Overview
Pulmonary arterial hypertension (PAH) is part of the spectrum of
pulmonary hypertension 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 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 PAH 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 SummaryThe Risk Factors section lists the different risk factors and associated conditions for PAH.
In the Classification section, PAH is classified under different groups depending on the cause.
History and Physical Examination
The Clinical Presentation section enumerates the typical symptoms of PAH. The signs seen in PAH 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 PAH.
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 PAH are also enumerated in this section.
As the symptoms of
PAH may overlap with other diseases, it is important to rule them out.
Conditions that should be considered are listed in the Differential Diagnosis
section.
Management
Clinical and hemodynamic assessments for the determination
of prognosis and management of PAH are discussed in
the Evaluation section.
The treatment goal and strategy in patients with PAH are stated in the Principles
of Therapy section.
The Pharmacological
Therapy section discusses in
detail the treatment options in patients with PAH,
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 PAH.
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 PAH based on the patient’s history and clinical
status.
