What are the diagnostic steps and treatment options for pulmonary hypertension?

From the Guidelines

Diagnosing and treating pulmonary hypertension requires a systematic approach beginning with clinical suspicion based on symptoms like shortness of breath, fatigue, and chest pain, with initial diagnostic steps including echocardiography to estimate pulmonary artery pressure, followed by right heart catheterization to confirm the diagnosis (defined as mean pulmonary artery pressure ≥20 mmHg at rest) 1.

Diagnostic Steps

The diagnostic process for pulmonary hypertension involves several key steps:

• Clinical suspicion based on symptoms such as dyspnea, fatigue, chest pain, syncope, or edema
• Echocardiography to estimate pulmonary artery pressure
• Right heart catheterization to confirm the diagnosis and assess hemodynamics
• Additional testing, including pulmonary function tests, chest imaging, ventilation-perfusion scanning, and blood tests to determine the underlying cause and classify the type of pulmonary hypertension
• Classification of pulmonary hypertension into different types, such as pulmonary arterial hypertension (PAH), pulmonary hypertension due to left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH)

Treatment Options

Treatment of pulmonary hypertension depends on the specific classification and underlying cause, but may include:

• Oxygen therapy for hypoxemia
• Diuretics, such as furosemide (20-80 mg daily), for fluid overload
• Anticoagulation with warfarin (target INR 2.0-3.0) in certain cases
• Specific pulmonary vasodilator therapies, including:

• Phosphodiesterase-5 inhibitors, such as sildenafil (20 mg three times daily)
• Endothelin receptor antagonists, such as bosentan (62.5-125 mg twice daily)
• Prostacyclin analogs, such as epoprostenol (starting at 2 ng/kg/min by continuous infusion)
• Soluble guanylate cyclase stimulators, such as riociguat (up to 2.5 mg three times daily)

• Combination therapy is often required for optimal management
• Regular monitoring with 6-minute walk tests, echocardiography, and laboratory tests to assess treatment response
• Lung transplantation may be considered for severe cases unresponsive to medical therapy 1.

Key Considerations

It is essential to rule out CTEPH, even in patients with underlying risk factors for PAH, as the therapeutic implications of the diagnosis are substantial 1. The treatment aims to reduce pulmonary vascular resistance, improve right ventricular function, and enhance quality of life by targeting the pathophysiological mechanisms of vasoconstriction, cellular proliferation, and thrombosis that characterize this progressive disease 1. Referral to a specialized center is strongly recommended for the management of patients with pulmonary hypertension due to the complexity of the diagnostic evaluation and treatment options available 1.

From the FDA Drug Label

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From the Research

Diagnostic Steps for Pulmonary Hypertension

• The diagnosis of pulmonary hypertension involves a stepwise approach, with the main aim of discerning those patients who need to be referred to a PH centre and who should undergo invasive haemodynamic assessment 2.
• Echocardiography is recommended as the initial step in the evaluation of patients with suspected pulmonary hypertension 3.
• A definitive diagnosis cannot be made on echocardiographic abnormalities alone, and some patients require invasive evaluation by right heart catheterization 3.
• Noninvasive examinations, such as computed tomography (CT) and magnetic resonance (MR) imaging, may support diagnosis of PH before proceeding to RHC and play an important role in management and treatment of the disease 4.

Treatment Options for Pulmonary Hypertension

• The development of disease-specific therapies for pulmonary arterial hypertension over the past decade underscores the importance of diagnosing pulmonary hypertension early in the course of the condition and implementing a treatment strategy that is based on the condition's cause and severity 5.
• For certain categories of pulmonary hypertension, particularly pulmonary arterial hypertension, treatment options are rapidly evolving, and early diagnosis and prompt referral to an expert center are critical to ensure the best prognosis 3.
• There are no directed therapies for many other categories of pulmonary hypertension; therefore, family physicians have a central role in managing contributing comorbidities 3.
• Other important considerations for patients with pulmonary hypertension include influenza and pneumonia immunizations, contraception counseling, preoperative assessment, and mental health 3.

Classification and Diagnosis

• Pulmonary hypertension (PH) is a haemodynamic condition characterised by elevation of mean pulmonary arterial pressure (mPAP) >20 mmHg, assessed by right heart catheterisation 2.
• Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH, isolated post-capillary PH and combined post- and pre-capillary PH 2.
• The core structure of the clinical classification of PH has been retained, including the five major groups, with some changes such as the re-introduction of "long-term responders to calcium channel blockers" as a subgroup of idiopathic pulmonary arterial hypertension 2.