Guidelines for Diagnosis and Treatment of Pulmonary Hypertension

Galiè N, Humbert M, Vachiery JL, et al.
2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Eur Heart J 2015;Aug 29:[Epub ahead of print].

The following are key points to remember from the European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension (PH):

  1. PH is a pathophysiological disorder that may involve multiple clinical conditions and can complicate the majority of cardiovascular and respiratory diseases.
  2. PH is defined as an increase in mean pulmonary artery (mPA) ≥25 mm Hg at rest, as assessed by right heart catheterization. The normal mPA is 14 ± 3 mm Hg with an upper limit of normal of approximately 20 mm Hg. The clinical significance of an mPA 21-24 mm Hg is unclear. Patients with a pulmonary artery pressure (PAP) in this range should be carefully followed because they are at risk for developing pulmonary arterial hypertension (PAH) (e.g., patients with connective tissue disease [CTD]).
  3. PAH describes a group of PH patients (e.g., idiopathic, heritable, congenital heart, CTD, human immunodeficiency virus, portal hypertension, drugs, and toxins) characterized hemodynamically by the presence of precapillary PH, defined by a pulmonary artery wedge pressure (PAWP) ≤15 mm Hg and a pulmonary vascular resistance (PVR) >3 Wood units (WU) in the absence of other causes of precapillary PH such as due to hypoxemia/lung diseases, or chronic thromboembolism. Post-capillary PH related to left heart and valve disease is defined as a PAWP >15 mm Hg with a diastolic pressure gradient (DPG = dPA – PCWP) <7 mm Hg and PVR ≤3 WU. Combined post- and precapillary PH is defined with PCWP >15 mm Hg with DPG ≥7 mm Hg and PVR >3 WU.
  4. In registries, around 50% of PAH patients have idiopathic, heritable, or drug-induced PAH. In the subgroup of associated PAH conditions (APAH), the leading cause is CTD, mainly systemic sclerosis. Up to 60% of patients with severe heart failure with reduced ejection fraction (HFrEF) and up to 70% of patients with HFpEF may present with PH, which is found in nearly all patients with severe symptomatic mitral valve disease and in up to 65% of those with symptomatic aortic stenosis. Mild PH is common in both severe chronic obstructive pulmonary disease and interstitial lung disease, but severe PH is uncommon unless the two conditions present together.
  5. Transthoracic echo is used to image the effects of PH on the heart and estimate right ventricular (RV) systolic pressure or PAP from continuous wave Doppler. Echo-Doppler should always be performed when PH is suspected. When treatment of PH is being considered, cardiac catheterization is required. The V/Q scan has been the screening method of choice for chronic thrombolic PH because of its higher sensitivity compared with computed tomography pulmonary angiogram. Cardiac magnetic resonance imaging is accurate and reproducible in the assessment of RV morphology and function, and allows noninvasive assessment and RV mass. It is particularly useful in detecting congestive heart failure and provides useful prognostic information at baseline and on treatment.
  6. High risk (>10% 1-year mortality) is defined as clinical signs of right heart failure, rapid progression, repeated syncope, World Health Organization functional class (WHO FC) IV, 6-minute walk (6MW) <165 m, B-type natriuretic peptide (BNP) 300 ng/L, pericardial effusion, right arterial pressure >14 mm Hg, cardiac index (CI) <2.0 L/m/m2, and mixed venous oxygen saturation (SvO2) <60%. Low risk (<5% in 1-year mortality) is defined as no signs of right heart failure, no progression of symptoms, WHO FC I, II, 6MW >440 m, BNP <50 pg/L, no pericardial effusion, normal right atrial (RA) size, RA pressure <8 mm Hg, CI ≥2.5 L/min/m2, and SvO2 >65%.
  7. General treatment measures include optimal weight, physical activity, and pulmonary rehabilitation, avoidance of pregnancy and combination birth control for prevention of pregnancy in women on PAH-specific treatment, psychosocial support, seasonal flu and pneumonia vaccine, oral anticoagulants for idiopathic and heritable PAH, diuretics, oxygen as determined in the 6MW and nocturnal monitoring, and digoxin (no clear evidence). Iron deficiency and associated anemia are common and should be treated when present. Consultation from PH experts should be obtained in conjunction with other specialists for care during pregnancy; elective, urgent, and emergency surgery; and PAH treatment perioperatively.
  8. Patients suspected to have PAH should be referred to an expert center for confirmation and treatment including acute vasoreactivity testing (idiopathic PAH/heritable PAH/drug-induced PAH only); risk stratification and selection, and institution of the most appropriate treatment(s). Patients on PAH-specific drugs should be followed by the expert center for clinical response and subsequent therapeutic decisions including various combination therapies, end-of-life decisions, and consideration of referral for lung transplantation.

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