Pulmonary Hypertension Associated with Left-Sided Heart Disease

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Pulmonary hypertension (PH) denotes a poor prognosis in patients with left-sided heart disease. No study has demonstrated long-term benefit from PH drugs such as prostanoids, endothelin antagonists, and phosphodiesterase-5 inhibitors. In some cases, cautious use of PH drugs may be indicated. However, theoretic and practical concerns limit any formal recommendations.

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Classification of pulmonary hypertension

In an attempt to unify the classification of various forms of PH among scientists and clinicians, a consensus meeting of PH experts was convened in 2003, known as the 3rd World Symposium on Pulmonary Arterial Hypertension [19]. At this meeting, experts revised the original consensus-based PH classification system [20], incorporating all forms of PH, including pulmonary arterial hypertension (PAH) and PH due to LHD (Box 1) [21]. Within the category of pulmonary venous hypertension, two

Left-sided heart diseases leading to pulmonary venous hypertension

Box 2 lists the various forms of LHD that have the potential for causing PH. In these patients, PH results from increased LV or left atrial filling pressure and is highly correlated with Doppler echocardiographic evidence of diastolic dysfunction [22]. In patients who have systolic LV dysfunction, the presence of PH is not only related to the associated diastolic dysfunction but is also influenced by the degree of associated mitral regurgitation present [23]. In patients who have diastolic

Pulmonary hypertension “out of proportion” to left-sided heart disease

The transpulmonary gradient (TPG), defined as the mean PAP minus the pulmonary capillary wedge pressure, is usually low (<12 mm Hg) [1], [26] in cases of LHD with PH, signifying that the elevation in PAP is passive. From a purely academic standpoint, even when the TPG is high, “isolated pulmonary arteriopathy cannot be diagnosed” [27]. It is well-known, however, that in some individuals who have LHD, the PAP is elevated out of proportion to that expected by the elevated left atrial pressure. In

Prognosis of pulmonary hypertension in patients who have left-sided heart disease

A study of nearly 200 patients who had left-sided heart failure showed PH to be an independent predictor of mortality [32]. This study also showed that when pulmonary artery pressure is followed over time, an increase in mean PAP of 30% or greater was incrementally predictive of poor prognosis beyond that of a single hemodynamic measurement.

In patients who have LV systolic dysfunction, pulmonary venous pressure is elevated to varying degrees, with higher left atrial and resultant higher

Diagnostic approach

For screening purposes, echocardiography is commonly performed when PH is suspected, and in such cases, it is recommended as a first-line test by PH experts [27]. Echocardiography offers the ability to noninvasively estimate PAP and can help identify LHD. In addition to evaluation of LV systolic and valvular function, echocardiography can identify patients who have isolated LV diastolic dysfunction, an increasingly prevalent clinical condition, especially in the aging population [47]. Some

Treatment

In most patients who have LHD and associated PH, the initial therapy should focus on the underlying LHD rather than the PH (which by itself may reduce or normalize PA pressure [36], [37], [38], [39], [41], [43]). There are no drugs approved by the Food and Drug Administration that have a specific indication for the treatment of PH associated with LHD. In addition, only a small number of studies of novel treatments have focused on treating PH in these patients.

Currently, for patients who have

Summary

The presence of PH in patients who have LHD predicts a poor outcome; however, no study to date has demonstrated long-term benefit from drugs specifically designed to improve pulmonary hemodynamics. There are currently no consensus recommendations on the management of PH when coexisting LHD is present. The underlying cause of LHD should be treated first. When PH appears to be out of proportion to the degree of LHD, cautious use of drugs approved for PAH may ameliorate some of the signs and

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