Right pulmonary artery agenesis with transient hypothyroidism in a newborn


  • Preeti Gupta Department of of Pediatrics and Neonatology, Shri Mata Vaishno Devi Narayana Hospital, Jammu, Jammu and Kashmir, India
  • Amitabha Chattopadhyay Department of Pediatric Cardiology, Narayana Hospital, Howrah, West Bengal, India
  • Geetanjali Gupta Department of Radiology, Shri Mata Vaishno Devi Narayana Hospital, Jammu, Jammu and Kashmir, India
  • Jayita Das Department of Pediatric Cardiology, Narayana Hospital, Howrah, West Bengal, India




Newborn, Pulmonary artery agenesis, Transient hypothyroidism


Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly that can present as an isolated lesion or may be associated with other Congenital heart diseases. It is usually detected in infancy or incidentally found on X ray chest in adulthood. The diagnosis of UAPA has rarely been made in a newborn. Only one case has been reported in newborn period from Belgium by F. Marchau who reported the rare occurrence of this anomaly in a 2-day old male baby. We report the rare occurrence of UAPA in a newborn female from India who presented with severe respiratory distress at birth with high cord blood TSH levels. Our case also reflects the importance of a high index of suspicion along with the value of X-ray chest and echocardiogram in the neonatal period in detection of this rare anomaly.


Fraentzel O. Ein Fall von abnormer communication der Aortamit der Arteria pulmonalis. Virchow’s Arch Path Anat. 1868;43:4201.

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

Chaudhry A, Rathore M, Banavaliker JN. Isolated UAPA with RUL Agenesis and RLL Bronchiectasis. Indian J Chest Dis Allied Sci. 2014;56:49-52.

Apostolopoulou SC, Kelekis N, Brountzos EN, Rammos S, Kelekis DA ‘Absent’ pulmonary artery in one adult andfive pediatric patients: imaging, embryology and therapeuticimplications. Am J Roentgen. 2002;179:1253-60.

Pool PE, Vogerl JHK, Blount SG Congenital unilateral absence of a pulmonary artery. The importance of flow in pul-monary hypertension. Am J Cardiol. 1962;10:706-32.

Presbitero P, Bull C, Haworth SG, de Leval MR. Absentor occult pulmonary artery. Br Heart J. 1984; 52:178-85.

Shakibi JG, Rastan H, Naziran I Isolated unilateral ab-sence of the pulmonary artery: review of the world literature and guidelines for surgical repair. Jpn Heart. 1978;19:439-51.

Ten Hakel DJ, Blom N, Ottenkamp J Isolated unilateral absence of a pulmonary artery; a case report and review of the literature. Chest. 2002;122:1471-7.

Marchau F, Boshoff D, Gewillig M, Mertens L. Excluded right pulmonary artery in a neonate.European Journal of Pediatrics. 2004;163(4-5):274-6.

Talwar S, Gupta A, Choudhary SK, Airan B. 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;8:277-9.

Singhi AK, Francis E, Kumar RK. Isolated absence of right pulmonary artery. Ann Pediatr Cardiol. 2010;3:119-22.

Sharma S, Kumar S, Yaduvanshi D, Chauhan D. Isolated unilateral pulmonary agenesis. Indian Pediatr. 2005;42:170-2.

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;4:32-7.

Cucci CE., Doyle EF, Lewis EW. Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation. 1964;29:124-31.

Bahler RC, Carson P, Traks E, Levene A, Gillespie D. Absent pulmonary artery. Problems in diagnosis and management. Am J Med. 1969;46:64-71.






Case Reports