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Therapeutic strategies in pulmonary hypertension.

Fuso L, Baldi F, Di Perna A - Front Pharmacol (2011)

Bottom Line: The management of patients with PH has advanced rapidly over the last decade and the introduction of specific treatments especially for PAH has lead to an improved outcome.These drugs act in several pathogenetic mechanisms of the PH and are specific for PAH although they might be used also in the other groups of PH.It is also possible to adopt a goal-oriented therapy in which the timing of treatment escalation is determined by inadequate response to known prognostic indicators.

View Article: PubMed Central - PubMed

Affiliation: Respiratory Disease Unit, Catholic University Rome, Italy.

ABSTRACT
Pulmonary hypertension (PH) is a life-threatening condition characterized by elevated pulmonary arterial pressure. It is clinically classified into five groups: patients in the first group are considered to have pulmonary arterial hypertension (PAH) whereas patients of the other groups have PH that is due to cardiopulmonary or other systemic diseases. The management of patients with PH has advanced rapidly over the last decade and the introduction of specific treatments especially for PAH has lead to an improved outcome. However, despite the progress in the treatment, the functional limitation and the survival of these patients remain unsatisfactory and there is no cure for PAH. Therefore the search for an "ideal" therapy still goes on. At present, two levels of treatment can be identified: primary and specific therapy. Primary therapy is directed at the underlying cause of the PH. It also includes a supportive therapy consisting in oxygen supplementation, diuretics, and anticoagulation which should be considered in all patients with PH. Specific therapy is directed at the PH itself and includes treatment with vasodilatators such as calcium channel blockers and with vasodilatator and pathogenetic drugs such as prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors. These drugs act in several pathogenetic mechanisms of the PH and are specific for PAH although they might be used also in the other groups of PH. Finally, atrial septostomy and lung transplantation are reserved for patients refractory to medical therapy. Different therapeutic approaches can be considered in the management of patients with PH. Therapy can be established on the basis of both the clinical classification and the functional class. It is also possible to adopt a goal-oriented therapy in which the timing of treatment escalation is determined by inadequate response to known prognostic indicators.

No MeSH data available.


Related in: MedlinePlus

Goal-oriented treatment algorithm for patients with pulmonary arterial hypertension (PAH). 6MWD, 6-min walking distance. Reproduced with permission from Hoeper et al. (2005).
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Figure 3: Goal-oriented treatment algorithm for patients with pulmonary arterial hypertension (PAH). 6MWD, 6-min walking distance. Reproduced with permission from Hoeper et al. (2005).

Mentions: This approach concerns patients with PAH in whom the objective of treatment is to reduce functional impairment and prolong life (Sitbon and Galiè, 2010). Goal-oriented therapy uses known prognostic indicators as treatment targets thus facilitating early intervention and therapeutic escalation before patients deteriorate. A crucial role in this strategy is played by the identification of parameters that correlate with the risk of deterioration and mortality. Several data indicate that WHO functional class is the most appropriate prognostic marker, with 6-min walking distance and some hemodynamic parameters representing alternatives (Sitbon and Galiè, 2010). The goal-oriented treatment algorithm proposed by Hoeper et al. (2005) identifies 6-min walking distance, peak oxygen consumption, and peak systolic blood pressure as prognostic indicators and set values for each of them that act as goals (Figure 3). According to this algorithm, patients were started on first-line bosentan monotherapy: if patients met the three treatment goals during follow-up, monotherapy was continued but, if a goal was not met, additional therapy was added. The authors reported that after 3 years of follow-up survival improved by using this goal-oriented therapy (80 versus 63% of historical controls; Hoeper et al., 2005).


Therapeutic strategies in pulmonary hypertension.

Fuso L, Baldi F, Di Perna A - Front Pharmacol (2011)

Goal-oriented treatment algorithm for patients with pulmonary arterial hypertension (PAH). 6MWD, 6-min walking distance. Reproduced with permission from Hoeper et al. (2005).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3108478&req=5

Figure 3: Goal-oriented treatment algorithm for patients with pulmonary arterial hypertension (PAH). 6MWD, 6-min walking distance. Reproduced with permission from Hoeper et al. (2005).
Mentions: This approach concerns patients with PAH in whom the objective of treatment is to reduce functional impairment and prolong life (Sitbon and Galiè, 2010). Goal-oriented therapy uses known prognostic indicators as treatment targets thus facilitating early intervention and therapeutic escalation before patients deteriorate. A crucial role in this strategy is played by the identification of parameters that correlate with the risk of deterioration and mortality. Several data indicate that WHO functional class is the most appropriate prognostic marker, with 6-min walking distance and some hemodynamic parameters representing alternatives (Sitbon and Galiè, 2010). The goal-oriented treatment algorithm proposed by Hoeper et al. (2005) identifies 6-min walking distance, peak oxygen consumption, and peak systolic blood pressure as prognostic indicators and set values for each of them that act as goals (Figure 3). According to this algorithm, patients were started on first-line bosentan monotherapy: if patients met the three treatment goals during follow-up, monotherapy was continued but, if a goal was not met, additional therapy was added. The authors reported that after 3 years of follow-up survival improved by using this goal-oriented therapy (80 versus 63% of historical controls; Hoeper et al., 2005).

Bottom Line: The management of patients with PH has advanced rapidly over the last decade and the introduction of specific treatments especially for PAH has lead to an improved outcome.These drugs act in several pathogenetic mechanisms of the PH and are specific for PAH although they might be used also in the other groups of PH.It is also possible to adopt a goal-oriented therapy in which the timing of treatment escalation is determined by inadequate response to known prognostic indicators.

View Article: PubMed Central - PubMed

Affiliation: Respiratory Disease Unit, Catholic University Rome, Italy.

ABSTRACT
Pulmonary hypertension (PH) is a life-threatening condition characterized by elevated pulmonary arterial pressure. It is clinically classified into five groups: patients in the first group are considered to have pulmonary arterial hypertension (PAH) whereas patients of the other groups have PH that is due to cardiopulmonary or other systemic diseases. The management of patients with PH has advanced rapidly over the last decade and the introduction of specific treatments especially for PAH has lead to an improved outcome. However, despite the progress in the treatment, the functional limitation and the survival of these patients remain unsatisfactory and there is no cure for PAH. Therefore the search for an "ideal" therapy still goes on. At present, two levels of treatment can be identified: primary and specific therapy. Primary therapy is directed at the underlying cause of the PH. It also includes a supportive therapy consisting in oxygen supplementation, diuretics, and anticoagulation which should be considered in all patients with PH. Specific therapy is directed at the PH itself and includes treatment with vasodilatators such as calcium channel blockers and with vasodilatator and pathogenetic drugs such as prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors. These drugs act in several pathogenetic mechanisms of the PH and are specific for PAH although they might be used also in the other groups of PH. Finally, atrial septostomy and lung transplantation are reserved for patients refractory to medical therapy. Different therapeutic approaches can be considered in the management of patients with PH. Therapy can be established on the basis of both the clinical classification and the functional class. It is also possible to adopt a goal-oriented therapy in which the timing of treatment escalation is determined by inadequate response to known prognostic indicators.

No MeSH data available.


Related in: MedlinePlus