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Abstract

We describe a unique case of congenital absence of a right pulmonary artery presenting in a patient of advanced age and initially misdiagnosed as coronary artery disease. Perfusion of the affected lung was accomplished via anomalous collaterals from right and left circumflex coronary arteries which induced myocardial ischemia, as demonstrated by myocardial perfusion scan. To our knowledge there are only three reports in the international literature, describing unilateral pulmonary artery agenesis with the coronary artery supplying the abnormal lung. All these reports described that the existence of such vessels does not affect the myocardial perfusion. However, here we describe, to our knowledge, for the first time that in a patient with unilateral pulmonary artery agenesis, the existence of collaterals from the coronary arteries to the affected lung can actually have a negative effect in myocardial perfusion and can induce myocardial ischemia. In conclusion, clinicians should be aware of the possibility of undiagnosed cases of unilateral pulmonary artery agenesis presenting with chest pain in advanced age. (c) 2007 S. Karger AG, Basel.

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Most cited references 12

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The varied manifestation of pulmonary artery agenesis in adulthood.

K Tsintiris, P Pare, P Panagou … (1995)

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. 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). 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. 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.