Natural history of pulmonary hemodynamics in primary pulmonary hypertension

doi:10.1016/0002-8703(87)90510-2

American Heart Journal

Volume 114, Issue 2, August 1987, Pages 407-413

https://doi.org/10.1016/0002-8703(87)90510-2 Get rights and content

Abstract

To study the relationship between the hemodynamic characteristics and prognosis in PPH, we analyzed the data on patients whose prognosis was well documented from the time of hemodynamic monitoring. Our subjects were 87 patients obtained from a nationwide survey in Japan. PCWPs were obtained in 44 patients and COs were measured in 59 patients. The average age was 33 years (range 14 to 69). Patients were followed prospectively for up to 100 months and were categorized based on the survival period from the time of catheterization. Hemodynamic variables that showed significant differences or prognostic trends were RVedp, pulmonary artery diastolic pressure, PCWP, CI, PVR, and PVR/SVR ratio. The CI correlated with the number of survival months (r = 0.583; p < 0.01). The hemodynamic state of patients who died suddenly within 1 year from the time of catheterization was no different from those who died of clinical right-sided heart failure. The partial pressure of oxygen of arterial blood was the only variable discriminating sudden deaths from right-sided heart failure deaths (54 ± 2 vs 66 ± 4 mm Hg;p < 0.05). The results of this study indicate that the major determinant of prognosis is right ventricular function and that the CI might be used as a prognostic indicator. In addition, sudden death is more likely to occur in patients with severe hypoxia.

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Four other studies were identified that did not find an association between CO and mortality.22–25 For RVEDP, only 3 studies were identified that supported this parameter as a prognostic factor for mortality in IPAH20,26 (Ref.15,18,38) (Table 7). However two of these (Rozkovec et al.20 and Kanemoto et al.26) also reported the absence of an association depending on the type of analysis performed or the definition of survival that was used.
There is a lack of consensus on factors that predict mortality in idiopathic pulmonary arterial hypertension (IPAH). Tests that can accurately predict prognosis are needed to guide treatment and counsel patients.
We conducted a systematic review to identify factors that prognosticate mortality in IPAH. Study design, cohort size, comparison method, measured value, and statistical significance was extracted for eight pre-selected parameters [pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), mean right atrial pressure (mRAP), cardiac output, right ventricular end diastolic pressure, functional class, 6 min walk distance (6MWD), and diffusing capacity of carbon monoxide].
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There is a large body of literature describing numerous factors that predict mortality in IPAH. Most factors have been assessed in very few studies. There are conflicting reports on the prognostic value of many factors. These discrepancies highlight the need to evaluate the literature in total when considering the utility of variables as prognostic factors in IPAH.
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2009, Journal of the American College of Cardiology
New and emerging therapies might provide benefit in patients with pulmonary arterial hypertension. Their efficacy and safety will be compared with existing combination therapies in randomized clinical trials. Appropriate end points for these trials need to be identified: these will include exercise testing, the composite end point of time to clinical worsening, and hemodynamic markers, including advanced imaging modalities and biomarkers. Quality-of-life questionnaires are useful and important secondary end points; pulmonary arterial hypertension-specific questionnaires are currently being developed. Advantages and disadvantages of various trial designs, including placebo-controlled monotherapy or add-on trials, noninferiority studies, and withdrawal trials are also discussed.
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Pulmonary arterial hypertension (PAH) is a rare but seemingly deadly condition of varying underlying aetiologies that result in an elevated mean pulmonary arterial pressure of > 25 mm Hg at rest, or 30 mm Hg with exercise and subsequent right heart failure [1]. Insidious in nature, PAH is often diagnosed long after pathological remodelling of all three layers of small pulmonary arteries causing vascular obstruction and raised pulmonary vascular resistance causing progressive right ventricular failure (the underlying reason for often unexplained dyspnoea equivalent to New York Heart Association Class II–IV symptoms) and a premature death [1]. PAH has attracted increasing interest with the combination of: a) the emergence of more effective therapies, b) greater efforts to standardise its detection and management and, finally, c) the introduction of a World Health Organisation sponsored classification system to better characterise the heterogeneous forms of pulmonary hypertension [2,3].
Although pulmonary arterial hypertension (PAH) is widely accepted as deadly, if not a rare disease, its prognostic impact beyond reports from specialist centres is unknown.
Using the unique Scottish Morbidity Record Scheme and linked survival data, we tracked the survival of all Scottish adults aged ≤ 65 years admitted for the first time during the period of 1986 to 2001 with a probable diagnosis of Idiopathic PAH and a PAH related to connective tissue disorders (Connective PAH) and congenital abnormalities (Congenital PAH) — the three most common forms of PAH.
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This population-based study has confirmed the deadly impact of the three most common forms of PAH. Overall, there are encouraging trends in relation to one and five year adjusted survival rates; particularly in relation to PAH related to connective tissue disorders.
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Citation Excerpt :
In the future, tricuspid DTI may be a useful adjunctive measure of PVR in these patients. In patients with primary pulmonary hypertension, an elevated PVR is an independent predictor of mortality.3,14,15 Invasive measurements of PVR are often followed to measure the efficacy of epoprostenol therapy.16,17
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Natural history of pulmonary hemodynamics in primary pulmonary hypertension

Abstract

Am J Med

Chest

Am J Med

Am J Cardiol

Am Heart J

Chest

Chest

Primary pulmonary hypertension: report of six cases and review of literature

Ann Intern Med

Primary pulmonary hypertension: a review and report of two cases

Asian Med J

Report on a WHO meeting: primary pulmonary hypertension