plexiform lesions pulmonary hypertension

Plaque-like lesions in pulmonary arterial hypertension (PAH) are a rare but severe pathological finding, often signaling disease progression and a poor prognosis. These lesions present as intimal thickening within the pulmonary arteries, leading to nodular protrusions that impede blood flow, thereby increasing pulmonary vascular resistance and right ventricular pressure.

The etiology of plaque-like lesions is multifactorial and complex, potentially involving genetic predisposition, autoimmune responses, and environmental exposures. They typically emerge in the advanced stages of PAH and are correlated with reduced survival rates. Therapeutically, while there are no drugs specifically targeting plaque-like lesions, the use of PAH-specific medications, such as endothelin receptor antagonists and phosphodiesterase-5 inhibitors, can enhance the overall condition and quality of life for patients. For those with severe disease refractory to medical treatment, lung transplantation may be considered as a final therapeutic option.

For individuals with PAH, regular follow-ups and disease monitoring are essential. If signs of plaque-like lesions are detected, immediate medical consultation is advised, and treatment regimens should be adjusted under a physician's supervision. Self-adjustment of medication dosages or discontinuation of treatment is strongly discouraged to prevent disease exacerbation. Additionally, maintaining a healthy lifestyle, including smoking cessation, sodium restriction, and regular physical activity, can aid in disease management.