Coarctation of Aorta with Supravalvular Pulmonary Stenosis in an Adult Patient: A Rare Exception of the Fetal Flow Pattern Theory

Manish Ruhela^1, , Harneesh Randhawa¹, Prachi Bagarhatta², Rajeev Bagarhatta¹ and Anoop Jain¹

¹Department of Cardiology, Sawai Man Singh Medical College and Hospital, Jaipur, Rajasthan, India

²Lady Hardinge Medical College, New Delhi, India

Cite this paper

Manish Ruhela, Harneesh Randhawa, Prachi Bagarhatta, Rajeev Bagarhatta and Anoop Jain. Coarctation of Aorta with Supravalvular Pulmonary Stenosis in an Adult Patient: A Rare Exception of the Fetal Flow Pattern Theory. American Journal of Medical Case Reports. 2015; 3(3):53-58. doi: 10.12691/AJMCR-3-3-1

Abstract

Coarctation of the aorta is a common congenital defect whose overall incidence is 5–8% of all congenital cardiac anomalies. Associated cardiac anomalies have been well described in previous studies examining specific subgroups of coarctation of aorta patients, particularly infants, excluding older children, adolescents, and adults. The association of coarctation with left-sided obstructive lesions ( Bicuspid aortic valve, Parachute mitral valve, Mitral atresia, Aortic atresia ) were well documented in the literature. The association of coarctation of aorta with right- sided obstructive lesions is rare and a hemodyanamic explanation based on fetal flow pattern was given for this. Herein we report a case of coarctation of aorta with right sided obstructive lesion in form of supravalvular pulmonary stenosis in an adult female. To the best of our knowledge this is the first case report where an adult female presented with coarctation of aorta along with supravalvular pulmonary stenosis.

Keywords

coarctation of aorta, supravalvular pulmonary stenosis, fetal flow pattern theory

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Sinha SN, Kardatzke ML, Cole RB, et al. Coarctation of the aorta in infancy. Circulation 1969;40: 385-398.
[2]	Kaemmerer H (2003) Aortic coarctation and interrupted arch. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and management of adult congenital heart disease. Churchill Livingstone, Edinburgh, pp 253-264.
[3]	Biffi M, Boriani G, Frabetti L, Bronzetti G, Branzi A (2001) Left superior vena cava persistence in patients undergoing pacemaker of cardioverter-defibrillator implantation. Chest 120:139-144.
[4]	Becker AE, Becker MJ, Edwards JE (1970) Anomalies associated with coarctation of aorta particular reference to infancy. Circulation 41:1067-1075.
[5]	Høimyr H, Christensen TD, Emmertsen K, Johnsen SP, Riis A, Hansen OK et al (2006) Surgical repair of coarctation of the aorta: up to 40 years of follow-up. Eur J Cardiothorac Surg 30:910-916.
[6]	Levy MJ, Levinsky L, Deviri E, Hauptman E, Blieden C (1982) Coarctation of the aorta in infancy. Tex Heart Inst J 10:57-62.
[7]	Shinebourne EA, Tam AS, Elseed AM, Paneth M, Lennox SC, Cleland WP (1976) Coarctation of the aorta in infancy and childhood. Br Heart J 38:375-380.
[8]	Rudolph, A. M., Heymann, M. A., and Spitznas, U. (1972). Hemodynamic considerations in the development of narrowing of the aorta. American Journal of Cardiology, 30, 514.
[9]	Kiraly L, Kornyei L, Mogyorossy G, Szatmari A. Hypoplastic aortic arch in newborns rapidly adapts to post-coarctectomy circulatory conditions. Heart 2005; 91: 233-234.
[10]	Beekman RH. Coarctation of the aorta. In: Allen HG, Gutgessell HP, Clark EB, Driscoll DJ, editors. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adults. 6th edition. Philadelphia: Lippincott Williams and Wilkins; 2001 p 988-1010.
[11]	Paladini D, Volpe P, Russo MG, et al. Aortic coarctation: prognostic indicators of survival in the fetus. Heart 2004;90: 1348-1349.
[12]	Sinha, S. N., Kardatzke, M. L., Cole, R. B., Muster, A. J., Wessel, H. U., and Paul, M. H. (I969). Coarctation of the aorta in infancy. Circulation, 40, 385.
[13]	Dawes, G. S., Mott, J. G., and Widdicombe, J. G. (1954). The foetal circulation in the lamb. Journal of Physiology, 126, 563.
[14]	Rudolph, A. M., Heymann, M. A., Teramo, K. A. W., Barrett, C. T., and Raiha, N. C. R. (I97I). Studies on the circulation of the previable human fetus. Pediatric Research, 5, 452.
[15]	Cook, C. D., Drinker, P. A., Jacobson, H. N., Levison, M., and Strang, L. B. (I963). Control of pulmonary blood flow in the foetal and newly born lamb. Journal of Physiology, I69, I0.
[16]	Lauer, R. M., Evans, J. A., Aoki, H., and Kittle, C. F. (I965). Factors controlling pulmonary vascular resistance in fetal lambs. Journal of Pediatrics, 67, 568.