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 specializing in pulmonary medicine, cardiothoracic surgery, interventional radiology, chest radiology, nuclear medicine, cardiovascular medicine, anesthesiology, and critical care medicine.

During the past 25 years, 273 PTE surgeries have been performed at Cleveland Clinic. Between 1995 and 2010, operative mortality (ie, in-hospital deaths) was 11.6%. Between 2011 and 2019, surgical volume more than doubled and the operative mortality decreased to 3.19%, 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 17 PTE surgeries in such patients, with excellent results and no operative mortality.

Pulmonary Thromboendarterectomy Experience (N = 273)

2011 - 2019

PTE = pulmonary thromboendarterectomy

For the 177 CTEPH patients operated on between 2011 and 2019 (excluding patients with normal resting pulmonary artery pressure), hemodynamic data show marked improvement in pulmonary artery pressure, pulmonary vascular resistance, and cardiac index in the immediate postoperative period as well as 6 months later.

Hemodynamics Before and After Pulmonary Thromboendarterectomy (N = 177)ᵃ

2011 - 2019

Preoperative Median (25th, 75th percentiles)Immediate Postoperative Median (25th, 75th percentiles)6 Months Postoperative (N = 63) (25th, 75th percentiles)
Mean PAP, mm Hg46 (39, 54)25 (20, 31)27 (20, 34)
CI, L/min/m²2.3 (1.8, 2.7)2.8 (2.4, 3.3)2.7 (2.4, 3.0)
PVR, Wood units6.9 (4.9, 9.8)2.6 (1.9, 3.5)2.4 (1.4, 4.3)

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 91.1%, compared with 68.2% for not operated patients between 2009 and 2019.

Pulmonary Thromboendarterectomy vs Medical Therapy (N = 276)ᵃ

2009 - 2019

PTE = pulmonary thromboendarterectomy

ᵃExcludes patients with normal resting pulmonary hemodynamics undergoing PTE

Balloon Pulmonary Angioplasty Experience (42 Patients and 165 Sessions)

2016 - 2019

BPA = balloon pulmonary angioplasty

For selected inoperable and residual CTEPH after PTE patients, balloon pulmonary angioplasty (BPA) has emerged as a potential treatment option. Cleveland Clinic performed its first BPA procedure in March 2016. Since then, 42 patients have undergone a total 165 BPA sessions, with a low complication rate of 4.8% and no mortality. While early, efficacy appears promising in terms of symptomatic and hemodynamic improvements.

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.