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). Since 2011, 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.

The COVID-19 pandemic created challenges never seen before to the CTEPH Program. The program resorted to virtual platforms to remain connected to our patients, while ensuring that those who needed in person care were appropriately identified and supported.

Over the past 26 years, 311 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 2020, surgical volume more than doubled and the operative mortality decreased to 3.1%, a rate comparable to or better than current published literature.1,2 (Figure 1).

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 (CTED) as evidenced by exercise studies.

Between 2011 and 2020, our program evaluated 355 patients: 316 with CTEPH and 39 with CTED. We performed 233 PTE surgeries (66% of evaluated patients): 214 in CTEPH and 19 in CTED patients.

Pulmonary Thromboendarterectomy Experience (N = 311)

2011 – 2020

PTE = pulmonary thromboendarterectomy

For the 214 CTEPH patients operated on between 2011 and 2020 (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 = 214)ᵃ

2011 – 2020

Preoperative Median (25th, 75th percentiles)Immediate Postoperative Median (25th, 75th percentiles)6 Months Postoperative (N = 63) (25th, 75th percentiles)
Mean PAP, mm Hg45 (38, 54)25 (20, 31)27 (20, 34)
CI, L/min/m²2.24 (1.8, 2.7)2.8 (2.4, 3.4)2.7 (2.3, 3.0)
PVR, Wood units6.6 (4.9, 9.7)2.5 (1.8, 3.5)2.6 (1.9, 4.5)

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 90.4%, compared with 71.6% for not operated patients between 2009 and 2020.

Pulmonary Thromboendarterectomy vs Medical Therapy (N = 322)ᵃ

2009 – 2020

PTE = pulmonary thromboendarterectomy

ᵃExcludes patients with normal resting pulmonary hemodynamics undergoing PTE

Balloon Pulmonary Angioplasty Experience (58 Patients and 216 Sessions)

2016 – 2020

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, 58 patients have undergone a total 216 BPA sessions. BPA was perfomed for technically inoperable disease in 75.8%, refusal of surgery in 3.2%, poor surgical candidacy in 8.1% and residual PH after PTE in 12.5%. The procedure is well tolerated, with a low complication rate (7.8% of sessions, 13% of patients, mild severity) and no mortality. Patients experience improvement in symptoms, oxygenation and hemodynamics.

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.