Discovery of agenesis of the right pulmonary artery on pulmonary scintigraphy in a context of pulmonary hypertension

We present pulmonary scintigraphy with computed tomography (SPECT-CT) images in a 68-year-old male with severe pulmonary hypertension (PAPs = 75 mmHg) discovered in the course of his mitral valvuloplasty follow-up (mitral valve prolapse by cord rupture), with no other particular medical history, necessary to rule out an embolic cause, which shows a complete absence of right lung perfusion with a normal ventilation. The CT slices of the SPECT-CT shows the absence of pulmonary artery, which explained the absence of functional perfusion of the lung. We concluded that the patient's pulmonary hypertension could be explained by this late-discovered agenesis that had been asymptomatic for more than 60 years.