Medway Maritime Hospital, Gillingham, Kent, UK
Medway Maritime Hospital, Gillingham, Kent, UK.
BMJ Case Rep. 2022 Apr 26;15(4):e244803. doi: 10.1136/bcr-2021-244803.
Portopulmonary hypertension (PoPH) is a poorly understood complication of liver disease which affects about 10% of patients with pulmonary hypertension. This case report outlines the difficulties in diagnosing and managing a patient with advanced disease, and the impact of these delays on the patient.PoPH has a significant risk of mortality with a 2-year survival rate of 67%. There are also few treatment options available and those which do exist are associated with multiple contraindications and risks. Patients with PoPH commonly present with dyspnoea, pulmonary hypertension and portal hypertension. The presence of coexisting chronic liver disease is also sometimes present. Traditional management for heart failure can temporarily alleviate symptoms but there is no proven long-term benefit. As a result, an understanding of the pathophysiology, diagnostics and management is crucial to ensure the best possible patient outcomes.
肝肺高血压（PoPH）是一种尚未完全了解的肝脏疾病并发症，影响约 10%的肺动脉高压患者。本病例报告概述了诊断和管理晚期疾病患者的困难，以及这些延迟对患者的影响。PoPH 的死亡率很高，2 年生存率为 67%。此外，治疗选择也很少，现有的治疗方法也存在多种禁忌症和风险。PoPH 患者通常表现为呼吸困难、肺动脉高压和门静脉高压。同时也存在共存的慢性肝脏疾病。心力衰竭的传统治疗方法可以暂时缓解症状，但没有长期获益的证据。因此，了解病理生理学、诊断和管理方法对于确保患者获得最佳治疗效果至关重要。
