Pulmonary Thromboendarterectomy and Pulmonary Artery Balloon Angioplasty for CTEPH; Respiratory Reader Fall 2019

November 26, 2019

Pulmonary Thromboendarterectomy and Pulmonary Artery Balloon Angioplasty for CTEPH

by Belinda Rivera-Lebron, MD, MS, FCCP

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by mechanical obstruction from residual thrombi in the pulmonary arteries resulting in increased pulmonary vascular resistance (PVR), pulmonary hypertension (PH), and right ventricular failure.1,2 Acute pulmonary embolism (PE) resolves in most cases. However, up to 4% of PE survivors develop CTEPH.3-5 Most patients with CTEPH have experienced a PE in their lifetime; despite this, up to 25% of patients have never reported a thrombotic event.6 In the U.S., approximately 600,000 people have an acute PE each year, of which up to 2,500 new cases are diagnosed with CTEPH each year.7

In addition to the transformation of incomplete resolution of thrombi into organized fibrotic scar tissue, CTEPH is a vascular disorder, with pulmonary arteriopathy in segments not affected by obstruction. The combination of thrombus obstruction and vascular remodeling results in PH, with increased right ventricle strain and eventual right-sided heart failure.

Patients may present with exertional dyspnea, fatigue, palpitations, lightheadedness, or syncope. CTEPH is diagnosed by precapillary pulmonary hypertension on right heart catheterization (mean pulmonary arterial pressure >25 mmHg and pulmonary arterial wedge pressure ≤15 mmHg) and abnormal ventilation perfusion scintigraphy (VQ scan) including at least one mismatched perfusion defect with confirmatory imaging by either computed tomography angiography (CTA) or pulmonary angiography after at least three months of effective anticoagulation.2

The Gold Standard: Pulmonary Thromboendarterectomy

Pulmonary thromboendarterectomy is the gold standard and only potentially curative therapy for CTEPH. Operability is determined by thrombus accessibility, hemodynamic severity, medical comorbidities, and expertise of the surgical team. However, not all patients are eligible for surgery. Furthermore, up to 35% of patients who undergo thromboendarterectomy may still have persistent PH after surgery.5

For inoperable patients and/or those with persistent pulmonary hypertension after thromboendarterectomy, medical therapy with riociguat, a soluble guanylate cyclase stimulator, has been shown to improve exercise capacity and pulmonary vascular resistance, and is the only FDA-approved drug for these patients.8 In a long-term follow-up study, riociguat was shown to have sustained beneficial effects and was well tolerated.9

Balloon Pulmonary Artery Angioplasty as an Alternative Therapy

Balloon pulmonary artery angioplasty (BPA) is emerging as an alternative therapy for patients with inoperable or persistent PH after thromboendarterectomy. BPA, a catheter-based intervention to treat pulmonary artery obstruction, was initially described in CTEPH patients in 2001 with favorable hemodynamic effects results, but with increased complication rates, including reperfusion pulmonary edema.10 With technique modifications, Japanese groups have dramatically improved outcomes by lowering the complication rate, while sustaining hemodynamic improvements. Serial BPA sessions have shown to improve mean pulmonary artery pressure, cardiac output, and functional class, and are a promising therapeutic strategy for the treatment of CTEPH.11,12,13

The UPMC CTEPH Program is a multidisciplinary team composed of pulmonary hypertension experts from pulmonary and critical care, cardiology, interventional cardiology, and cardiac surgery. Our team has the expertise in medical, surgical, and interventional treatments, including BPA, for CTEPH. Please refer your patients by calling 1-877-PH4-UPMC (744-8762).

References

1. Simonneau G, Robbins IM, Beghetti M, et al. “Updated clinical classification of pulmonary hypertension.” J Am Coll Cardiol. 2009;54(1 Suppl):S43-54.
2. Kim NH, Delcroix M, Jenkins DP, et al. “Chronic thromboembolic pulmonary hypertension.” J Am Coll Cardiol. 2013;62(25 Suppl):D92-99.
3. Auger WR, Fedullo PF. “Chronic thromboembolic pulmonary hypertension.” Semin Respir Crit Care Med. 2009;30(4):471-483.
4. Pengo V, Lensing AW, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism.” N Engl J Med. 2004;350(22):2257-2264.
5. Fedullo P, Kerr KM, Kim NH, Auger WR. “Chronic thromboembolic pulmonary hypertension.” Am J Respir Crit Care Med. 2011;183(12):1605-1613.
6. Pepke-Zaba J, Delcroix M, Lang I, et al. “Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry.” Circulation. 2011;124(18):1973-1981.
7. Tapson VF, Humbert M. “Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism.” Proc Am Thorac Soc. 2006;3(7):564-567.
8. Ghofrani HA, D’Armini AM, Grimminger F, et al. “Riociguat for the treatment of chronic thromboembolic pulmonary hypertension.” N Engl J Med. 2013;369(4):319-329.
9. Simonneau G, D’Armini AM, Ghofrani HA, et al. “Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2).” Eur Respir J. 2015;45(5):1293-1302.
10. Feinstein JA, Goldhaber SZ, Lock JE, Ferndandes SM, Landzberg MJ. “Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension.” Circulation. 2001;103(1):10-13.
11. Mizoguchi H, Ogawa A, Munemasa M, Mikouchi H, Ito H, Matsubara H. “Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension.” Circ Cardiovasc Interv. 2012;5(6):748-755.
12. Kataoka M, Inami T, Hayashida K, et al. “Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension.” Circ Cardiovasc Interv. 2012;5(6):756-762.
13. Ogo T, Fukuda T, Tsuji A, et al. “Efficacy and safety of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension guided by cone-beam computed tomography and electrocardiogram-gated area detector computed tomography.” Eur J Radiol. 2016.