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Category: Volume 11 Issue 07 July 2023
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Title: Double Outlet Right Ventricle with Pulmonary Atresia and Antero-Posterior Malposition of Great Arteries: Transthoracic Echocardiographic Evaluation of a rare cyanotic congenital heart disorder

Authors: Akhil Mehrotra, Ujala Shakya, Mohammed Shaban, Shubham Kacker

 DOI: https://dx.doi.org/10.18535/jmscr/v11i7.08

Abstract

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle, This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We are reporting a case of seven year old deeply cyanotic child with DORV, subaortic VSD with rare anatomic features of antero-posterior malposition of great arteries (GA) and pulmonary vavular atresia, with conspicuous absence of patent ductusarteriosus (PDA) or major aorto-pulmonary collaterals (MAPCAs), despite the presence of atretic pulmonary valve.

Keywords: DORV, Pulmonary atresia, TGA, VSD, subaortic VSD, overriding of Aorta, cyanotic congenital heart defects.

References

  1. Shuler CO, Black GB, Jerrell JM. Population- based treated prevalence of congenital heart disease in a pediatric cohort. PediatrCardiol. 2013; 34:606-11.
  2. Imaging in Pediatrics, Book 2017, edited by: A. Carlson Merrow, Jr. and Selena Hariharan.
  3. Hagler DJ. Double outlet right ventricle. In: Allen HD, Drisscoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams' Heart Disease in Infants, Children, and Adolescents. 7th. Philadelphia: Lippincott Williams & Wilkins; 2007. p. 1103-27.
  4. Lev M, Bharati S, Meng CC, Liberthson RR, Paul MH, Idriss F. 1972. A concept of double-outlet right ventricle. J Thorac Cardio- vascSurg 64:271-281.
  5. Guerin R, Soto B, Karp RB, Kirklin JW, Barcia A. Transposition of the great Determination of the position of the great arteries in conventional chest roentgenograms. Am J Roentgenol Radium Ther Nucl Med. 1970 Dec; 110(4):747-56. 
  6. Pang KJ, Meng H, Hu SS, Wang H, Hsi D, Hua ZD, et al. Echocardiographic classification and surgical approaches to double-outlet right ventricle for great arteries arising almost exclusively from the right ventricle. Tex Heart Inst J 2017:44:245-51.
  7. Jaggers JJ, Cameron DE, Herlong JR, Ungerleider RM: Congenital Heart Surgery Nomenclature and Database Project: transposition of the great arteries. AnnThoracSurg 2000, 69:S205-35.
  8. Sondheimer HM, Freedom RM, Olley PM. Double outlet right ventricle: Clinical spectrum and prognosis. Am J Cardiol 1977:39:709-14.
  9. Wright GE, Maeda K,Silverman NH, Hanley FL, Roth SJ. Double outlet right ventricle. In: Allen HD, Shaddy RE, Driscoll DJ, Feltes TF, editors. Moss and Adams' Heart Disease in Infants, Children, and Adolescents. 8th. Philadelphia: Lippincott Williams & Wilkins; 2013. p. 1161-74.
  10. Stidaromont S, Ritter DG, Feldt RH, Davis GD, Edwards JE. Double-outlet right ventricle. Anatomic and angiocardiographic correlations. Mayo ClinProc 1978; 53:555-77.
  11. Aoki M, Forbess JM, Jonas RA, Mayer Jr JE, Castaneda A. Result of biventricular repair for double outlet right ventricle. J Thorac Cardiovasc Surg 1994; 107:338-50.
  12. Sakata R, Lecompte Y, Batisse A, Borromee L, Durandy Y. Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. I. Criteria of surgical decision. J Thorac CardiovascSurg 1988; 95: 90-5.
  13. Peixoto LB, Leal SMB, Silva CES et al, Double outlet right ventricle with anterior and left sided aorta and subpulmonary ventricular septal defect, Araq Bras Cardiol, 1999; 75:446-450.
  14. Thiene G, Bortolotti U, Gallucci V, Valente ML, Volta SD. Pulmonary atresia with ventricular septal defect: further anatomical ob- servations. Br Heart J 1977; 39:1223-33.
  15. Allanby KD, Brinton WD, Campbell M, Gardner F. Pulmonary atresia and the collateral circulation to the lungs. Guys Hosp Rep 1950; 99:110-52.
  16. Schneeweiss A, Shem-Tov A, Dicker D, Blieden LC, Deutsch V, Neufeld HN. The pulmonary circulation and its systemic arterial supply in pulmonary atresia. ClinCardiol 1982; 5:489-92.
  17. Tynan MJ, Gleeson JA. Pulmonary atresia with bronchial arteries arising from the subclavian arteries. Br Heart J 1966; 28:573-6.
  18. Jefferson K, Rees S, Somerville J. Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 1972; 34:418-27.
  19. Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC. Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates. Radiology 1978; 128:133-44.
  20. McGooh DC, Baird DK, Davis GD. Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia. Circulation 1975; 52:109-18.

Corresponding Author

Dr. Akhil Mehrotra

Chief, Non Invasive Cardiologist, Pediatric and Adult Cardiology. Prakash Heart Station, D-16 Nirala Nagar, Lucknow, UP India