Unilateral absence of a pulmonary artery (UAPA) is a rare congenital condition and coexists with cardiovascular abnormalities such as cyanotic heart disease or cardiac septal defects; however, it can also occur in isolation. Patients with isolated UAPA can progress without symptoms into late adulthood but usually present with dyspnea, chest pain, hemoptysis, or recurrent infections. There are no reported guidelines on the treatment of UAPA. The plan of treatment is based on the presenting symptoms, pulmonary artery (PA) anatomy, aortopulmonary collaterals, associated cardiovascular anomalies, and pulmonary hypertension.
The objectives of unifocalization are to consolidate the multiple sources of pulmonary blood flow, to unite the multiple pulmonary collateral arteries, and to remodel a new central artery that can be created with a prosthetic conduit. Usually, a modified Blalock-Taussig shunt, a surgical systemic pulmonary shunt, is used to perfuse the pulmonary arteries. The perioperative course and management of a case of UAPA for unifocalization have rarely been reported in the literature. Here, we discuss the perioperative management of a case of unilateral absence of the right PA (RPA) for unifocalization.
Bouros D, Pare P, Panagou P, et al. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995;108(3):670–676. DOI: 10.1378/chest.108.3.670
Presbitero P, Bull C, Haworth SG, et al. Absent or occult pulmonary artery. Br Heart J 1984;52(2):178–185. DOI: 10.1136/hrt.52.2.178
Pfefferkorn JR, Loser H, Pech G, et al. Absent pulmonary artery. A hint to its embryogenesis. Pediatr Cardiol 1982;3(4):283–286. DOI: 10.1007/BF02427028
Frentzel O. Ein Fall von anormer communicationder aorta mit der arteria Pulmonalis. Respir Med Case Rep 2017;22:238–242. DOI: 10.1016/j.rmcr.2017.09.004. PMID: 28951831
Welch K, Hanley F, Johnston T, et al. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg 2005;79(4):1399–1402. DOI: 10.1016/j.athoracsur.2003.10.037
Kruzliak P, Syamasundar RP, Novak M, et al. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis 2013;106(8–9):448–454. DOI: 10.1016/j.acvd.2013.05.004
Kucera V, Fiser B, Tůma S, et al. Unilateral absence of pulmonary artery: a report on 19 selected clinical cases. Thorac Cardiovasc Surg 1982; 30(3):152–158. DOI: 10.1055/s-2007-1022234
Shakibi JG, Rastan H, Nazarian I, et al. Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair. Jpn Heart J 1978;19(3):439–451. DOI: 10.1536/ihj.19.439
Hayashi Y, Takaki O, Uchida O, et al. Anesthetic management of patients undergoing bilateral unifocalization. Anesth Analg 1993;76(4):755–759.
Cook DR, Davis PJ. Pediatric anesthesia pharmacology. In: Lake CL, ed. Pediatric cardiac anesthesia. 1st ed. East Norwalk. Appleton & Lange 1988:121–154.
Sawatari K, Imai Y, Kurosawa H, et al. [Staged operation for pulmonary atresia with a ventricular septal defect and major aorto-pulmonary collateral arteries: a case report]. J Thorac Cardiovasc Surg 1989;98(4):738–750. DOI: 10.1016/S0022-5223(19)34296-5