A late diagnosis of a congenital absence of the left pulmonary artery (E-Poster Presentations)
Dublin Core
Title
A late diagnosis of a congenital absence of the left pulmonary artery (E-Poster Presentations)
Subject
congenital absence of the left pulmonary artery
Description
Introduction
Congenital absence of left pulmonary artery is very rare, but this case is of particular interest due to the patients age and the
diagnosis being missed in previous imaging.
Case presentation
A 79-year-old female presented to the emergency department with fluid overload and a 2-day history of shortness of breath. She
denies chest pain, palpitations, fever, cough, and weight loss. Her past medical history is significant of unexplained JAK-2 negative
polycythaemia, hypertension, an old left occipital infarct, and a new left parietal infarct. Examination was remarkable for reduced
saturations (74%), raised JVP, tachycardia, and pedal oedema to knees. Bloods were unremarkable except for Hb of 177g/L
(normal 120-160g/L) and NT-Pro BNP of 8258ng/L (normal < 400ng/L). COVID and flu swabs were negative. A CTPA was performed
which ruled out pulmonary embolism, but noted the absence of a left pulmonary artery and a hypoplastic left lung, and severe
anatomical distortion with the heart displaced to the left and the right lung extending into much of the left thoracic cavity. TTE
showed a high probability of pulmonary hypertension with disproportionate RV dysfunction and only mildly reduced LV function. A
right-to-left shunt is also suspected, which helps explain the two strokes. The patient denied any previous thoracic surgery. She
was treated with Furosemide for fluid overload and discharged with home oxygen.
Discussion
Congenital absence of the left pulmonary artery is a rare condition with a wide range of presentations. It is believed to result from
an embryological defect in the sixth aortic arch, with an estimated incidence of 1 in 200,000. The working diagnosis is chronic
hypoxaemia secondary to the above anatomical variation (which explains the long-standing polycythaemia), with consequential
pulmonary hypotension leading to decompensated right ventricular failure. This patient, therefore, has likely been hypoxemic for
some time, highlighting the body’s remarkable ability to adapt to chronic conditions. Given the significant sequelae of this
condition (notably RV failure and two strokes), the patient’s age, and a history of previous CT CAPs, we must give further thought
to the reasons for a lack of earlier diagnosis.
Conclusion
Although very rare, a unilateral absence of pulmonary artery has many significant clinical sequalae, so an early diagnosis is
important to avoid complications. Whilst unusual, the condition may remain minimally symptomatic or asymptomatic until late adulthood, increasing the difficulty of diagnosis.
Congenital absence of left pulmonary artery is very rare, but this case is of particular interest due to the patients age and the
diagnosis being missed in previous imaging.
Case presentation
A 79-year-old female presented to the emergency department with fluid overload and a 2-day history of shortness of breath. She
denies chest pain, palpitations, fever, cough, and weight loss. Her past medical history is significant of unexplained JAK-2 negative
polycythaemia, hypertension, an old left occipital infarct, and a new left parietal infarct. Examination was remarkable for reduced
saturations (74%), raised JVP, tachycardia, and pedal oedema to knees. Bloods were unremarkable except for Hb of 177g/L
(normal 120-160g/L) and NT-Pro BNP of 8258ng/L (normal < 400ng/L). COVID and flu swabs were negative. A CTPA was performed
which ruled out pulmonary embolism, but noted the absence of a left pulmonary artery and a hypoplastic left lung, and severe
anatomical distortion with the heart displaced to the left and the right lung extending into much of the left thoracic cavity. TTE
showed a high probability of pulmonary hypertension with disproportionate RV dysfunction and only mildly reduced LV function. A
right-to-left shunt is also suspected, which helps explain the two strokes. The patient denied any previous thoracic surgery. She
was treated with Furosemide for fluid overload and discharged with home oxygen.
Discussion
Congenital absence of the left pulmonary artery is a rare condition with a wide range of presentations. It is believed to result from
an embryological defect in the sixth aortic arch, with an estimated incidence of 1 in 200,000. The working diagnosis is chronic
hypoxaemia secondary to the above anatomical variation (which explains the long-standing polycythaemia), with consequential
pulmonary hypotension leading to decompensated right ventricular failure. This patient, therefore, has likely been hypoxemic for
some time, highlighting the body’s remarkable ability to adapt to chronic conditions. Given the significant sequelae of this
condition (notably RV failure and two strokes), the patient’s age, and a history of previous CT CAPs, we must give further thought
to the reasons for a lack of earlier diagnosis.
Conclusion
Although very rare, a unilateral absence of pulmonary artery has many significant clinical sequalae, so an early diagnosis is
important to avoid complications. Whilst unusual, the condition may remain minimally symptomatic or asymptomatic until late adulthood, increasing the difficulty of diagnosis.
Creator
Glen Reynolds
Source
Ibrahim, H. A. K. (Ed.). (2024). Abstract and Poster Presentations Book: 3rd Emergency Physician’s International Conference (EPIC24), 10 July 2024. From Zero to Hero Medical Education LTD
Publisher
From Zero to Hero Medical Education LTD
Date
10 July 2024.
Contributor
Sri Wahyuni
Format
PDF
Language
English
Type
Text
Files
Citation
Glen Reynolds, “A late diagnosis of a congenital absence of the left pulmonary artery (E-Poster Presentations),” Repository Horizon University Indonesia, accessed April 15, 2026, https://repository.horizon.ac.id/items/show/11982.