Treatment of pediatric pulmonary hypertension

Abstract

Pulmonary hypertension was once thought to be a rare condition and only managed in specialized centers. Now however, with the advent of echocardiography, it is found in many clinical scenarios, in the neonate with chronic lung disease, in the acute setting in the intensive care unit, in connective tissue disease and in cardiology pre-and postoperatively. We have a better understanding of the pathological process and have a range of medication which is starting to be able to palliate this previously fatal condition. This review describes the areas that are known in this condition and those that are less familiar. The basic physiology behind pulmonary hypertension and pulmonary vascular disease is explained. The histopathologic process and the various diagnostic tools are described and are followed by the current and future therapy at our disposal.

Survival in Childhood Pulmonary Arterial Hypertension

“…1,2 Common symptoms include DOE, fatigue, syncope and presyncope, poor appetite, and poor growth. 1,2 Echocardiography is considered the best screening tool while right heart catheterization with vasoreactivity testing remains the gold standard for diagnosis and prognosis. 2,3 Acute management may include oxygen, inhaled nitric oxide, milrinone, mechanical ventilation to control pH and pCO 2 , sedation, and even extracorporeal membrane oxygenation.…”

“…2,3 Acute management may include oxygen, inhaled nitric oxide, milrinone, mechanical ventilation to control pH and pCO 2 , sedation, and even extracorporeal membrane oxygenation. 2 Potential maintenance therapies include digoxin, calcium channel blockers, phosphodiesterase V inhibitors, prostacyclin analogues, and endothelin receptor antagonists, though many of these therapies are not well studied in pediatrics. [1][2][3] Corticosteroids and anticoagulants are also indicated in some patients.…”

“…2 Potential maintenance therapies include digoxin, calcium channel blockers, phosphodiesterase V inhibitors, prostacyclin analogues, and endothelin receptor antagonists, though many of these therapies are not well studied in pediatrics. [1][2][3] Corticosteroids and anticoagulants are also indicated in some patients. 2 Surgical therapies include atrial septostomy and lung or heart/lung transplantation for severe disease.…”

“…[1][2][3] Corticosteroids and anticoagulants are also indicated in some patients. 2 Surgical therapies include atrial septostomy and lung or heart/lung transplantation for severe disease.…”