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; José Eduardo Riceto Loyola Junior, M.D.[2]
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 receive 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 receive 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
Complications of pulmonary hypertension include:[2]
- Right-sided heart failure (cor pulmonale)
- Blood clots
- Arrhythmia
- Hemoptysis
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).
- Elevated pulmonary arterial pressure on heart catheterization is a predictor of mortality, especially if it is happening not only in the setting of myocarditis or decreased right ventricular ejection fraction, but also COPD. Treatment of pulmonary hypertension in these morbidities does not affect outcomes.[3]
- Survival rate at 5 years is 57%.[3]
- Persistently elevated BNP or NT-proBNP levels, PAH associated with connective tissue disease or portal hypertension indicate poorer prognosis.[3]
- 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.
- Mortality rate is 5.2-5.4 per 100,000 and is more common in African-Americans and women.
- The most common cause of hospitalization is heart failure.
- The most common cause of death is right ventricular failure and not chronic lower respiratory disease as was once thought.
- Surprisingly, patients with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.[4]
Indicators of Poor Prognosis
Indicators of poor prognosis include:
- Poor functional class[2]
- Poor exercise tolerance as assessed by the 6-minute-walk distance (6MWD)[5]
- Elevated brain natriuretic peptide (BNP)[6] or NT-proBNP levels.[3]
- Pericardial effusion[6]
- Persistently elevated right atrial size and pressure[7]
- Significant right ventricular dysfunction or failure[6]
- Low cardiac index[7]
- Underlying connective tissue disease[6]
- Decreased predicted carbon monoxide diffusing capacity[6]
- PAH associated with connective tissue disease or portal hypertension.
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.
- ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 3.2 3.3 Poch D, Mandel J (2021). "Pulmonary Hypertension". Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check
|pmid=value (help). - ↑ Hopkins WE,Ochoa LL, Richardson GW, Trulock EP(1996) Comparison of the hemodynamics and survival or patients with severe pulmonary hypertension or Eisenmenger Syndrome.
- ↑ 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.
- ↑ 6.0 6.1 6.2 6.3 6.4 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.
- ↑ 7.0 7.1 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.