Pharmacotherapy Of Pulmonary Hypertension Research Articles

Updates in the Pharmacotherapy of Pulmonary Hypertension in Patients with Heart Failure with Preserved Ejection Fraction.

Pulmonary hypertension (PH) associated with left heart disease (LHD) is a complex cardiopulmonary condition where a variable degree of pulmonary congestion, arterial vasoconstriction and vascular remodeling can lead to PH and right heart strain. Right heart dysfunction has a significant prognostic impact on these patients. Therefore, preserving right ventricular (RV) function is an important treatment goal. However, the treatment of PH in patients with left heart disease has produced conflicting evidence. The transition from pure LHD to LHD with PH is a continuum and clinically challenging. The heart failure with preserved ejection fraction (HFpEF) patient population is heterogeneous when it comes to PH and RV function. Appropriate clinical and hemodynamic phenotyping of patients with HFpEF and concomitant PH is paramount to making the appropriate treatment decision. This manuscript will summarize the current evidence for the use of pulmonary arterial vasodilators in patients with HFpEF.

Severe Pulmonary Hypertension Secondary to Concomitant Mitral Stenosis with Veno-occlusive Disease in the Context of Systemic Sclerosis: Importance of Careful and Comprehensive Assessment

Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/ hemodynamic criteria that are a consequence of several etiologies. Confirmation of pulmonary hypertension is based on right heart catheterization.
 Pulmonary hypertension is a devastating condition that can lead to considerable morbidity and premature mortality. In the last few decades, significant advancement in the pharmacotherapy of pulmonary hypertension has resulted from better understanding of the complex pathogenesis and pathophysiology of this dreaded disease. Despite these accomplishments, pharmacotherapy of pulmonary hypertension is still far from perfect, and the mortality in this modern treatment era is still unacceptably high.
 We report a complex clinical presentation characterized by severe pulmonary hypertension secondary to concomitant mitral stenosis with veno-occlusive disease in the context of systemic sclerosis.
 Our case highlights the importance of a systematic and comprehensive diagnostic approach to avoid missing an underlying pathology.

Pharmacotherapy for Pulmonary Hypertension in Infants with Bronchopulmonary Dysplasia: Past, Present, and Future.

Approximately 8-42% of premature infants with chronic lung disease of prematurity, bronchopulmonary dysplasia (BPD), develop pulmonary hypertension (PH). Infants with BPD-PH carry alarmingly high mortality rates of up to 47%. Effective PH-targeted pharmacotherapies are desperately needed for these infants. Although many PH-targeted pharmacotherapies are commonly used to treat BPD-PH, all current use is off-label. Moreover, all current recommendations for the use of any PH-targeted therapy in infants with BPD-PH are based on expert opinion and consensus statements. Randomized Control Trials (RCTs) are needed to determine the efficacy of PH-targeted treatments in premature infants with or at risk of BPD-PH. Prior to performing efficacy RCTs, studies need to be conducted to obtain pharmacokinetic, pharmacodynamic, and safety data for any pharmacotherapy used in this understudied and fragile patient population. This review will discuss current and needed treatment strategies, identify knowledge deficits, and delineate both challenges to be overcome and approaches to be taken to develop effective PH-targeted pharmacotherapies that will improve outcomes for premature infants with or at risk of developing BPD-PH.

Recent advances in the pharmacotherapy of pulmonary hypertension: practical considerations.

Pulmonary arterial hypertension (PAH) is a rare disease characterized by pulmonary vascular remodeling leading to increased vascular resistance. The increased afterload contributes to the development of right ventricular dysfunction and heart failure, which is the leading cause of death among patients with PAH. The development of specific treatments has markedly improved the prognosis of this population. However, PAH continues to be an incurable, life‑limiting condition, which creates a major burden for healthcare systems. This review describes the currently used treatments for PAH and provides insight into novel therapeutic targets that aim to reduce vascular remodeling, which ultimately leads to right ventricular failure.

Update on pharmacotherapy for pulmonary hypertension.

Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis if not treated. Pharmacological treatment options for PAH have increased significantly over the past 10 years, with availability of intravenous, oral and inhaled drugs targeting the nitric oxide, endothelin and prostacyclin pathways. Treatment with these therapies in specialised pulmonary hypertension centres has resulted in reductions in patient symptoms, disease progression and mortality, and improved exercise capacity. Recognition of chronic thromboembolic pulmonary hypertension is important, as this cause of pulmonary hypertension may be amenable to surgical treatment. Several new oral drugs, including macitentan, riociguat and selexipag, some of which have novel modes of action, and the use of combinations of PAH drugs have recently been shown to be beneficial in treating PAH and are likely to change treatment for this condition in the future.

Dynamics of clinical and immunological parameters of pharmacotherapy of pulmonary hypertension in patients with CHD in the surgical treatment stages

Dynamics of clinical and immunological parameters of pharmacotherapy of pulmonary hypertension in patients with CHD in the surgical treatment stages

Pharmacotherapy of pulmonary hypertension (according to the 2015 ESC/EAS Guidelines for the diagnosis and treatment of pulmonary hypertension)

Indications and efficacy of drugs used for the treatment of pulmonary hypertension are considered. Stages of the treatment of pulmonary arterial hypertension are discussed, including maintenance therapy, specific therapy with calcium channel blockers, prostacyclin analogues, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclin agonists, guanylate cyclase stimulators, combination treatment.

Primary Antiphospholipid Syndrome and Pulmonary Hypertension

Primary antiphospholipid syndrome (APS) is a disease characterized by the presence of autoantibodies reacting with proteins bound to phospholipids, leading to thrombosis and gestation abnormalities. Prothrombotic states and impaired clot dissolution are believed to contribute to the occurrence of chronic thromboembolic pulmonary hypertension (CTEPH) in APS. Whether preventive anticoagulation therapy in patients with antiphospholipid autoantibodies without a history of thrombosis reduces the risk of thrombosis is currently unclear. The diagnosis and treatment of CTEPH in APS is similar to CTEPH complicated by other predisposing conditions, with surgical treatment being the most effective. However, not every patient with CTEPH is suitable for pulmonary thromboendartarectomy and such individuals may benefit from pharmacotherapy of pulmonary hypertension, given the presence of pulmonary microvascular abnormalities similar to those in idiopathic pulmonary hypertension. Anticoagulation therapy is the mainstay of management because of the high risk of recurrent embolization and local in-situ thrombosis.

Abstract 609: Potential Role Of Adipose Derived Stem Cells In Attenuating Pulmonary Hypertension

Pulmonary hypertension (PH) is a devastating cardiopulmonary disorder, which often results in right heart failure. In spite of recent advancements in the pharmacotherapy of pulmonary hypertension, the mortality rate has not been significantly reduced. Recently stem cell therapy has gained more attention for the treatment of cardiopulmonary diseases. Despite the promising results shown by bone marrow derived stem cells in attenuating right heart failure in PH, it remains a highly invasive and laborious procedure. On the contrary, adipose derived stem cell (ADSC) presents a less complicated option. Therefore, we proposed to use ADSC in attenuating the progression of PH. Monocrotaline (MCT) at a 50 mg/kg dose was injected to eight-week old male Sprague-Dawley rats to induce PH. Four weeks post MCT injection; animals developed PH with elevated right ventricular systolic pressure (RVSP, Control: 28.65±0.40; MCT: 78.06±6.4 mmHg; n=6-8), and right ventricular hypertrophy (RVH, Control; 0.22±0.005; MCT: 0.48±0.054 [ratio, AU]; n=6-8). ADSC (isolated from inguinal fat of healthy male rat) were characterized using flow cytometry (CD44 + , CD90 + , CD34 - and CD45 - ) and their differentiation to adipocyte was confirmed by Oil Red O staining. ADSCs (1x10 6 ; passage 2-4) were injected through the jugular vein, 14 days post MCT injection. After 2 weeks following ADSC therapy, RVSP (MCT+ADSC: 55.67±8.99 mmHg; n=6-8) and RVH (MCT+ADSC: 0.32±0.062 [AU]; n=6-8) was significantly reduced. Moreover, the elevated right ventricular end diastolic pressure (RVEDP) and ±dP/dT associated with PH were attenuated significantly in ADSC treated MCT animals. An additional group of animals received only the ADSC conditioned media (CM) from 1x10 6 cells. After 2 weeks of CM administration, RVSP (MCT+CM: 42.11±4.66 mmHg; n=5) and RVH (MCT+CM: 0.29±0.020 [AU]; n=5) was significantly reduced. Further, the RVEDP and ± dP/dT were also significantly reduced in CM treated MCT animals. Collectively, either ADSC or conditioned media alone can attenuate the progression of PH, which suggest that paracrine-like substances may be responsible for the beneficial effects observed and may be considered as a potential therapeutic alternative for the treatment of right heart failure.