Unusual, Rare Case of Ascending Aortic Atresia and Right Ventricle Dependent Coronary Circulation in a Newborn
DOI:
https://doi.org/10.14205/2310-9394.2014.02.01.1Keywords:
RV Dependent, Pulmonary Atresia/Intact Ventricular Septum, Complete Atrioventricular Canal.Abstract
We describe a newborn with complex, congenital cardiac anomalies, most notably single ventricle physiology, interruption/atresia of the ascending aorta and a coronary circulation that was totally Right Ventricle (RV) dependent. This arrangement left the coronary supply in a very precarious situation, theoretically much worse than most patients with Pulmonary Atresia/Intact Ventricular Septum (PA/IVS), since the entire coronary circulation was RV dependent. The patient underwent a palliative surgical correction, but expired the following day. We believe this is the first reported case in the literature with this constellation of lesions.
References
Schwartz SM, Dent CL, Musa NL, Nelson DP. Singleventricle physiology. Crit Care Clin 2003; 19: 393-411. http://dx.doi.org/10.1016/S0749-0704(03)00007-1
Celoria G C, Patton R B: Congenital absence of the aortic arch. Am Heart J 1959; 58: 407-13. http://dx.doi.org/10.1016/0002-8703(59)90157-7
Van Praagh R, Bernhard W F, Rosenthal A, et al. Interrupted aortic arch: surgical treatment. Am J Cardiol 1971; 27: 200- 11. http://dx.doi.org/10.1016/0002-9149(71)90259-1
Weidenbach M, Daehnert I, Razek V, Beller A, Janousek J, Kostelka M, Anderson RH. Interruption of the Ascending Aorta: A Hitherto Undescribed Lesion. Ann Thorac Surg 2008; 85: 1451-3. http://dx.doi.org/10.1016/j.athoracsur.2007.10.078
Jux C, Kaulitz R, von Waseilewski R, Peuster M, Fink C, Paul T, Hausdorf G. Antegrade flow in the aorta ascendens despite aortic atresia: 2 case reports with retrograde coronary perfusion through coronary fistulas and sinusoids. Z Kardiol 2000; 89(6): 502-7. http://dx.doi.org/10.1007/s003920070221
Baker-Smith CM, Milazzo AS, Frush DP, Jaggers J, Kirby ML, Kanter RJ, Barker PC. Double aortic arch with aortic atresia and left-sided type B interruption. Congenit Heart Dis 2010; 5(3): 316-20. http://dx.doi.org/10.1111/j.1747-0803.2009.00341.x
Giglia TM, Mandell VS, Connor AR, Mayer JE, Jr., Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992; 86: 1516-28. http://dx.doi.org/10.1161/01.CIR.86.5.1516
Berne RM, Levy MN. Cardiovascular Physiology. 4th edition, pp. 215, St. Louis, CV Mosby, 1981.
Lajos P, Love J, Salim MA, Wang W, Cardarelli MG. Total Right Ventricular Dependent Coronary Circulation in Pulmonary Atresia With Intact Ventricular Septum. Ann Thorac Surg 2004; 77: 1087-8. http://dx.doi.org/10.1016/S0003-4975(03)01259-1
