64 years old male was referred to our clinic for assessment of dyspnea on exertion.
His past medical history included hypertension and hyperlipidemia.
His social history was significant for long standing smoking.
His medications included Amlodipine 5 mg, Losartan 50 and Atorvastatin 40 mg
Physical examination: Normal S1,S2; grade 2/6 SM at LSB; EDM at Left 2nd ICS
Echo: Normal LV and RV function; dilated ascending aorta (4.5cm), moderate AI; mild TR; mild pulmonary hypertension (PA pressure 35-40 mmHg) 1 year later repeat Echo Normal LV and RV function; mild to moderate pulmonary hypertension (PA pressure 50-55 mmHg) Following year echo shows: Normal LV function but dilated RV with mildly reduced systolic function; moderately severe pulmonary hypertension (PA pressure 65-70 mmHg)
Underwent CT chest which revealed moderate to severe cardiomegaly; dilated MPA which can be seen in setting of pulmonary arterial HTN; areas of mosaic attenuation predominantly left upper lobe; no evidence of pulmonary fibrosis.
Underwent Right and Left heart catheterization which revealed:
Patent coronaries; PAP 83/25; mean PAP 49 mmHg; PCWP10 mmHg.
RA mean 7 mmHg; C.O. 2.8l/m; CI 1.6l/m/m2.
Findings suggestive of primary pulmonary hypertension.
Vasoreactivity test was negative (Epoprosterenol did not change mean PAP).
Pulmonary Wedge arteriography in right lower lobe suggesting arteriolar fibrosis.
Patient was started on Macitentan 10 mg po qd.
In about a week patient had acute shortness of breath and syncope.
Was transferred to nearest Hospital ER.
Echo performed in ER showed dilated right ventricle with severe hypokinesis with apical sparing (McConnell's sign); septal flattening; moderate TR with severe pulmonary hypertension (Fig. 1, Fig. 2, Fig. 3, Fig. 4, Fig. 5).
CT showed pulmonary emboli.
Patient underwent thrombolysis and subsequent treatment with Xarelto 20 mg po qd for 6 months.
Repeat CT angio showed no evidence of thrombus.
Continued to be treated with Macitentan 10 mg po qd (ETA and ETB blocker).
Repeat Echo showed remarkable improvement in RV function with mild pulmonary hypertension (PA pressure 41-46 mmHg).
Most recent echo showed moderate pulmonary hypertension (PA pressure 50-55 mmHg).
Patient also felt dyspnea on exertion. Due to worsening of his symptom and his higher PA pressure, pulmonologist added Riociguat 0.5 mg po tid to his current regimen which is to be titrated up as tolerated.