Unilateral Absence of a Pulmonary Artery (UAPA): Case Report and Literature Review

  • Hosseinali Jelvehmoghadam Anesthesiology Research Center, Loghman Hakim Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  • Maziar Mobasher Department of Critical Care, Shahid Madani Medical Center, Alborz University of Medical Sciences, Karaj, Iran.
  • Sevak Hatamian Alborz University of Medical Sciences
  • Hooman Bahrami Motlagh Beheshti University of Medical Sciences
  • Masoum Khoshfetrat Zahedan University of Medical Sciences
  • Mohammadreza Hajiesmaeili Shahid Beheshti University of Medical Sciences
  • Abdorahim Ghasemi Shahid Beheshti University of Medical Sciences
  • Elham Samami Shahid Beheshti University of Medical Sciences
  • Farshid Rahimibashar Hamadan University of Medical Sciences
  • Shahram Saifi Babol University of Medical Sciences
  • Ali Amirsavadkouhi Department of Anesthesiology and Critical Care Medicine, Mehrad Hospital, Tehran, Iran
  • Mehdi Ghahremani Shahid Beheshti University of Medical Sciences
  • Seyedmohamadmasoud Moosavinasab Shahid Beheshti University of Medical Sciences
  • Hosna Forooghirad Department of Biology, Payame Noor University, Tehran, Iran
  • Mohammadali Khabirikhatiri Shahid Beheshti University of Medical Sciences
  • Alireza Sedaghat Mashhad University of Medical Sciences
Keywords: case report, respiratory symptoms, unilateral pulmonary artery agenesis

Abstract

One of the more infrequent congenital diseases with a wide spectrum of symptoms and more complications of the right side of pulmonary artery (63% according to Ten Harkel) is unilateral pulmonary artery agenesis (UPAA). The clinical presentation is variable, and many patients can be asymptomatic for many years and even throughout their lives. Although the disease more frequently appears in childhood with moderate to severe respiratory symptoms, some patients continue living with the disease up to adulthood. The abnormality is sometimes concurrent with cardiac anomalies. Patients with UAPA may be asymptomatic or may present with recurrent pulmonary infections. In a report, a 25-year old woman who had inductional termination of pregnancy at 35 week last menstrual period (LMP) in a rural hospital due to preeclampsia reported as the UAPA patient.

References

Pac A, Ozme S, Celiker A, Ozkutlu S. Absent pulmonary valve syndrome with agenesis of the left pulmonary artery. Turk J Pediatr. 1994; 36(3):249-53.

Vázquez SJ, Pinto TR, Testelli M, Herrera E. [Unilateral agenesis of the main branch of the pulmonary artery]. Arch Inst Cardiol Mex. 1983; 53(4):321-6.

Moosavi SAJ, Iranpour A. Unilateral Pulmonary Artery Agenesis in an Adult Patient with Cough and Hemoptysis: A Case Report. Tanaffos. 2014; 13(4):58-60.

Kilickiran Avci B, Karadag B, Tuzun H, Ongen Z. Unilateral absence of the left pulmonary artery with patent ductus arteriosus and interrupted inferior vena cava. Turk Kardiyol Dern Ars. 2014; 42(4):399-402

Takahashi T, Endo H, Ito T, Takei T, Yagi K. Isolated unilateral absence of the left pulmonary artery: a case report. Ann Vasc Dis. 2014; 7(2): 178–182.

Muthusami P, Ananthakrishnan R, Elangovan S. Incidentally detected unilateral pulmonary artery agenesis with pulmonary hypoplasia in a 67 year old woman. J Radiol Case Rep. 2010; 4(11): 32–37.

Koga H, Hidaka T, Miyako K, Suga N, Takahashi N. Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. Pediatr Cardiol. 2010 Nov;31(8):1186-90.

Reading DW, Oza U. Unilateral absence of a pulmonary artery: a rare disorder with variable presentation. Proc (Bayl Univ Med Cent). 2012; 25(2):115-8.

Griffin N, Mansfield L, Redmond K, Dusmet M, Goldstraw P, Mittal T, et al. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases. Clin Radiol. 2007; 62(3):238-44.

Ozcan A, Yilmaz I, Kahveci S, Kose M. A rare cause of recurrent respiratory tract infection: isolated absence of the right pulmonary artery. Turk J Pediatr. 2014; 56(2):186-8.

Kadi H, Kurtoglu N, Karadag B. Congenital absence of the right pulmonary artery with coronary collaterals supplying the affected lung: effect on coronary perfusion. Cardiology. 2007; 108(4):314-6.

Kayemba-Kay's S, Couvrat-Carcauzon V, Goua V, Podevin G, Marteau M, Sapin E, et al. Unilateral pulmonary agenesis: a report of four cases, two diagnosed antenatally and literature review. Pediatric pulmonology. Pediatr Pulmonol. 2014; 49(3):E96-102.

Singh U, Jhim D, Kumar S, Mittal V, Singh N, Gour H, et al. Unilateral agenesis of the lung: a rare entity. Am J Case Rep. 2015; 16:69-72.

Gunbey HP, Gunbey E, Sayit AT, Bulut T. Unilateral right pulmonary agenesis in adulthood. J Clin Diagn Res. 2014; 8(6): RD01–RD02.

Osborne J, Masel J, McCredie J. A spectrum of skeletal anomalies associated with pulmonary agenesis: possible neural crest injuries. Pediatr Radiol. 1989; 19(6-7):425-32.

Cunningham ML, Mann N. Pulmonary agenesis: a predictor of ipsilateral malformations. Am J Med Genet. 1997; 70(4):391-8.

Knowles S, Thomas RM, Lindenbaum RH, Keeling JW, Winter RM. Pulmonary agenesis as part of the VACTERL sequence. Arch Dis Child. 1988; 63(7 Spec No):723-6.

Kaya IS, Dilmen U. Agenesis of the lung. Eur Respir J. 1989; 2(7):690-2.

Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am. 1994; 41(3):453-72.

Schneider P, Schwalbe E. Die Morphologie der Missbildungen Des Menschen Under Thiere, 1912; Vol. 3, Part 2. Jena: Gustav Fischar.817-22.

Sbokos CG, McMillan I. Agenesis of the lung. British journal of diseases of the chest. 1977; 71:183-97.

Furuya T, Iida R, Konishi J, Kato J, Suzuki T. [Anesthesia for Cesarean section in a patient with isolated unilateral absence of a pulmonary artery]. Rev Bras Anestesiol. 2015; pii: S0034-7094(15)00018-5.

Fahimi F, Ghafari S, Jamaati H, Banaiasadi S, Tabarsi P, Najafi A, et al. Continuous versus intermittent administration of piperacillin-tazobactum in intensive care unit patients with ventilator-associated pneumonia. Indian J Crit Care Med. 2012; 16(3): 141-147.

Furuya T, Iida R, Konishi J, Kato J, Suzuki T. [Anesthesia for Cesarean section in a patient with isolated unilateral absence of a pulmonary artery]. Rev Bras Anestesiol. 2015; pii: S0034-7094(15)00018-5

Published
2016-11-22
How to Cite
1.
Jelvehmoghadam H, Mobasher M, Hatamian S, Bahrami Motlagh H, Khoshfetrat M, Hajiesmaeili M, Ghasemi A, Samami E, Rahimibashar F, Saifi S, Amirsavadkouhi A, Ghahremani M, Moosavinasab S, Forooghirad H, Khabirikhatiri M, Sedaghat A. Unilateral Absence of a Pulmonary Artery (UAPA): Case Report and Literature Review. AACC. 2(4):258-62.
Section
Review Article(s)