43-year-old female with idiopathic PAH

Images obtained in a 43-year-old female with idiopathic PAH. (A) S1Q3T3 pattern noted consistent with right ventricle strain. T-wave inversion on anterior leads and ST depression is also suggestive of RVH with strain (note early R wave predominance in V1-V2 consistent with RVH). (B) Chest X-ray showing enlarged pulmonary artery and pruning of the distal pulmonary vasculature. (C) Lateral chest X-ray showing filling of the retrosternal space by an enlarged RV. (D) Parasternal long axis echocardiography view showing dilated RV. (E) Parasternal short axis echocardiography view showing flatted interventricular septum (thin white arrow), severely dilated RV and pericardial effusion (thick white arrow). (F) Tricuspid regurgitation velocity (represented by *) is proportional to right ventricular systolic pressure and estimated by Bernoulli's equation. (G) Measurement of PAAT (time from onset of flow to peak velocity) (thin white arrow). Note the notching of the PA Doppler envelope, suggestive of pulmonary vascular hypertension (thick white arrow). (H) "Moth eaten" appearance of ventilation/ perfusion scan in idiopathic PAH with patchy perfusion defects, in this case predominantly in the left lower lobe. (I, J) Histopathology from a different patient with Category 1 PAH showing medial hypertrophy and intimal fibrosis of small (<200 m) PAs

Additional keywords: mPAP,electrocardiogram,echocardiography,x-ray,histology