肺動脈高壓與左側心臟疾病引起的肺高壓

肺高壓是指平均肺動脈壓大於或等於2525mmHg 的病理異常，為一種複雜的疾病，且和多種的臨床病變相關。近年來由於診斷工具和治療藥物不斷進步，分類及治療準則也作了大幅修正；肺高壓共分為5群，第1群為肺動脈高壓，屬肺微血管前之肺高壓，且肺血管阻力大於3個伍的單位，並排除其他原因。第2及第3群分別為左側心臟疾病和肺病所引起，第4群是慢性肺栓塞導致，而第5群則屬多重或不明原因之肺高壓。因為對病生理的進一步了解，新的血流動力學參數被採用來定義一群微血管後肺高壓的次分組。新的臨床分類無論成人或兒童皆可適用。詳細的診斷流程表及篩檢策略已經更新，且強調轉診到專家中心的重要性。所有疑似肺高壓的患者，都應該接受心臟超音波檢查，以判別肺高壓可能性高低，要確診肺動脈高壓及慢性血栓栓塞性肺高壓，需要作右側心導管檢查，對於原發性肺動脈高壓(IPAH)，遺傳性肺動脈高壓(HPAH)或是毒藥物引起的肺動脈高壓這三類病患，須同時加作肺血管反應性測試，以找出適合使用高劑量的鈣離子通道阻斷劑治療的患者。肺動脈高壓疾病嚴重度評估指標及治療目標之設定，也一併更新。加入了新的標靶藥物以及新的治療流程。有關左心疾病及肺病引起之肺高壓也更新。所謂的「不成比例的肺高壓」一詞已被丟棄不用。治療左心疾病引起的肺高壓，仍是以治療共病為主，標靶藥物目前仍不建議使用。

關鍵字

肺高壓；肺動脈高壓；治療準則；診斷流程；右側心導管檢查；血管反應性測試；基因測試及諮詢

並列摘要

Pulmonary hypertension (PH) is a pathophysiologic disorder characterized by an increase in mean pulmonary arterial pressure≧25 mmHg that may involve multiple clinical conditions. The diagnostic tools and therapeutic drug for pulmonary hypertension have evolved progressively in recent years so the classification and treatment guideline are revised accordingly. Pulmonary hypertension is classified into 5 groups. Group 1 is pulmonary arterial hypertension (PAH) described a group of PH characterized by the presence of pre-capillary PH , defined by a pulmonary artery wedge pressure PAWP ≦15 mmHg and a pulmonary vascular resistance PVR ＞3 WU in the absence of other cause. Group 2 and 3 are PH due to left heart disease and lung disease or hypoxia, Group 4 are chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary artery obstruction. Group 5 is PH due to multifactorial or unclear mechanism. New wordings and parameters for the hemodynamic definition of post-capillary PH subgroups have been adopted. An updated common clinical classification for adult and pediatric patients is reported. An updated diagnostic algorithm has been provided. The importance of expert referral centers in the management of PH patients has been highlighted in both the diagnostic and treatment algorithms. Echocardiography should always be performed when PH is suspected and may be used to infer a diagnosis of PH in patients in whom multiple different echocardiographic measurements are consistent with this diagnosis. Right heart catheterization is required to confirm the diagnosis of PAH and CTEPH and to assess the severity of hemodynamic impairment and to undertake vasoreactivity testing of the pulmonary circulation in selected patients; idiopathic or hereditary PAH and drugs or toxin induced PAH. New developments on PAH severity evaluation and on treatments and treatment goals are reported. The chapters on PH due to left heart disease and lung diseases have been updated. The term ‘out of proportion PH’ has been abandoned in both conditions. The primary goal of therapy in PH due to left heart disease must be to improve global management of the underlying condition and co-morbidity and there is still no new evidence supporting the use of PAH therapies in this condition.

並列關鍵字

pulmonary hypertension ； pulmonary arterial hypertension ； guideline ； diagnostic algorithm ； right heart catheterization ； vasoreactivity test ； genetic testing

參考文獻

Galiè N, Humbert M, Vachiery JL, et al: 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2015;46:903-75.

Ivy DD, Abman SH, Barst RJ, et al: Pediatric pul- monary hypertension. J Am Coll Cardiol 2013;62:D117-26. doi: 10.1016/j.jacc.2013.10.028

Humbert M, Sitbon O, Chaouat A, et al: Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006;173:1023-30. doi:10.1164/rccm.200510-1668OC

Vahanian A, Alfieri O, Andreotti F, et al: Guidelines on the management of valvular heart disease (version 2012). Eur Heart J 2012;33: 2451-96.

Trip P, Nossent EJ, de Man FS, et al: Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses. Eur Respir J 2013;42:1575-85. doi: 10.1183/09031936.00184412

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肺動脈高壓與左側心臟疾病引起的肺高壓

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