Adempas: Uses, Dosage & Side Effects
A soluble guanylate cyclase (sGC) stimulator used to treat chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH)
Adempas (riociguat) is a first-in-class soluble guanylate cyclase (sGC) stimulator used to treat two forms of pulmonary hypertension: chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH). It works through a unique dual mechanism of action, both sensitizing sGC to endogenous nitric oxide and directly stimulating the enzyme independently of nitric oxide. This leads to increased production of cyclic GMP (cGMP), causing vasodilation of the pulmonary arteries and reducing the strain on the right side of the heart. Adempas is the only approved medical therapy for inoperable or persistent/recurrent CTEPH and has demonstrated significant improvements in exercise capacity and hemodynamic parameters in clinical trials.
Quick Facts: Adempas
Key Takeaways
- Adempas (riociguat) is the first and only approved soluble guanylate cyclase (sGC) stimulator for pulmonary hypertension, working through a unique dual mechanism that increases cGMP production to dilate pulmonary blood vessels.
- It is approved for chronic thromboembolic pulmonary hypertension (CTEPH) in adults who are inoperable or have persistent disease after surgery, and for pulmonary arterial hypertension (PAH) in adults and children aged 6 years and older.
- Adempas must never be combined with PDE5 inhibitors (sildenafil, tadalafil, vardenafil), nitrates, or other sGC stimulators such as vericiguat, as this can cause dangerously low blood pressure.
- Treatment begins at a low dose (1 mg three times daily) and is gradually increased every two weeks to a maximum of 2.5 mg three times daily, guided by blood pressure monitoring.
- Adempas is strictly contraindicated in pregnancy, and women of childbearing potential must use effective contraception and undergo monthly pregnancy testing throughout treatment.
What Is Adempas and What Is It Used For?
Adempas contains the active substance riociguat, a soluble guanylate cyclase (sGC) stimulator. This represents an entirely novel pharmacological class that addresses a fundamental pathological mechanism in pulmonary hypertension. The nitric oxide–soluble guanylate cyclase–cyclic GMP (NO-sGC-cGMP) pathway plays a critical role in regulating vascular tone, and dysfunction of this pathway is a key feature of both chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH). Riociguat acts directly on this pathway through a unique dual mechanism: it both sensitizes sGC to endogenous nitric oxide (NO) and directly stimulates the enzyme independently of NO, leading to increased intracellular cGMP levels.
The increased cGMP triggers a cascade of downstream effects in the pulmonary vasculature. It activates protein kinase G, which promotes smooth muscle relaxation and vasodilation, inhibits smooth muscle cell proliferation, and reduces platelet aggregation. These combined effects lead to a decrease in pulmonary vascular resistance, reduced pulmonary arterial pressure, and improved blood flow through the lungs. Because the right side of the heart no longer needs to work as hard to pump blood through the lungs, patients experience relief from symptoms such as breathlessness, fatigue, and dizziness, along with improved exercise tolerance.
Adempas is approved for use in adults and children from 6 years of age with the following forms of pulmonary hypertension:
- Chronic thromboembolic pulmonary hypertension (CTEPH): In CTEPH, the blood vessels in the lungs become blocked or narrowed by organized blood clots that have not dissolved after pulmonary embolism. The resulting obstruction and secondary vascular remodeling lead to progressive increases in pulmonary vascular resistance and right heart failure. Adempas is used for adult patients with CTEPH who cannot undergo pulmonary endarterectomy surgery (the procedure to remove clot material from pulmonary arteries), as well as for those whose pulmonary hypertension persists or recurs after surgery. The landmark CHEST-1 trial demonstrated that riociguat significantly improved the primary endpoint of 6-minute walking distance (6MWD) by 39 meters compared with placebo, along with meaningful improvements in pulmonary vascular resistance and WHO functional class.
- Pulmonary arterial hypertension (PAH): In PAH, the walls of the small pulmonary arteries become thickened and stiff due to endothelial dysfunction, smooth muscle cell proliferation, and fibrosis, causing the vessels to narrow progressively. This increases pulmonary vascular resistance and places an unsustainable workload on the right ventricle. Adempas is used for adults and children from 6 years of age with PAH, typically in combination with endothelin receptor antagonists (such as bosentan or macitentan). Adult patients may also receive Adempas as monotherapy. The PATENT-1 trial showed that riociguat improved 6MWD by 36 meters compared with placebo, with additional benefits in pulmonary vascular resistance, NT-proBNP levels, and WHO functional class.
Pulmonary hypertension is a serious, progressive condition in which elevated blood pressure in the pulmonary arteries forces the right ventricle to work increasingly harder to pump blood through the lungs. Over time, this leads to right ventricular hypertrophy, right heart failure, and ultimately death if left untreated. Patients typically present with exertional dyspnea (shortness of breath during physical activity), fatigue, syncope (fainting), chest pain, and peripheral edema (swelling of the ankles and legs). The diagnosis is confirmed by right heart catheterization showing a mean pulmonary arterial pressure of 20 mmHg or higher.
Before Adempas, there was no approved medical therapy specifically for inoperable CTEPH, making this drug a significant therapeutic advance for this patient population. In PAH, Adempas provides an additional treatment option that targets a pathway complementary to the endothelin and prostacyclin pathways addressed by other PAH-specific therapies.
Unlike PDE5 inhibitors (such as sildenafil or tadalafil) that work by preventing the breakdown of cGMP, Adempas stimulates sGC to produce more cGMP in the first place. This is why Adempas and PDE5 inhibitors must never be used together—the combined effect on the cGMP pathway could cause dangerously low blood pressure. Adempas also has anti-proliferative and anti-fibrotic properties in the pulmonary vasculature, which may help to reverse some of the pathological vascular remodeling seen in pulmonary hypertension.
What Should You Know Before Taking Adempas?
Contraindications
There are specific situations in which Adempas must not be used. Understanding these absolute contraindications is essential before initiating treatment, as they are based on serious safety concerns identified during clinical development and post-marketing surveillance.
- Are taking PDE5 inhibitors such as sildenafil, tadalafil, or vardenafil (used for pulmonary hypertension or erectile dysfunction)
- Are taking nitrates or nitric oxide donors such as amyl nitrite (including recreational drugs known as “poppers”)
- Are taking other sGC stimulators such as vericiguat
- Are pregnant or may become pregnant
- Have severe hepatic (liver) impairment (Child-Pugh C)
- Have low blood pressure (systolic <95 mmHg in patients aged 12 or older; <90 mmHg in children aged 6 to under 12) before starting treatment
- Have pulmonary hypertension associated with idiopathic interstitial pneumonia (scarring of the lungs of unknown cause)
- Are allergic to riociguat or any of the other ingredients
The contraindication with PDE5 inhibitors is particularly important. Both riociguat and PDE5 inhibitors act on the cGMP pathway, albeit through different mechanisms: riociguat increases cGMP production while PDE5 inhibitors prevent cGMP degradation. The combination leads to an excessive and potentially life-threatening accumulation of cGMP, resulting in severe systemic hypotension. Similarly, nitrates and other nitric oxide donors potentiate the cGMP pathway and are absolutely contraindicated. The combination with other sGC stimulators such as vericiguat (used in heart failure) is also prohibited for the same pharmacological reasons.
Warnings and Precautions
Before and during treatment with Adempas, your doctor needs to be aware of various medical conditions and risk factors that may affect how you respond to treatment or require special monitoring:
- Pulmonary veno-occlusive disease (PVOD): If you have or are suspected of having PVOD, a condition characterized by fluid accumulation in the lungs due to obstruction of the pulmonary veins, your doctor may choose a different treatment. Signs of PVOD include acute onset of dyspnea, pulmonary edema, and hypoxemia. If these develop during treatment, Adempas may need to be discontinued.
- Hemoptysis and pulmonary hemorrhage: Serious pulmonary bleeding (coughing up blood) has been reported in patients taking Adempas. If you have recently experienced significant lung bleeding or have had bronchial artery embolization treatment, inform your doctor. The risk may be higher in patients taking anticoagulant medications concurrently.
- Hypotension (low blood pressure): Adempas lowers blood pressure, which is part of its therapeutic effect but can also cause symptomatic hypotension, particularly during the initial dose-titration phase. Symptoms may include dizziness, lightheadedness, or fainting. The risk is increased in patients who are already taking other blood-pressure-lowering medications, diuretics, or in elderly patients (over 65 years). Your doctor will monitor your blood pressure regularly, particularly during the first weeks of treatment.
- Smoking: Smoking significantly affects the blood levels of riociguat. Smokers tend to have lower plasma concentrations of the drug, which may reduce its effectiveness. You are strongly encouraged to stop smoking before and during treatment. If you start or stop smoking while taking Adempas, your doctor may need to adjust your dose accordingly.
- Renal impairment: If your kidneys do not function properly or if you are on dialysis, Adempas is not recommended due to limited clinical experience in this population. Your doctor will assess your kidney function before and during treatment.
- Hepatic impairment: If your liver function is reduced (mild to moderate impairment), your doctor will exercise caution with dosing. Adempas is contraindicated in severe liver impairment.
Pregnancy and Breastfeeding
Adempas is strictly contraindicated during pregnancy. Based on its mechanism of action and animal data, riociguat may cause serious harm to the developing fetus. Women and adolescent girls of childbearing potential must use effective contraception during treatment. A pregnancy test must be performed monthly while taking Adempas. Discuss appropriate contraceptive methods with your doctor before starting treatment.
Breastfeeding is not recommended during treatment with Adempas. It is not known whether riociguat or its metabolites are excreted in human breast milk, but animal studies have shown excretion in milk. Given the potential risk to the nursing infant, your doctor will help you decide whether to discontinue breastfeeding or stop the medication.
Driving and Operating Machinery
Adempas has a moderate effect on the ability to drive and use machines. It can cause dizziness, which is one of the most common side effects. You should be aware of how you respond to this medication before driving, cycling, or operating machinery. If you experience dizziness or other symptoms that could impair your ability to concentrate, refrain from these activities until the symptoms resolve.
Important Information About Ingredients
Adempas tablets contain lactose monohydrate as an excipient. If you have been told by your doctor that you have an intolerance to certain sugars, consult your doctor before taking this medication. Each tablet also contains less than 1 mmol (23 mg) of sodium, meaning it is essentially sodium-free and suitable for patients on sodium-restricted diets.
How Does Adempas Interact with Other Drugs?
Drug interactions with Adempas primarily involve two pharmacological mechanisms: additive effects on the cGMP pathway (which can cause dangerous hypotension) and alterations in the metabolism of riociguat through the cytochrome P450 enzyme system and other clearance pathways. Riociguat is metabolized by CYP1A1, CYP3A4, CYP2C8, and CYP2J2, and is also a substrate for P-glycoprotein (P-gp) and breast cancer resistance protein (BCRP). Medications that affect these enzymes or transporters can alter riociguat blood levels.
Major Interactions (Contraindicated)
| Interacting Drug | Effect | Clinical Significance |
|---|---|---|
| Sildenafil, Tadalafil, Vardenafil (PDE5 inhibitors) | Excessive cGMP accumulation leading to severe, potentially life-threatening hypotension | Absolute contraindication – never combine |
| Nitrates (e.g., nitroglycerin, isosorbide mononitrate/dinitrate) | Potentiation of hypotensive effects via enhanced NO-cGMP signaling | Absolute contraindication – never combine |
| Amyl nitrite (including recreational “poppers”) | Severe hypotension through NO-mediated vasodilation | Absolute contraindication – never combine |
| Vericiguat (another sGC stimulator) | Additive sGC stimulation with unpredictable cGMP elevation | Absolute contraindication – never combine |
Significant Interactions (Use with Caution)
| Interacting Drug | Effect | Clinical Significance |
|---|---|---|
| Ketoconazole, Itraconazole, Posaconazole (azole antifungals) | Inhibit CYP3A4 and P-gp, increasing riociguat blood levels significantly | Increased risk of adverse effects; close monitoring required |
| Ritonavir, Atazanavir, Cobicistat (HIV protease inhibitors/boosters) | Strong CYP3A4 inhibition increases riociguat exposure | Increased risk of adverse effects; monitor closely for hypotension |
| Phenytoin, Carbamazepine, Phenobarbital (antiepileptics) | CYP3A4 induction may decrease riociguat blood levels | Reduced efficacy possible; dose adjustment may be needed |
| St. John’s wort (Hypericum perforatum) | CYP3A4 induction may lower riociguat blood levels | Avoid concurrent use; may reduce therapeutic effect |
| Ciclosporin (immunosuppressant) | Inhibits P-gp and BCRP, may increase riociguat absorption | Monitor for increased side effects |
| Antacids (aluminium/magnesium hydroxide) | Reduce absorption of riociguat by raising gastric pH | Take antacids at least 2 hours before or 1 hour after Adempas |
Adempas can be taken together with certain other pulmonary hypertension medications, including endothelin receptor antagonists such as bosentan and prostacyclin analogues such as iloprost. In PAH, combination therapy with an endothelin receptor antagonist is common and has been studied in clinical trials. However, you should always inform your doctor about all medications, supplements, and herbal products you are taking to ensure safe and effective treatment.
If switching from a PDE5 inhibitor to Adempas, wait at least 24 hours after the last dose of sildenafil or at least 48 hours after the last dose of tadalafil before starting Adempas. If switching from Adempas to a PDE5 inhibitor, stop Adempas at least 24 hours before starting sildenafil or tadalafil. These washout periods are essential to prevent dangerous overlap of cGMP-elevating effects.
What Is the Correct Dosage of Adempas?
Adempas treatment should only be initiated and monitored by a physician experienced in the management of pulmonary hypertension. The dose is carefully titrated upward during the first weeks of treatment, guided by regular blood pressure measurements, to find the highest tolerated dose for each individual patient. This individualized approach helps to maximize therapeutic benefit while minimizing the risk of hypotension.
Adults
Starting Dose
Dose: 1 mg taken orally three times daily for the first 2 weeks
Route: Oral (swallowed whole, or crushed and mixed with water or soft food)
Frequency: Three times daily, approximately every 6–8 hours
Blood pressure should be measured at least every 2 weeks during the titration phase to guide dose adjustments.
Dose Titration
Increase: The dose is increased by 0.5 mg every 2 weeks if systolic blood pressure remains at or above 95 mmHg and the patient has no signs or symptoms of hypotension
Target: Maximum dose of 2.5 mg three times daily (7.5 mg total daily dose)
If blood pressure drops too low: The dose should not be increased. If the patient experiences symptomatic hypotension, the current dose should be reduced by 0.5 mg three times daily.
Your doctor will select the highest dose that you tolerate well. For some patients, lower doses three times daily may be the optimal maintenance dose.
| Week | Dose Per Administration | Total Daily Dose | Action |
|---|---|---|---|
| Weeks 1–2 | 1 mg | 3 mg | Starting dose; monitor blood pressure |
| Weeks 3–4 | 1.5 mg | 4.5 mg | Increase if SBP ≥95 mmHg |
| Weeks 5–6 | 2 mg | 6 mg | Increase if SBP ≥95 mmHg |
| Weeks 7–8+ | 2.5 mg | 7.5 mg | Maximum dose if tolerated |
Children (6 Years and Older with PAH)
Adempas is approved for children aged 6 years and older with pulmonary arterial hypertension (PAH). For pediatric patients, the dosing is weight-based and must be carefully managed by a specialist. Children weighing 50 kg or more may use tablets, while those weighing less than 50 kg should use the granules for oral suspension formulation to allow more precise dosing. The starting dose and titration follow similar principles to adult dosing, with blood pressure thresholds adjusted for age (systolic blood pressure must be at least 90 mmHg for children aged 6 to under 12 years). Adempas is not recommended for children under 6 years due to safety concerns, and it is not approved for CTEPH in patients under 18 years.
Elderly Patients (65 Years and Older)
Elderly patients may be at increased risk of hypotension during treatment with Adempas. While no specific dose adjustment is required based on age alone, your doctor may exercise additional caution during dose titration and monitor blood pressure more frequently. The dose should be increased more conservatively if there are any signs of low blood pressure.
Missed Dose
If you forget to take a dose of Adempas, do not take a double dose to make up for the missed one. Simply continue with your next scheduled dose as planned. It is important to maintain a consistent dosing schedule, taking the medication approximately every 6–8 hours, three times daily.
Overdose
If you take more Adempas tablets than prescribed, contact your doctor immediately. An overdose is likely to cause a significant drop in blood pressure (hypotension), which may manifest as dizziness, lightheadedness, fainting, or a rapid heartbeat. If blood pressure drops dangerously low, you may need immediate medical attention. There is no specific antidote for riociguat; treatment of overdose is supportive and focused on maintaining adequate blood pressure.
Treatment Interruption
Do not stop taking Adempas without consulting your doctor first. Abrupt discontinuation may lead to a worsening of your pulmonary hypertension symptoms. If you have not taken Adempas for 3 days or more, inform your doctor before resuming treatment, as re-titration of the dose may be necessary.
If you have difficulty swallowing whole tablets, Adempas can be crushed and mixed with water or soft food immediately before taking. Speak with your doctor or pharmacist about alternative administration methods if needed.
Adempas can generally be taken with or without food. However, if you tend to have low blood pressure, you should be consistent—always take it with food or always without food—to ensure stable drug absorption and minimize blood pressure fluctuations.
What Are the Side Effects of Adempas?
Like all medicines, Adempas can cause side effects, although not everybody gets them. The side effects are generally related to the vasodilatory (blood vessel-widening) mechanism of the drug. Most side effects are mild to moderate in severity and tend to occur more frequently during the initial dose-titration phase. Your doctor will monitor you closely and may adjust your dose if side effects become troublesome.
Serious Side Effects Requiring Immediate Medical Attention
- Hemoptysis (coughing up blood): Common (may affect up to 1 in 10 people). Contact your doctor immediately.
- Pulmonary hemorrhage (acute lung bleeding): Uncommon (may affect up to 1 in 100 people). This is potentially life-threatening and requires urgent medical care.
Side Effects in Adults
Very Common
May affect more than 1 in 10 people
- Dizziness
- Headache
- Indigestion (dyspepsia)
- Diarrhea
- Nausea
- Vomiting
- Peripheral edema (swelling of the arms and legs)
Common
May affect up to 1 in 10 people
- Gastroenteritis (inflammation of the digestive tract)
- Anemia (low red blood cell levels) – may cause pale skin, fatigue, or breathlessness
- Palpitations (irregular, hard, or fast heartbeat)
- Hypotension (low blood pressure)
- Epistaxis (nosebleeds)
- Nasal congestion (blocked nose)
- Gastritis (stomach inflammation)
- Gastroesophageal reflux (heartburn)
- Dysphagia (difficulty swallowing)
- Gastrointestinal and abdominal pain
- Constipation
- Abdominal distension (bloating)
- Hemoptysis (coughing up blood)
Uncommon
May affect up to 1 in 100 people
- Pulmonary hemorrhage (acute lung bleeding) – may be life-threatening
Side Effects in Children
The side effects observed in children aged 6 to under 18 years treated with Adempas were generally similar to those seen in adults. The most frequently reported side effects in the pediatric population were:
Very Common in Children
May affect more than 1 in 10 children
- Hypotension (low blood pressure)
Common in Children
May affect up to 1 in 10 children
- Headache
If you experience any side effects, even those not listed here, inform your doctor or pharmacist. Reporting side effects helps to continuously monitor the benefit-risk balance of the medication and contributes to the overall safety knowledge of the drug.
How Should You Store Adempas?
Adempas tablets do not require any special storage conditions. They should be stored at room temperature in their original packaging to protect them from moisture. Always keep the medication out of the sight and reach of children to prevent accidental ingestion, which could be particularly dangerous given the blood pressure-lowering effects of riociguat.
Check the expiry date on the carton before taking the medication. The expiry date refers to the last day of the stated month. Do not use Adempas if the expiry date has passed, as the quality, safety, and efficacy of the medication may no longer be guaranteed.
When you no longer need Adempas or if the medication has expired, do not throw it in household waste or flush it down the toilet. Return unused medication to your pharmacist for proper disposal. These measures help protect the environment from pharmaceutical contamination of water and soil.
What Does Adempas Contain?
Adempas is available as film-coated tablets in five different strengths, each containing a different amount of the active substance riociguat. The tablets are round, biconvex, 6 mm in diameter, and are marked with the Bayer cross on one side and the strength value plus an “R” on the other.
| Strength | Color | Imprint |
|---|---|---|
| 0.5 mg | White | Bayer cross / 0.5 R |
| 1 mg | Pale yellow | Bayer cross / 1 R |
| 1.5 mg | Yellow-orange | Bayer cross / 1.5 R |
| 2 mg | Light orange | Bayer cross / 2 R |
| 2.5 mg | Red-orange | Bayer cross / 2.5 R |
Active substance: Riociguat (0.5 mg, 1 mg, 1.5 mg, 2 mg, or 2.5 mg per tablet depending on the strength).
Inactive ingredients (excipients):
- Tablet core: Microcrystalline cellulose, crospovidone (type B), hypromellose 5 cP, lactose monohydrate, magnesium stearate, and sodium lauryl sulfate
- Film coating: Hydroxypropyl cellulose, hypromellose 3 cP, propylene glycol (E 1520), and titanium dioxide (E 171)
- Additional colorants: The 1 mg and 1.5 mg tablets also contain iron oxide yellow (E 172). The 2 mg and 2.5 mg tablets contain both iron oxide yellow (E 172) and iron oxide red (E 172).
Adempas is available in packs of 42, 84, 90, or 294 tablets in transparent calendar blister packs. Not all pack sizes may be available in all countries. The marketing authorization holder is Bayer AG, Leverkusen, Germany.
Frequently Asked Questions About Adempas
Adempas (riociguat) and sildenafil both act on the nitric oxide–cGMP pathway, but through different mechanisms. Sildenafil is a PDE5 inhibitor that prevents the breakdown of cGMP, while Adempas stimulates soluble guanylate cyclase (sGC) to produce more cGMP in the first place. Importantly, Adempas is the only approved drug treatment for inoperable CTEPH, whereas sildenafil is approved only for PAH. These two drugs must never be used together because the combined effect on cGMP levels can cause dangerously low blood pressure. If switching between them, a washout period is required.
Adempas begins working shortly after ingestion, with peak blood levels reached within 1–1.5 hours. However, the clinical benefits in terms of improved exercise capacity and symptom relief develop gradually over the dose-titration period, which typically takes 8 weeks as the dose is increased every 2 weeks. In the CHEST-1 and PATENT-1 clinical trials, the primary efficacy assessments were performed at 16 weeks. Full therapeutic benefit is usually apparent after several weeks at the optimal maintenance dose.
There is no specific prohibition on alcohol consumption with Adempas. However, alcohol can lower blood pressure, and since Adempas also reduces blood pressure, the combination may increase the risk of hypotension (dizziness, lightheadedness, or fainting). If you choose to drink alcohol, do so in moderation and be cautious about potential additive blood pressure-lowering effects. Discuss your alcohol consumption with your doctor.
There is limited data on the effect of riociguat on human fertility. Animal studies have not shown adverse effects on male or female fertility at clinically relevant doses. However, because Adempas is strictly contraindicated in pregnancy, women of childbearing potential must use effective contraception and undergo monthly pregnancy tests during treatment. If you have concerns about fertility, discuss them with your healthcare provider before starting treatment.
Dizziness is one of the most common side effects of Adempas and is typically related to its blood pressure-lowering effect. If you experience severe dizziness, sit or lie down immediately and avoid sudden changes in position. Do not drive or operate machinery. Contact your doctor, who may need to reduce your dose or adjust the titration schedule. If the dizziness is accompanied by fainting, rapid heartbeat, or confusion, seek medical attention promptly. Taking Adempas consistently with or without food (not alternating) can help minimize blood pressure fluctuations.
No, Adempas is not a cure for pulmonary hypertension. It is a chronic treatment that manages the symptoms and slows disease progression by reducing pulmonary vascular resistance and improving exercise capacity. Treatment is typically continued long-term, and stopping the medication may lead to deterioration of the condition. For CTEPH, pulmonary endarterectomy (surgical removal of clots) remains the only potentially curative treatment for eligible patients. Adempas plays an important role for patients who cannot undergo surgery or who have persistent disease after surgery.
References
- European Medicines Agency (EMA). Adempas (riociguat) – Summary of Product Characteristics. Last updated 2025. Available at: www.ema.europa.eu
- U.S. Food and Drug Administration (FDA). Adempas (riociguat) tablets – Prescribing Information. Last updated 2024. Available at: www.fda.gov
- Ghofrani H-A, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319–329. doi:10.1056/NEJMoa1209657 (CHEST-1 trial)
- Ghofrani H-A, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4):330–340. doi:10.1056/NEJMoa1209655 (PATENT-1 trial)
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618–3731. doi:10.1093/eurheartj/ehac237
- Kim NH, Delcroix M, Jais X, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(1):1801915. doi:10.1183/13993003.01915-2018
- Simonneau G, D’Armini AM, Ghofrani H-A, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45(5):1293–1302. doi:10.1183/09031936.00087114
- World Health Organization (WHO). WHO Model List of Essential Medicines – 23rd list. 2023. Available at: www.who.int
- British National Formulary (BNF). Riociguat. Available at: bnf.nice.org.uk
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