Acute decompensated pulmonary hypertension in ICU

Meet the patient

A woman with idiopathic PAH on background therapy arrives with hours of worsening dyspnoea and presyncope after missing iloprost doses yesterday. She is hypotensive in new atrial fibrillation, hypoxic on room air, with a raised JVP, cool peripheries and a high lactate. Bedside echo shows a dilated RV with septal shift.^[1]

The two questions that decide her next hour are the two that decide every PH crisis: why did the RV fail now? (a missed prostacyclin dose, new AF, hypoxia, acidosis, sepsis — the trigger you must find and reverse) and how do I drop the afterload the RV faces without dropping the systemic pressure it depends on for coronary perfusion? That tension — lowering PVR while defending SVR — is the entire management problem in one sentence.^[1]

References24ShowHide

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