What is the best management approach for a 50-year-old male with hypertension, experiencing dyspnea on exertion, palpitations, dry cough, and edema, with normal chest X-ray and electrocardiogram findings?

Management of Suspected Pulmonary Hypertension in a Hypertensive Patient with Dyspnea on Exertion

This patient requires transthoracic echocardiography as the immediate next step to screen for pulmonary hypertension, followed by right heart catheterization if echocardiography suggests elevated pulmonary pressures, as normal chest X-ray and ECG do not exclude pulmonary hypertension. 1

Initial Diagnostic Approach

Why Standard Tests Are Insufficient

• Normal chest X-ray does not exclude pulmonary hypertension (PH), though 90% of patients with idiopathic PAH have abnormal findings at diagnosis, including central pulmonary arterial dilatation with peripheral vessel pruning and right heart enlargement 1
• Normal ECG similarly does not rule out PH, as ECG abnormalities (right axis deviation, RV hypertrophy, RV strain) are more likely in severe rather than mild disease, with insufficient sensitivity (55%) despite high specificity (70%) 1
• The combination of dyspnea on exertion, palpitations, dry cough, and edema in a hypertensive patient raises concern for either pulmonary arterial hypertension or PH secondary to left heart disease 1

Essential First-Line Testing

Transthoracic echocardiography must be performed immediately as the noninvasive screening test to detect pulmonary hypertension and evaluate right ventricular function 1

Key echocardiographic findings to assess:

• Estimated right ventricular systolic pressure (RVSP) >40 mmHg warrants further evaluation in patients with unexplained dyspnea 1
• Right atrial or right ventricular enlargement 1
• Interventricular septal flattening 1
• Left ventricular systolic and diastolic function to distinguish Group 2 PH (left heart disease) from other causes 1

Critical Diagnostic Pathway

If Echocardiography Suggests Pulmonary Hypertension

Right heart catheterization (RHC) is mandatory before initiating any PH-specific therapy, as echocardiography alone is insufficient to confirm diagnosis and guide treatment 1, 2

RHC establishes:

• Mean pulmonary artery pressure (mPAP) ≥25 mmHg at rest 1, 2
• Pulmonary capillary wedge pressure (PCWP) ≤15 mmHg to exclude left heart disease 1, 2
• Pulmonary vascular resistance (PVR) >3 Wood units to confirm true pulmonary vascular disease rather than passive elevation from high cardiac output or left heart dysfunction 1, 2

Distinguishing PH Etiologies

The transpulmonary gradient (mPAP minus wedge pressure) and PVR are critical because they distinguish passive PH from pulmonary vascular disease 1

For this hypertensive patient specifically:

• If PCWP >15 mmHg: PH is likely secondary to left heart disease (Group 2), which is the most common cause in general cardiology populations 1
• If PCWP ≤15 mmHg and PVR >3 Wood units: Consider pulmonary arterial hypertension or other causes requiring additional workup 1, 2

Additional Required Testing

To Exclude Chronic Thromboembolic PH

Ventilation-perfusion (V/Q) scanning must be performed to rule out chronic thromboembolic pulmonary hypertension (CTEPH), as a normal scan effectively excludes this diagnosis with sensitivity >90% and specificity >94% 1, 2, 3

• CTEPH requires specific surgical management (pulmonary thromboendarterectomy) and has different therapeutic implications 1, 3
• CT pulmonary angiography is less sensitive than V/Q scanning for CTEPH detection 1

Pulmonary Function Testing

Pulmonary function tests with DLCO should be obtained to evaluate for underlying obstructive or restrictive lung disease (Group 3 PH) 1, 2

• Patients with PAH typically show mild-to-moderate reduction in lung volumes and decreased DLCO 1
• Arterial blood gases usually demonstrate normal or slightly decreased PaO2 with decreased PaCO2 due to alveolar hyperventilation 1

Screening for Associated Conditions

Testing for connective tissue disease and HIV infection should be performed in patients with unexplained PAH 1

Additional laboratory evaluation:

• Liver function tests 4
• Complete blood count 4
• Thyroid function tests 4
• Antinuclear antibody serology if connective tissue disease suspected 1

Management Based on Etiology

If PH Secondary to Left Heart Disease (Most Likely)

Optimize treatment of the underlying hypertension and any left ventricular dysfunction identified on echocardiography 3, 4

• Continue current antihypertensive medications with goal BP <140/90 mmHg 5
• Consider increasing losartan dose to 100 mg daily if currently on lower dose 5
• Add diuretics for volume management if edema persists 3, 4
• Do not use PAH-specific vasodilators (phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclins) for Group 2 PH, as these target pulmonary vascular disease, not left heart disease 3

If Pulmonary Arterial Hypertension Confirmed

Immediate referral to a pulmonary hypertension specialist center is mandatory 1, 3, 4

• Vasoreactivity testing with short-acting agents (IV epoprostenol, adenosine, or inhaled nitric oxide) should be performed by experienced physicians 1
• Patients demonstrating favorable acute response (fall in mPAP ≥10 mmHg to ≤40 mmHg with increased or unchanged cardiac output) may be candidates for calcium channel blocker therapy 1
• Calcium channel blockers should never be used empirically without demonstrated acute vasoreactivity 1
• Functional class III patients not responding to calcium channel blockers require targeted PAH therapies (endothelin receptor antagonists, prostacyclin analogues, phosphodiesterase-5 inhibitors) 1, 3

If CTEPH Identified

Referral to centers experienced in pulmonary thromboendarterectomy is essential, as surgical endarterectomy is the treatment of choice for eligible patients 1, 3

Common Pitfalls to Avoid

• Never rely on echocardiography alone to initiate PH-specific therapy—right heart catheterization is required for definitive diagnosis 1, 2
• Do not assume normal chest X-ray and ECG exclude pulmonary hypertension—these tests lack sufficient sensitivity 1
• Avoid empiric use of PAH-specific vasodilators in patients with left heart disease, as the underlying pathophysiology differs and these medications are not indicated 3
• Do not skip V/Q scanning—missing CTEPH means missing a potentially curable form of PH with surgical intervention 1, 2
• Recognize that blood pressure control alone may not resolve symptoms if true pulmonary vascular disease is present, necessitating specialist evaluation 1, 3