A Case of Isolated Congenital Absence of Right Pulmonary Artery

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18∼20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

keywords: Pulmonary artery, Agenesis, Hemoptysis

Reference

1. Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002;122:1471-7.

2. Kim H, Kang JH, Wee JS, Kim YH, Chung WS, Nam S. Right pulmonary artery agenesis: a case report. Korean J Thorac Cardiovasc Surg 2007;40:52-5.

3. Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995;108:670-6.

4. Shakibi JG, Rastan H, Nazarian I, Paydar M, Aryanpour I, Siassi B. Isolated unilateral absence of the pulmonary artery: review of the world literature and guidelines for surgical repair. Jpn Heart J 1978;19:439-51.

5. Krall WR, Ploy-Song-Sang Y. Unilateral pulmonary artery aplasia presenting with chest pain and pleural effusion. South Med J 1980;73:233-6.

6. Choi YW, Seo HS, Hahm CK, Choi CS, Bae OK, Jeon SC. Unilateral absence of a pulmonary artery: report of 3 cases. J Korean Radiol Soc 1994;31:87-90.

7. Simsek PO, Ozcelik U, Celiker A, Yalcin E, Cobanoglu N, Pekcan S, et al. A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis. Eur J Pediatr [serial online] 2008 May [cited 2008 May]. Avalable from: http://www.springerlink.com/content/d7206224 32r7742m/

8. Hentrich F, Stoermer J, Wiesemann G. Unilateral proximal aplasia of the pulmonary artery: studies on the clinical significance and embryologic interpretation. Klin Padiatr 1984;196:311-4.

9. Thompson JW, Nguyen CD, Lazar RH, Stocks RM, Schoumacher RA, Hamdan F, et al. Evaluation and management of hemoptysis in infants and children: a report of nine cases. Ann Otol Rhinol Laryngol 1996;105:516-20.

10.

10. Herraiz Sarachaga I, Pérez González W, Vergara Rodríguez F, Alonso Lastra MJ, Acerete Guillén F, Bermúdez- Cañete Fernández R, et al. Unilateral agenesis of the pulmonary artery: experience with 4 cases. An Esp Pediatr 1993;38:139-44.

11.

11. Rene M, Sans J, Dominguez J, Sancho C, Valldeperas J. Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol 1995;18:251-4.

12.

12. Campbell KR, Krasuski R, Wang A, O'Laughlin MP, Harrison JK. Congenital agenesis of the right pulmonary artery. Catheter Cardiovasc Interv 2000;51:460-3.

13.

13. Wyman SM. Congenital absence of pulmonary artery: its demonstration by roentgenography. Radiology 1954;62:321-8.

14.

14. Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999;99:1197-208.

15.

15. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-65.

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Article Contents

Vol.65 No.5

A Case of Isolated Congenital Absence of Right Pulmonary Artery

Abstract

Reference

Tuberculosis & Respiratory Diseases