Abstract
Congenital unilateral intrapericardial pulmonary artery absence is a rare heart anomaly resulting from the involution of the proximal right or left sixth aortic arch. The incidence of the anomaly in general population is 1:150,000-200,000. Distal part of the sixth aortic arch evolves to patent ductus arteriosus, which exists in each case of the anomaly and gives origin to the distal part (hilar artery) of absent pulmonary artery. The anomaly can be either an isolated form or associated with other congenital intracardiac lesions such as tetralogy of Fallot. The latter form accounts for 75% of all anomaly cases. In this report we present a unique method of pulmonary arteries reimplantation technique in a patient with tetralogy of Fallot associated with unilateral left pulmonary artery absence by using extracardiac conduit.
References
- Bouros D., Pare P., Panagou P., Tsintiris K., Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest. 1995; 108: 670–6. DOI: 10.1378/chest.108.3.670
- Devulapally K.K., Bhatt N., Allen J.N. A 36-year-old woman with a history of dextrocardia and dyspnea. Diagnosis: unilateral absence of a pulmonary artery (UAPA) with right hypoplastic lung. Respiration. 2010; 79: 81–4. DOI: 10.1159/000232393
- Sotomora R.G., Edwards J.E. Anatomic identfication of so called absent pulmonary artery. Circulation. 1978; 57: 624–33. DOI: 10.1161/01.CIR.57.3.624
- Welch K., Hanley F., Johnston T., Cailes C., Shah M.J. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann. Thorac. Surg. 2005; 79: 1399–402.
DOI: 10.1016/j.athoracsur.2003.10.037
- Bockeria L.A., Makhachev O.A., Khiriev T.K., Abramyan M.A. Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung. Interactive Cardiovasc. Thorac. Surg. 2011; 12: 509–10. DOI: 10.1510/icvts.2010.250795A
- Fadel E., Wijtenburg E., Michel R., Mazoit J.X., Bernatchez R., Decante B. et al. Regression of the systemic vasculature to the lung after removal of pulmonary artery obstruction. Am. J.
Resp. Crit. Care Med. 2006; 173: 345–9. DOI: 10.1164/rccm.200506-894OC
- Bockeria L.A., Podzolkov V.P., Makhachev O.A., Zelenikin M.A., Alekian B.G., Ilyin V.N. et al. Surgical correction of tetralogy of Fallot with unilateral absence of pulmonary artery. Ann.
Thorac. Surg. 2007; 83: 613–8. DOI: 10.1016/j.athoracsur.2006.08.022
- Toews W.H., Pappas G. Surgical management of absent right pulmonary artery with associated pulmonary hypertension. Chest. 1983; 84: 497–9. DOI: 10.1378/chest.84.4.497
- Moreno-Cabral R.J., McNamara J.J., Reddy V.J., Caldwell P. Unilateral absent pulmonary artery: surgical repair with a new technique. J. Thorac. Cardiovasc. Surg. 1991; 102: 463–5.
DOI: 10.1016/s0022-5223(19)36540-7
- Hiramatsu T., Komori S., Okamura Y., Suzuki H., Takeuchi T., Shibuta S. Surgical correction of isolated unilateral absence of right pulmonary artery. Heart Vessels. 2010; 25: 353–5. DOI: 10.1007/s00380-009-1207-5
- El-Hattab A.Y., Calcaterra D., Parekh K.R., Rossi N.P., Davis J.E., Turek D.W. Semiautologous repair for congenital discontinuous right pulmonary artery. Ann. Thorac. Surg. 2013; 96: 2230–1. DOI: 10.1016/j.athoracsur.2013.02.057
- Kim G.B., Ban J.E., Bae E.J., Noh C.I., Kim W.H., Lee J.R., Kim Y.J. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. The J. Thorac. Cardiovasc. Surg. 2011; 1: 171–8. DOI: 10.1016/j.jtcvs.2009.09.072
- Bilal M.S., Yildirim O., Avsar M., Ozyuksel A. Repair of unilateral absence of right pulmonary artery with contralateral pulmonary artery autograft interposition in an infant. Ann. Thorac. Surg. 2015; 99: 1467–9. DOI: 10.1016/j.athoracsur.2014.12.036
- Giamberti A., Huseinov F., Yuzbashova M., Musayev S., Piazza L., Mahmudov R. Tetralogy of Fallot with left pulmonary artery discontinuity and totally anomalous pulmonary venous drainage to the azygos vein. World J. Pediatr. Congenit. Heart Surg. 2016; 7 (4): 506–8. DOI: 10.1177/2150135115603333
About the authors
- Konstantin V. Shatalov, Dr. Med. Sc., Professor, Head of Department, ORCID
- Konstantine M. Dzhidzhikhiya, Cand. Med. Sc, Cardiac Surgeon, ORCID
- Magomed A. Abdurazakov, Postgraduate, ORCID
