Treatmentof

Pulmonary Arterial Hypertensionin children

Mohammad Rezaei

Pediatric Pulmonologist

General Measures

children often have a more reactive pulmonary vascular bed than adult

aggressive therapy for acute pulmonary hypertensive crises

Influenza and pneumococcal vaccinations

Antipyretics

URI (antitussive medications / Decongestants with pseudoefedrine)

prevent constipation (& Valsalva maneuvers)

Anticoagulation

lung histopathology: often demonstrates thrombotic lesions in small pulmonary arterioles

Poor right ventricular function → thrombi in ventricle

Anticoagulation??

Warfarin (pediatric study??) / LMWH?

Our approach: hypercoagulable or in right-sided heart failure

Antiplatelet therapy ??

Calcium Channel Blockade

Acute vasodilator testing (Responder or Non-responder)

Response to vasodilator testing:

(decrease in mean PAP by ≥20% from baseline with no clinically significant

decrease in cardiac output, and a decrease or no change in the ratio of PVR to

SVR)

current definition for an acute responder in adult PAH:

(decrease in mean PAP by >10mm Hg to an absolute value of <40mm Hg with

no clinically significant decrease in cardiac index)

Calcium Channel Blockade

Advers effects

Dosage

Serial Reevaluations

not all acute “responders” have a sustained long-term response

Prostacyclin Analogs

(1) be a pulmonary vasodilator

(2) inhibit platelet aggregation

(3) inhibit proliferation of vascular smooth muscle

(4) improve endothelial dysfunction

(5) be a possible cardiac inotrope.

Prostacyclin Analogs

Epoprostenol

Approved:1995 / gold standarad /improves…../

↓ PAP , ↑ cardiac/output , ↑O2 transport

These effects occur with long-term use, even if there is no acute response to

vasodilator testing

Dosage / rout of use / complication

Iloprost

Treprostinil

Endothelin Receptor Antagonists

Endothelin → vasoconstrictor

ETA & ETB

Bosentan

sitaxsentan

Ambrisentan

Check LFT

Nitric Oxide

Selective relaxing pulmonary vessels (inhaled NO)

Antiprolifrative effect on smooth muscle

Inhibit PLT adhision

Usage: treatment PPHN , IPAH exacerbation/ determine operability /

perioperative pulmonary HTN/ other form of PAH

Not approved

Phosphodiesterase Inhibitors

PDE-5 → ↑cGMP → ↑NO

Sildenafil

Tadalafil

Gene Therapy

HPAH gene (mutation in the BMPR2 protein at 2q33)

induce the overexpression of vasodilator genes

Oxygen

Sleep → hypoventilation → modest systemic arterial o2 desaturation

Usually during the early morning houers

Usage of supplemental oxygen

Digoxin

Diuretics

antiarrhythmic

Inotropics

Nitrates

Atrial septostomy

Transplatation

Treatment algorithm

ACCF/AHA 2009

Expert Consensus Document on

Pulmonary HypertensionA Report of the American College of Cardiology Foundation Task Force

onExpert Consensus Documents and the American Heart Association

Treatment algorithm