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Paediatric pulmonary hypertension (PPH) is a complex and potentially life-threatening condition characterized by elevated blood pressure within the pulmonary arteries that supply blood to the lungs in children. This condition arises from various underlying causes, including congenital heart defects, chronic lung diseases, and genetic predispositions. In PPH, the increased pressure in the pulmonary arteries imposes a significant burden on the right side of the heart, leading to progressive damage and, if left untreated, eventual heart failure. The symptoms of PPH are often subtle and nonspecific, making early diagnosis challenging. Children affected by PPH may exhibit fatigue, shortness of breath, fainting episodes, and difficulty thriving. Diagnostic evaluation involves a comprehensive approach, including medical history, physical examination, echocardiography, and cardiac catheterization to assess the severity and underlying causes of the condition. Treatment strategies for PPH aim to alleviate symptoms, improve quality of life, and slow disease progression. Therapeutic interventions may include medications such as prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase inhibitors, which target pathways involved in regulating blood vessel tone. In severe cases, surgical procedures or lung transplantation may be considered. Despite advances in medical management, PPH poses significant challenges, and ongoing research is focused on better understanding its pathophysiology and developing novel therapeutic approaches. A multidisciplinary approach involving Paediatric cardiologists, pulmonologists, and other specialists is essential to provide comprehensive care for children with pulmonary hypertension, emphasizing the importance of early detection, personalized treatment plans, and ongoing monitoring to optimize outcomes and enhance the overall well-being of affected individuals.
