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Secondary Pulmonary Hypertension Medication

  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Ryland P Byrd, Jr, MD  more...
 
Updated: Jan 27, 2016
 

Medication Summary

Although treatment of secondary pulmonary arterial hypertension (SPAH) consists primarily of that necessary for the underlying disease, several medications are used in different clinical settings, as is oxygen supplementation. Currently, definite proof of effectiveness is lacking for several of these treatments.

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Calcium Channel Blockers

Class Summary

The efficacy of calcium channel blockers (CCBs) has been evaluated primarily in patients with idiopathic pulmonary arterial hypertension (IPAH). The efficacy of CCBs in patients with SPH remains unclear. In selected patients (eg, patients with scleroderma), these agents may be tried only after a vasodilator response is demonstrated. CCBs act by inhibiting calcium ions from entering slow channels or select voltage-sensitive areas of vascular smooth muscle.

Nifedipine (Adalat, Procardia, Nifedical)

 

Nifedipine is a vasodilator that dilates both systematic and pulmonary vascular beds. Higher than usual doses are required for optimal vasodilation of pulmonary arteries.

Diltiazem (Cardizem, Dilacor, Cartia XT, Tiazac)

 

During depolarization, diltiazem inhibits calcium ions from entering slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium. It produces vasodilation but causes less reflex tachycardia than nifedipine does. Diltiazem may be useful if patients develop excessive hypotension with nifedipine.

Amlodipine (Norvasc)

 

Amlodipine has a longer duration of action than nifedipine or diltiazem and requires less frequent dosing. Experience with this agent in pulmonary hypertension is not as extensive as that with other agents. Amlodipine has fewer effects on conduction and infrequently causes atrioventricular (AV) block.

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Vasodilators

Class Summary

Prostacyclin is a strong vasodilator of all vascular beds and a potent endogenous inhibitor of platelet aggregation. Platelet effects result from activation of intracellular adenylate cyclase and from increased cyclic adenosine monophosphate (cAMP) concentrations within platelets. Prostacyclin may decrease thrombogenesis and platelet clumping in the lungs by inhibiting platelet aggregation.

Epoprostenol (Flolan, Veletri)

 

Long-term infusion of epoprostenol improves outcome in patients with primary pulmonary arterial hypertension and selected patients with SPAH. The short-term vasodilator response appears to be unrelated because a favorable impact on disease progression occurs with long-term therapy. The dosage is determined during a dose-effect study performed in a catheterization laboratory or intensive care unit (ICU). The selected dosage produces maximum vasodilation with minimum systemic hypotension.

Treprostinil (Remodulin, Tyvaso, Orenitram)

 

Treprostinil is used to treat PAH. It elicits direct vasodilation of pulmonary and systemic arterial vessels and inhibits platelet aggregation. Vasodilation reduces right and left ventricular afterload and increases cardiac output and stroke volume.

Inhaled treprostinil is indicated for PAH in patients with New York Heart Association (NYHA) class III symptoms. It elicits direct vasodilation of pulmonary and systemic arterial vessels and inhibits platelet aggregation. Vasodilation reduces right and left ventricular afterload and increases cardiac output and stroke volume. It is available as a SC injection, oral inhalation, and oral extended-release tablet.

Treprostinil (Remodulin, Tyvaso)

 

Treprostinil is used to treat PAH. It elicits direct vasodilation of pulmonary and systemic arterial vessels and inhibits platelet aggregation. Vasodilation reduces right and left ventricular afterload and increases cardiac output and stroke volume.

Inhaled treprostinil is indicated for PAH in patients with New York Heart Association (NYHA) class III symptoms. It elicits direct vasodilation of pulmonary and systemic arterial vessels and inhibits platelet aggregation. Vasodilation reduces right and left ventricular afterload and increases cardiac output and stroke volume.

Iloprost (Ventavis)

 

Iloprost is a synthetic analogue of prostacyclin that dilates systemic and pulmonary arterial vascular beds. It is indicated for World Health Organization (WHO) class I PAH in patients with NYHA class III or IV symptoms to improve exercise tolerance and symptoms and to delay deterioration.

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PAH, Prostacyclin Agonists

Class Summary

Selexipag is the first prostacyclin agonist approved in the United States.

Selexipag (Uptravi)

 

Selectively activates the prostacyclin receptor (ie, IP-receptor), one of 5 types of prostanoid receptors. Unlike prostacyclin analogs, selexipag is selective for the IP receptor over other prostanoid receptors (ie, EP1-4, DP, FP, TP). Activating the IP receptor induces vasodilation and inhibits proliferation of vascular smooth muscle cells. It is indicated for adults with WHO group I PAH to delay disease progression and reduce the risk of hospitalization.

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Endothelin-Receptor Antagonists

Class Summary

Endothelin-receptor antagonists competitively bind to the endothelin-1 receptors EtA and EtB, causing reductions in pulmonary arterial pressure, pulmonary vascular resistance, and mean right atrial pressure.

Bosentan (Tracleer)

 

Bosentan is indicated for the treatment of PAH in patients with WHO class III or IV symptoms to improve exercise ability and decrease the rate of clinical decline. It inhibits vessel constriction and elevation of blood pressure by competitively binding to EtA and EtB receptors in endothelium and vascular smooth muscle. This leads to a significant increase in cardiac index associated with significant reductions in pulmonary arterial pressure, pulmonary vascular resistance, and mean right atrial pressure.

Because of its teratogenic potential, bosentan can be prescribed only through the Tracleer Access Program (phone: 1-866-228-3546).

Ambrisentan (Letairis)

 

Ambrisentan is indicated for the treatment of WHO group 1 PAH in patients to 1) improve exercise ability and delay clinical worsening; and 2) in combination with tadalafil to reduce the risks of disease progression and hospitalization for worsening PAH, and to improve exercise ability. It inhibits vessel constriction and elevation of blood pressure by competitively binding to EtA and EtB receptors in endothelium and vascular smooth muscle. This leads to a significant increase in cardiac index associated with significant reductions in pulmonary arterial pressure, pulmonary vascular resistance, and mean right atrial pressure.

Because of the risks of hepatic injury and potential teratogenesis, ambrisentan is available only through the Letairis Education and Access Program (LEAP). Prescribers and pharmacies must register with LEAP in order to prescribe and dispense. For more information, see http://www.letairis.com or call (866) 664-LEAP (5327).

Macitentan (Opsumit)

 

Macitentan is a dual endothelin receptor antagonist that prevents binding of ET1 to both ETA and ETB receptors. It is indicated to delay disease progression of pulmonary arterial hypertension (WHO Group I).

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Phosphodiesterase-5 Enzyme Inhibitors

Class Summary

The antiproliferative effects of the phosphodiesterase type 5 (PDE5) pathway, which regulates cyclic guanosine monophosphate (cGMP) hydrolysis, may be significant in the long-term treatment of PAH with PDE5 inhibitors such as sildenafil.

Sildenafil (Revatio)

 

Sildenafil promotes selective smooth-muscle relaxation in lung vasculature, possibly by inhibiting PDE5. This results in a subsequent reduction of blood pressure in pulmonary arteries and an increase in cardiac output.

Tadalafil (Adcirca)

 

Tadalafil is a PDE5 inhibitor indicated for improving exercise capacity in patients with WHO class 1 PAH. It increases cGMP, the final mediator in the nitric oxide pathway.

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Soluble Guanylate Cyclase (sGC) Stimulators

Class Summary

Soluble guanylate cyclase (sGC) is an enzyme in the cardiopulmonary system and the receptor for nitric oxide (NO). Pulmonary arterial hypertension (PAH) is associated with endothelial dysfunction, impaired synthesis of NO, and insufficient stimulation of the NO-sGC-cGMP pathway.

Riociguat (Adempas)

 

Riociguat elicits a dual mode of action. It sensitizes sGC to endogenous NO by stabilizing the NO-sGC binding, and it directly stimulates sGC via a different binding site, independently of NO. It is indicated for chronic thromboembolic pulmonary hypertension and PAH.

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Cardiac Glycosides

Class Summary

Cardiac glycosides are used for prevention and treatment of supraventricular arrhythmias associated with SPAH and for patients who have concomitant left-sided heart failure. Digoxin is not useful in the treatment of right-sided ventricular failure.

Digoxin (Lanoxin)

 

Digoxin is a cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. It acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

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Diuretics, Loop

Class Summary

Diuretics should be employed in patients who develop right-sided heart failure and those who have systemic congestion manifested by hepatomegaly, ascites, and marked lower extremity edema. Severe right-sided heart failure may also compromise function of left ventricle leading to pulmonary congestion. Therefore, judicious use of diuretics helps reduce systemic congestion and edema. However, excessive hypovolemia may lower cardiac output further and interfere with tissue oxygenation.

Furosemide (Lasix)

 

Furosemide increases excretion of water by interfering with the chloride-binding cotransport system, thereby, in turn, inhibiting sodium and chloride reabsorption in the ascending loop of Henle and the distal renal tubule. The dosage must be individualized to the patient. Depending on the response, administer furosemide in increments of 20-40 mg, no sooner than 6-8 hours after the previous dose, until the desired diuresis occurs. When treating infants, titrate with increments of 1 mg/kg until a satisfactory effect is achieved.

Bumetanide

 

Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium, potassium, and chloride reabsorption in ascending loop of Henle. These effects increase urinary excretion of sodium, chloride, and water, resulting in profound diuresis. Renal vasodilation occurs following administration, renal vascular resistance decreases, and renal blood flow is enhanced.

Individualize dose to patient. Start at 1-2 mg IV; titrate to as high as 10 mg/day. Rarely, doses as high as 24 mg/day are used for edema but generally are not required for treatment of hyperkalemia.

One mg of bumetanide is equivalent to approximately 40 mg of furosemide.

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Anticoagulants

Class Summary

Long-term anticoagulation with warfarin should be considered in selected patients with SPAH. These include patients with chronic pulmonary embolism (PE), pulmonary veno-occlusive disease, and atrial fibrillation induced by left- or right-sided heart failure who are at high risk for developing venous thromboembolism (eg, those with cor pulmonale or immobility secondary to severe dyspnea).

Warfarin (Coumadin, Jantoven)

 

Warfarin interferes with hepatic synthesis of vitamin K–dependent coagulation factors. It is used for prophylaxis and treatment of deep vein thrombosis (DVT), PE, and thromboembolic disorders.

Tailor the dose to maintain an international normalized ratio (INR) in the range of 2-3. Recurrence of DVT and PE increases dramatically when the INR drops below 2 and decreases when it is kept between 2 and 3. Serious bleeding risk (including hemorrhagic stroke) is approximately constant when the INR is between 2.5 and 4.5 but rises dramatically when it exceeds 5.

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Contributor Information and Disclosures
Author

Nader Kamangar, MD, FACP, FCCP, FCCM Professor of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Chief, Division of Pulmonary and Critical Care Medicine, Vice-Chair, Department of Medicine, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM is a member of the following medical societies: Academy of Persian Physicians, American Academy of Sleep Medicine, American Association for Bronchology and Interventional Pulmonology, American College of Chest Physicians, American College of Critical Care Medicine, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, Association of Pulmonary and Critical Care Medicine Program Directors, Association of Specialty Professors, California Sleep Society, California Thoracic Society, Clerkship Directors in Internal Medicine, Society of Critical Care Medicine, Trudeau Society of Los Angeles, World Association for Bronchology and Interventional Pulmonology

Disclosure: Nothing to disclose.

Coauthor(s)

Sat Sharma, MD, FRCPC Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, World Medical Association

Disclosure: Nothing to disclose.

Kelvin Chan, MD Resident Physician, Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Ryland P Byrd, Jr, MD Professor of Medicine, Division of Pulmonary Disease and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University

Ryland P Byrd, Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

Oleh Wasyl Hnatiuk, MD Program Director, National Capital Consortium, Pulmonary and Critical Care, Walter Reed Army Medical Center; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences

Oleh Wasyl Hnatiuk, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Shahriar Pirouz, MD Resident Physician, Department of Internal Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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Gross pathology on patient who died of severe pulmonary arterial hypertension secondary to persistent patent ductus arteriosus.
Close-up view of gross pathology on patient who died of severe arterial pulmonary hypertension secondary to persistent patent ductus arteriosus.
During pulmonary arterial thromboendarterectomy, bilateral proximal thrombus was carefully dissected and extracted, leading to resolution of secondary pulmonary arterial hypertension.
Chest radiograph of patient with secondary pulmonary arterial hypertension shows enlarged pulmonary arteries. This patient had atrial septal defect.
54-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. The patient was found to have severe pulmonary arterial hypertension.
54-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened on exertion. Spiral CT showed enlarged pulmonary arteries but no evidence of thromboembolism.
Ventilation-perfusion scan of bilateral mismatched segmental and subsegmental defects, suggesting chronic thromboembolic hypertension.
Left pulmonary arterial angiogram shows large central pulmonary arteries and attenuation of peripheral vessels, but thrombosis cannot be identified, because it has organized along vessel walls.
Bilateral angiography should be performed in patients suspected of having chronic thromboembolic pulmonary arterial hypertension. This right pulmonary arterial angiogram shows no evidence of filling defect, therefore excluding acute thrombosis. Angioscopy is potentially useful in this setting.
 
 
 
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