Belgian Pulmonary Hypertension Registry

Pulmonary hypertension or PH is a disease were the pressure in the lung vessels (small circulation) is elevated. This should not be confused with increased blood pressure in the large circulation (arterial hypertension). It is a severe and progressive disease, leading to exercise limitations, right heart failure and ultimately death. The signs and symptoms of pulmonary hypertension in its early stages might not be noticeable for months or even years. As the disease progresses, symptoms become worse. Pulmonary hypertension symptoms include: shortness of breath (dyspnea), initially during exercise and eventually at rest, fatigue, dizziness or fainting (syncope), chest pain, swelling (edema) of the ankles and legs, bluish color of the lips and skin (cyanosis), racing pulse or heart palpitations. PH is classified in five groups, based on underlying condition. Group 1: pulmonary arterial hypertension, group 2: pulmonary hypertension caused by left-sided heart disease, group 3: pulmonary hypertension caused by lung disease, group 4: pulmonary hypertension caused by chronic blood clots, group 5: pulmonary hypertension with unclear or multiple mechanisms. Group one is a rare disease and the care is managed in specialized expert center with the collaboration of a multidisciplinary team. Given the rarity of the disorder, its complexity and the anticipated cost of therapy to the NHS, specialist centers working in a collaborative way, offers best patient care. PH is hard to diagnose early because it's not often detected in a routine physical exam. Even when the condition is more advanced, its signs and symptoms are similar to those of other heart and lung conditions. The diagnosis procedure consist of: an echocardiography, blood sampling, right heart catheterization, ECG, .. etc. PH can't be cured, but specialized medical treatment and surgery (only for patients of group 4) can improve the symptoms and slow down the progression of the disease.肺动脉高压（Pulmonary Hypertension，PH）是一类以肺血管（小循环）压力升高为特征的疾病，需注意切勿将其与体循环（大循环）血压升高（动脉高血压）相混淆。 该病属于严重且呈进行性发展的疾病，可引发运动耐量受限、右心衰竭，最终导致死亡。肺动脉高压的早期体征与症状可能在数月乃至数年内均无明显表现；随着病情进展，症状会逐渐加重。其典型症状包括：呼吸困难（dyspnea），初期仅在运动时发作，后期静息状态下也会出现；疲劳感；头晕或晕厥（syncope）；胸痛；踝部与下肢水肿（edema）；口唇及皮肤发绀（cyanosis）；脉搏急促或心悸（heart palpitations）。 依据潜在病因，PH可分为五大类别：第1类为肺动脉性高血压（pulmonary arterial hypertension）；第2类为左心疾病相关性肺动脉高压；第3类为肺部疾病相关性肺动脉高压；第4类为慢性血栓相关性肺动脉高压；第5类为发病机制不明或存在多种机制的肺动脉高压。其中第1类属于罕见病，其诊疗需在专科中心开展，并由多学科团队协作完成。鉴于该病的罕见性、诊疗复杂性以及国民保健制度（NHS）的预期治疗成本，以协作模式运作的专科中心可为患者提供最优的诊疗服务。 肺动脉高压早期诊断难度较大，常规体检通常难以检出该病；即便病情进展至较严重阶段，其体征与症状也与其他心肺疾病高度相似。其诊断流程包括：超声心动图检查、血液采样、右心导管术、心电图（ECG）等。 肺动脉高压目前无法根治，但通过专科药物治疗与手术治疗（仅适用于第4类患者）可改善症状、延缓病情进展。