Correct
4. 1 and 3
Formally assessing the patient for pulmonary hypertension with right heart catheterization is appropriate given the remote history of fenfluramine/phentermine (“Fen-Fen”) use as well as two echocardiograms, and a chest CT, showing features suggesting pulmonary hypertension. Cerebral angiography is appropriate to assess for vasculitis and embolic sources, particularly given the brain MRI showing embolic phenomenon. While the recent brain and head and neck MRA showed no evidence of either vasculitis or embolic sources, catheter cerebral angiography may be more sensitive for the detection of such abnormalities. Brain biopsy is not indicated as the patient reportedly responded well to immunosuppressive therapy for presumed central nervous system vasculitis and the risk of performing a brain biopsy may not be outweighed by any information obtained by the procedure, at least at this patient with the patient’s presentation. Furthermore, the appearance of the brain MRI is more suggestive of an embolic phenomenon than a cerebral vasculitis, and the cerebral angiogram results may further inform the need for brain biopsy and should be performed before this invasive procedure is undertaken in this circumstance.
Cerebral angiography was performed and showed no abnormalities. Neurosurgery was consulted but declined brain biopsy, and neurology concurred with this assessment. Cardiology was consulted and right heart catheterization was performed, which showed a low cardiac index of 1.5 L/min/m2 and a mean pulmonary arterial pressure of 62 mmHg, the latter unaffected by nitric oxide infusion.
The pulmonary capillary wedge pressure was normal. At this point, which of the following represents the most appropriate step in this patient’s management? (Click on the correct answer to proceed to the eleventh of twelve pages)