.
- Kaufman S., Kan J. Embolisation of pulmonary artery
collaterals in the management of gemoptysis in pulmonary
atresia. Am. J. Cardiol. 1985; 58: 1130–2.
DOI: 10.1016/0002-9149(86)90138-4
- Mitchell S., Kan J., White R. Interventional techniques
in congenital heart desease. Semin. Roentgenol. 1985; 20:
290–311. DOI: 10.1016/0037-198x(85)90012-4
- Gianturco C., Anderson J.A., Wallace S. Mechanical
devices for arterial occlusion. Am. J. Roentgenol. 1975;
124: 428–35. DOI: 10.2214/ajr.124.3.428
- Puga F.J., Uretzky G. Establishment of right ventricle
hypoplastic pulmonary artery continuity without the use
of extracorporeal circulation. J. Thorac. Cardiovasc.
Surg. 1982; 83 (1): 74–80.
- Puga F.J., McGoon D.C., Julsrud P.R., Danielson G.K.,
Mair D.D. Complete repair of pulmonary atresia with
nonconfluent pulmonary arteries. Ann. Thorac. Surg. 1983;
35 (1): 36–44. DOI: 10.1016/s0003-4975(10)61428-2
- Nevvazhay T.I., Chernogrivov A.E., Biktasheva L.Z.,Karchevskaya K.V., Ekimenko L.V., Paramonova T.I.et al. A case of single-stage bilateral pulmonary bloodflow unification in type 4 pulmonary atresia with ventri-cular septal defect. Russian Journal of Thoracic andCardiovascular Surgery.2009; 5: 70–2 (in Russ.).
- Cheban V.N., Samsonov V.B., Kokshenev I.V., Shin-kareva T.V. The case of successful unifocalisation of thepulmonary blood flow after repair of the right ventricularoutflow tract in the condition of pulmonary artery atresiawith ventricular septal defect. Children's Heart andVascular Diseases.2013; 1: 48–51 (in Russ.).
- Gadzhiev A.A., Kokshenev I.V., Samsonov V.B.,Pursanov M.G., Shamrin Yu.N., Mchedlishvili C.A. et al.Types of collateral blood flow of the lungs and their diag-nosis in pulmonary atresia with ventricular septal defectin combination with great aortopulmonary collateralarteries. Russian Journal of Thoracic and CardiovascularSurgery.2004; 2: 29–36 (in Russ.).
- Lillehei C.W., Cohen M.C., Warden H.E., Read R.C.,
Aust J.B., DeWall R.A., Varco R.L. Direct vision intracardiac
surgical correction of the tetralogy of Fallot, pentalogy
of Fallot, and pulmonary atresia defects. Repot
of first ten cases. Ann. Surg. 1955; 142: 418–42.
DOI: 10.1097/00000658-195509000-00010
- Liava'a M., Brizard C.P., Konstantinov I.E., Robertson T.,
Cheung M.M., Weintraub R., d'Udekem Y. Pulmonary
atresia, ventricular septal defect, and major aortopulmonary
collaterals: neonatal pulmonary artery rehabilitation
without unifocalization. Ann. Thorac. Surg. 2012;
93: 185–92. DOI: 10.1016/j.athoracsur.2011.08.082
- Yonghui Zhang, Zhongdong Hua, Keming Yang, Hao
Zhang, Jun Yan, XuWang et al. Outcomes of the rehabilitative
procedure for patients with pulmonary atresia,
ventricular septal defect and hypoplastic pulmonary
arteries beyond the infant period. Eur. J. Cardiothorac.
Surg. 2014; 46: 297–303. DOI: 10.1093/ejcts/ezt622
- Lenoir M., Pontailler M., Gaudin R., Gerelli S., Tamisier
D., Bonnet D. et al. Outcomes of palliative right
ventricle to pulmonary artery connection for pulmonary
atresia with ventricular septal defect. Eur. J. Cardiothorac.
Surg. 2017; 52 (3): 590–8. DOI: 10.1093/ejcts/ezx194
- Jae Gun Kwak, Chang-Ha Lee, Cheul Lee, Chun Soo
Park. Surgical management of pulmonary atresia with ventricular
septal defect: early total correction versus shunt.
Ann. Thorac. Surg. 2011; 91: 1928–35. DOI: 10.1016/j.athoracsur.2011.01.014
- Watanabe N., Mainwaring R.D., Mohan Reddy V.,
Palmon M., Hanley F.L. Early complete repair of pulmonary
atresia with ventricular septal defect and major
aortopulmonary collaterals. Ann. Thorac. Surg. 2014; 97:
909–15. DOI: 10.1016/j.athoracsur.2013.10.115
- Mainwaring R.D., Patrick W.L., Punn Rajesh, Palmon M.,
Mohan Reddy V., Hanley F.L. Fate of right ventricle to
pulmonary artery conduits after complete repair of pulmonary
atresia and major aortopulmonary collaterals.
Ann. Thorac. Surg. 2015; 99: 1685–91. DOI: 10.1016/j.athoracsur.2014.12.071
- Kaskinen A.K., Happonen J.-M., Mattila I.P., Pitkänen
O.M. Long-term outcome after treatment of pulmonary
atresia with ventricular septal defect: nationwide
study of 109 patients born in 1970–2007. Eur. J. Cardiothorac.
Surg. 2016; 49 (5): 1411–8. DOI: 10.1093/ejcts/ezv404
- Chen Q., Ma K., Hua Zh., Yang K., Zhang H., Wang X.
et al. Multistage pulmonary artery rehabilitation in
patients with pulmonary atresia, ventricular septal defect
and hypoplastic pulmonary artery. Eur. J. Cardiothorac.
Surg. 2016; 50 (1): 160–6. DOI: 10.1093/ejcts/ezw069
About the authors
Aleksandr A. Chvamaniya, Researcher; orcid.org/0000-0002-8011-4993
