Management of Severe Pulmonary Hypertension Secondary to Heart Failure with Preserved Ejection Fraction

Diagnostic Confirmation and Treatment Strategy

The European Respiratory Society recommends confirming the diagnosis of pulmonary hypertension secondary to left heart disease (PH-LHD) through right heart catheterization with careful measurement of pulmonary capillary wedge pressure (PCWP) and left ventricular end-diastolic pressure (LVEDP) 5, 6
The American College of Cardiology suggests that a PCWP >15 mmHg confirms post-capillary pulmonary hypertension secondary to left heart disease 4, 5, 6
The clinical presentation of PH-LHD, including age >65 years, concentric LV hypertrophy, symptoms of heart failure, and dilated right heart chambers, is supported by the European Respiratory Journal 1, 2, 7

The European Respiratory Journal recommends initiating loop diuretics immediately to reduce left ventricular filling pressures and pulmonary venous congestion 1, 2, 3
The American College of Cardiology suggests starting with furosemide 40-80 mg IV daily (or equivalent bumetanide/torsemide) and titrating to the lowest effective dose that relieves congestion without causing hypotension or worsening renal function 8, 9
The European Journal of Heart Failure recommends adding a thiazide diuretic (e.g., metolazone 2.5-5 mg daily) for sequential nephron blockade if there is an inadequate response to loop diuretics 9

The European Respiratory Journal suggests starting dapagliflozin 10 mg daily or empagliflozin 10 mg daily as first-line disease-modifying therapy 8
The DELIVER trial showed that dapagliflozin reduced worsening HF and cardiovascular death (HR 0.82, 95% CI 0.73-0.92) 8
The EMPEROR-PRESERVED trial showed that empagliflozin reduced HF hospitalization and CV death (HR 0.79, 95% CI 0.69-0.90) 8

The American College of Cardiology recommends optimizing blood pressure control targeting <130/80 mmHg using ACE inhibitors or ARBs 10, 5, 6
The European Respiratory Journal suggests starting with ACE inhibitor (e.g., lisinopril 10-40 mg daily) or ARB if ACE inhibitor intolerant (e.g., losartan 50-100 mg daily) 10, 6

The European Journal of Heart Failure recommends adding spironolactone 12.5-25 mg daily, particularly given the severe pulmonary hypertension 9, 8
The TOPCAT trial showed that spironolactone reduced HF hospitalizations (HR 0.83, 95% CI 0.69-0.99) 8

The European Respiratory Journal recommends avoiding pulmonary arterial hypertension-specific therapies (prostanoids, endothelin receptor antagonists, PDE-5 inhibitors) in patients with PH-LHD 1, 2, 3, 4
The American College of Cardiology suggests avoiding nondihydropyridine calcium channel blockers (diltiazem, verapamil) due to their negative inotropic effects 10, 8

The Journal of the American College of Cardiology recommends considering inhaled nitric oxide or inhaled epoprostenol if acute RV decompensation occurs 11

The European Respiratory Journal suggests screening for and aggressively managing common comorbidities that worsen PH-LHD, including sleep apnea syndrome, COPD, and metabolic syndrome 1, 2, 3

The American College of Cardiology recommends reassessing volume status, symptoms, and functional capacity every 2-4 weeks initially 8
The European Respiratory Journal suggests repeating echocardiography in 3-6 months to assess response (LV mass regression, RV function, estimated PA pressures) 8

The European Respiratory Journal recommends referring patients to an advanced heart failure center for consideration of LVAD or heart transplantation if they remain severely symptomatic despite maximal medical therapy 1, 2, 7
The Journal of the American College of Cardiology suggests that LVAD implantation has been shown to lower pulmonary pressures through LV unloading 1, 2, 7