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Pulmonary atresia with intact ventricular septum

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Congenital Heart Defects
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Abstract

The pulmonary valve annulus is generally small, rarely hypoplastic, and the pulmonary valve leaflets are generally well formed but fused. The main pulmonary artery is small but rarely is atretic as seen with pulmonary atresia and ventricular septal defect. The patent ductus arteriosus is usually small because it carries blood from the aorta to the pulmonary arteries in utero and not the other way around as in normal fetuses; therefore, much less blood travels through the patent ductus arteriosus in utero.

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References

  • Adachi I, Yagihara T, Kagisaki K, Hagino I, Ishizaka T, Kobayashi J, Kitamura S, Uemura H (2007) Preoperative small pulmonary artery did not affect the midterm results of Fontan operation. Eur J Cardiothorac Surg 32:156–162

    Article  PubMed  Google Scholar 

  • Choi BW, Park YH, Lee JK, Kim MJ, Kim DJ, Ryu SJ, Cho BK, Choe KO (2003) Evaluation of the postshunt status with electron beam computed tomography in cyanotic congenital heart disease. Yonsei Med J 44:249–258

    PubMed  Google Scholar 

  • Corno AF, Mazzera E, Marino B, Parisi F, Marcelletti C (1986) Simultaneous patency of ductus arteriosus and surgical shunt in pulmonary atresia with intact ventricular septum. A cause of acute myocardial failure? Scand J Thorac Cardiovasc Surg 20:123–127

    PubMed  CAS  Google Scholar 

  • De Leval MR, Bull C, Stark J, Anderson RH, Taylor JFN, Macartney FJ (1982) Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification. Circulation 66: 272–280

    PubMed  Google Scholar 

  • Freedom RM (1983) The morphological variations of pulmonary atresia with intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 4:183–188

    Article  PubMed  CAS  Google Scholar 

  • Geva T, Greil GF, Marshall AC, Landzberg M, Powell AJ (2002) Gadolinium-enhanced three-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with X-ray angiography. Circulation 106:473–478

    Article  PubMed  Google Scholar 

  • Giannico S, Corno AF, Marino B, Cicini MP, Gagliardi MG, Amodeo A, Picardo S, Marcelletti C (1992) Total extracardiac right heart bypass. Circulation 86(Suppl2):110–117

    Google Scholar 

  • Giannico S, Hammad F, Amodeo A, Michielon G, Drago F, Turchetta A, di Donato R, Sanders SP (2006) Clinical outcome of 193 extracardiac Fontan patients. J Am Coll Cardiol 47:2065–2073

    Article  PubMed  Google Scholar 

  • Gray RG, Altmann K, Mosca RS, Prakash A, Williams IA, Quaegebeur JM, Chen JM (2007) Persistent antegrade pulmonary blood flow post-Glenn does not alter early post-Fontan outcomes in single-ventricle patients. Ann Thorac Surg 84:888–893

    Article  PubMed  Google Scholar 

  • Hirata Y, Chen JM, Quaegebeur JM, Hellenbrand WE, Mosca RS (2007) Pulmonary atresia with intact ventricular septum: limitations of catheter-based intervention. Ann Thorac Surg 84:574–580

    Article  PubMed  Google Scholar 

  • Hosein RBM, Clarke AJB, McGuirk SP, Griselli M, Stumper O, De Giovanni JV, Barron DJ, Brawn WJ (2007) Factors influencing early and late outcome following the Fontan procedure in the current era. The “Two Commandments”? Eur J Cardiothorac Surg 31:344–353

    Article  PubMed  Google Scholar 

  • Marcelletti C, Corno AF, Giannico S, Marino B (1990) Inferior vena cava to pulmonary artery extracardiac conduit: a new form of right heart bypass. J Thorac Cardiovasc Surg 100:228–232

    PubMed  CAS  Google Scholar 

  • Qureshi SA (2001) Collaborative approach in the management of pulmonary atresia with intact ventricular septum. J Interv Cardiol 14:377–384

    Article  PubMed  CAS  Google Scholar 

  • Zhang H, Li SJ, Li YQ, Wang H, Hu SS (2007) Hybrid procedure for the neonatal management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 133:1654–1656

    Article  PubMed  Google Scholar 

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© 2009 Steinkopff Verlag

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(2009). Pulmonary atresia with intact ventricular septum. In: Congenital Heart Defects. Steinkopff. https://doi.org/10.1007/978-3-7985-1719-6_10

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  • DOI: https://doi.org/10.1007/978-3-7985-1719-6_10

  • Publisher Name: Steinkopff

  • Print ISBN: 978-3-7985-1718-9

  • Online ISBN: 978-3-7985-1719-6

  • eBook Packages: MedicineMedicine (R0)

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