Pulmonary hypertension natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor(s)-in-Chief: Ralph Matar
Overview
In the NHLBI registry for primary pulmonary hypertension, the mean interval from the onset of symptoms to diagnosis was 2 years, and the most common initial symptoms were dyspnea, fatigue, and syncope. There was an estimated median survival of 2.8 years for symptomatic patients who do not recieve any treatment, with the most common cause of death as cor pulmonale.
Natural History
- The National Institutes of Health (NIH) Registry estimated a median survival of 2.8 years for symptomatic patients with idiopathic pulmonary hypertension who do not recieve any treatment, with the cause of death usually being right ventricular failure (cor pulmonale).[1] The 1, 3,and 5-year survival rates for untreated patients with idiopathic pulmonary hypertension were 68%, 48%, and 34%, respectively.[1]
- The median survival duration was even lower for patients with pulmonary hypertension that was associated with other diseases like portal hypertension, and scleroderma (2-year survival of 40% if untreated).
- A recent outcome study of those patients who had started treatment with bosentan demonstrated that 86% patients were alive at 3 years.
- With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently. It would not be unreasonable to expect median survival to extend past 10 years in the near future.
Complications
- Right-sided heart failure (cor pulmonale).
- Blood clots.
- Arrhythmia (irregular heart beats).
- Bleeding into the lungs and coughing up blood.
Prognosis
- The long-term prognosis has been known to be poor, however outcomes have changed dramatically over the last two decades. This may be attributed to the use of newer drug therapy, better overall care, and earlier diagnosis (lead time bias).
- Some people with this condition may have heart failure that could lead to death. Assessment of prognosis in patients with pulmonary arterial hypertension (PAH) is important since it influences both medical therapy and referral for transplantation.
- Surprisingly, patients with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.[2]
Indicators of Poor Prognosis
- Poor functional class[3]
- Poor exercise tolerance as assessed by the 6-minute-walk distance (6MWD)[4]
- Elevated brain natriuretic peptide (BNP)[5]
- Pericardial effusion[5]
- Elevated right atrial size and pressure[6]
- Significant right ventricular dysfunction or failure[5]
- Low cardiac index[6]
- Underlying connective tissue disease[5]
- Decreased predicted carbon monoxide diffusing capacity[5]
ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [7]
Recommendations for Pulmonary Arterial Hypertension
| Class III |
| "1. Prophylactic antiarrhythmic therapy generally is not indicated for primary prevention of SCD in patients with pulmonary arterial hypertension (PAH) or other pulmonary conditions.(Level of Evidence: C)" |
References
- ↑ 1.0 1.1 Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM; et al. (1987). "Primary pulmonary hypertension. A national prospective study". Ann Intern Med. 107 (2): 216–23. PMID 3605900.
- ↑ Hopkins WE,Ochoa LL, Richardson GW, Trulock EP(1996) Comparison of the hemodynamics and survival or patients with severe pulmonary hypertension or Eisenmenger Syndrome.
- ↑ McLaughlin VV, Presberg KW, Doyle RL, Abman SH, McCrory DC, Fortin T; et al. (2004). "Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines". Chest. 126 (1 Suppl): 78S–92S. doi:10.1378/chest.126.1_suppl.78S. PMID 15249497.
- ↑ Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB; et al. (1996). "A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension". N Engl J Med. 334 (5): 296–301. doi:10.1056/NEJM199602013340504. PMID 8532025.
- ↑ 5.0 5.1 5.2 5.3 5.4 Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS; et al. (2010). "Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)". Circulation. 122 (2): 164–72. doi:10.1161/CIRCULATIONAHA.109.898122. PMID 20585012.
- ↑ 6.0 6.1 D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM; et al. (1991). "Survival in patients with primary pulmonary hypertension. Results from a national prospective registry". Ann Intern Med. 115 (5): 343–9. PMID 1863023.
- ↑ Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.