• Journal of Chest Surgery
• /
• v.24 no.1
• /
• pp.83-87
• /
• 1991

A unilateral pulmonary artery agenesis, which develops occasionally as one of the associated anomalies in congenital cardiovascular defect like as tetralogy of Fallot, is very rare anomaly as an isolated congenital defect. The diagnostic approach for the pulmonary artery agenesis is first suggested by the unique appearance of the involved lung on a routinely checked chest roentgenogram, because most patients are asymptomatic unless pulmonary infection had been superimposed. We have recently experienced a case of left pulmonary artery agenesis, which was diagnosed by perfusion scan, digital subtraction angiogram and then treated by left pneumonectomy in a 9 year-old boy, and presented hereby with the review of relevant literature.

• Tuberculosis and Respiratory Diseases
• /
• v.52 no.3
• /
• pp.288-293
• /
• 2002

A 32-year-old woman presented with cough and hemoptysis. The radiologic findings showed increased interstitial markings in the right lung, a slightly decreased lung volume in the RLL and a hypoplastic right pulmonary artery with collaterals in the mediastinum and subpleural area. The pulmonary angiography showed an abrupt occlusion of the right lower pulmonary artery. The echocardiographic findings indicated pulmonary hypertension. A doppler leg ultrasonograph disclosed that the left popliteal vein was occluded with collateral veins, not filling the defect in the venous lumen. The D-dimer increased 1.0 ug/ml. This condition was initially misdiagnosed as a congenital pulmonary artery agenesis. Finally, a chronic pulmonary thromboembolism with a deep vein thrombosis was confirmed.

• Journal of Chest Surgery
• /
• v.30 no.1
• /
• pp.108-111
• /
• 1997

Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

• Tuberculosis and Respiratory Diseases
• /
• v.57 no.5
• /
• pp.489-493
• /
• 2004

Unilateral pulmonary artery agenesis is a rare congenital anomaly usually associated with other cardiovascular anomalies such as tetralogy of Fallot or septal defect. Unilateral pulmonary artery agenesis without other coexisting cardiovascular abnormality (isolated unilateral pulmonary artery) is extremely rare and often asymptomatic until adulthood. In these patients, diagnostic clue is found in a plain chest roentgenogram, showing a hyperlucent contracted hemithorax. We have recently experienced a case of isolated right pulmonary artery agenesis, which was diagnosed by chest dynamic CT, perfusion scan, echocardiogram and 3-dimensional reconstruction cardiac CT angiography in a 50-year old female who had suffered from mild dyspnea on exertion and improved with conservative treatment. We report this case with a brief review of the relevant literature.

• Journal of Chest Surgery
• /
• v.40 no.1 s.270
• /
• pp.52-55
• /
• 2007

Unilateral pulmonary artery agenesis is a rare congenital malformation usually associated with other cardiovascular anomaly such as Tetralogy of Fallot. Isolated pulmonary artery agenesis is very rare, and usually asymptomatic. It is usually highly suspected by routine chest X-ray, and associated symptoms are hemoptysis, blood tinged sputum, repeated pulmonary infection, and dyspnea on exertion. We have recently experienced the right pulmonary artery agenesis in 27 year-old male patient, complaining of minimal hemoptysis and sustained blood tinged sputum. He was successfully treated by right pneumonectomy, so we report this case with the review of associated literature.

• Tuberculosis and Respiratory Diseases
• /
• v.65 no.5
• /
• pp.430-434
• /
• 2008

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.