The Varied Manifestation of Pulmonary Artery Agenesis in Adulthood

doi:10.1378/chest.108.3.670

Chest

Volume 108, Issue 3, September 1995, Pages 670-676

https://doi.org/10.1378/chest.108.3.670 Get rights and content

Objective: Unilateral pulmonary artery agenesis (UPAA), a rare congenital anomaly that is frequently associated with other cardiovascular abnormalities, is usually diagnosed in childhood. Most patients who have no associated cardiac anomalies have only minor or absent symptoms and survive into adulthood. The conditions of such patients are frequently misdiagnosed in adulthood. In this report, we describe six patients with UPAA in whom the diagnosis was first established in adulthood. The varied clinical presentation of these patients is reviewed and the relative effectiveness of a variety of diagnostic tests is compared.

Subjects and methods: During the period January 1987 through December 1990, six male patients, aged 17 to 20 years, were found to have UPAA at the time of their medical screening for enrollment into the armed forces. The diagnosis was based on history, clinical and imaging examinations, including chest radiography, ventilation-perfusion lung scan, digital subtraction angiography (DSA), computed tomography (CT), and magnetic resonance imaging (MRI).

Results: In four of the patients, the UPAA was on the left side and in two it was on the right. A right aortic arch was present in three patients and other cardiovascular anomalies were found in three. Pulmonary function studies showed a mild restrictive pattern in four. In contrast to previous reports, the ventilation scan showed a diminished “wash in” and “equilibrium” phase without a delayed “wash out” phase on the affected side in all patients. Selective bronchography through the fiberoptic bronchoscope revealed ipsilateral mixed-type bronchiectasis in two of four patients studied, a finding of clinical significance that has not been described previously. In all cases, the diagnosis was made by DSA. CT of the thorax (n=6) and MRI (n=4) were diagnostic in all cases in which they were performed, but added no significant information. Conclusion: UPAA is frequently misdiagnosed in adulthood and is often not considered in the differential diagnosis of the unilateral hyperlucent lung. Clinicians and radiologists should be aware of the possibility of undiagnosed cases in adults, with many atypical characteristics.

Section snippets

METHODS

Between 1987 and 1990, six male patients who had a final diagnosis of UPAA were seen in our 500-bed hospital, which is a tertiary referral center for respiratory disease. All patients were admitted to the hospital for evaluation of an abnormal chest radiograph taken a few days after their enrollment in the army. Approximately 600,000 recruits were evaluated during this period, giving an incidence of 1 in 150,000 men. The patients' UPAAs were undiagnosed until they presented to us, although some

History

All but one patient had a history of recurrent mild respiratory infections since childhood, which were occasionally treated with antibiotics. One patient (case 2) had mild dyspnea on exertion (grade 2/4) and an episode of blood-tinged sputum in the previous 2-year period. None of the patients were cigarette smokers and none had been exposed to a noxious environment in their occupation.

Clinical Findings

A consistent clinical finding was decreased breath sounds on the involved side and slight ipsilateral deviation

DISCUSSION

The diagnosis of UPAA is difficult, especially when chest radiographic abnormalities are first noted in adulthood. The diagnosis is based on history, physical, and laboratory examination, coupled with a high index of suspicion. A characteristic feature is that the abnormalities on the chest radiograph are more extensive than would be predicted by the relatively benign clinical history. Symptoms, when they occur, are most often related to recurrent respiratory tract infections and there may be

ACKNOWLEDGMENTS

We thank Nestor L. Müller, MD, PhD, of the Department of Radiology at the University of British Columbia for his comments in preparing the manuscript.

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: Presented in part at the annual Congress of the European Respiratory Society, Firenze, Italy, September 25–29, 1993.

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Clinical Investigations: CardiologyThe Varied Manifestation of Pulmonary Artery Agenesis in Adulthood

Section snippets

METHODS

History

Clinical Findings

DISCUSSION

ACKNOWLEDGMENTS

Chest

Chest

Chest

Chest

Am Heart J

Pulmonary artery agenesis: diagnosis with ventilation and perfusion scintiphotography.

Circulation

Angeborener Defect der rechten Lungenarterie.

Virchows Arch Path Anat

Absent or occult pulmonary artery.

Br Heart J

Congenital unilateral absence of pulmonary artery: report of two adults cases.

Am Rev Respir Dis

Unilateral hyperlucent lung: noninvasive diagnosis of pulmonary artery agenesis.

Angiology

Unilateral absence of a pulmonary artery: congenital disease or embolic occlusion?

Eur Respir J

Agenesis of the right pulmonary artery in a young asymptomatic girl.

Eur Respir J

Congenital absence of a pulmonary artery: its demonstration by roentgenology.

Radiology

Clinical Investigations: Cardiology
The Varied Manifestation of Pulmonary Artery Agenesis in Adulthood