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Hinweis der Redaktion
- What is PAH? PAH is a syndrome characterised by a progressive increase in pulmonary vascular resistance (PVR), which leads to right ventricular overload and eventually to right ventricular failure and premature death. 1 Increased PVR is related to a number of progressive changes in the pulmonary arterioles, including vasoconstriction, obstructive remodelling of the pulmonary vessel wall through proliferation in the various layers of the blood vessel wall (smooth muscle cell and endothelial cell proliferation), inflammation and in-situ thrombosis. If untreated, PAH carries a very poor prognosis with a median survival of 2.8 years after diagnosis 2 . This survival figure is comparable with some malignancies. References Sitbon et al. Circulation 2005; 111 : 3105-3111. 2. D’Alonzo GE, Barst RJ. Ann Intern Med 1991; 115 : 343-349.
- PAH: histopathology The main histological features of PAH include medial hypertrophy, intimal thickening, adventitial thickening, plexiform lesions and in-situ thrombosis. The plexifom lesion represents a focal proliferation of endothelial and smooth muscle cells resulting in a complex 3-Dimensional deformation of the vessel and is pathognomonic of PAH.
- PAH: definition PAH is defined as a sustained elevation of mean pulmonary arterial pressure (mPAP) of > 25 mmHg at rest or > 30 mmHg while exercise in the presence of a normal pulmonary capillary wedge pressure (PCWP) of 15 mmHg. 1 The earliest symptom of PAH is often dyspnoea experienced on physical exertion. Other symptoms may include syncope or near syncope and fatigue. 1,2 Chest tightness and pain similar to angina may occur, particularly on physical exertion. In severe disease, signs of right heart failure (RHF) may emerge. References Gali è N et al. European Heart Journal . 2004;25:2243-2278. Gaine SP, Rubin LJ. Lancet 1998; 352 : 719 725. Barst RJ et al. J Am Coll Cardiol 2004; 43 (Suppl S) : 40S 47S.
- Early diagnosis and therapeutic intervention is therefore crucial. Marked improvements in prognosis and survival have been shown for patients who begin targeted therapy in a less severe stage (WHO FCI/II) of PAH compared with those in a more severe stage (WHO FCIII/IV). 5 References D'Alonzo GE, Barst RJ et al. Ann Intern Med 1991; 115 :343-349. Kato I, Severson RK and Schwartz AG. Cancer 2001; 92: 2211-2219. Bjoraker JA, Ryu JH et al. Am J Respir Crit Care Med 1998; 157 :199-203. Gaine SP, Rubin LJ. Lancet 1998; 352 : 719 725. Humbert M, Sitbon O et al. Am J Respir Crit Care Med 2006; 173: 1023-1030. Sitbon O, Humbert M et al. J Am Coll Cardiol 2002; 40 :780-788.
- PAH: how is it treated? Treatment options have progressed considerably in the last decade, especially those which target the underlying mechanisms of the disease. The main medical treatment options for patients with PAH are: 1 Treatments that are routinely used but with little evidence of an impact on the disease: anticoagulants , such as warfarin, to address the observed thrombotic changes and potential predisposition in the pulmonary microcirculation for in-situ thrombosis, diuretics , for treatment of right heart failure, oxygen therapy , to maintain oxygen saturation at > 90% at all times and calcium-channel blockers (CCBs) . However, less than 10% of IPAH patients benefit from CCBs therapy. This figure is even lower in other forms of PAH. If not used in appropriate candidates (patients with demonstrated vasoreactivity during right heart catheterisation), CCBs can decrease cardiac output and systemic vascular resistance without any improvement in PAP and PVR and therefore may be deleterious. 2 References Humbert H et al. N Engl J Med 2004; 351 : 1425-1436. 2. Sitbon O et al. Circulation 2005; 11 : 3105-11.
- Treatments that have been specifically studied in PAH: endothelin receptor antagonists - endothelin is implicated in the pathogenesis of PAH through actions on the pulmonary vasculature. Endothelin is found to be elevated in patients with PAH and levels of endothelin are directly related to disease severity and prognosis. Endothelin receptor antagonists (ERAs) are oral treatments that either block the ET A receptor alone or both the ET A and ET B receptors, 1,2 prostacyclin analogues - may be delivered by continuous intravenous or subcutaneous infusion or via an intermittent nebuliser. 3 To date, oral agents have shown limited effectiveness and phosphodiesterase 5 inhibitors -induce relaxation and antiproliferative effects on vascular smooth muscle cells by preventing the reduction in levels of cGMP. 4 In very severe cases surgical options may be considered: balloon atrial septostomy and heart and lung transplantation. However, the use of transplantation is constrained by the limited number of donor organs. References Channick RN et al. Lancet 2001; 358: 1119-23. Galie et al. European Heart Journal 2004; 25 : 2243-2278. Humbert H et al. N Engl J Med 2004; 351 ; 1425-36. 4. Galiè N et al. N Engl J Med 2005; 353 : 2148-57.
- PAH: Screening high risk populations The key to early diagnosis is introducing screening for high risk patient populations if they are asymptomatic. High risk patient populations include: Family members of a patient with familial Pulmonary Arterial Hypertension (FPAH) Patients with systemic sclerosis (SSc) Patients with HIV Patients with portopulmonary hypertension (PoPH) International guidelines now recommend annual screening high-risk groups with Doppler echocardiography. 1-3 Doppler echocardiography is currently the most effective method for screening, however, for a definitive diagnosis right heart catheterisation has to be performed. References Hachulla E and Coghlan JG. Ann Rheum Dis 2004; 63: 1009-1014. Galiè N, Torbicki A, Barst RJ, et al. European Heart Journal 2004; 25 :2243-2278. McGoon M, Gutterman D, Steen V, et al. Chest 2004; 126 :14S-34S.
- Echocardiography Echocardiography is one of the techniques available in the screening and subsequent diagnosis of PAH. Compared with a normal heart, the heart of a PAH patient shows considerable enlargement of the right ventricle, a decrease in the size of the left ventricle and an abnormal septal configuration that is consistent with right ventricular overload.
- Echocardiography: its value as a screening tool Transthoracic Doppler-echocardiography (TTE) is able to estimate pulmonary arterial systolic pressure, which is equivalent to right ventricular systolic pressure in the absence of pulmonary outflow obstruction, and can provide additional information about the cause and consequences of PH, including right and left ventricular dimensions and function, heart valve abnormalities, right ventricular ejection and left ventricular filling characteristics and presence of a pericardial effusion. In the initial investigation of patients with PAH it is important to obtain adequate images of the right heart . Pulmonary arterial pressure can be estimated from the tricuspid regurgitant jet. References Galie et al. European Heart Journal 2004; 25: 2243-2278.
- Right heart catheterisation: the diagnostic gold standard While echocardiography and chest X-ray are useful in the overall evaluation of a patient, right heart catheterisation is required to confirm a diagnosis of PAH 1 , to assess the severity of haemodynamic impairment and to test the vasoreactivity of the pulmonary circulation. As described in earlier slides, PAH is defined as an mPAP of > 25 mmHg at rest and > 30 mmHg with exercise but with a PCWP of 15 mmHg. PVR is also > 3 Woods units. 1 References Barst RJ et al. J Am Coll Cardiol 2004; 43 (Suppl S) : 40S 47S.
- Right heart catheterisation: the diagnostic gold standard This diagram shows the relative positions of the catheter to record standard haemodynamic measurements.
- 6-minute walk test: evaluation of exercise capacity The 6-minute walk test (6-MWT) is reflective of a patient’s ability to exercise. The distance a PAH patient can walk in 6 minutes is a critical endpoint in studies evaluating the benefit of different therapeutic options. To allow meaningful comparisons, it is important that the 6-MWT be performed under supervision according to a standardised protocol. 1 A 30 m corridor should be available, marked at 3 m intervals The patient should rest for at least 10 minutes prior to the test and should not have performed any rigorous exercise within the previous 2 hours The patient should be asked to rate their baseline dyspnoea The patient should be instructed to walk to their maximum capacity but not to run or jog; they should be permitted to rest as necessary The supervisor should count each lap as the patient finishes it The patient should be asked to rate their dyspnoea at the end of the test The test should be repeated at approximately the same time of day on each occasion References ATS. ATS statement: guidelines for the 6-minute walk test. Am J Crit Care Med 2002; 166 : 111 117.