Prognosis
Prognosis of Pulmonary Hypertension
The prognosis of PPH is variable but generally poor. Patients often are diagnosed with a mean of 2 years delay from the onset of symptoms and present with already severe PH and right ventricular dysfunction / decompensation.
Survival correlates with the severity of pulmonary hypertension and right ventricular dysfunction, NYHA classification, exercise tolerance, hemodynamic parameters and on the response to vasodilator therapy.
Series from the United Kingdom, Japan and France reported a median survival of approximately 3 years from diagnosis (Oakley CW, 1994; Brenot F, 1994; Okada O et al., 1999).
Patients with moderate elevations in pulmonary artery pressure (mean pressure less than 55 mmHg) and preserved right heart function (normal right atrial pressure and cardiac index) had a median survival in slight excess of 2,5 years in the National Institutes of Health (NIH) Registry (D'Alonzo GE et al., 1991).
Patients with severe pulmonary hypertension or right heart failure tend to die within one year.
Assessing functional status using the New York Heart Association classification can also be useful in determining prognosis and making decisions regarding therapy. Patients who fall into functional classes I and II tend to live far longer than those who fall into classes III and IV.
Since survival in patients who fall into NYHA classes III or IV is poor (9-18 months), patients with severe symptoms and evidence of impaired cardiac function, which is refractory to conventional therapy or epoprostenol infusion should be considered for transplantation.
Alternatively, those patients with mild exertional symptoms, preserved exercise tolerance, and stable cardiac function (NYHA classes I and II) appear to have a 5 year survival more than 50 percent with medical therapy alone.
Pericardial effusion, which corellates with elevated right atrial pressure, is a prognostically unfavorable sign (Hinderliter AL et al.,1999).
Oral anticoagulation and vasodilator therapy have improved the prognosis of PPH . Long-term infusion of epoprostenol may also be beneficial in treating severe secondary pulmonary hypertension due to congenital heart disease, collagen vascular disease, sarcoidosis, distal thromboembolic disease, or portopulmonary hypertension (McLaughlin VV et al., 2000).
References:
Altman RD; Medsger TA Jr; Bloch DA; Michel BA. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 1991 Apr;34(4):403-13
Brenot F. Primary pulmonary hypertension. Case series from France.
Chest 1994 Feb;105(2 Suppl):29S-32S
Chest 1994 Feb;105(2 Suppl):33S-36S
D'Alonzo GE et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991 Sep 1;115(5):343-9
Hinderliter AL et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Am J Cardiol 1999 Aug 15;84(4):481-4, A10
Kunieda T et al. Prognoses of primary pulmonary hypertension and chronic majorvessel thromboembolic pulmonary hypertension determined from cumulative survival curves. Intern Med 1999 Jul;38(7):543-6
McLaughlin VV et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med. 2000 Jan 18;132(2):165
Oakley CW. Primary pulmonary hypertension. Case series from the United Kingdom.
Okada O et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990. Intern Med 1999 Jan;38(1):12-6
The prognosis of PPH is variable but generally poor. Patients often are diagnosed with a mean of 2 years delay from the onset of symptoms and present with already severe PH and right ventricular dysfunction / decompensation.
Survival correlates with the severity of pulmonary hypertension and right ventricular dysfunction, NYHA classification, exercise tolerance, hemodynamic parameters and on the response to vasodilator therapy.
Series from the United Kingdom, Japan and France reported a median survival of approximately 3 years from diagnosis (Oakley CW, 1994; Brenot F, 1994; Okada O et al., 1999).
Patients with moderate elevations in pulmonary artery pressure (mean pressure less than 55 mmHg) and preserved right heart function (normal right atrial pressure and cardiac index) had a median survival in slight excess of 2,5 years in the National Institutes of Health (NIH) Registry (D'Alonzo GE et al., 1991).
Patients with severe pulmonary hypertension or right heart failure tend to die within one year.
Assessing functional status using the New York Heart Association classification can also be useful in determining prognosis and making decisions regarding therapy. Patients who fall into functional classes I and II tend to live far longer than those who fall into classes III and IV.
Since survival in patients who fall into NYHA classes III or IV is poor (9-18 months), patients with severe symptoms and evidence of impaired cardiac function, which is refractory to conventional therapy or epoprostenol infusion should be considered for transplantation.
Alternatively, those patients with mild exertional symptoms, preserved exercise tolerance, and stable cardiac function (NYHA classes I and II) appear to have a 5 year survival more than 50 percent with medical therapy alone.
Pericardial effusion, which corellates with elevated right atrial pressure, is a prognostically unfavorable sign (Hinderliter AL et al.,1999).
Oral anticoagulation and vasodilator therapy have improved the prognosis of PPH . Long-term infusion of epoprostenol may also be beneficial in treating severe secondary pulmonary hypertension due to congenital heart disease, collagen vascular disease, sarcoidosis, distal thromboembolic disease, or portopulmonary hypertension (McLaughlin VV et al., 2000).
References:
Altman RD; Medsger TA Jr; Bloch DA; Michel BA. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 1991 Apr;34(4):403-13
Brenot F. Primary pulmonary hypertension. Case series from France.
Chest 1994 Feb;105(2 Suppl):29S-32S
Chest 1994 Feb;105(2 Suppl):33S-36S
D'Alonzo GE et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991 Sep 1;115(5):343-9
Hinderliter AL et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Am J Cardiol 1999 Aug 15;84(4):481-4, A10
Kunieda T et al. Prognoses of primary pulmonary hypertension and chronic majorvessel thromboembolic pulmonary hypertension determined from cumulative survival curves. Intern Med 1999 Jul;38(7):543-6
McLaughlin VV et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med. 2000 Jan 18;132(2):165
Oakley CW. Primary pulmonary hypertension. Case series from the United Kingdom.
Okada O et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990. Intern Med 1999 Jan;38(1):12-6
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SGPH-Symposium, 2. September 2010, Hotel Bristol in Genf:
"Hypertension artérielle pulmonaire: Comment ne pas passer à côté d’une maladie émergente?" Mehr
Umfrage:
Helfen Sie uns, den Bereich "Patienten" weiter zu verbessern und Ihren Ansprüchen anzupassen indem Sie an unserer Umfrage teilnehmen.
Vereinsgründung:
Schweizer PH-Verein für Menschen mit pulmonaler Hypertonie gegründet. Pressemitteilung PDF
Next SSPH Workshop: October 28th, 2010
Haus der Universität in Berne, details will follow.
SSPH Workshop 2010: May 7-8, 2010
The SSPH workshop on May 7-8, 2010 in Lucerne did consist of an update of the website with the following topics:
- Diagnosis
- Treatment
- Follow-up
- Patients part
4th international Congress of the SSPH
October 30 - 31, 2009
Link to the congress presentations as video streaming on swiss-webconferencing.ch .
Link to the congress photo-gallery
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