Exploring Treatment Options for Pulmonary Hypertension: From Pharmacological Therapies to Surgery
June 11th, 2026
Pulmonary hypertension (PH) is a serious and progressive condition characterised by elevated blood pressure within the pulmonary arteries. Current estimates suggest that approximately 1% of the global population may be affected by PH, with prevalence rising to nearly 10% among individuals over the age of 65 [1].
PH includes several subgroups with different underlying causes, and treatment depends on the specific type and severity. In the UK, PH caused by left-sided heart disease (Group 2) is the most common subgroup, followed closely by PH associated with lung disease and/or hypoxia (Group 3) [2]. Treatment options for PH currently range from pharmacological therapies and oxygen support to advanced surgical interventions and transplantation.
Given its heterogeneous causes and clinical impact, a clear understanding of management strategies is essential to optimise patient outcomes. This blog explores the treatment approaches available for PH and how they are used across the different subgroups.
Pharmacological therapies
Pharmacological therapies are central to managing PH, working either to ease symptoms or to directly target the processes that lead to increased pulmonary artery pressures. These therapies can be broadly divided into general measures, such as anticoagulants and diuretics and more targeted therapies, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostaglandins, soluble guanylate cyclase stimulators, and calcium channel blockers. Targeted therapies are primarily used for Group 1 PH (pulmonary arterial hypertension, PAH), where they can improve exercise capacity and delay disease progression. In other PH subgroups, treatment is focused on the underlying condition. For example, patients with Group 2 PH typically receive a combination of diuretics and standard heart failure medications to manage fluid overload and reduce cardiac strain.
Oxygen Therapy
Low oxygen levels are common in PH, particularly those with Group 1 and Group 3 PH (PH associated with lung disease), although they can also occur in other PH subgroups [3]. One study found that 57% of patients with PAH used home oxygen therapy [4]. Current guidelines recommend starting oxygen therapy when oxygen levels fall below a certain threshold: a PaO2 < 8kPa (60 mmHg; alternatively, SaO2 92%) at rest [3]. For patients with PAH, home oxygen therapy has been associated with reduced pulmonary vascular resistance (PVR) and improved exercise tolerance [3]. However, while oxygen can help manage symptoms, it has not been shown to improve long-term survival [3].
Surgical Options
In some cases, more advanced interventions are needed. Surgical options include pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), atrial septostomy, and transplantation. PEA is primarily used to treat Group 4 PH (chronic thromboembolic PH) and involves removing organised blood clots and scar tissue from the pulmonary arteries. Around 60% of eligible Group 4 patients may undergo this procedure, and many experience improvements in symptoms, quality of life, and survival [5–8]. However, PEA is not suitable for everyone, particularly those with inaccessible obstructions or significant comorbidities [9].
BPA may be considered for Group 4 PH patients who are not suitable candidates for PEA or who have persistent PH after surgery. BPA is a catheter-based procedure that involves the use of a balloon to widen narrowed pulmonary arteries and improve blood flow. Approximately 18% of Group 4 PH patients may undergo BPA [5]. Although BPA has been shown to improve haemodynamic parameters and exercise capacity, it carries risks such as vascular injury and lung complications [10,11].
Atrial septostomy is reserved for severe Group 1 PH that does not respond to medical therapy, particularly in patients with right ventricular failure. The procedure involves the creation of a small opening between the heart’s upper chambers to reduce right-sided pressure and improve cardiac output. Because it is performed in only a small subset of patients, evidence regarding long-term survival benefits is currently limited.
Transplantation may be considered as a final treatment option in very severe cases. Heart transplantation may be appropriate for carefully selected Group 2 PH patients whose pulmonary pressures are adequately controlled. However, those with high pulmonary vascular resistance may require combined heart-lung transplantation [12]. Lung transplantation is more commonly performed in patients with Group 1 and Group 3 PH who remain severely symptomatic despite maximal therapy. In appropriate candidates, lung transplantation can significantly improve pulmonary pressures, quality of life, and survival [13].
In conclusion, PH is a complex condition for which many different treatment options are available. While many therapies are directed at Group 1 PH, treatment in other PH subgroups focuses on addressing the underlying cause. The careful selection of patients, early specialist referral, and a personalised treatment approach remain central to improving outcomes and quality of life for patients with PH.
References
[1] Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med 2016; 4: 306–322. doi:10.1016/S2213-2600(15)00543-3
[2] Wijeratne DT, Lajkosz K, Brogly SB, et al. Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension. Circ Cardiovasc Qual Outcomes 2018; 11. doi:10.1161/CIRCOUTCOMES.117.003973
[3] 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: 3618–3731. doi:10.1093/eurheartj/ehac237
[4] Farber HW, Badesch DB, Benza RL, et al. Use of supplemental oxygen in patients with pulmonary arterial hypertension in REVEAL. The Journal of Heart and Lung Transplantation 2018; 37: 948–955. doi:10.1016/j.healun.2018.03.010
[5] Delcroix M, Pepke-Zaba J, D’Armini AM, et al. Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy. Circulation 2024; 150: 1354–1365. doi:10.1161/CIRCULATIONAHA.124.068610
[6] Quadery SR, Swift AJ, Billings CG, et al. The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension. European Respiratory Journal 2018; 52: 1800589. doi:10.1183/13993003.00589-2018
[7] Kallonen J, Corbascio M, Rådegran G, et al. Quality of life and functional status after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: A Swedish single‐center study. Pulm Circ 2023; 13. doi:10.1002/pul2.12219
[8] Mathai SC, Ghofrani H-A, Mayer E, et al. Quality of life in patients with chronic thromboembolic pulmonary hypertension. European Respiratory Journal 2016; 48: 526–537. doi:10.1183/13993003.01626-2015
[9] Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011; 124: 1973–1981. doi:10.1161/CIRCULATIONAHA.110.015008
[10] Kiko T, Asano R, Endo H, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary disease without pulmonary hypertension. Pulm Circ 2024; 14. doi:10.1002/pul2.12409
[11] Maneiro Melon NM, Velazquez Martin M, Huertas Nieto S, et al. Effectiveness and Safety of Balloon Pulmonary Angioplasty for the Treatment of Patients with Persistent Pulmonary Hypertension after Pulmonary Endarterectomy. J Clin Med 2023; 12: 905. doi:10.3390/jcm12030905
[12] MEHRA M, KOBASHIGAWA J, STARLING R, et al. Listing Criteria for Heart Transplantation: International Society for Heart and Lung Transplantation Guidelines for the Care of Cardiac Transplant Candidates—2006. The Journal of Heart and Lung Transplantation 2006; 25: 1024–1042. doi:10.1016/j.healun.2006.06.008
[13] George MP, Champion HC, Pilewski JM. Lung Transplantation for Pulmonary Hypertension. Pulm Circ 2011; 1: 182–191. doi:10.4103/2045-8932.83455