Unilateral hypoplasia or absence of pulmonary artery is a rare pulmonary vascular anomaly that is usually associated with other cardiacanomalies like Tetralogy of Fallot or cardiac septal defects, but can also occur as an isolated anomaly. We present a case of 6-montholdinfant who presented with recurrent respiratory tract infection. On work up, chest X-ray showed ipsilateral small hemithorax withhyperluscency in the left lung field. Findings were confirmed with 2D echocardiography and pulmonary angiography which weresuggestive of hypoplasia of left pulmonary artery

Gupta, Kirti

Mehariya, K M

Patel, Priyansi

Purani, Charul

English

Atharva Scientific Publications (E-Jounals)

Vol 2 | Issue 1 | Jan - Mar 2015 Indian J Child Health 31
Purani et al. Hypoplastic left pulmonary artery
Case Report
Hypoplasia of left pulmonary artery: A rare congenital heart disease
Charul Purani, K M Mehariya, Kirti Gupta, Priyansi Patel
From Department of Paediatrics, B J Medical College, Ahmedabad, Gujarat, India
Correspondence to: Dr. Charul Purani, A/201, Akash-4 Apartments, Opposite Paavan Party Plot, Ankur, Naranpura, Ahmedabad, 
Gujarat, India. Phone: +91-9825612393, E-mail: charulpurani@yahoo.com
Received ‒ 12 October 2014 Initial Review ‒ 20 November 2014 Published Online ‒ 14 February 2015
Abstract
Unilateral hypoplasia or absence of pulmonary artery is a rare pulmonary vascular anomaly that is usually associated with other cardiac 
anomalies like Tetralogy of Fallot or cardiac septal defects, but can also occur as an isolated anomaly. We present a case of 6-month-
old infant who presented with recurrent respiratory tract infection. On work up, chest X-ray showed ipsilateral small hemithorax with 
hyperluscency in the left lung field. Findings were confirmed with 2D echocardiography and pulmonary angiography which were 
suggestive of hypoplasia of left pulmonary artery.
Key words: Congenital heart disease, Hemithorax, Hypoplasia of left pulmonary artery, Multislice computed tomography angiography
Unilateral absence or hypoplasia of a pulmonary artery is a rare condition with an estimated prevalence of 1 in 2,00,000 [1]. Isolated hypoplasia involves right lung 
in about 2/3rd of the cases in which hypoplasia is on the side 
of the chest opposite to the aortic arch. The prognosis depends 
upon the associated cardiovascular anomaly and a degree of 
pulmonary hypertension (PAH). Patients may present with 
chronic dyspnea and exercise intolerance. Sometimes, it can 
present with more serious complication such as hemoptysis. 
Diagnosis of hypoplasia of pulmonary artery can be difficult, 
but important clues are present in chest radiographs. Patients 
who develop PAH can be treated medically with vasodilator. 
Complication like the hemoptysis can be treated with 
embolization, lobectomy or pneumectomy.
CASE REPORT
A 6-month-old male infant presented with cough, fever and 
breathlessness for 4 days. He was first born child to non-
consanguineous parents. There was history of hospitalization 
thrice with similar complaints starting from the neonatal period. 
The general examination and anthropometric measurements 
were unremarkable. There was no cyanosis or clubbing, and the 
pulse oximetry showed normal arterial saturation. On systemic 
examination, respiratory examination showed tachypnea, 
subcostal and intercostal retraction. Auscultation of the chest 
showed fine crepitations and wheezing in the right lung field. On 
cardiovascular examination, he had systolic murmur grade 2/6 
in 3rd and 4th left intercostal space and 2nd left intercostal space.
On laboratory workup, hemogram showed mild anemia 
with polymorphonuclear leukocytosis. Chest X-ray (Fig. 1) 
was suggestive of asymmetric lung fields, with an ipsilateral 
small hemithorax with hyperlucency in the left lung field. 
Considering the recurrence of pneumonia in the same site in all 
consecutive hospitalizations and presence of the murmur, 2D 
echocardiography was done, which showed? partial anomalous 
pulmonary venous connection of right upper pulmonary 
vein, patent foramen ovale with left to right shunt, moderate 
tricuspid regurgitation, and mild PAH. For further confirmation 
of findings, angiography was advised. Computed tomography 
(CT) thorax was done, which showed bilateral upper lobe 
consolidation. Multislice CT pulmonary angiography (Figs. 2 
and 3) was done, which showed small (atretic) left pulmonary 
artery with prominent left superior intercostal vein.
The patient was treated with intravenous antimicrobial 
therapy along with other supportive measures for 
bronchopneumonia after which he improved gradually with 
resolution of respiratory symptoms. The cardiothoracic 
surgeon opinion was sought for the underlying heart condition 
and was advised regular follow up. The patient was immunized 
with H. influenzae type B, Pneumococcal and Meningococcal 
vaccines (in addition to routine immunization) on discharge 
to protect the child from common pathogens. Parents were 
counseled regarding the disease, associated complications 
and prognosis. The child came for follow-up regularly up to 
10 months of age, he was growing well till then.
DISCUSSION
Unilateral absence or hypoplasia of a pulmonary artery is a rare 
condition with an estimated prevalence of 1 in 2,00,000 [1]. 
Pulmonary artery arises from sixth arch artery and opens into the 
Vol 2 | Issue 1 | Jan - Mar 2015 Indian J Child Health 32
Purani et al. Hypoplastic left pulmonary artery
dorsal part of the aortic sac. The spiral septum, which is formed in 
the truncus arteriosus and extends into the aortic sac, fuses with 
its posterior wall in such a way that blood from the pulmonary 
trunk arches into the sixth arch artery. Most commonly it occurs 
in conjunction with cardiovascular anomalies such as Tetralogy 
of Fallot or cardiac septal defects, but can also occur an isolated 
anomaly [1,2], as was seen in our patient.
Isolated unilateral absence or hypoplasia of pulmonary 
artery involves right lung in about 2/3rd of the cases in which 
hypoplasia of pulmonary artery is on the side of chest opposite 
to the aortic arch [3-4]. These patients are usually asymptomatic, 
which may delay the diagnosis. However, they may present 
with recurrent respiratory infections, chronic dyspnea, exercise 
intolerance, and sometimes with hemoptysis and high altitude 
pulmonary edema. The prognosis depends on the associated 
cardiovascular anomalies and the degree of PAH.
Various mechanisms have been proposed for many of the 
common sequelae of this condition. PAH may result from blood 
flow directed away from absent pulmonary artery to remaining 
pulmonary artery. Increased blood flow leads to sheer stress on 
the endothelium, which results in the release of vasoconstrictors 
such as endothelin [5]. Chronic vasoconstriction of the 
pulmonary arterioles lead to remodeling resulting in increased 
resistance in the pulmonary vasculature and PAH [5].
The etiology of recurrent infections observed in these patients 
is likely to be multifactorial. Lack of arterial blood flow to the 
affected lung may result in poor delivery of inflammatory cells 
to the sites of inflammation and impaired ciliary function [6]. 
In addition, poor blood supply to the affected lung may result in 
alveolar hypocapnea, leading to secondary bronchoconstriction 
and mucous trapping [6]. Hemoptysis is a serious complication 
and appears to be caused by large collateral circulation that 
subject venous systems to unusually high pressures.
Diagnosing unilateral absence or hypoplasia of a pulmonary 
artery can be difficult, but important clues are present in chest 
radiographs. X-ray chest may show asymmetric lung fields, 
with an ipsilateral small hemithorax holding a hyperlucent lung, 
which was present in our patient [1,7]. Definite diagnosis can 
be made by CT, magnetic resonance imaging or transthoracic 
echocardiogram. Pulmonary angiography is considered the 
gold standard for diagnosis.
Treatment should be based on the symptoms and pulmonary 
hemodynamic status. Asymptomatic patients should be 
followed regularly for the development of PAH. Patients 
who develop PAH can be treated medically with vasodilator 
therapy, which was not used in our as PAH was mild [3,5]. 
Alternatively, revascularization of peripheral branches 
of the affected pulmonary artery to the pulmonary hilum 
can be attempted [2,8,9]. Hemoptysis may be treated with 
embolization, lobectomy or pneumonectomy [10,11].
CONCLUSION
Though the disease is rare, we should suspect this disease is patients 
of recurrent respiratory infections, and chest X-ray showing 
Figure 1: Small hemithorax with hyperlucency in left lung 
field
Figure 2: Small (atretic) left pulmonary artery with 
prominent left superior intercostal vein
Figure 3: Small (atretic) left pulmonary artery with 
prominent left superior intercostal vein
Vol 2 | Issue 1 | Jan - Mar 2015 Indian J Child Health 33
Purani et al. Hypoplastic left pulmonary artery
abnormality at the same site. Disease should be diagnosed by 2D 
echocardiography and then managed medically or surgically.
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Funding: None; Conflict of Interest: None Stated
How to cite this article: Purani C, Mehariya KM, Gupta K, 
Patel P. Hypoplasia of left pulmonary artery: A rare congenital 
heart disease. Indian J Child Health. 2015;2(1):31-3.


Hypoplasia of left pulmonary artery: A rare congenital heart disease

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Hypoplasia of left pulmonary artery: A rare congenital heart disease

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