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 Table of Contents  
Year : 2017  |  Volume : 5  |  Issue : 1  |  Page : 35-38

A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch

Department of Respiratory, Critical Care and Sleep Medicine, Indraprastha Apollo Hospitals, New Delhi, India

Date of Web Publication29-Dec-2016

Correspondence Address:
Dr. Trilok Chand
Department of Respiratory, Critical Care and Sleep Medicine, Indraprastha Apollo Hospitals, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-8775.196654

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The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

Keywords: Absence of pulmonary artery, aortic arch, hemoptysis, hypoplasia of lung, recurrent pneumothorax

How to cite this article:
Chand T, Bansal A, Mansuriya J. A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch. J Assoc Chest Physicians 2017;5:35-8

How to cite this URL:
Chand T, Bansal A, Mansuriya J. A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch. J Assoc Chest Physicians [serial online] 2017 [cited 2022 Jun 24];5:35-8. Available from: https://www.jacpjournal.org/text.asp?2017/5/1/35/196654

  Introduction Top

The congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. It is thought to be the result of a failure in the connection of the sixth aortic arch with the pulmonary trunk during embryologic development.[1] The absence of a left pulmonary artery is less frequent and occurs in one-third of all unilateral absence of a pulmonary artery.[2] The UAPA most commonly occurs with cardiovascular malformations such as tetralogy of fallot or cardiac septal defects, but it can also occur in isolated form.[3],[4] In two-thirds of cases, isolated UAPA occurs in the right lung,[2] probably because of UAPA usually occurs on the opposite side of aortic arch due to embryologic relationships,[5] but in our case, it was on the left side, which have been less frequently reported.[6] Our case was presented with a history of recurrent pneumothorax and hemoptysis and on thorough investigations, he found to has an absence of left pulmonary artery and hypoplasia of the left lung with the right side aortic arch.

  Case Report Top

Eighteen-year-old male with a history of recurrent cough and cold symptoms since childhood, was presented with left-sided chest pain and shortness of breath for 3 days. On examination, breath sounds and air entry decreased on the left side. His chest X-ray (CXR) confirmed left side pneumothorax [Figure 1], and Intercostal drainage (ICD) was put on the same side. Patient’s contrast enhanced computed tomography (CT) chest was ordered which showed volume loss in the left hemithorax with ipsilateral mediastinal shifting and minimal left pleural thickening and a bulla in the left lung [Figure 2]. The right lung was hyperinflated and right-sided aortic arch seen in mirror image branching pattern [Figure 3]. After the ICD removal, patient was discharged in stable condition.
Figure 1: Chest X-rays showing the left side pneumothorax and hyperinflated right lung and ICD in the left lung

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Figure 2: Computed tomography scan section of upper lobes show volume loss of the left lung and bulla in left lung

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Figure 3: Computed tomography scan showing cystic changes in the left lower lobe (left image) and single right pulmonary artery (right image)

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Patient’s past history was suggestive of a similar event of pneumothorax on the left side, 3 years back and was managed with ICD in an another hospital.

The patient was again presented in our hospital after 1 month with the complaint of 400–500 cc single episode of hemoptysis. He was managed conservatively, and all routine investigations, including coagulation profile, antinuclear antibody, antineutrophil cytoplasmic antibodies, and alpha antitrypsin were sent which showed normal or negative results. His bronchoscopy was done which showed blood on both sides and oozing continuously from the left lower lobe. After stabilization, his pulmonary function test was done which showed mild restrictive defect with small airway defect with reversibility in the smaller airways. Patient’s CT angiography was done which showed the absence of the left pulmonary artery and blood supply of the left lung from systemic circulation and venous drainage to pulmonary vein with associated congenital anomaly of right aortic arch [Figure 4]. The ventilation-perfusion scan showed a mismatch on the left side. The patient was discharged on request, but he again came into hospital with cough and sputum, hemoptysis, and left side pneumothorax. His routine investigations were carried out, and ICD was secured on the left side of chest. All blood tests found within normal range, two-dimensional (2D) echo was also done which revealed normal findings except right aortic arch and absent left pulmonary artery.
Figure 4: Images showing the absence of the left pulmonary artery (left image) and blood supply of the left lung from systemic circulation (right image)

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The patient was evaluated further and reviewed all previous reports. Opinion was sought from thoracic surgeon and interventional radiologist. The final diagnosis of congenital absence of the left pulmonary artery with hypoplastic left lung with associated congenital anomaly of a right-sided aortic arch and no associated intracardiac defect was made. The patient was advised for surgical management, but he refused for that and he was discharged after stabilization and left side pleurodesis.

  Discussion Top

In 1868, UAPA was first described as a rare developmental anomaly.[7] In 2011, Bockeria et al. found 352 cases of UAPA in the world literature.[8] The estimated prevalence of single UAPA is 1/200,000 patients.[3] There is no predilection for the right or left side, although the condition is somewhat more common on the right. However, left-sided agenesis seems to be more frequently associated with cardiac abnormalities.[9]

We herein presenting a case with congenital absence of the left pulmonary artery and hypoplasia of the left lung with right-sided aortic arch detected incidentally after episodes of recurrent pneumothorax and hemoptysis. The exact pathogenesis or developmental cause of UAPA is still not confirmed. However, Pfefferkorn et al. stated that altered development of a sixth aortic arch segment is thought to result in a ductal origin to a pulmonary artery that leads to the proximal interruption of that vessel when the ductal tissue regresses at the time of birth.[5] The distal intrapulmonary branches of the involved artery usually found normal, and they supplied by collateral vessels from bronchial, intercostals, internal mammary, subdiaphragmatic, subclavian, or coronary arteries.[8],[10] Some suggested that chromosomal origin, Vitamin A deficiency, intrauterine infections, and environmental factors are responsible for the etiology of congenital lung malformations.

Pulmonary hypoplasia is usually associated with conditions which causing fetal lung compressions such as diaphragmatic defects, excess pleural fluid, intrathoracic tumors or cysts, oligohydramnios, and chronic elevation of hemidiaphragm.[11]

These patients can be present with congestive heart failure or pulmonary hypertension in infancy[2],[12] or combination of symptoms in older, such as recurrent chest infections, chest pain, pleural effusion and dyspnea or exercise intolerance, hemoptysis, and high-altitude pulmonary edema.[2]

Our case was presented with recurrent pneumothorax and hemoptysis. His 2D echo revealed no pulmonary hypertension, but associated cardiac anomaly was present. Patient’s hypoplastic lung with bulla may be responsible for recurrent pneumothorax, and recurrent hemoptysis is explained by large collateral circulations of the left lung under high venous systemic pressures.

Diagnosing UAPA based on chest radiographic findings, which classically shows asymmetric lung fields, ipsilateral small hemithorax with hyperlucent lung fields.[3],[13] Moreover, mediastinum shifting toward the same side with decreased or absent hilar vasculature on the affected side. Other radiographic findings include elevated ipsilateral diaphragm, pulmonary pseudofibrosis of lung apices,[3] plethoric and hyperinflated contralateral lung. The confirm diagnosis made by CT angiography or magnetic resonance imaging, which shows an absent pulmonary artery, terminating within 1 cm of its expected origin from the main pulmonary artery.[3] Transthoracic echocardiogram can also help to diagnose UAPA and coexisting cardiac abnormalities. Pulmonary artery angiography is the gold standard for the diagnosis of UAPA and is usually considered before surgery in a patient who has developed hemoptysis or severe infection.[13]

The management of UAPA consists routine echocardiographic monitoring of asymptomatic patients for the development of pulmonary hypertension[14] and vasodilator therapy if pulmonary hypertension established.[2],[15] Palliative surgical treatments of congenital heart defects associated with UAPA is systemic to pulmonary artery shunt and transluminal balloon pulmonary valvuloplasty in patients with nonsevere hypoplasia of the single pulmonary artery. Palliative reconstruction of the right ventricular outflow tract is a more favorable procedure for patients with a severe hypoplasia of the single pulmonary artery.[16]

Severe infections sometimes require lobectomy or pneumonectomy, and any pulmonary surgery in a patient with UAPA may be complicated by the presence of systemic collaterals.[10] Hemoptysis can be treated with embolization, lobectomy or pneumonectomy.[17],[18]

In our rare case of the left-sided absent pulmonary artery with cardiac anomaly, surgical management was advised in view of recurrent pneumothorax and hemoptysis, and to prevent future complications, but unfortunately, patient’s family refused for any major surgery, and he was managed conservatively. It is necessary for this patient to be followed up closely, especially for the observation of his pulmonary hemodynamics.

  Conclusion Top

The agenesis of the left pulmonary artery is a rare anomaly and patient may remain asymptomatic till adulthood. Imaging will helpful in diagnosis and detecting associated anomaly in the heart and lungs. For patients, who present with a recurrent pneumothorax, hemoptysis, chest infection and an abnormal CXR, UAPA anomaly should be considered in the differential diagnosis. An early diagnosis and treatment can prevent serious complications.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Cucci CE, Doyle EF, Lewis EW Jr. Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 1964;29:124‑31.  Back to cited text no. 1
Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: A case report and review of the literature. Chest 2002;122:1471‑7.  Back to cited text no. 2
Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995;108:670‑6.  Back to cited text no. 3
Presbitero P, Bull C, Haworth SG, de Leval MR. Absent or occult pulmonary artery. Br Heart J 1984;52:178‑85.  Back to cited text no. 4
Pfefferkorn JR, Löser H, Pech G, Toussaint R, Hilgenberg F. Absent pulmonary artery. A hint to its embryogenesis. Pediatr Cardiol 1982;3:283‑6.  Back to cited text no. 5
Fraser RS, Müller NL, Colman N, Pare PD. Developmental anomalies affecting the pulmonary vessels. In: Fraser RS, Pare PD, editors. Fraser and Pare's Diagnosis of Disease of the Chest. 4th ed. Philadelphia: WB Saunders Company; 1999. p. 637‑75.  Back to cited text no. 6
Frantzel O. Angeborener defect der rechten lungenarterie. Virchows Arch Pathol Anat 1868;43:420.  Back to cited text no. 7
Bockeria LA, Makhachev OA, Khiriev TK, Abramyan MA. Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung. Interact Cardiovasc Thorac Surg 2011;12:509‑10.  Back to cited text no. 8
Vergauwen S, Bracke P, De Schepper A. Unilateral absence of a pulmonary artery. J Belge Radiol 1998;81:254.  Back to cited text no. 9
Kadir IS, Thekudan J, Dheodar A, Jones MT, Carroll KB. Congenital unilateral pulmonary artery agenesis and aspergilloma. Ann Thorac Surg 2002;74:2169‑71.  Back to cited text no. 10
Green RA, Shaw DG, Haworth SG. Familial pulmonary hypoplasia: Plain film appearances with histopathological correlation. Pediatr Radiol 1999;29:455‑8.  Back to cited text no. 11
Bahler RC, Carson P, Traks E, Levene A, Gillespie D. Absent right pulmonary artery. Problems in diagnosis and management. Am J Med 1969;46:64‑71.  Back to cited text no. 12
Griffin N, Mansfield L, Redmond KC, Dusmet M, Goldstraw P, Mittal TK, et al. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: A review of four cases. Clin Radiol 2007;62:238‑44.  Back to cited text no. 13
Turner D, Vincent J, Epstein M. Isolated right pulmonary artery discontinuity. Images Paediatr Cardiol 2000;2:24‑30.  Back to cited text no. 14
Shostak E, Sarwar A. A 50‑year‑old woman with dyspnea, lower extremity edema, and volume loss of the right hemithorax. Chest 2009;136:628‑32.  Back to cited text no. 15
Bockeria LA, Podzolkov VP, Makhachev OA, Alekyan BG, Khiriev TK, Zelenikin MA, et al. Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery. Interact Cardiovasc Thorac Surg 2013;16:286‑92.  Back to cited text no. 16
Reñé M, Sans J, Dominguez J, Sancho C, Valldeperas J. Unilateral pulmonary artery agenesis presenting with hemoptysis: Treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol 1995;18:251‑4.  Back to cited text no. 17
Bekoe S, Pellegrini RV, DiMarco RF Jr., Grant KJ, Woelfel GF. Pneumonectomy for unremitting hemoptysis in unilateral absence of pulmonary artery. Ann Thorac Surg 1993;55:1553‑4.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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