Chronic Thromboembolic Pulmonary Hypertension

Chronic Thromboembolic Pulmonary Hypertension

Pulmonary hypertension due to unresolved pulmonary emboli that narrow pulmonary arteries, also known as chronic thromboembolic pulmonary hypertension (CTEPH), is a deadly disease that can be effectively treated with a complex surgical procedure called pulmonary thromboendarterectomy (PTE). Cleveland Clinic has a multidisciplinary team dedicated to the evaluation and treatment of CTEPH patients. The team includes clinicians from Pulmonary Medicine, Cardiothoracic Surgery, Interventional Radiology, Chest Radiology, Nuclear Medicine, Cardiovascular Medicine, Anesthesiology, and Critical Care Medicine.

Over the past 24 years, 230 PTE surgeries have been performed at Cleveland Clinic. Between 1995 and 2010, operative mortality (i.e., in-hospital deaths) was 11.6%. Between 2011 and 2018, surgical volume increased dramatically and the operative mortality decreased to 3.4%, a rate comparable to or better than current published literature.¹ ²

Treatment options are expanding. Cleveland Clinic now offers PTE surgery to carefully selected patients with normal resting pulmonary hemodynamics who are symptomatic from chronic thromboembolic disease as evidenced by exercise studies. Since 2014, Cleveland Clinic has performed 14 PTE surgeries in such patients, with excellent results and no operative mortality.

Pulmonary Thromboendarterectomy Experience (N = 133)

2012 - 2018

PTE = pulmonary thromboendarterectomy

For the 133 CTEPH patients operated on between 2012 and 2018 (excluding patients with normal resting pulmonary artery pressure), hemodynamic data show marked improvement in artery pressure, pulmonary vascular resistance, and cardiac index.

References

  1. Mayer E, Jenkins D, Lindner J, D’Armini A, Kloek J, Meyns B, Ilkjaer LB, Klepetko W, Delcroix M, Lang I, Pepke-Zaba J, Simonneau G, Dartevelle P. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011 Mar;141(3):702-710.
  2. Madani MM, Auger WR, Pretorius V, Sakakibara N, Kerr KM, Kim NH, Fedullo PF, Jamieson SW. Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg. 2012 Jul;94(1):97-103; discussion 103.
Hemodynamics Before and After Pulmonary Thromboendarterectomy (N = 135)ᵃ

2011 - 2018

Preoperative Median (25th, 75th percentiles) Postoperative Median (25th, 75th percentiles)
Mean PAP, mm Hg 45 (38.5, 53.5) 25 (20, 31)
CI, L/min/m² 2.2 (1.8, 2.7) 2.8 (2.4, 3.4)
PVR, Wood units 6.6 (4.7, 9.7) 2.4 (1.8, 3.3)

CI = cardiac index, PAP = pulmonary artery pressure, PVR = pulmonary vascular resistance

ᵃExcludes patients with normal resting pulmonary hemodynamics undergoing PTE

Not only are hemodynamic results remarkable and operative mortality low, but long-term outcomes are excellent, with a 5-year survival rate of 91.6%, compared with 67.7% for patients treated with medical therapies (P < 0.001) between 2009 and 2018.

Pulmonary Thromboendarterectomy vs Medical Therapy (N = 212)ᵃ

2009 - 2018

PTE = pulmonary thromboendarterectomy

ᵃExcludes patients with normal resting pulmonary hemodynamics undergoing PTE

Balloon Pulmonary Angioplasty Experience (24 Patients and 78 Sessions)

2016 - 2018

For inoperable CTEPH, balloon pulmonary angioplasty (BPA) has emerged as a potential treatment option. Cleveland Clinic performed its first BPA procedure in March 2016. Since then, 24 patients have undergone a total 78 BPA sessions, without any major complications. While early, efficacy appears promising in terms of symptomatic and hemodynamic improvements.