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Congenital Heart Disease and Surgical Footprints

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The Operated Heart at Autopsy

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Abstract

The purpose of this chapter is to illustrate and discuss a series of cases of congenital heart disease that will provide for some pathologists in practice, and particularly for those in training, a point of reference by which skills can be developed in this area of complex morphologic changes of the heart and what they mean at autopsy. Images of a collection of formalin-fixed hearts with congenital defects of variable complexity will be presented. Surgical approaches will be discussed in an attempt to reinforce the need for the pathologist to identify those “footprints” described earlier in this manuscript. The final case in this chapter, although not an example of congenital heart disease, will signal caution to the observer to maintain an overall perspective when examining the heart.

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References

  1. Gruber PJ, Epstein JA. Development gone awry. Congenital heart disease. Circ Res 2004;94:273–83.

    Article  PubMed  CAS  Google Scholar 

  2. Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E, McGee G, Sable CA, Srivastava D, Webb CL. Genetic basis of congenital heart defects: current knowledge. Circulation 2007;115:1–24.

    Article  Google Scholar 

  3. Fost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein’s anomaly—review of a multifaceted congenital cardiac condition. Swiss Med Wkly 2005;135:269–81.

    Google Scholar 

  4. Carpentier A, Chauvaud S, Mace L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101.

    PubMed  CAS  Google Scholar 

  5. Moodie DS, Ritter DG, Tajik AJ, O’Fallon WM. Long-term follow-up in the unoperated univentricular heart. Am J Cardiol 1984;53:1124–28.

    Article  PubMed  CAS  Google Scholar 

  6. Krishnan U. Univentricular heart: management options. Indian J Pediatr 2005;72:519–24.

    Article  PubMed  Google Scholar 

  7. Malhotra SP, Ivy DD, Mitchell MB, Campbell DN, Dines ML, Miyamoto S, Kay J, Clarke DR, Lacour-Gayet F. Performance of cavopulmonary palliation at elevated altitude: midterm outcomes and risk factors for failure. Circulation 2008;118:S177–81.

    Article  PubMed  Google Scholar 

  8. Margossian RE, Solowiejczyk D, Bourlon F, Apfel H, Gersony WM, Hordof AJ, Quaegebeur J. Septation of the single ventricle: revisited. J Thorac Cardiovasc Surg 2002;124:442–7.

    Article  PubMed  Google Scholar 

  9. Obler D, Juraszek AL, Smoot LB, Natowicz MR. Double outlet right ventricle: aetiologies and associations. J Med Genet 2008;45:481–97. Review.

    Article  PubMed  CAS  Google Scholar 

  10. Lacour-Gayet LF. Intracardiac repair of double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2008; 39–43.

    Google Scholar 

  11. Rodefeld MD, Ruzmetov M, Vijay P, Fiore AC, Turrentine MW, Brown JW. Surgical results of arterial switch operation for Taussig-Bing anomaly: is position of the great arteries a risk factor? Ann Thorac Surg 2007 Apr;83(4):1451–7.

    Article  PubMed  Google Scholar 

  12. Bradley TJ, Karamlou T, Kulik A, Mitrovic B, Vigneswaran T, Jaffer S, Glasgow PD, Williams WG, Van Arsdell GS, McCrindle BW. Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle. J Thorac Cardiovasc Surg 2007;134:967–73.

    Article  PubMed  Google Scholar 

  13. Rastelli G, Kirklin JW, Titus JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc 1966;41:296–308.

    PubMed  CAS  Google Scholar 

  14. Squarcia U, Squarcia lA. Giancarlo Rastelli: the scientist, the man. Clin Cardiol 2007;30: 485–7.

    Article  PubMed  Google Scholar 

  15. Mavroudis C, Weinstein G, Turley K, Ebert PA. Surgical management of complete atrioventricular canal. J Thorac Cardiovasc Surg 1982;83:670–9.

    PubMed  CAS  Google Scholar 

  16. Minette MS, Sahn DJ. Ventricular septal defects. Circulation 2006;114:2190–7.

    Article  PubMed  Google Scholar 

  17. Barratt-Boyes BG, Neutze JM, Clarkson PM, Shardey GC, Brandt PW. Repair of ventricular septal defect in the first two years of life using profound hypothermia-circulatory arrest techniques. Ann Surg 1976;184:376–90.

    Article  PubMed  CAS  Google Scholar 

  18. Schwerzmann M, Samman AM, Salehian O, Holm J, Provost Y, Webb GD, Therrien J, Siu SC, Silversides CK. Comparison of echocardiographic and cardiac magnetic resonance imaging for assessing right ventricular function in adults with repaired tetralogy of Fallot. Am J Cardiol 2007;99:1593–7.

    Article  PubMed  Google Scholar 

  19. Saleeb SF, Solowiejczyk DE, Glickstein JS, Korsin R, Gersony WM, Hsu DT. Frequency of development of aortic cuspal prolapse and aortic regurgitation in patients with subaortic ventricular septal defect diagnosed at <1 year of age. Am J Cardiol 2007;99:1588–92.

    Article  PubMed  Google Scholar 

  20. Verheugt CL, Uiterwaal CS, Grobbee DE, Mulder BJ, Verheugt CL, Uiterwaal CS, Grobbee DE, Mulder BJ. Long-term prognosis of congenital heart defects: a systematic review. Int J Cardiol 2008 Aug 5 [Epub ahead of print].

    Google Scholar 

  21. Bartz PJ, Cetta F, Cabalka AK, Reeder GS, Squarcia U, Agnetti A, Aurier E, Carano N, Tachana B, Hagler DJ. Paradoxical emboli in children and young adults: role of atrial septal defect and patent foramen ovale device closure. Mayo Clin Proc 2006;81:615–8.

    Article  PubMed  Google Scholar 

  22. Wechsler LR. PFO and stroke: what are the data? Cardiol Rev 2008;16 (1):53–7. Review.

    Article  PubMed  Google Scholar 

  23. Takenaka T, Horimoto M, Fujiwara M. Anomalous origin of the left anterior descending coronary artery from the right sinus of Valsalva associated with effort angina pectoris. Eur Heart J 1993;14:129–31.

    PubMed  CAS  Google Scholar 

  24. Turkoglu S, Ozdemir M. Anomalous origin of the left circumflex coronary artery from the right coronary artery and the left anterior descending artery from the right coronary sinus. J Invasive Cardiol 2006;18:E214–6.

    PubMed  Google Scholar 

  25. Kimbiris D. Anomalous origin of the left main coronary artery from the right sinus of Valsalva. Am J Cardiol 1985;55:765–9.

    Article  PubMed  CAS  Google Scholar 

  26. Shirani J, Roberts WC. Origin of the left main coronary artery from the right aortic sinus with retroaortic course of the anomalistically arising artery. Am Heart J 1992;124: 1077–8.

    Article  PubMed  CAS  Google Scholar 

  27. Rigatelli G, Rigatelli G. Coronary artery anomalies: what we know and what we have to learns. A proposal for a new clinical classification. Ital Heart J 2003;4:305–10.

    PubMed  Google Scholar 

  28. Angelini P. Coronary artery anomalies. An entity in search of an identity. Circulation 2007;115:1296–305.

    PubMed  Google Scholar 

  29. Frescura C, Basso C, Thiene G, Corrado D, Pennelli T, Angelini A, Daliento L. Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol 1998;29:689–95.

    Article  PubMed  CAS  Google Scholar 

  30. Goland S, Czer LS, De Robertis MA, Mirocha J, Kass RM, Fontana GP, Chang W, Trento A. Risk factors associated with reoperation and mortality in 252 patients after aortic valve replacement for congenitally bicuspid aortic valve disease. Ann Thorac Surg 2007;83: 931–7.

    Article  PubMed  Google Scholar 

  31. Cosgrove DM, Rosenkranz ER, Hendren WG, Bartlett JC, Stewart WJ. Valvuloplasty for aortic insufficiency. J Thorac Cardiovasc Surg 1991;102:571–6; discussion 576–7.

    PubMed  CAS  Google Scholar 

  32. Guntheroth WG. A critical review of the American College of Cardiology/American Heart Association practice guidelines on bicuspid aortic valve with dilated ascending aorta. Am J Cardiol 2008;102:107–10. Review.

    Article  PubMed  Google Scholar 

  33. Della Corte A, De Santo LS, Montagnani S, Quarto C, Romano G, Amarelli C, Scardone M, De Feo M, Cotrufo M, Caianiello G. Spatial patterns of matrix protein expression in dilated ascending aorta with aortic regurgitation: congenital bicuspid valve versus Marfan’s syndrome. J Heart Valve Dis 2006;15:20–7; discussion 27.

    PubMed  Google Scholar 

  34. Nascimento D, Nunes L, Oliveira F, Vencio E, Teixeira V, Reis M. Pulmonary atherosclerosis associated with an atrial septal defect in old age: Case report of an elderly autopsied patient. Pathol Res Pract 2008 Oct 21 [Epub ahead of print].

    Google Scholar 

  35. Giroud JM, Jacobs JP. Evolution of strategies for management of the patent arterial duct. Cardiol Young 2007;17 Suppl 2:68–74. Review.

    PubMed  Google Scholar 

  36. Tekin Y, Ozer S, Murat B, Hulusi UM, Timucin ON. Closure of adult patent ductus arteriosus under cardiopulmonary bypass by using foley balloon catheter. J Card Surg 2007;22: 219–20.

    Article  PubMed  Google Scholar 

  37. Wong D, Golding F, Hess L, Caldarone CA, Van Arsdell G, Manlhiot C, McCrindle BW, Miner SE, Nield LE. Intraoperative coronary artery pulse Doppler patterns in patients with complete transposition of the great arteries undergoing the arterial switch operation. Am Heart J 2008;156:466–72.

    Article  PubMed  Google Scholar 

  38. Love BA, Mehta D, Fuster VF. Medscape. Evaluation and management of the adult patient with transposition of the great arteries following atrial-level (Senning or Mustard) repair. Nat Clin Pract Cardiovasc Med 2008;5:454–67. Review.

    Article  PubMed  Google Scholar 

  39. Graham Jr TP, Markham L, Parra DA, Bichell D, Graham Jr TP, Markham L, Parra DA, Bichell D. Congenitally corrected transposition of the great arteries: an update. Curr Treat Options Cardiovasc Med 2007;9:407–13.

    Article  PubMed  Google Scholar 

  40. Reddy VM, McElhinney DB, Silverman NH, Hanley FL. The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children. Eur Heart J 1997;18:1470–7.

    PubMed  CAS  Google Scholar 

  41. Gagliardi MG, Adorisio R, Crea F, Versacci P, Di Donato R, Sanders SP. Abnormal vasomotor function of the epicardial coronary arteries in children five to eight years after arterial switch operation: an angiographic and intracoronary Doppler flow wire study. J Am Coll Cardiol 2005;46:1565–72.

    Article  PubMed  Google Scholar 

  42. Midgley FM, Scott LP, Perry LW, Shapiro SR, McClenathan JE. Subclavian flap aortoplasty for treatment of coarctation in early infancy. J Pediatr Surg 1978;13:264–8.

    Article  PubMed  CAS  Google Scholar 

  43. Caminos OW. Congenital heart diseases: their study and treatment. MCI Publications, Sønderborg, 1999.

    Google Scholar 

  44. Backer CL, Stewart RD, Kelle AM, Mavroudis C. Use of partial cardiopulmonary bypass for coarctation repair through a left thoracotomy in children without collaterals. Ann Thorac Surg 2006;82:964–72.

    Article  PubMed  Google Scholar 

  45. Uddin MJ, Haque AE, Salama AL, Uthman BC, Abushaban LA, Shuhaiber HJ. Surgical management of coarctation of the aorta in infants younger than five months: a study of fifty-one patients. Ann Thorac Cardiovasc Surg 2000;6:252–7.

    PubMed  CAS  Google Scholar 

  46. Ciotti GR, Vlahos AP, Silverman NH. Morphology and function of the bicuspid aortic valve with and without coarctation of the aorta in the young. Am J Cardiol 2006;98: 1096–102.

    Article  PubMed  Google Scholar 

  47. Carvalho MV, Pereira WL, Gandra SM, Rivetti LA. Aortic coarctation in the adult: regarding a case and extra-anatomic bypass approaches. Rev Bras Cir Cardiovasc 2007;22:501–4.

    Article  PubMed  Google Scholar 

  48. Yang Y, Zhou Y, Ma L, Ni Y. Unruptured aneurysm of the sinus of Valsalva presenting with thrombosis and right ventricular outflow obstruction. J Card Surg 2008;23:782–4.

    Article  PubMed  Google Scholar 

  49. Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, Holmes D. Sinus of Valsalva aneurysms-47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159–64.

    Google Scholar 

  50. Jung SH, Yun TJ, Im YM, Park JJ, Song H, Lee JW, Seo DM, Lee MS. Ruptured sinus of Valsalva aneurysm: transaortic repair may cause sinus of Valsalva distortion and aortic regurgitation. J Thorac Cardiovasc Surg 2008;135:1153–8.

    Article  PubMed  Google Scholar 

  51. Dodd JD, Aquino SL, Holmvang G, Cury RC, Hoffmann U, Brady TJ, Abbara S. Cardiac septal aneurysm mimicking pseudomass: appearance on ECG-gated cardiac MRI and MDCT. AJR Am J Roentgenol 2007;188:W550–3. Review.

    Article  PubMed  Google Scholar 

  52. Ossemann M, Laloux P, Marchandise B, Jamart J. Association between stroke and atrial septal aneurysm assessed by transesophageal echocardiography in a cardiologic population. Acta Neurol Belg 1995;95:170–7. Review.

    PubMed  CAS  Google Scholar 

  53. Harkness JR, Fitton TP, Barreiro CJ, Alejo D, Gott VL, Baumgartner WA, Yuh DD. A 32-year experience with surgical repair of sinus of Valsalva aneurysms. J Card Surg 2005;20: 198–204.

    Article  PubMed  Google Scholar 

  54. Cury RC, Abbara S, Sandoval LJ, Houser S, Brady TJ, Palacios IF. Images in cardiovascular medicine. Visualization of endomyocardial fibrosis by delayed-enhancement magnetic resonance imaging. Circulation 2005;111:e115–7.

    Article  PubMed  Google Scholar 

  55. Boudoulas H. Etiology of valvular heart disease. Expert Rev Cardiovasc Ther 2003;1: 523–32. Review.

    Article  PubMed  Google Scholar 

  56. Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH. A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med 2008;359:43–9.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Boston, MA 02114, USA

    Dr. Stuart Lair Houser

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Correspondence to Stuart Lair Houser .

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Houser, S.L. (2009). Congenital Heart Disease and Surgical Footprints. In: The Operated Heart at Autopsy. Humana Press. https://doi.org/10.1007/978-1-60327-808-9_6

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