Isolated right pulmonary artery agenesis with agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply from celiac axis with normal venous drainage.

Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly. When detected in infancy, the condition is commonly associated with cardiovascular defects which are more frequently associated with left pulmonary artery agenesis. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms and are diagnosed incidentally on the chest radiographs. We report a case of a 19-year-old female patient who presented to us with recurrent haemoptysis. She was symptomatic since the age of four years. We report the rare occurrence of UAPA on right side, agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply of right lung from coeliac axis in this patient.

Left pulmonary artery agenesis with pulmonary hypoplasia in an elderly patient: a rare case report.

Proximal interruption of the unilateral pulmonary artery is a rare congenital anomaly, which is often associated with other cardiovascular abnormalities. It is usually diagnosed in children but rarely discovered in adulthood as an isolated phenomenon, occurring more frequently on the right side and is often associated with a contralateral aortic arch. We are presenting a rare case of a sixty year old male who was diagnosed with left lung hypoplasia due to proximal interruption of left pulmonary artery with left sided aortic arch without any associated cardiovascular anomalies.

A Case of Isolated Right Pulmonary Artery Agenesis / 결핵및호흡기질환

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.

Left Pulmonary Artery Agenesis Accompanied with Fistula of Left Circumplex Artery to Left Bronchial Artery

Left pulmonary artery agenesis, accompanied by a coronary arterial fistula, is a very rare anomaly. Although unilateral pulmonary artery agenesis is associated with other cardiovascular defects, like as ventricular septal defect, patent ductus arteriosus, and tetralogy of fallot, this anomaly, accompanied by a coronary arterial fistula, has not yet been reported. Most patients with no associated cardiac anomalies have only minor, or absent, symptoms, and survive to adulthood, but some patients may suffer from recurrent respiratory infections and hemoptysis. The vessel to the affected lung in many of the proved cases has been described as arising from either the bronchial artery or the aortic arch. The blood supply from the coronary artery to the affected lung has never been reported. Recently, a case of left pulmonary artery agenesis, accompanied with a coronary arterial fistula was experienced, which was diagnosed by coronary angiography and a chest CT, which is presented, with the review of relevant literature.

A 32 Years-old Female Accompanied by the Loss of Lung Volume, Complained of Hemoptysis / 결핵

A 32-year-old woman presented with cough and hemoptysis. The radiologic findings showed increased interstitial markings in the right lung, a slightly decreased ling volume in the RLL and a hypoplastic right pulmonary arte ry 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 politeal vein was occluded with collateral veins, not filling the defect in the venous lumen. The D-dimer increased 1.0 micro gram/ml. This condition was initially misdiagnosed as a congenital pulmonary artery agenesis. Finally, a chronic pulmonary thromboembolism with a deep vein thrombosis was confirmed.

Right Pulmonary Artery Agenesis: A Case Report / 대한흉부외과학회지

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 anomaly is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film sonsists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced right 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.