Unilateral Pulmonary Artery Agenesis: Noninvasive Diagnosis With Dual-Source Computed Tomography
This article represents an abstract from the original case report. To read the whole article, please visit Circulation. 2009 Mar 3;119(8):1158-60.
History
A 46-year-old female patient presented at the emergency department with hemoptysis of unknown cause. Past medical history was significant for pulmonary hypertension with Eisenmenger syndrome due to a patent ductus arteriosus that had been diagnosed in childhood. (…)
Diagnosis
The patient was referred for CT angiography of the pulmonary arteries to locate the origin of hemoptysis. A CT scan was performed on a dual-source CT system in dual-energy mode at 140 and 80 kVp tube potential after injection of 80 mL of high concentration iodinated contrast material. This recently developed CT technique makes it possible to detect iodine by its spectral properties, thus enabling direct visualization of the perfusion of lung parenchyma.
Results
CT angiography images showed the known patent ductus arteriosus but surprisingly revealed an absence of the right pulmonary artery. Blood supply to the right lung was only provided by compensatorily enlarged bronchial arteries, whereas the vascular morphology of the left lung, with tortuous, dilated central arteries, was typical for pulmonary arterial hypertension. (…)
The spectral differentiation of iodine in the lung parenchyma on the basis of the dual-energy information showed an asymmetrical perfusion of the lungs with very limited enhancement of the right lung and normal perfusion on the left side.
Discussion
Unilateral agenesis of the pulmonary artery was first described in 1868, and some 90 cases have been reported since then. It represents a rare developmental anomaly due to a failure in the connection of the sixth aortic arch with the pulmonary trunk. (…)
Hemoptysis has been described as a clinical symptom in up to 10% of patients, originating from either hypertrophied bronchial collateral vessels or peripheral arteriovenous fistulas ipsilateral to the absent pulmonary artery, as well as from rupture of chronically hyperperfused vessels on the contralateral side. (…)
In our patient, the combination of continued hemoptysis, pulmonary hypertension, and exertion on exercise indicated that heartlung transplantation was the most suitable therapeutic option.
