Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: A rare case diagnosed during the postpartum period (original) (raw)
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Congenital Right Pulmonary Artery Agenesis with Atrial Septal Defect and Pulmonary Hypertension
Congenital Heart Disease, 2012
Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.
Acta Cardiologica Sinica, 2015
UNLABELLED The congenital unilateral agenesis of pulmonary artery is a congenital cardiovascular anomaly rarely seen in adulthood. A 21-year-old asymptomatic male was admitted to our hospital to obtain a routine health report to accompany a job application. Posteroanterior chest radiograph revealed a mediastinal shift to the left, with increased radiopacity in the left lung and increased radiolucency in the right lung. Thoracoabdominal computed tomography revealed hypoplasia of the left pulmonary artery. Transthoracic echocardiography excluded any accompanying cardiac abnormalities. Pulmonary angiography was undertaken and confirmed diffuse hypoplasia of the left pulmonary artery while right pulmonary artery was significantly enlarged. The patient's pulmonary artery pressure was within the normal limits, after which he decided to be carefully followed-up. KEY WORDS Cardiovascular anomaly; Hypoplasia; Pulmonary artery.
The rare manifestation of pulmonary artery agenesis
Srpski arhiv za celokupno lekarstvo, 2019
Introduction. Unilateral absence of pulmonary artery is a rare vascular malformation. Because of this anomaly, the lungs are supplied by the system of collateral arteries. Case outline. We present a case of the right pulmonary artery agenesis in a female patient. She was admitted to the hospital because of hemoptysis. A computed tomography scan revealed a congenital malformation ? the right lung was smaller in size, the right principal pulmonary artery had not been developed along with aberrant tortuous blood vessels. Conclusion. Symptomatic therapy was applied in the case of our patient. There was no need for any surgical treatment. However, in case of massive hemoptysis embolisation or lobectomy/ pneumonectomy will probably be applied.
Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report
Journal of Interdisciplinary Medicine, 2016
Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH), that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complicati...
Pulmonary Artery Agenesis: A Rare Case Report
2020
Pulmonary artery agenesis is one of the rare anomalies of unknown etiology. It was first described by D. Pozze in 1673 during a women's autopsy [1]. Bilateral pulmonary artery agenesis is incompatible with life. Genetic and teratogenic factors have been implicated in the unilateral pulmonary artery agenesis. Approximately 50% of pulmonary artery agenesis may be associated with congenital malformations such as cardiovascular, gastrointestinal, neuro-musculoskeletal and genitourinary system [2,3]. The diagnosis of unilateral pulmonary artery agenesis can be made by various radiological imaging methods such as posteroanterior and lateral lung direct radiography, Computed Tomography (CT), Magnetic Resonance (MR), bronchography and angiography. It is common opinion that the majority of isolated unilateral pulmonary artery agenesis is infant-child and is a very rare congenital pathology [1]. In this disease, it does not contain a specific hereditary component but in some case reports ...