What is the initial management for a 60-year-old male with bilateral leg swelling, concentric Left Ventricular Hypertrophy (LVH), grade 1 diastolic dysfunction, Left Ventricular Ejection Fraction (LVEF) of 56%, mild Tricuspid Regurgitation (TR), and moderate Pulmonary Arterial Hypertension (PAH)?

Initial Management of HFpEF with Moderate PAH and Bilateral Leg Edema

The initial management should prioritize aggressive diuretic therapy to relieve congestion, strict blood pressure control targeting underlying left ventricular hypertrophy, and critically—avoid PAH-specific therapies as this patient has pulmonary hypertension secondary to left heart disease (Group 2 PH), not primary pulmonary arterial hypertension.

Critical First Step: Confirm the Diagnosis

This patient has HFpEF (heart failure with preserved ejection fraction) with secondary pulmonary hypertension due to left heart disease, NOT primary pulmonary arterial hypertension. The clinical picture—60-year-old with concentric LVH, grade 1 diastolic dysfunction, LVEF 56%, and bilateral leg edema—strongly suggests Group 2 PH (PH-LHD) rather than Group 1 PAH 1. The presence of concentric LVH is a key structural left heart abnormality that raises suspicion for PH-LHD 1. Right heart catheterization is mandatory before considering any PAH-specific therapy to confirm the hemodynamic classification and measure pulmonary artery wedge pressure (PAWP) 2, 3.

Immediate Management Priorities

1. Aggressive Volume Management with Diuretics

Start intravenous loop diuretics at a dose of at least twice the patient's home oral dose if previously on diuretics, or initiate with furosemide 40-80 mg IV if diuretic-naive 1. The bilateral leg swelling indicates significant volume overload, which is a major determinant of ventricular filling pressure 1.

• Meticulous identification and control of fluid retention is the cornerstone of management 1
• Diuretics improve breathlessness and reduce cardiac filling pressures in HFpEF patients 1
• Monitor daily weights, strict intake/output, and electrolytes (sodium, potassium, magnesium) closely 1
• Caution: Patients with HFpEF may be particularly sensitive to preload reduction and can develop hypotension, especially in elderly patients 1
• Titrate diuretics to achieve euvolemia while avoiding excessive volume depletion 1

2. Blood Pressure and Heart Rate Control

Aggressive blood pressure control is essential as hypertension is the primary driver of concentric LVH and diastolic dysfunction 1.

• Target blood pressure should follow published guidelines for patients with structural heart disease 1
• Beta-blockers can provide symptomatic relief in HFpEF patients and help control heart rate 1
• Control of blood pressure, heart rate, blood volume, and myocardial ischemia are physiological factors known to exert important effects on ventricular relaxation 1
• The concentric LVH increases chamber stiffness and impairs diastolic function, making blood pressure control critical 4, 5

3. Identify and Treat Underlying Causes

Diseases known to cause HFpEF should be aggressively treated, including coronary artery disease, hypertension, and metabolic syndrome features 1.

• Evaluate for coronary artery disease if chest pain or ischemia is present 1
• Screen for metabolic syndrome components (obesity, diabetes, dyslipidemia) which are associated with PH-LHD 1
• Check thyroid function, complete blood count, renal function, liver function, and lipid profile 1
• Obtain 12-lead ECG looking for LVH, left atrial abnormality, atrial fibrillation, or Q waves 1

What NOT to Do: Avoid PAH-Specific Therapies

PAH-specific drugs (phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogs) are NOT recommended and may be harmful in patients with PH due to left heart disease 1, 2, 6.

• The ESC/ERS guidelines explicitly state there is no strong evidence-based recommendation for PAH therapies in PH-LHD 1
• A multicenter trial with riociguat showed no benefit in PH due to systolic heart failure 1
• Starting PAH-specific drugs empirically without confirming diagnosis via right heart catheterization can delay appropriate treatment and cause harm 2
• The primary goal must be to improve management of the underlying left heart condition prior to considering any specific measures to treat PH 1

Supportive Measures

Volume Status Optimization

• Assess volume status through jugular venous pressure, presence of pulmonary crackles, hepatomegaly, and peripheral edema 1
• Measure orthostatic blood pressure changes, weight, height, and calculate body mass index 1
• Obtain chest radiograph (PA and lateral) to assess for pulmonary edema, pleural effusion, and Kerley B lines 1

Echocardiographic Monitoring

• The initial echo shows grade 1 diastolic dysfunction with E/e' ratio that should be monitored 1
• Elevated E/e' ≥15 or other evidence of elevated filling pressures supports HFpEF diagnosis 1
• Monitor for progression of diastolic dysfunction, as LVH is a predictor for future development of severe diastolic dysfunction 4

Address Comorbidities

• Varicose veins are a common independent predictor of bilateral leg edema in cardiovascular disease patients and should be evaluated 7
• Exclude other causes of leg edema including venous insufficiency, medications (calcium channel blockers, NSAIDs), renal disease, or liver disease 8, 7
• The mild tricuspid regurgitation should be monitored but is likely secondary to elevated right ventricular pressures from PH-LHD 9

Follow-Up and Monitoring

Clinically target symptom reduction, principally by reducing cardiac filling pressures at rest and during exertion 1.

• Reassess volume status, symptoms, and functional capacity regularly 1
• Repeat echocardiography to monitor LV filling pressures, diastolic function, and pulmonary pressures 1
• Consider natriuretic peptide levels (BNP/NT-proBNP) for monitoring, though baseline values should be established 1
• If symptoms persist despite optimal medical management of left heart disease, then and only then should right heart catheterization be performed to definitively characterize the pulmonary hypertension 1, 2

Common Pitfalls to Avoid

• Do not empirically start sildenafil or other PAH medications without hemodynamic confirmation via right heart catheterization 2, 10
• Avoid excessive diuresis leading to hypotension, renal dysfunction, and electrolyte abnormalities 1
• Do not overlook atrial fibrillation, as patients with HFpEF may be particularly sensitive to loss of atrial kick 1
• Recognize that the moderate PAH is likely secondary to elevated left-sided filling pressures, not primary PAH 1