Abstract
Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital malformation. We describe a 26‐year‐old woman with isolated UAPA who presented with hemoptysis. Since massive hemoptysis may mandate selective embolization of collaterals or surgical treatment, early diagnosis of UAPA is pivotal.
Keywords: collateral artery, congenital heart disease, hemoptysis
Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital malformation. Since massive hemoptysis may mandate selective embolization of collaterals or surgical treatment, early diagnosis of UAPA is pivotal.
A 26‐year‐old woman was referred for hemoptysis, which persisted for 11 days and improved spontaneously. Her medical histories were childhood asthma and dysmenorrhea. The results of physical assessment and laboratory tests were not remarkable. Chest X‐ray showed ipsilateral cardiac and mediastinal displacement and the absence of pulmonary artery shadow (Figure 1A). Chest computed tomography (CT) indicated left pulmonary hemorrhage. Contrast‐enhanced CT revealed an isolated left‐sided unilateral absence of the pulmonary artery (UAPA) and right‐sided aortic arch (Figure 1B,C). CT aortography showed the left lung had collateral arteries, disruption of which conceivably led to hemorrhage (Figure 1D). Heart ultrasonography did not indicate pulmonary hypertension or cardiac anomalies. We made a diagnosis of isolated left‐sided UAPA. Since her symptom was self‐limiting, we followed her up without interventional treatment.
FIGURE 1.
(A) Ipsilateral cardiac and mediastinal displacement and the absence of pulmonary artery shadow are shown by chest X‐ray. (B, C) Contrast‐enhanced computed tomography (CT) (B) and three‐dimensional reconstructed CT (C) show the absence of the left pulmonary artery and the left pulmonary vascular bundles. The asterisk indicates the right pulmonary artery (B). Right‐sided aortic arch is also shown. (D) CT aortography showed tortuous collateral vessels from an inferior phrenic artery and an internal thoracic artery.
Isolated UAPA is a rare congenital malformation. 1 Adult patients with isolated UAPA are asymptomatic or present hemoptysis, exertional dyspnea, and recurrent respiratory infection. 2 Since massive hemoptysis mandates surgery or selective embolization of collaterals, 1 early diagnosis of UAPA is pivotal.
AUTHOR CONTRIBUTIONS
Koichiro Yamamoto: Writing – original draft. Yasuhiro Nakano: Writing – review and editing. Fumio Otsuka: Supervision.
CONFLICT OF INTEREST
The authors declare no conflicts of interest.
CONSENT
Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.
ACKNOWLEDGMENTS
None.
Yamamoto K, Nakano Y, Otsuka F. Isolated unilateral absence of adult pulmonary artery. Clin Case Rep. 2022;10:e06652. doi: 10.1002/ccr3.6652
DATA AVAILABILITY STATEMENT
Not applicable.
REFERENCES
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Data Availability Statement
Not applicable.