At the Bedside

  • Think of PH in dyspnea, syncope/presyncope, chest pain, edema, exertional intolerance, known connective tissue disease, congenital heart disease, portal HTN, HIV, stimulant use, chronic thromboembolic disease, or known PAH on prostacyclin/vasodilators.
  • Initial assessment: ABCs, monitor, IV access, pulse ox, telemetry. Avoid overexertion and unnecessary delays; these patients can deteriorate with stress and hypoxemia.
  • Exam clues: loud P2, RV heave, JVD, hepatomegaly, peripheral edema, cool extremities, hypotension, signs of low output.
  • Testing: ECG (right axis deviation, RV strain, RVH, RBBB), CXR (enlarged pulmonary arteries, RV enlargement, alternative diagnoses), BNP/troponin, VBG/ABG if ill, CMP/CBC, lactate if shock, bedside echo if available. Echo findings that matter: RV dilation, septal flattening, reduced RV function, elevated RV pressures, small LV from underfilling.
  • Search for triggers: PE, pneumonia, ACS, arrhythmia, anemia, pregnancy-related strain, medication interruption, volume overload, sepsis.
  • Oxygenation/ventilation: give oxygen for hypoxemia. Avoid intubation if possible; positive pressure and induction can precipitate collapse by dropping preload and worsening RV afterload. If ventilatory failure is unavoidable, involve expert help early and prepare for hemodynamic collapse.
  • Hemodynamics: in decompensated RV failure, cautious fluids only if clearly preload responsive; many patients worsen with volume loading. Start vasopressors early if hypotensive—norepinephrine is commonly first-line; vasopressin can be helpful as an adjunct.
  • Pulmonary vasodilator rescue: if patient already uses inhaled nitric oxide or epoprostenol, continue it if possible; abrupt interruption can cause severe rebound PH. Consider ICU-level rescue therapies and specialist consultation.
  • Disposition: most symptomatic, hypoxemic, hypotensive, or decompensated patients need ICU/step-down with pulmonary/cardiology involvement. Stable known PH with mild symptoms still deserves a low threshold for admission if there is syncope, biomarker elevation, RV dysfunction, or trigger requiring treatment.
A Classic Presentation
A 34-year-old woman with known idiopathic pulmonary arterial hypertension on continuous prostacyclin infusion presents with progressive dyspnea, lightheadedness, and near-syncope after her pump alarmed overnight. She is tachycardic, mildly hypoxic, with elevated JVD, a loud P2, cool extremities, and borderline blood pressure. ECG shows right axis deviation and RV strain, BNP is elevated, and bedside echo demonstrates a dilated RV with septal flattening. She is admitted to the ICU, prostacyclin infusion is immediately restored, oxygen is given, and norepinephrine is started for shock.
Patient Presentation to Attending
How you’d present this patient on the floor
This is a 34-year-old woman with known pulmonary arterial hypertension on prostacyclin presenting with dyspnea and presyncope since overnight, and my main concern is acute RV failure from decompensated pulmonary hypertension, especially because her pump alarmed and she missed therapy. She’s had worsening exertional intolerance, lightheadedness, and mild chest pressure, but no fever or pleuritic pain to make me think infection or PE is the primary driver right now. On exam she’s tachycardic, mildly hypoxic, has JVD and a loud P2, and her extremities are cool with borderline blood pressure. ECG shows RV strain/right axis deviation, BNP is up, and bedside echo shows a dilated RV with septal flattening. Overall this looks like decompensated PH with RV failure, so we’ve restored her prostacyclin, given oxygen, and I’m starting vasopressor support while involving ICU and PH/cardiology for disposition.

Study Directive

  • Draw the RV failure spiral from memory: hypoxia/acidosis → ↑PVR → RV dilation → septal shift → ↓LV filling → hypotension → coronary hypoperfusion → worse RV failure.
  • Practice a 3-minute decompensated PH algorithm: oxygen, minimize agitation, restore PH meds, early pressors, cautious fluids, ICU consult.
  • Review the bedside echo signs of RV strain: RV/LV ratio, septal flattening, TAPSE, TR jet, IVC congestion.
  • Write out which conditions to actively search for in PH decompensation: PE, sepsis, arrhythmia, anemia, pregnancy, medication interruption.
  • Memorize the first-line pressor choices and why norepinephrine/vasopressin are preferred over pure beta-agonists in hypotensive RV failure.

Recent Literature