Tuberc Respir Dis.  2008 Nov;65(5):430-434.

A Case of Isolated Congenital Absence of Right Pulmonary Artery

• La SS
• Kim SM
• Kim DH

Abstract

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

Keyword

Pulmonary artery; Agenesis; Hemoptysis

MeSH Terms

Hand
Heart Septal Defects, Ventricular
Hemoptysis
Humans
Korea
Lung
Male
Pulmonary Artery
Sputum
Stress, Psychological
Tetralogy of Fallot
Tolnaftate
Tolnaftate

Figure

• 

  Figure 1 Chest radiograph shows an enlarged left lung and small, hypoplastic right lung with increased interstitial markings in right lung zone.

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  Figure 2 (A) Chest CT scan shows absence of Rt. pulmonary artery with ipsilateral hypoplastic lung and contralateral hyperinflation. (B) Rt. pulmonary vessels are diminished and Rt. bronchial artery is dilated, which makes collateral vessels along the right peribronchial spaces. There are also increased interstitial thickening, which represented numerous collaterals from parietal pleura (arrow).

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  Figure 3 A perfusion scan shows perfusion defect in Rt. lung, consistent with total obstruction of the Rt. pulmonary artery.

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  Figure 4 Pulmonary angiography demonstrates absence of pulmonary artery arising from pulmonary trunk. There is no evidence of congenital defects between Rt. and Lt. heart chambers. Dilated bronchial artery and collaterals are not present in Rt. lung region.

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