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6.3.1.3 Diuretics

Right HF is associated with systemic fluid retention, reduced renal blood flow, and activation of the renin–angiotensin–aldosterone system. Increased right-sided filling pressures are transmitted to the renal veins, increasing interstitial and tubular hydrostatic pressure within the encapsulated kidney, which decreases net glomerular filtration rate and oxygen delivery.119 Avoiding fluid retention is one of the key objectives in managing patients with PH. Once these patients develop signs of right-sided HF and oedema, restricting fluid intake and using diuretics is recommended. The three main classes of diuretics—loop diuretics, thiazides, and mineralocorticoid receptor antagonists—are used as monotherapy or in combination, as determined by the patient’s clinical need and kidney function. Patients requiring diuretic therapy should be advised to regularly monitor their body weight and to seek medical advice in case of weight gain. Close collaboration between patients, PH centres, especially PH nurses, and primary care physicians plays a vital role. Kidney function and serum electrolytes should be regularly monitored, and intravascular volume depletion must be avoided as it may cause a further decline in CO and systemic blood pressure. Physicians should bear in mind that fluid retention and oedema may not necessarily signal right-sided HF, but may also be a side effect of PAH therapy.323

6.3.1.4 Oxygen

Although oxygen administration reduces PVR and improves exercise tolerance in patients with PAH, there are no data to suggest that longterm oxygen therapy has sustained benefits on the course of the disease. Most patients with PAH, except those with CHD and pulmonary-to-systemic shunts, have minor degrees of arterial hypoxaemia at rest, unless they have a patent foramen ovale. Data show that nocturnal oxygen therapy does not modify the natural history of advanced Eisenmenger syndrome.324 In the absence of robust data on the use of oxygen in patients with PAH, guidance is based on evidence in patients with COPD;325 when PaO2 is ,8 kPa (60 mmHg; alternatively, SaO2 ,92%) on at least two occasions, patients are advised to take oxygen to achieve a PaO2 .8 kPa. Ambulatory oxygen may be considered when there is evidence of symptomatic benefit and correctable desaturation on exercise.326,327 Nocturnal oxygen therapy should be considered in case of sleep-related desaturation.328

6.3.1.5 Cardiovascular drugs

No data from rigorous clinical trials are available on the usefulness and safety of drugs that are effective in systemic hypertension or leftsided HF, such as angiotensin-converting enzyme inhibitors, angiotensin receptor blockers (ARBs), angiotensin receptor–neprilysin inhibitors (ARNIs), sodium–glucose cotransporter-2 inhibitors (SGLT-2is), beta-blockers, or ivabradine in patients with PAH. In this group of patients, these drugs may lead to potentially dangerous drops in blood pressure, heart rate, or both. Likewise, the efficacy of digoxin/digitoxin has not been documented in PAH, although these drugs may be administered to slow ventricular rate in patients with PAH who develop atrial tachyarrhythmias.

6.3.1.6 Anaemia and iron status

Iron deficiency is common in patients with PAH and is defined by serum ferritin ,100 μg/L, or serum ferritin 100–299 μg/L and transferrin saturation ,20%.329 The underlying pathological mechanisms are complex.330–333 In patients with PAH, iron deficiency is associated with impaired myocardial function, aggravated symptoms, and increased mortality risk.333,334 Based on these data, regular monitoring of iron status (serum iron, ferritin, transferrin saturation, soluble transferrin receptors) is recommended in patients with PAH.

In patients with severe iron deficiency anaemia (Hb ,7–8 g/dL), i.v. supplementation is recommended.335–337 Oral iron formulations containing ferrous (Fe2+) sulfate, ferrous gluconate, and ferrous fumarate are often poorly tolerated, and drug efficacy may be impaired in patients with PAH.330,331 Ferric maltol is a new, orally available formulation of ferric (Fe3+) iron and maltol. One small, open-label study suggested good tolerability and efficacy in patients with severe PH with mild-to-moderate iron deficiency and anaemia.338 In contrast, two small, 12 week, randomized, cross-over trials studying iron supplementation in PAH patients without anaemia provided no significant clinical benefit.339 Randomized controlled trials comparing oral and i.v. iron supplementation in patients with PAH are lacking.

6.3.1.7 Vaccination

As a general health care measure, it is recommended that patients with PAH be vaccinated at least against influenza, Streptococcus pneumoniae, and SARS-CoV-2.

6.3.1.8 Psychosocial support

Receiving a diagnosis of PH—often after a substantial delay—and experiencing the physical limitations have a substantial impact on psychological, emotional, and social aspects of patients and their families. Symptoms of depression and anxiety, as well as adjustment disorders, have a high prevalence in patients with PAH. Pulmonary arterial hypertension also has grave repercussions on ability to work and income.281,340–344 Empathic and hopeful communication is essential for physicians caring for patients with PAH. Awareness and knowledge about the disease and its treatment options empower patients to engage in shared decision-making. Adequate diagnostic screening tools are the key to identifying patients in need of referral for psychological/ psychiatric support, including psychopharmacological medication,345 or social assistance. Patient support groups may play an important role, and patients should be advised to join such groups. Given the life-limiting character of PAH, advanced care planning with referral to specialist palliative care services should be supported at the right time.346

6.3.1.9 Adherence to treatments

Adhering to medical therapy is key to successfully managing PAH. In general, factors that affect adherence are patient related (e.g. demographics, cognitive impairment, polypharmacy, adverse reactions/ side effects, psychological health, health literacy, patient understanding of the treatment rationale, and comorbidities), physician related (expertise, awareness of guidelines, and multidisciplinary team approach), and health care system related (work setting, access to treatments, and cost).347 Recent studies have indicated that adherence to drug therapy in patients with PAH may be suboptimal.348,349 Given the complexity of PAH treatment, potential side effects, and risks associated with treatment interruptions, adherence should be periodically Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026