Correct!
3. Cor pulmonale
The thoracic CT, performed as a CT pulmonary angiogram, shows the pre-exiting fibrotic lung disease to be stable, without new infiltrative abnormalities, but with new pericardial effusion, enlargement of the right atrium and right ventricle with leftward bowing of the interventricular septum; the latter findings are consistent with acutely worsening pulmonary hypertension and right ventricular failure. The absence of new ground-glass opacity and / or consolidation superimposed on the fibrotic lung disease, excludes an acute exacerbation of the fibrotic lung disease. The left ventricle does not appear enlarged at CT and no interlobular septal thickening is evident, which argues against increased pressure edema. Pulmonary arterial dissection is a rare entity that is most commonly encountered in the context of pulmonary hypertension, but the characteristic appearance of pulmonary arterial dissection- a linear “intimal flap” within the pulmonary artery, is not present.
The patient underwent repeat transthoracic echocardiography which showed an estimated right ventricular systolic pressure of 115 mmHg, an estimated mean pulmonary arterial pressure of 78 mmHg, and severe right ventricular enlargement and dysfunction with a “D-shaped” right ventricle and a moderate pericardial effusion. The patient’s oxygen saturation was 76% on room air, increasing to 91% by mask. Repeat right heart catheterization showed a mean pulmonary arterial pressure of 62 mmHg, pulmonary capillary wedge pressure of 32 mmHg (thought to be attributable to impaired left ventricular filling owing to leftward bowing of the interventricular septum and marked enlargement of the right ventricle), and a pulmonary vascular resistance of 12 Wood units. The patient was treated with aggressive diuresis, maintained on mycophenolate mofetil and oxygen therapy, and inhaled prostacyclin therapy was started, with a good response. After three months, repeat transthoracic echocardiography showed reduction in the now-mild right ventricular enlargement and return of a normal configuration of the interventricular septum, with right ventricular end-systolic pressure decreased to 63 mmHg.
Diagnosis: Interstitial lung disease associated with systemic sclerosis with development of pulmonary hypertension
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