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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
Disclosures
• No disclosures relevant to this presentation
• Consulting/Speaking: United Therapeutics, Gilead, Actelion, Bayer
Pulmonary Hypertension
evolution
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
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
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. 2013.62(suppl).D92-99.
Incidence of CTEPH after PE?
Pengo, V, et al. N Engl J Med 2004.350:2257-2264.
1% at 6 months
3.1% at 1 year
3.8% at 2 years
• Prospective • N = 314 • Median f/u 8 years • Risk predictors:
• Idiopathic • Young age • Large defect • Hx multiple PEs
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: 351-360.
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
V/Q Scan
Fedullo PF. N Engl J Med 2001; 345:1455-1472.
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.
Treatment Evaluation
• Guidelines recommend referral to expert PTE/CTEPH Program
• Multidisciplinary care: Cardiology/PH, Chest Radiology, Cardiac Surgery, Interventional Cardiology
• Coordinators
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
Jamieson SW, et al. Ann Thoracic Surg.2003;76:1457-1464. Galie N, et al. Eur Heart J. 2009;30:2493-2537
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;124:1973-1981
CTEPH treatment algorithm
Pulmonary Embolectomy
Pulmonary Thromboendarterectomy
Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery. 2017 Sep 4.10188-6.
N = 108 PTEs Mortality = 3.7% Last year: 40 PTEs
Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery. 2017 Sep 4.10188-6.
Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery. 2017 Sep 4.10188-6.
Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery. 2017 Sep 4.10188-6.
Raza F, Vaidya A, Forfia P. J Cardiovascular Surgery. 2017 Sep 4.10188-6.
• 68 yo M with hx NICM, EF 10%
• Progressive DOE • Remote PE 40 years ago • Referred to TUH for OHT • Severely elevated PVR
Balloon Pulmonary Angioplasty
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!
Drug Pathways in PAH
CHEST-1
NEJM 2013
Improved: 6MW, PVR, NT-ProBNP, Functional Class
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?
Drug Pathways in PAH
MERIT-1 Data - Macitentan
• Phase 2, DBRCT vs placebo, inoperable CTEPH
• N = 80; 16 weeks
Ghofrani HA, et al. Lancet. 2017.5(10):785-794.
Improved:PVR 16% reduction, 6MW distance (34 meters)
Thank You!
Pulmonary Hypertension, Right Heart Failure, and Pulmonary
Thromboendarterectomy Program P: 215-707-7636; F: 215-707-9977
Cell: 215-531-2297 [email protected]
9th Floor Parkinson Pavilion 3401 North Broad Street Philadelphia, PA 19140