Tuberc Respir Dis.  2011 Aug;71(2):126-133. 10.4046/trd.2011.71.2.126.

Isolated Right Pulmonary Artery Hypoplasia with Retrograde Blood Flow in a 68-Year Old Man

• Chang Y
• Ra SW
• Chae EJ
• Seo JB
• Kim WY
• Na S
• Kim JH
• Park TS
• Park SK
• Park SJ
• Lee T
• Ahn YC
• Lee SD

• KMID: 1846420
• DOI: http://doi.org/10.4046/trd.2011.71.2.126

Abstract

Unilateral pulmonary artery hypoplasia (UPAH) is a rare disease in adults and is frequently accompanied by a congenital cardiac anomaly at a young age. The diagnosis is usually based on computed tomography (CT), angiography, and magnetic resonance imaging (MRI). However, no reports are available on retrograde flow in patients with UPAH. We describe a 68-year-old man with isolated UPAH and retrograde blood flow. He was admitted for dyspnea on exertion for the past 23 years. His diagnosis was delayed, as his symptoms and signs mimicked his underlying pulmonary diseases, such as emphysema and previous tuberculous pleurisy sequelae. A discrepancy was detected between the results of a ventilation-perfusion scan and the CT image. This was resolved by MRI, which showed retrograde blood flow from the right to the left pulmonary artery. Using MRI, we diagnosed this patient with isolated pulmonary artery hypoplasia and retrograde flow.

Keyword

Pulmonary Artery; Hypoplasia; Magnetic Resonance Imaging; Regional Blood Flow

MeSH Terms

Adult
Aged
Angiography
Dyspnea
Emphysema
Humans
Lung Diseases
Magnetic Resonance Imaging
Pulmonary Artery
Rare Diseases
Regional Blood Flow
Tuberculosis, Pleural

Figure

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  Figure 1 Chest radiography. Mild emphysema is observed in both lung fields. Abnormal linear opacities are observed in the right lung field, as well as blunting of the right costophrenic angle, indicative of previous pulmonary tuberculosis and tuberculous pleurisy. Increased pulmonary vascularity is observed in the left lung.

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  Figure 2 Ventilation/Perfusion scan. The inhalation scan is normal, but the perfusion scan shows a total perfusion defect in his right lung.

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  Figure 3 Chest CT (axial image). (A) Chest CT shows emphysema in both lung with hypoplastic right lung. (B) Decreased pulmonary vasculature is observed in right lung with pleural thickening (arrowheads). CT: computed tomography.

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  Figure 4 Chest CT (coronary image). (A, B) The right pulmonary artery is markedly decreased in diameter and has a streaky appearance (arrow). This artery, however, can be traced to the peripheral portion of the right lung (arrowheads). (C, D) Hypertrophy of the right intercostal, internal mammary, phrenic and bronchial arteries is observed (arrows). CT: computed tomography.

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  Figure 5 Dynamic MR angiography. In early phase dynamic MR angiography, the right pulmonary artery is not visualized, whereas the entire left pulmonary artery (arrow) is clearly visualized. After systemic circulation was confirmed by opacification of the aorta (arrowhead), we observed a streaky right pulmonary artery (dotted circle). MR: magnetic resonance.

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  Figure 6 Flow analysis on MRI. (A) Flow in the main PA shows an up-sloping curve above zero, indicating an antegrade flow and a peak velocity of approximately 200 mL/s. (B) Flow in the right PA shows a down-sloping curve below zero, indicating a retrograde flow and a peak velocity of approximately 6.0 mL/s. PA: pulmonary artery; MRI: magnetic resonance imaging.

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