|Year : 2018 | Volume
| Issue : 2 | Page : 72-75
Absent Unilateral Pulmonary Artery: A Report of Two Cases
Govind S Rajawat1, Manoj Meena2, Manohar L Gupta3, Piyush Arora2
1 Department of Chest & Tuberculosis, SMS Medical College, Jaipur, India
2 Department of Respiratory Medicine, Jawahar Lal Nehru Medical College, Ajmer, India
3 Department of Pulmonary Medicine, SDMH Hospital, Jaipur, Rajasthan, India
|Date of Web Publication||10-Jul-2018|
Department of Respiratory Medicine, Jawahar Lal Nehru Medical College, Ajmer, Rajasthan
Source of Support: None, Conflict of Interest: None
Unilateral absent pulmonary artery is an uncommon condition and may be associated with other congenital cardiac defect. Patients without associated cardiac abnormality usually present in adulthood with symptoms of dyspnea, hemoptysis, etc. We encountered two adults with absent right pulmonary artery who presented with hemoptysis and on investigations found to have absent right pulmonary artery. Considering the rarity of this entity, we found it worthy of reporting.
Keywords: Congenital cardiac defect, hemoptysis, unilateral absent pulmonary artery
|How to cite this article:|
Rajawat GS, Meena M, Gupta ML, Arora P. Absent Unilateral Pulmonary Artery: A Report of Two Cases. J Assoc Chest Physicians 2018;6:72-5
| Introduction|| |
Absence of one or other main pulmonary artery is a consequence of failure of either branch of sixth brachial arch to develop in the embryo. This entity may be seen as an isolated condition or in association with other congenital heart defect. Although this situation occurs equally on both the sides, but right pulmonary artery agenesis is more often seen than left. This is because, absent left pulmonary artery is often associated with severe congenital cardiac defects. Therefore, patients with absent left pulmonary artery do not survive long enough to report late in life. On the contrary, right pulmonary artery agenesis has no strong relationship with congenital cardiac defect; therefore, these individuals survive long and are diagnosed late in the life, often without any symptoms. We diagnosed two patients with absent right pulmonary artery at the age of 19 and 52, respectively. Considering the rarity, they are reported.
| Case History|| |
A 19-year-old male presented with history of hemoptysis since 4 days. There was no cough, expectoration, chest pain, breathlessness, palpitation, or syncope. Family history was negative for any congenital heart disease. Patient denied any history of smoking, alcohol, or drug abuse. Past medical history was unrewarding.
Physical examination on the patient revealed him as a fairly built adult. His vitals were within normal limits. Clubbing, cyanosis, hepatomegaly, and edema feet were absent. Jugular venous pressure was normal. Respiratory and cardiovascular examinations were normal except shift of apex beat to medially in fifth intercostal space just lateral to left sternal border.
Electrocardiogram, two dimensional echocardiography, and resting arterial blood gases analysis were within normal limits. Spirometry was not performed as patient was having hemoptysis. X-ray posteroanterior [Figure 1] showed reduced right lung volume with shift of mediastinium toward same side. Right lung field appeared hazy due to diffuse reticulations in it.
|Figure 1: X-ray posteroanterior view showing small right hemi thorax with shift of mediastinal toward right side with diffuse parenchymal haziness|
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Contrast-enhanced computed tomography of thorax in mediastinal window [[Figure 2]a] showed absent right pulmonary artery. Rest of the major mediastinal vessels and trachea appeared normal. Lung window [[Figure 2]b] just above the level of diaphragm showed hypoplastic right lung with compensatory hyperinflation of left lung. Left lung field vasculature was within normal limits. Perfusion scan with 99mTc macroaggregated albumin showed absent right lung perfusion also. A diagnosis of absent right pulmonary artery was made, and symptomatic treatment in form of cough sedative rest, etc. was given to the patient. Hemoptysis subsided in next 24 h.
|Figure 2: (a) Contrast enhanced computed tomography of thorax showing absent right pulmonary artery (arrow). Rest other mediastinal structures including left pulmonary artery appears normal. (b) High-resolution section just above the diaphragm showing hypoplastic right lung with compensatory hyperinflation of the contralateral lung|
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A 52-year-old non-smoker male was presented with history of hemoptysis since 2 days. Patient denied any cough, expectorations, chest pain, and breathlessness. Vital signs, for example, temperature, pulse, blood pressure, and respiratory rate were within normal limits. Examination of respiratory system was normal except decreased breath sounds on right side. Cardiovascular, abdominal and central nervous system examinations were essentially within normal limits. Electrocardiogram and two-dimensional echocardiography did not show any abnormality.
Skiagram chest posteroanterior view showed a small right hemithorax with ipsilateral shift of mediastinum. Increased markings were present in the whole of the right lung field. Contrast-enhanced computed tomography of chest [Figure 3] showed an absence of right pulmonary artery. Prominent reticular markings were evident in subpleural region of the right lung. Tubular form of bronchiectasis was also present in right upper lobe.
|Figure 3: Contrast enhanced computed tomography of the chest showing absent right pulmonary artery and increase reticular markings in sub pleural region of right lung. A small area of tubular bronchiectasis (T) is also present|
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A diagnosis of absent right pulmonary was made, and symptomatic treatment was advised. Hemoptysis subsided with symptomatic treatment.
| Discussion|| |
Pulmonary vasculature is derived from three principal sources. Main pulmonary artery is derived from the arterial portion of truncoaortic sac. Extra pulmonary portion of pulmonary arteries develops from respective 6th brachial arch. Intrapulmonary pulmonary vasculature develops from their respective mesenchymal tissue surrounding the lung buds. Absent pulmonary artery is the result of involution of 6th brachial arch. Because, intrapulmonary vasculature which develops from respiratory mesenchyma is normal in these patients, lung receives blood retrogradely from collateral arteries and from persistent ductus arteriosus; therefore, the term interruption is preferred to absent pulmonary artery by some authors. Common collaterals supplying to lung include bronchial arteries, transpleural branches of intercostals, internal mammary, subclavian, and innominate artery. Rarely, the collaterals from coronary artery have also shown to supply ipsilateral lung of the patient with unilateral absent pulmonary artery.
During first half of 20th century, all the recorded cases of absent pulmonary artery were from the autopsy series. Subsequently with the availability of cardiac surgery in early 70s for cyanotic heart diseases, many more cases were identified/diagnosed at operations. After the development of angiographic techniques, the diagnosis of this entity was possible without operation or autopsy.
Advent of computed tomography in last quarter of 20th century made the diagnosis of this entity easy. Patients were diagnosed to have this abnormality when computed tomography was performed for the evaluation of some or other unrelated chest problems or during the workup of symptoms related to this abnormality, for example, dyspnea/hemoptysis.
Occasionally, this entity can also be suspected, when skiagram chest is performed as a part of routine checkup, or during preemployment examination. Small hemi thorax with diffuse reticulations due to collateral blood supply from systemic circulation to lung points toward the possibility of absent pulmonary artery and need of further workup. Although smaller hemithorax may also be seen in scimitar syndrome and hypoplastic lung, but the presence of other associated findings, for example, anomalous venous drainage and hypoplastic rather than absent unilateral absent pulmonary artery in former and hypoplastic ipsilateral pulmonary artery without collateral systemic blood supply in the later entity may help in arriving correct diagnosis.
On computed tomography, the affected part of pulmonary artery gets terminated with in 1 cm of its origin. Direct anastomosis of transpleural collateral vessels with peripheral branches of the pulmonary artery causes serrated thickening of pleura and sub pleural, parenchyma seen on computed tomography (CT) film. Such CT appearance may mimic interlobular septal thickening as seen in idiopathic pulmonary fibrosis. Contrast enhanced computed tomography of the chest not only diagnoses absent pulmonary artery but also provides information about mediastinal structures and lung parenchyma. In contrast, having an invasive angiography is better in certain aspects, because it provides better delineation and hemodynamic data. Considering the availability, expense, and risk, pulmonary angiography is to be reserved only for those having structural cardiac anomaly on echocardiography.
A patient with isolated unilateral absent pulmonary artery may experience recurrent respiratory infections, dyspnea on exertion, high-altitude pulmonary edema, pulmonary hypertension in contra lateral lung or hemoptysis. Hemoptysis occurs in about 20% of cases and is usually selflimiting. An occasional case report of massive hemoptysis and death is available in literature. Rupture of an aneurysm between systemic to pulmonary collateral is said to be responsible for hemoptysis.
There is no consensus regarding the treatment of this condition. However, those who are asymptomatic or have minor symptoms should be observed closely for any development of pulmonary arterial hypertension. Medical treatment of pulmonary hypertension in patients with unilateral absent pulmonary artery without associated congenital heart disease is not available yet. Surgical option to correct pulmonary hypertension when the present patient includes anastomosis of the hilar arteries of affected lung to the main pulmonary artery. Pneumnectomy or lobectomy is to be considered in cases with massive hemoptysis. In patients having poor pulmonary reserve and or co morbid conditions, selective embolization of the systemic artery is indicated for the management of hemoptysis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Balci TA, Koc ZP. Isolated left pulmonary artery agenesis: A case report. Mol Imaging Radionucl Ther 2012;21:80-3.
Sotiria CA, Nikolaos LK, Elias NB, Spyridon R, Dimitrios AK. Absent pulmonary artery in one adult and five pediatric patients: Imaging, embryology and therapeutic implication. Am J Roentgenol 2002;179:1253-60.
Steiropoulos P, Archontogeorgis K. Unilateral pulmonary artery agenesis: A case series. Hippocratia 2013;17:73-6.
Harkel DJ, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: A case report and review of literature. Chest 2002;122:1471-7.
Gupta K, James J, Livesay JJ, Lufschanowski R. Absent right pulmonary artery with coronary collaterals supplying the affected lung. Circulation 2001;104:12-3.
Mishra M, Nayyar P. Absent unilateral pulmonary artery in a adult. A rare case entity. IOSR J Dent Med Sci 2015;14:76-8.
Kempny A, Dimopoulos K, Uebing A, Diller GP, Rosendahl U, Belitsis G et al.
Outcome of cardiac surgery in patients with congenital heart disease in England between 1997 and 2015. PLoS One 2017;12:e0178963. doi: 10.1371/journal.pone.0178963
Gupta ML, Bagarhatta R, Sinha J. Scimitar syndrome: A rare disease with unusual presentation. Lung India 2009;26:26-9.
] [Full text]
Castaner E, Gallardo X, Rimolo J, Pallardo Y, Mata JM, Perendreu J et al.
Congenital and acquired pulmonary artery anomalies in the adult: Radiologic overview. Radiographic 2006;26:349-71.
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.
Toews H, Pappas G. Surgical management of absent right pulmonary artery with associated pulmonary hypertension. Chest 1983;84:497-9.
Yoon W, Kim FK, Kim YH, Chung TW, Kang HK. Bronchial and nonbronchial systemic artery embolization for life-threatening hemoptysis: A comprehensive review. Radiographics 2002;22:1395-409.
[Figure 1], [Figure 2], [Figure 3]