Anjali Vaidya, MD, FACC, FASE, FACP Associate Director, Pulmonary Hypertension, Right Heart Failure, and Pulmonary Thromboendarterectomy Program
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1 Anjali Vaidya, MD, FACC, FASE, FACP Associate Director, Pulmonary Hypertension, Right Heart Failure, and Pulmonary Thromboendarterectomy Program Advanced Heart Failure & Cardiac Transplant Temple University Lewis Katz School of Medicine
2 Disclosures No disclosures relevant to this presentation Consulting/Speaking: United Therapeutics, Gilead, Actelion, Bayer
3 Pulmonary Hypertension evolution
4 Pulmonary Hypertension I: Pulmonary Arterial HTN (PAH) Idiopathic/Familial Portal HTN Drugs/Toxins Collagen vascular disease HIV Systemic-Pulm Shunt Schistosomiasis PPHN II: Pulmonary Venous HTN (Left Heart Disease) Left ventricular or atrial disease Mitral or Aortic valve disease III: Chronic Respiratory Disorders Interstitial lung disease COPD Sleep disordered breathing (Who Group II?) Chronic high altitude exposure Alveolar hypoventilation disorders IV: Chronic thromboembolic disease (CTEPH) V: Miscellaneous External compression, Tumor obstruction Fibrosing mediastinitis Langerhans cell histiocytosis Lymphangiomatosis Hematologic disorders Sarcoidosis J Am Coll Cardiol. 2013;62(25_S) 5th WSPH Nice 2013
5 Hemodynamics: PAH = CTEPH Defined as: mpap (mean pressure) 25 mmhg - HIGH PAWP (L heart pressure) 15 mmhg - LOW PVR (vascular resistance) > 3 Wood Units - HIGH PH Specific Medical Therapy approved for: WHO Group I (PAH) - all WHO Group IV (CTEPH) riociguat
6 CTEPH Diagnosis PAH hemodynamics (PVR >3, PCW < 15) VQ with unmatched perfusion defects CTA Chest with vascular abnormalities Anticoag > 3-6 months Kim, NH et al. J Am Coll Cardiol (suppl).D92-99.
7 Incidence of CTEPH after PE? Prospective N = 314 Median f/u 8 years Risk predictors: Idiopathic Young age Large defect Hx multiple PEs 3.8% at 2 years 3.1% at 1 year 1% at 6 months Pengo, V, et al. N Engl J Med :
8 Risk Factors for CTEPH PE specific: Recurrent, unprovoked Young age at PE diagnosis PASP > 50 mm Hg at time of PE Hypercoagulable: Lupus anticoagulant, antiphospholipid Protein C, S deficiency Beta 2 Microglobulin Prothrombin gene mutation Medical conditions: Cancer Indwelling pacemaker, ports Splenectomy Piazza G, Goldhaber SZ. N Engl J Med 2011;364:
9 Risk Factors for CTEPH PE specific: Hypercoagulable: Medical conditions: Anatomic conditions: May-Thurner Syndrome Uterine fibroids Pelvic vein compression, stenosis Lacharite-Roberge A, Vaidya A. CTEPH National Proceedings. 2017c
10 V/Q Scan Fedullo PF. N Engl J Med 2001; 345:
11 CTEPH Evaluation Left image courtesy of Dr Nick Kim, University of California, San Diego. Right image courtesy of the PTE Program at University of California, San Diego.
12 Treatment Evaluation Guidelines recommend referral to expert PTE/CTEPH Program Multidisciplinary care: Cardiology/PH, Chest Radiology, Cardiac Surgery, Interventional Cardiology Coordinators
13 Jamieson SW, et al. Ann Thoracic Surg.2003;76: Galie N, et al. Eur Heart J. 2009;30: Pulmonary Thromboendarterectomy First line treatment Similar to transplant, outcomes correlate with volume and experience Deep hypothermia (20 C) Extracorporeal circulation Circulatory arrest (20 minutes) ICU Perioperatively: Reperfusion pulmonary lung injury VV ECMO Bleeding Hemodynamics Neurologic; Sedation Arrhythmia
14 Post-Operatively Improvements in 6MW distance Improved RV function, TR Improved hemodynamics Improved quality of life Life-long anticoagulation Inoperable? Are you sure?? (expert center referral) Distal disease Comorbidities PH out of proportion to thromboembolic burden Kim NH, et al. J Am Coll Cardiol. 2013;62(suppl) D92-D99. Pepke-Zaba J, et al. Circulation. 2011;124:
15 CTEPH treatment algorithm
16 Pulmonary Embolectomy
17 Pulmonary Thromboendarterectomy
18 N = 108 PTEs Mortality = 3.7% Last year: 40 PTEs Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery Sep
19 Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery Sep
20 Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery Sep
21 Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery Sep
22 Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery Sep
23 Balloon Pulmonary Angioplasty 68 yo M with hx NICM, EF 10% Progressive DOE Remote PE 40 years ago Referred to TUH for OHT Severely elevated PVR
24 PH: Medical Therapy PDE-5 inhibitors sildenafil, tadalafil Guanylate cyclase stimulator riociguat Endothelin receptor antagonists bosentan, ambrisentan, macitentan Prostacyclin therapy treprostinil, epoprostenol, iloprost Prostacyclin receptor agonist selexipag CTEPH!
25 Drug Pathways in PAH
26 CHEST-1 Improved: 6MW, PVR, NT-ProBNP, Functional Class NEJM 2013
27 PH: Medical Therapy PDE-5 inhibitors sildenafil, tadalafil Guanylate cyclase stimulator riociguat Endothelin receptor antagonists bosentan, ambrisentan, macitentan Prostacyclin therapy treprostinil, epoprostenol, iloprost Prostacyclin receptor agonist selexipag CTEPH?
28 Drug Pathways in PAH
29 MERIT-1 Data - Macitentan Phase 2, DBRCT vs placebo, inoperable CTEPH N = 80; 16 weeks Improved:PVR 16% reduction, 6MW distance (34 meters) Ghofrani HA, et al. Lancet (10):
30 Thank You! Pulmonary Hypertension, Right Heart Failure, and Pulmonary Thromboendarterectomy Program P: ; F: Cell: th Floor Parkinson Pavilion 3401 North Broad Street Philadelphia, PA 19140
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