Pulmonary Vascular Disease as a Systemic and Multisystem Disease

Area of Science:

• Cardiology
• Pulmonology
• Vascular Biology

Background:

• Pulmonary arterial hypertension (PAH) involves progressive pulmonary vascular remodeling.
• It stems from abnormal cell proliferation and endothelial dysfunction.
• PAH presents as a multisystem disease with systemic effects.

Purpose of the Study:

• To comprehensively review the chronic heart failure syndrome in PAH.
• To explore the systemic manifestations and complications of PAH.

Main Methods:

• Literature review of studies on PAH and its systemic effects.
• Synthesis of information on cardiovascular, muscular, inflammatory, endocrine, and neurological aspects.
• Analysis of the liver-gut axis, sleep, and metabolic changes.

Main Results:

• PAH leads to right ventricle-pulmonary artery uncoupling and neurohormonal activation.
• Systemic consequences include effects on skeletal/respiratory muscles, endothelial dysfunction, and coronary artery disease.
• Other manifestations involve inflammation, infection, endocrine/metabolic changes, liver-gut axis disruption, sleep disturbances, neurologic issues, and skin/iron metabolism alterations.

Conclusions:

• PAH extends beyond the pulmonary vasculature, significantly impacting systemic physiology.
• Understanding these multisystem effects is crucial for managing PAH-associated chronic heart failure.
• Comprehensive management strategies must address the diverse systemic complications of PAH.