Abstract

UNRAVELING PULMONARY ARTERIAL HYPERTENSION: ETIOLOGY, DRUG ASSOCIATIONS, AND ADVANCES IN DIAGNOSIS AND MANAGEMENT

Muzaffar Khushboo*, Fazili Aamirah and Shafi Tawqeer

Abstract

Pulmonary Arterial Hypertension (PAH) is an uncommon yet severe pulmonary condition marked by increased pressure within the arteries of the pulmonary system, which transport blood from the circulatory system to the pulmonary. The heart must work harder to pump blood because of the increased pressure, which eventually strains the right side of the heart and may result in right-sided heart failure. PAH can be categorized into several types, each with its own specific causes and risk factors. Idiopathic PAH, the most common form, has no known cause. Heritable PAH is a genetic form of the disease, often passed down through families. Drug-induced PAH can occur as a side effect of certain medications, particularly appetite suppressants. PAH may also occur in conjunction with other medical conditions, including coagulation problems such as sclerosis, SLE and HIV infection. The symptoms of Pulmonary Arterial Hypertension (PAH) can differ significantly from one person to another and may encompass breathlessness, exhaustion, chest discomfort, a rapid pulse, syncope spells, and swelling in the lower limbs or ankles. Diagnosing PAH generally requires a thorough assessment, including a detailed medical history, physical examination, and diagnostic tests such as an echocardiogram, electrocardiogram, chest radiography, and lung function tests. A conclusive diagnosis is obtained via right heart catheterization—a specialized procedure in which a slender catheter is guided through a blood vessel in the neck or groin and advanced into the heart to precisely measure blood pressure within the pulmonary artery. The treatment approach for Pulmonary Arterial Hypertension (PAH) focuses on alleviating symptoms, slowing disease progression, and enhancing overall quality of life. Therapeutic agents, such as vasodilators and endothelin receptor blockers, work by relaxing vascular tissues and lowering pressure within the pulmonary arteries. In certain cases, surgical intervention may be warranted to repair or replace compromised heart valves. Additionally, lifestyle adjustments—including consistent physical activity, a balanced diet, and avoiding tobacco use—can aid in managing the condition. Although a definitive cure for PAH remains elusive, ongoing research and medical advancements are paving the way for more promising outcomes for individuals facing this challenging disease.

Keywords: Pulmonary Hypertension, Catheterization, Endothelin receptor blockers.