Tetralogy of Fallot with unilateral absence of pulmonary artery (PA) is a rare congenital anomaly that has been reported in isolated case reports and small case series. There is no well-defined treatment algorithm for these patients, and repair has been associated with high mortality, although survival is improving in the more recent era. Recent reports suggest strict case selection criteria based on PA dimensions and size of the left ventricle.
FraentzelO.Ein fall von abnormer communication der aorta mit der arteria pulmonalis. VirchowsArch Pathol Anat. 1868;43(3):420–426.
2.
KuceraVFiserSTumaSHucinB. Unilateral absence of pulmonary artery: a report on 19 selected clinical cases. Thorac Cardiovasc Surg. 1982;30(3):152–158.
3.
PoolPEVogelJHBlountSGJr. Congenital unilateral absence of a pulmonary artery. Am J Cardiol. 1962;10:706-732.
4.
XiaG. Tetralogy of Fallot with absence of the left pulmonary artery [in Chinese]. Zhonghua Yi Xue Za Zhi. 1990;70(3):128–129.
5.
LiuJSWangZW. Correction of tetralogy of Fallot in patients with a single pulmonary artery. Zhonghua Wai Ke Za Zhi. 1994;32(3):172–174.
6.
ZhangGCWangZWZhangRFZhuHYYiDH. Surgical repair of patients with tetralogy of Fallot and unilateral absence of pulmonary artery. Ann Thorac Surg. 1997;64(4):1150–1153.
7.
EmanuelRWPattinsonJN. Absence of the left pulmonary artery in Fallot’s tetralogy. Br Heart J. 1956;18(3):289–295.
8.
BeyerJBrunnerLKlinnerW. Surgical treatment for tetralogy of Fallot with unilateral absence of a pulmonary artery. Thoraxchir Vask Chir. 1976;24(5):431–437.
9.
GambaAVillaniMTiraboschiR. Surgical treatment of tetralogy of Fallot with a single pulmonary artery[in Italian]. G Ital Cardiol. 1984;14(7):499–504.
10.
DonahooJCBrawleyRKHallerAJElkinsRCBenderHWGottVL. Correction of tetralogy of Fallot in patients with one pulmonary artery in continuity with the right ventricular outflow tract. Surgery. 1973;74(6):887–893.
11.
LabordeFde RiberollesCEscandeGLinoRHazanENeveuxJY. Tetralogy of fallot with congenital absence of a pulmonary artery. Apropos of 4 cases[in French]. Sem Hop. 1983;59(44):3043–3046.
12.
BockeriaLAPodzolkovVPMakhachevOA. Surgical correction of tetralogy of Fallot with unilateral absence of pulmonary artery. Ann Thorac Surg. 2007;83(2):613–618.
13.
WaldmanJDKarpRBGittenberger-de GrootACAgarwalaBGlagovS. Spontaneous acquisition of discontinuous pulmonary arteries. Ann Thorac Surg.1996;62(1):161–168.
14.
SherrickDWKincaidOWDushaneJW. Agenesis of a main branch of the pulmonary artery. Am J Roentgenol Radium Ther Nucl Med. 1962;87:917–928.
15.
CucciCEDoyleEFLewisEWJr. Absence of a primary division of the pulmonary trunk: an ontogenetic theory. Circulation. 1964;29:124–131.
16.
Grosse-WortmannLAl-OtayAGooHW. Anatomical and functional evaluation of pulmonary veins in children by magnetic resonance imaging. J Am Coll Cardiol. 2007;49(9):993–1002.
17.
HonjoOAl-RadiOOMacdonaldC. The functional intraoperative pulmonary blood flow study is a more sensitive predictor than preoperative anatomy for right ventricular pressure and physiologic tolerance of ventricular septal defect closure after complete unifocalization in patients with pulmonary atresia,ventricular septal defect and major aortopulmonary collaterals. Circulation. 2009;120(suppl 10):S46–S52.
18.
BockeriaLAPodzolkovVPMakhachevOA, et al. Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery. Interact Cardiovasc Thorac Surg. 2013;16(3):286–292.
19.
WerberJRamiloJLLondonRHarrisVJ. Unilateral absence of pulmonary artery. Chest. 1983;84(6):729–732.
20.
DeslauriersJUgaldePMiroS. Adjustments in cardiorespiratory function after pneumonectomy: results of the pneumonectomy project. J Thorac Cardiovasc Surg. 2011;141(1):7–15.
21.
FerenczC.The pulmonary vascular bed in tetralogy of Fallot. I. Changes associated with pulmonic stenosis. Bull Johns Hopkins Hosp. 1960;106:81–99.
22.
WilliamsGDDunganWTCampbellGS. Surgical treatment of tetralogy of Fallot with unilateral absence of a pulmonary artery. Ann Thorac Surg. 1972;14(5):483–493.
23.
HislopAReidL. Structural changes in pulmonary arteries and veins in Tetralogy of Fallot. Br Heart J. 1973;35(11):1178–1183.
24.
JohnsonRJHaworthSG. Pulmonary vascular and alveolar development in tetralogy of Fallot: a recommendation for early correction. Thorax. 1982;37(12):893–901.
25.
MistrotJJBernhardWFRosenthalACastañedaA. Tetralogy of Fallot with a single pulmonary artery: operative repair. Ann Thorac Surg. 1977;23(3):249–253.
26.
TurinettoBColìGDonatiAGalliRMikusPPierangeliA. Absent right pulmonary artery complicating tetralogy of Fallot. JCardiovasc Surg (Torino). 1975;16(3):322–326.
27.
GoldsmithMFarinaMAShaherRM. Tetralogy of Fallot with atresia of the left pulmonary artery: surgical repair using a homograft aortic valve. J Thorac Cardiovasc Surg. 1975;69(3):458–466.
SakamotoTKurosawaHShin'okaTAokiMIsomatsuY. The influence of pH strategy on cerebral and collateral circulation during hypothermic cardiopulmonary bypass in cyanotic patients with heart disease: results of a randomized trial and real-time monitoring. J Thorac Cardiovasc Surg. 2004;127(1):12–19.
NewburgerJWJonasRASoulJ. Randomized trial of hematocrit 25% versus 35% during hypothermic cardiopulmonary bypass in infant heart surgery. J Thorac Cardiovasc Surg. 2008;135
(2):347–354.
32.
BabuBBhatSPrabuswamyHP. Controlling oxygenation during initiation of cardiopulmonary bypass: can it improve immediate postoperative outcomes in cyanotic children undergoing cardiac surgery? A prospective randomized study. World J Pediatr Congenit Heart Surg. 2012;3(3):310–316.
33.
ImanakaKShimizuSMatsumotoJHashizumeKTsuchiyaKTakemuraT. Unilateral absence of pulmonary artery and ventricular septal defect in an infant. Ann Thorac Surg. 1998;66(1):251–252.
34.
ReñéMSansJDominguezJSanchoCValldeperasJ. Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol. 1995;18(4):251–254.
35.
MaxeyTSFernandezLGReeceTBKeelingWBKronILLaubachVE. Endothelial nitric oxide synthase is essential for postpneumonectomy compensatory vasodilation. Ann Thorac Surg. 2006;81(4):1234–1238.
36.
BrownLMRannelsSRRannelsDE. Implications of post-pneumonectomy compensatory lung growth in pulmonary physiology and disease. Respir Res. 2001;2(6):340–347.
37.
KadirISThekudanJDheodarAJonesMTCarrollKB. Congenital unilateral pulmonary artery agenesis and aspergilloma. Ann Thorac Surg. 2002;74(6):2169–2171.
38.
YamakiSSuzukiYIshizawaEKagawaYHoriuchiTSatoT.Isolated aortic origin of right pulmonary artery. Report of a case with special reference to pulmonary vascular disease in the left and right lung. Chest. 1983;83(3):575–578.
39.
ReddyVMPetrossianEMc ElhinneyDBMoorePTeitelDFHanleyFL. One-stage complete unifocalisation in infants: when should be the ventricular septal defect be closed?J Thorac Cardiovasc Surg. 1997;113(5):858–868.
40.
ShakibiJGRastanHNazarianIPaydarMAryanpourISiassiB.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.
41.
ToewsWHPappasG. Surgical management of absent right pulmonary artery with associated pulmonary hypertension. Chest. 1983;84(4):497–499.
42.
Moreno-CabralRJMcNamaraJJReddyVJCaldwellP. Unilateral absent pulmonary artery: surgical repair with a new technique. J Thorac Cardiovasc Surg. 1991;102(3):463–465.
43.
KosakaYKurosawaHHoshinoSShin'okaTIsomatsuYTsujiY. Surgery for unilateral absence of pulmonary artery using autologous tissue. Ann Thorac Surg. 2003;76(4):1281–1283.
44.
KimGBBanJEBaeEJ. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg. 2011;141(1):171–178.
45.
MurphyDNWinlawDSCooperSGNunnGR. Successful early surgical recruitment of the congenitally disconnected pulmonary artery. Ann Thorac Surg. 2004;77(1):29–35.
46.
PriftiECruceanABonacchiMBonacchibM. Postoperative outcome in patients with anomalous origin of one pulmonary artery branch from the aorta. Eur J Cardiothorac Surg. 2003;24(1):21–27.
47.
TalwarSGuptaAChoudharySKAiranB. Absent left pulmonary artery and double aortic arch in tetralogy of Fallot: reconstruction using homograft saphenous vein or iliac artery. Interact Cardiovasc Thorac Surg. 2009;8(2):277–279.
48.
MalhotraSPHanleyFL. Surgical management of pulmonaryatresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;12(1):145–151.
49.
KouchoukosNTBlackstoneEHDotyDBHanleyFLKarpRB. Origin of right or left pulmonary artery from ascending aorta. In: KouchoukosNTBlackstoneEHDotyDBHanleyFLKarpRB. Kirklin/Baratt-Boyes Cardiac Surgery. 3rd ed. Philadelphia, PA: Elsevier Science; 2003:1233.
