It is increasingly recognised that diverse genetic respiratory disorders present as severe pulmonary hypertension (PH) in the neonate and young infant, but many controversies and uncertainties persist regarding optimal strategies for diagnosis and management to maximise long-term outcomes. To better define the nature of PH in the setting of developmental lung disease (DEVLD), in addition to the common diagnoses of bronchopulmonary dysplasia and congenital diaphragmatic hernia, we established a multidisciplinary group of expert clinicians from stakeholder paediatric specialties to highlight current challenges and recommendations for clinical approaches, as well as counselling and support of families. In this review, we characterise clinical features of infants with DEVLD/DEVLD-PH and identify decision-making challenges including genetic evaluations, the role of lung biopsies, the use of imaging modalities and treatment approaches. The importance of working with team members from multiple disciplines, enhancing communication and providing sufficient counselling services for families is emphasised to create an interdisciplinary consensus.

人们越来越认识到，新生儿和小婴儿中多种遗传性呼吸系统疾病表现为严重肺动脉高压（PH），但关于诊断和治疗的最佳策略以最大限度地提高长期结果仍然存在许多争议和不确定性。为了更好地定义发育性肺疾病 (DEVLD) 中 PH 的性质，除了支气管肺发育不良和先天性膈疝的常见诊断之外，我们还建立了一个由利益相关者儿科专业的临床专家组成的多学科小组，以强调当前的挑战和建议用于临床方法以及家庭的咨询和支持。在这篇综述中，我们描述了 DEVLD/DEVLD-PH 婴儿的临床特征，并确定了决策挑战，包括遗传评估、肺活检的作用、成像方式的使用和治疗方法。强调与多学科团队成员合作、加强沟通、为家庭提供充分的咨询服务的重要性，以建立跨学科共识。