Pulmonary Hypertension

Classification and Guidelines

Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Repir J. 2019;53(1). The 6th World Symposium on Pulmonary Hypertension proposes a new threshold of mPAP of 20mmHg as the upper limit of normal value.

PMID: 30545968

Free Full Text

Klinger JR, Elliott CG, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST guideline and expert panel report. Chest. 2019;155:565-586.  This is a timely update to the 2014 guideline that summarizes the indications for each of the many therapeutic classes based on severity of symptoms, vasoreactivity, and prior treatment. There is a new emphasis on earlier use of combination therapy.

PMID: 30660783

Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37(1):67-119. This consensus document is still included as it contains recommendations on diagnostic evaluation.

PMID: 26320113

Free Full Text


Arcasoy SM, Christie JD, Ferrari VA, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003; 167:735-40. The cardiology literature indicates echocardiography-derived estimates of pulmonary artery pressures are accurate. This study found 52% of echo estimates were inaccurate (off by > 10 mmHg) in 166 lung transplant candidates and the difference was > 20 mmHg in 28%. In patients without hypertension, echo was more likely to overestimate pressures while in patients with pulmonary hypertension; it was as likely to over as underestimate. Accuracy and ability to obtain an estimate varied with the underlying disease.

PMID: 12480614

Fisher MR, Forfia PR, Chamera E, et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179:615-21. This prospective study of 65 patients undergoing right-heart catheterization for the diagnosis or management of pulmonary hypertension extends the findings of Arcasoy et al. above to patients without end-stage lung disease. Echo over or underestimated pulmonary artery pressure by > 10 mm Hg 52% of the time. The majority of patients had pulmonary arterial hypertension (WHO Group 1).

PMID: 19164700

Free Full Text

Treatment of pulmonary arterial hypertension

Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369:809-18. Unlike previous therapeutic trials, this placebo-controlled RCT is noteworthy for using a primary composite endpoint which included death, atrial septostomy, lung transplantation, initiation of treatment with intravenous or subcutaneous prostanoids, or worsening of pulmonary arterial hypertension.  The composite endpoint occurred in 46.4% of placebo group vs. 31.4% of the higher dose of macidentan. Macitentan carries lower risk of hepatotoxicity compared to bosentan.

PMID: 23984728

Free Full Text

Galiè N, Barberà JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. New Engl J Med 2015: 373:834-844. This randomized trial is novel for focusing on the role of upfront combination therapy in symptomatic treatment-naive patients.  Patients received ambrisentan and tadalafil alone or in combination. The primary outcome (a composite of death, hospitalization for worsening PAH, disease progression, or unsatisfactory long-term clinical response) occurred in 18% of the combination group vs 31% in the pooled monotherapy group (hazard ratio 0.50, p < .001). This was driven by a decrease in hospitalization. There was no mortality difference or change in WHO functional class.

PMID: 26308684

Free Full Text

Thromboendarterectomy for chronic thromboembolic disease

Mayer E, Jenkins D, Lindner J, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg 2011;141:702-10. This description of experience within an international, multi-institutional setting offers a comparison with the San Diego experience. Preoperative PVR was somewhat lower in this group, circulatory arrest time was the same, and one-year mortality was 7%. An interesting discussion, including a member of the San Diego group, follows the references.

PMID: 21335128