Ashu Sharma, MD
Evan H. Dillon, MD
, Department of Radiology, Lenox Hill Hospital; and
Frank Bieuei, MD
, Department of Pathology, North Shore University Hospital, New
A 48-year-old man presented to the hospital with gross painless
hematuria. He underwent a radiologic evaluation, including renal
sonography and computed tomography (CT) of the abdomen and pelvis.
These studies indicated a solid mass in the mid- to lower-pole of
the right kidney. Chest radiographs revealed a right-sided aortic
arch, deviation of the trachea to the left, and an abnormal left
pulmonary branching pattern. During his hospital stay, the patient
developed shortness of breath and underwent a ventilation-perfusion
scan. This revealed absent perfusion of the left lung with
diminished ventilation. The study was interpreted as suspicious for
a pulmonary embolism to the left pulmonary artery. A
contrast-enhanced CT of the chest using a pulmonary embolism
protocol was then performed (Figure 1).
Agenesis of the left pulmonary artery, with patent ductus
arteriosus and hypoplasia of the left lung
Contrast-enhanced CT scans of the chest (Figure 1) reveal
agenesis of the left pulmonary artery, a hypoplastic left lung
perfused by bronchial collaterals, unilateral emphysema involving
the left lung, and a right-sided aortic arch with aberrant origin
of the left subclavian artery.
The patient subsequently underwent a radical nephrectomy that
revealed renal cell carcinoma. Postoperatively, he developed
progressive pulmonary infiltrates, which were more evident on the
right side with evidence of bronchial occlusion. He was placed on a
ventilator, but eventually died due to cardiac arrythmia, despite
At autopsy in this patient, the aortic arch was found to be
right-sided with aberrant origin of the left subclavian artery. A
patent ductus arteriosus with right ventricular hypertrophy was
seen. The left pulmonary artery was absent, and bronchial
collaterals were found to be supplying the left lung. The left lung
was hypoplastic, with evidence of bullous emphysematous changes and
fibrosis (Figure 2). The right lung displayed changes consistent
with acute respiratory distress syndrome.
Congenital absence of a pulmonary artery can affect either the
right or left side; it was first described by Fraentzel in 1868.
In his initial report, he indicated that 30% of patients with this
finding could remain asymptomatic until adult life. To date, 168 to
170 patients with this disease have been described in the
This case is unusual because imaging observations have been
correlated with autopsy findings.
Left pulmonary artery agenesis has been reported with a variety
of cardiac malformations and usually presents with symptomatic
disease. The associated cardiac malformations include tetralogy of
Fallot, atrial septal defect, coarctation of aorta, right aortic
arch, and Eisenmenger's syndrome.
Rarely, this abnormality has been reported in combination with
patent ductus arteriosus.
Absent right pulmonary artery is more commonly present as an
isolated, asymptomatic finding without associated congenital
cardiac malformations. In the case reported here, an adult patient
was diagnosed with asymptomatic left pulmonary artery agenesis in
combination with hypoplasia of the left lung, right aortic arch,
and patent ductus arteriosus on the basis of the CT scan.
According to previously published reports, isolated pulmonary
artery agenesis can present clinically in a variety of ways. In a
recent series, Bouros et al
described 6 cases of isolated pulmonary artery agenesis in
adulthood. Only 1 case was asymptomatic, while the others had
recurrent respiratory tract infections. Additionally, 1 patient had
hemoptysis and dyspnea.
Chest radiography findings may include absence of pulmonary
markings on the affected side, displacement of cardiac and
mediastinal shadow, elevation of the diaphragm on the affected
side, and contralateral lung hyperinflation. Ventilation-perfusion
scans usually reveal absence of lung perfusion on the affected
side. On CT scans, absence of the affected pulmonary artery is
observed. The affected lung is usually supplied by systemic
vasculature including bronchial, subclavian, intercostal, and
subdiaphragmatic branches. In this case, the collateral circulation
involved bronchial arteries.
Other evaluation techniques, including physiologic measurements,
cardiac catheterization, pulmonary angiography, and magnetic
resonance imaging, have also been used to diagnose this condition.
However, as this case illustrated, a CT scan is quite useful in
revealing the features of this disorder.
Rarely, left pulmonary artery agenesis in combination with
patent ductus arteriosus can present as an asymptomatic condition
in adult life. Characteristic findings on contrast-enhanced CT
allow accurate characterization of the anomaly.