Cureus

. 2022 Oct 23;14(10):e30622.

 doi: 10.7759/cureus.30622. eCollection 2022 Oct. https://pubmed.ncbi.nlm.nih.gov/36426339/

Comprehensive Review of Pulmonary Hypertension and Treatment Options in the Paediatric Population

Pragyna D Parmita ¹, Archana R Thool ²

Affiliations expand

• PMID: 36426339
• PMCID: PMC9681719
• DOI: 10.7759/cureus.30622

Free PMC article

Abstract

Pulmonary hypertension (PH) is a complex condition that can occur as a result of a wide range of disorders, including left heart disease, lung disease, and chronic pulmonary thromboembolism. Multiple improvements have been made in the diagnosis and treatment of pulmonary arterial hypertension (PAH) including a greater understanding of the involvement of extrapulmonary vascular organ systems, validated point of care, clinical assessment tools, and a focus on the initial exposure of numerous pharmacotherapeutics in the appropriate level of care. To achieve a minimal symptom burden, improve the patient’s biochemical, hemodynamic, and functional profile, and reduce adverse impact, early diagnosis of PAH is a key objective today. The preferred method of management for thromboembolic PH, which is chronic, is pulmonary endarterectomy since the majority of affected patients are operable. The timing of pulmonary endarterectomy should never be delayed for medical reasons, and risk stratification can enable us to select patients who have a high chance of success. Patients who are not qualified for endarterectomy should be referred for drug trials. Even though there are more effective ways to guarantee a sufficient, long-lasting septostomy, atrial septostomy is promising but undervalued. The procedure’s indications remain the same and need to be taken into account more frequently. Class III or IV patients who are not improving need to be consulted at a transplant centre as soon as possible as they may be candidates for potential recipients of bilateral sequential lung or heart-lung transplants, which is a significant choice for some people. PH is rarely linked to other conditions like connective tissue or thromboembolic disease. It is either idiopathic or linked to congenital heart disease. Infants and children with PH are more frequently recognised in conjunction with a congenital diaphragmatic hernia and developmental lung diseases like bronchopulmonary dysplasia. Although the underlying disease has not yet been treated and advanced structural changes have not yet been reversed, the value of natural life and survival have suggestively increased. Children’s haemodynamic and functional outcomes have improved as a result of endothelin receptor antagonists, prostacyclin analogues, and phosphodiesterase type 5 inhibitors, which are examples of targeted pulmonary vasodilator therapies. The health maintenance of paediatric PH is still difficult because treatment decisions are largely based on the findings of adult studies that have been supported by evidence and the clinical expertise of paediatric specialists.

Keywords: congenital heart disease; endarterectomy; pulmonary hypertension; septostomy; thromboembolic disease.

Copyright © 2022, Parmita et al.