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Pulmonary Hypertension - Scottish Pulmonary Vascular Unit - Health Care Professional Section

 

WHAT IS PH?

REFERRAL GUIDELINES

TREATMENT

CLINICAL TRIALS

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WHAT IS PH? : MANAGEMENT

DEFINITION AND CLASSIFICATION / PATHOPHYSIOLOGY / EPIDEMIOLOGY / CLINICAL PRESENTATION / MANAGEMENT / NATURAL HISTORY


The management of a patient with pulmonary hypertension depends upon the aetiology and the broad principles of management are shown in the table below. Patients who could possibly be in classes I, IV and V should be referred to the Scottish Pulmonary Vascular Unit for further investigation.

 

Class Treatment
I. Pulmonary arterial hypertension Refer to SPVU
Prostanoids, endothelin receptor antagonists, phosphodiesterase inhibitors
II. PH due to left heart disease Optimise treatment of underlying disease (by patient's local cardiologist)
III. PH due to lung diseases / hypoxaemia Optimise treatment of underlying disease (by patient's local respiratory physician)
IV. Chronic thromboembolic pulmonary hypertension Refer to SPVU
All CTEPH patients are referred to the national Pulmonary Endarterectomy MDT for decision regarding operability, otherwise treated as PAH
V. Miscellaneous Refer to SPVU
 
Supportive therapies are considered for all patients in groups 1, 4 and 5. These include diuretics and LTOT. The expert consensus is that anticoagulation with warfarin should be considered for all IPAH patients. Lifelong anticoagulation for all patients with CTEPH is essential

Further general measures include managing arrhythmias, avoiding pregnancy and improving physical fitness with exercise/pulmonary rehabilitation (within the limitations of symptoms). Suitability for lung transplantation is also considered in those with advanced or deteriorating PH.

We view our involvement in clinical trials as a key role in the management of PH. See ?Clinical Trials? pages for more information.

 

 

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