Pulmonary hypertension medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: Ralph Matar,

Overview

Treatment of pulmonary hypertension has passed through a dramatic evolution in the past few years,in part owing to advances in the understanding of the basic pathophysiological contributors to the disease. However, despite all the modern therapeutic agents, pulmonary hypertension remains a chronic disease with no cure.

Before prescribing any medication, the physician must assess the severity, consider supportive treatment and lifestyle changes, then consider the combination of different drugs and the possibility of further interventions.

Treatment Goals

• Improving the patient's symptoms.
• Enhancing functional capacity.
• Lowering Pulmonary arterial pressure and normalizing cardiac output.
• Prevent or at least slow the progression of the disease.
• Decrease the hospitilization rate.
• Improve Survival.

ESC/ERS Recommendations for General measures:

“

Class I 1. It is recommended to avoid pregnancy in patients with PAH. (Level of Evidence: C) 2. Immunization of PAH patients against influenza and pneumococcal infections is recommended (Level of Evidence: C) Class IIa 1. Physically deconditioned PAH patients should be considered for supervised exercise rehabilitation (Level of Evidence: B) 2. Psychosocial support should be considered in patients with PAH (Level of Evidence: C) 3. In-flight oxygen administration should be considered for patients in WHO-FC III and IV and those with arterial oxygen pressure consistently less than 60mmHg (Level of Evidence: C) 4. Epidural anesthesia instead of general anesthesia should be utilised if possible for elective surgery (Level of Evidence: C) Class III 1. Excessive physical activity that leads to distressing symptoms is not recommended in patients with PAH(Level of Evidence: C)'

”

ESC/ERC Recommendations for supportive therapy:

“

Class I 1.Diuretic treatment is indicated in PAH patients with signs of RV failure and fluid retention. (Level of Evidence: C) 2. Continous long-term oxygen therapy is indicated in PAH patients when arterial oxygen pressure is consistently less than 60mmHg. (Level of Evidence: C) Class IIa 1. Oral anticoagulant treatment should be considered in patients with IPAH, heritable PAH, and PAH due to use of anorexigens (Level of Evidence: C) Class IIb 1. Oral anticoagulant treatment should be considered in patients with APAH (Level of Evidence: C) 2. Digoxin may be considered in patients with PAH who develop atrial tachyarrhythmias to slow ventricular rate. (Level of Evidence: C)

”

Recommendations for specific medical therapy

Table 1:Recommendation for specific drug therapy according to WHO-FC

Medication	WHO-FC II	WHO-FC III	WHO-FC IV
Calcium channel blockers	I-C	I-C	--
Ambrisentan	I-A	I-A	IIa-C
Bosentan	I-A	I-A	IIa-C
Sitaxentan	IIa-C	I-A	IIa-C
Sildenafil	I-A	I-A	IIa-C
Tadalafil	I-B	I-B	IIa-C
Beraprost	--	IIb-B	--
Epoprostenol(IV)	--	I-A	I-A
Iloprost(inhaled)	--	I-A	IIa-C
Iloprost(IV)	--	IIa-C	IIa-C
Treprostinil(subcutaneous)	--	I-B	IIa-C
Treprostinil(IV)	--	IIa-C	IIa-C
Treprostinil(Inhaled)	--	I-B	IIa-C
Initial drugs combination therapy	--	--	IIa-C
Sequential drugs combination therapy	IIa-C	IIa-B	IIa-B

Recommendations for PAH associated with congenital cardiac shunts

“

Class I 1. Bosentan(Endothelin receptor antagonist) is indicated in WHO-FC III patients with Eisenmenger syndrome(Level of Evidence: B) Class IIa 1. Other endothelin receptor antagonist, phosphodiesterase inhibitors, and prostanoids should be considered in patients with Eisenmenger's syndrome (Level of Evidence: C) 2. In the absence of significant haemoptysis, oral coagulant treatment should be considered in patients with PA thrombosis or signs of heart failure.(Level of Evidence: C) 3. The use of supplemental oxygen therapy should be considered in cases in which it produces a consistent increase in arterial oxygen saturation and reduces symptoms (Level of Evidence: C) 4. If symptoms of hyperviscosity are present, phlebotomy with isovolumic replacement should be considered usually when the haematocrit is >65%(Level of Evidence: C) Class IIb 1. Combination therapy may be considered in patients with Eisenmenger's syndrome. (Level of Evidence: C) Class III 1. The use of CCB is not recommended in patients with Eisenmenger's syndrome.(Level of Evidence: C)'

”