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Unilateral interruption of pulmonary artery with pulmonary hypertension: a case for heart lung transplantation?

  • Roopesh Singhal (a1)


Unilateral interruption of pulmonary artery is a rare congenital anomaly which is usually associated with other congenital heart disease. Even more rarely it may occur in isolation. Most of the cases are incidentally detected in adulthood. Some cases develop pulmonary hypertension for yet unknown reasons; such cases usually present in infancy with right heart failure. Surgical correction in such cases is associated with adverse outcomes. Heart lung transplantation should be considered in such patients. We report a 3-year-old boy with interruption of right pulmonary artery with severe pulmonary hypertension and right heart failure who was considered for heart lung transplantation.


Corresponding author

Author for correspondence: R. Singhal, Department of Cardiology, U. N. Mehta Institute of Cardiology and Research Center, Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India. Tel: +91-9099016535; Fax: +91-79-22682092; E-mail:


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1. Fraentzel, O. Ein Fall von abnormer Communication der Aorta mit der Arteria pulmonalis. Arch Für Pathol Anat Physiol Für Klin Med 1868; 43: 420426.
2. 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: 439451.
3. Turner, D, Vincent, J, Epstein, M. Isolated right pulmonary artery discontinuity. Images Paediatr Cardiol 2000; 2: 2430.
4. Pfefferkorn, JR, Löser, H, Pech, G, Toussaint, R, Hilgenberg, F. Absent pulmonary artery. A hint to its embryogenesis. Pediatr Cardiol 1982; 3: 283286.
5. Cucci, CE, Doyle, EF, Lewis, EW. Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 1964; 29: 124131.
6. Ten Harkel, ADJ, Blom, NA, Ottenkamp, J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002; 122: 14711477.
7. Pool, PE, Vogel, JH, Blount, SG. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 1962; 10: 706732.
8. Haworth, SG, de Leval, M, Macartney, FJ. Hypoperfusion and hyperperfusion in the immature lung. Pulmonary arterial development following ligation of the left pulmonary artery in the newborn pig. J Thorac Cardiovasc Surg 1981; 82: 281292.
9. Malcon, MC, Malcon, CM, Cavada, MN, Caruso, PEM, Real, LF. Unilateral pulmonary agenesis. J Bras Pneumol 2012; 38: 526529.
10. Singh, U, Jhim, D, Kumar, S, et al. Unilateral agenesis of the lung: a rare entity. Am J Case Rep 2015; 16: 6972.
11. DeRuiter, MC, Gittenberger-de Groot, AC, Poelmann, RE, VanIperen, L, Mentink, MM. Development of the pharyngeal arch system related to the pulmonary and bronchial vessels in the avian embryo. With a concept on systemic-pulmonary collateral artery formation. Circulation 1993; 87: 13061319.
12. Franke, U, Wiebe, K, Harringer, W, et al. Ten years experience with lung and heart-lung transplantation in primary and secondary pulmonary hypertension. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg 2000; 18: 447452.
13. Goerler, H, Simon, A, Gohrbandt, B, et al. Heart-lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg 2007; 32: 926931.
14. Goldstein, BS, Sweet, SC, Mao, J, Huddleston, CB, Grady, RM. Lung transplantation in children with idiopathic pulmonary arterial hypertension: an 18-year experience. J Heart Lung Transplant 2011; 30: 11481152.


Unilateral interruption of pulmonary artery with pulmonary hypertension: a case for heart lung transplantation?

  • Roopesh Singhal (a1)


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