Pulmonary Arterial Hypertension Disease Summary

Overview

Pulmonary arterial hypertension is part of the spectrum of pulmonary hypertension (PH) which is a hemodynamic and pathophysiological condition, as stated in the Introduction section. The different types of pulmonary hypertension based on the mean PA pressure are enumerated in this section.

Pulmonary hypertension (PH) affects approximately 1% of the global population. A detailed discussion about the prevalence of pulmonary hypertension is in the Epidemiology section.

The Pathophysiology section states that pulmonary arterial hypertension is a syndrome resulting from restricted flow through the pulmonary arterial (PA) circulation, resulting in increased pulmonary vascular resistance and remodeling, and ultimately leading to right heart failure. 

Pulmonary Arterial Hypertension_Disease Summary

History and Physical Examination

The Clinical Presentation section enumerates the typical symptoms of pulmonary arterial hypertension. The signs seen in pulmonary arterial hypertension are listed in the Physical Examination.

Diagnosis

The Screening section discusses the recommendation as regards to at-risk individuals for early diagnosis, initiation of treatment and improvement of prognosis of patients with pulmonary arterial hypertension.

Discussion on non-invasive and invasive diagnostic tests for the detection of pulmonary hypertension is in the Laboratory Tests and Ancillaries and Imaging sections.  Diagnostic tests for pulmonary hypertension clinical classification and determination of associated causes of pulmonary arterial hypertension are also enumerated in this section.

Management

Clinical and hemodynamic assessments for the determination of prognosis and management of pulmonary arterial hypertension are discussed in the Evaluation section.

The treatment goal and strategy in patients with pulmonary arterial hypertension are stated in the Principles of Therapy section.

The Pharmacological Therapy section discusses in detail the treatment options in patients with pulmonary arterial hypertension, whether with positive or negative acute vasoreactivity. Options for monotherapy, combination therapy and supportive therapy are also explained in this section.

The Nonpharmacological section includes general care and things to educate the patient about the management of pulmonary arterial hypertension.

Interventional therapies such as balloon atrial septostomy (BAS), Potts shunt and lung transplantation are in the Surgery section.

The Monitoring section identifies follow-up recommendations and assessments of patients with pulmonary arterial hypertension based on the patient’s history and clinical status.