Takayasu’s Arteritis Mimicking Unilateral Pulmonary Artery Agenesis in a Child With Severe Pulmonary Hypertension and Right Heart Failure: A Diagnostic Dilemma
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Affliction of the pulmonary arteries in Takayasu’s arteritis is uncommon. Moreover the incidence of pulmonary artery involvement in this condition is often underestimated because of asymptomatic nature in most patients. Severe involvement may however present with pulmonary artery hypertension and hemoptysis, which may prove to be fatal. This case report describes a 9-year-old girl with severe pulmonary hypertension and right heart failure secondary to total occlusion of the right pulmonary artery. Detailed clinical examination and computed tomography (CT) angiography confirmed this diagnosis.
KeywordsPulmonary artery agenesis Pulmonary hypertension Takayasu’s arteritis
The authors thank Dr. Amithab Arya from Department of Nuclear Medicine, SGPGIMS, for providing SPECT CT lung perfusion images.
Video 1 Transthoracic echocardiogram in basal short-axis view showing dilated main and left pulmonary arteries with no right pulmonary artery shadow. (WMV 866 kb)
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