Two patients with severe PAH secondary to two different etiology, one is due to Systemic sclerosis and other one is due to COPD....presented with cor pulmonale and evidence of right heart failure.
In the first patient, I would test reacitivity of pulmonary vessels with prostacyclin and the stardt treatment with specific drugs (depending on severity of PH and reaction during test). In the second patient, oxygen administration would be probably the best choice and should be again tested acutely. Dosing depends on type of respirátory insufficiency (partial or global).
The SSc patients are (most of the time) difficult cases:
- Some patients may have myocardial involvment and HFpEF
- Some patients may have ILD
As a consequence and depending on patients age and general condition, I would suggest to perform a left + right heart cath with fluid challenge or exercise. Vasoreactivity testing received only a class IIb recommandation in PAH-SSc as these patients are classicaly not vasoreactive. If the diagnosis is confirmed, I would also treat (after control of cardiac rythme and appropriate volume depletion) with a specific drug with a preference for an initial upfront bitherapy (as Group 1.4.1 PAH present usually with severe hemodynamic impairment and worse prognosis) and no hesitation to move with a triple combination if unmet treatment goals.
The COPD case is more debatable. No doubt oxygen therapy is part of the treatment if PO2 is below 60 mmHg, with diuretics. The question here is mainly the "COPD-PH" discrepancy...
Best regards.
FL
In SScl PAH is indeed a complex diagnosis; next to myocardial involvement also the pulmonary venous compartment can de involved; ie pulmonary venous occlusive disease. And, ILD may be present and complicate the clinical picture.
So, full work-up should include left and right heart cath; be careful with vasoreactivity given the possibility for PVOD. In case of PAH without any signs of PVOD treat as suggested. In case of PVOD refer to a highly experienced PH center.In case of concomitant ILD PAH treatment may de of benefit in selected cases.
COPD-PH; treat the COPD and the cor pulmonale with the usual medication and oxygen. No prove that PAH-drugs have a role in the treatment of these patients.
Best regards, Paul Bresser
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