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Management of patients with chronic thromboembolic pulmonary hypertension

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Management of patients with chronic thromboembolic pulmonary hypertension

  1. 1. Management of patients with chronic thromboembolic pulmonary hypertension Anastasia Bykova, NP-Adult, MN Nurse Practitioner Marc de Perrot, MD, MSc, FRCSC Associate Professor of Surgery Director, Toronto Pulmonary Endarterectomy Program Division of Thoracic Surgery Toronto General Hospital
  2. 2. Thromboembolic pulmonary disease • The incidence of venous thromboembolism is high with about 240 cases per 100,000 person/ year • The diagnosis and management of patients with acute pulmonary emboli (PE) is well characterized • However, the incidence and management of patients with residual pulmonary hypertension after acute PE is still unclear • Over the past 10 years, pulmonary endarterectomy has become the mainstay of therapy for patients with severe pulmonary hypertension and unresolved PE • The incidence of CTEPH appears to be about 10x higher than previously estimated  Annual incidence of up to 25 cases per million inhabitants (incidence of idiopathic PAH is 2-3 cases per million inhabitants)
  3. 3. Definition of chronic thromboembolic pulmonary hypertension (CTEPH) • CTEPH is defined by the presence of: – Precapillary pulmonary hypertension (mean PA pressure >25 mmHg, PCWP or LVEDP <15 mmHg) – Combined with the presence of perfusion defects in the pulmonary arterial tree – Despite at least 3 months of anticoagulation
  4. 4. Pulmonary hypertension is due to: • Obstruction of PA bed by thrombo-embolic material • Vascular remodeling • in situ thrombosis • Overflow vasculopathy • Post-obstructive vasculopathy Mechanisms leading to pulmonary hypertension Localized phenomenon  PH progresses despite adequate anticoagulation!!!
  5. 5. A Closer Look at CTEPH Pathophysiology • Fibrous obstruction of the pulmonary vascular bed  increase in PVR. • Chronic thromboembolic obstruction  small vessel arteriolar vasculopathy:  Excessive vascular and inflammatory cell proliferation  High flow through the pulmonary vasculature induces medial hypertrophy  These changes resemble the arteriopathy noted in idiopathic pulmonary arterial hypertension  Second process that contributes to elevated PVR and disease progression in CTEPH  PH develops because of 1) primary obstruction by embolus and adjacent remodeling or 2) combination of proximal obstruction and secondary small vessel vasculopathy
  6. 6. Natural Course of the Disease Residual pulmonary vascular obstruction with PH Thrombus organization in occluded vessels Increasing shear stress in non-occluded vessels RV function is compensated Progression of vascular remodeling Progressive right ventricle dysfunction Death 1. VTE/DVT - PE 2. Honey moon period 3. Clinical deterioration
  7. 7. A Closer Look at Natural Course of the Disease with Focus on RV Failure Initial stage - Compensatory RV pressure overload  RV hypertrophy *RV is not capable of sustaining a long term pressure overload * Later Stage – Decompensatory RV dilation with ↑ oxygen demand and ↓ perfusion  vicious circle of compromised contractility and dilation RV failure: ↑ filling pressure, diastolic dysfunction, diminished cardiac output (CO), hypertrophy LV diastolic dysfunction through ventricular interdependence and leftward septal displacement
  8. 8. Microvascular disease and RV dysfunction directly impacts on results of pulmonary endarterecomy • Peri-operative mortality reduced to 2-3% • Mortality increases with RV failure • Results of PEA are best during “honey moon” period – Minimal distal vasculopathy – Good RV function  Reduced risk of post- operative RV failure  Reduced risk of residual PH in the long-term
  9. 9. Clinical Presentation of Patients with CTEPH Clinical Symptoms Early • Progressive dyspnea on exertion Later • Dyspnea at rest • Chest tightness, pain • Syncope • Hemoptysis • Early satiety Caveat: History of acute pulmonary emboli present in <50% of the patients Clinical Signs Early • Accentuated pulmonic component of S2 Later • Jugular venous distention • RV heave / lift • Fixed splitting of S2 • Tricuspid regurgitation • S3 RV gallop • Hepatomegaly • Peripheral edema • Ascites
  10. 10. Diagnostic Work up 1. Diagnosis • V/Q scan 2. Evaluation for medical/surgical therapy • Angio CT scan (64 slices) • Right heart cath • Pulmonary angiography • TTE ECHO 3. Pre-operative evaluation • Coronary angiogram (>45 years old or risk factors) • Venous/arterial Doppler • Pulmonary function tests • 6 minutes walk test
  11. 11. Diagnostic Algorithm CTEPH ruled out Normal perfusion scan Multidisciplinary discussion with Respirologists/cardiologists, surgeons, and radiologists Further imaging with: CT angiography, Pulmonary angiogram, Right heart cath Indeterminate or multiple perfusion defects Ventilation/perfusion scan Patients with unexplained PH or with PH and history of PE
  12. 12. Ventilation-perfusion scan • V/Q scan is the most reliable test to rule out CTEPH • V/Q is excellent for diagnosis of CTEPH, but has no role to determine feasibility of surgery • Pulmonary angiogram if mismatched perfusion defect presents on V/Q scan
  13. 13. CT pulmonary angiogram • CT pulmonary angio is important to assess the possibility of PEA • The presence of intra-luminal webs (*) is pathognomonic of the disease • However, a normal CT does not rule out the diagnosis of CTEPH • CTPA complements the pulmonary angiogram *
  14. 14. Pulmonary angiogram and right heart cath • Webs and bands • Pouches • Irregularities • Narrowed and truncated vessels • Usually no residual clot is visible in the lumen
  15. 15. Pulmonary endarterectomy (PEA) • Therapy of choice for patients with CTEPH – Major clinical improvement – Resolution of PH – Excellent long term survival – Only treatment is life-long anticoagulation • The goal of the surgery is curative by: – Reverse RV compromise caused by high PVR and improve CO – Complete reperfusion of previously occluded PA and treatment of dyspnea – Prevention of progressive RV dysfunction and secondary vasculopathy in the remaining patent vessel
  16. 16. Indications for pulmonary endarterectomy • Diagnosis of CTEPH must be established Importance of adequate imaging • Surgically accessible disease • Symptoms related to CTEPH (usually PVR>300 • Absence of significant co-morbidities
  17. 17. Pulmonary endarterectomy (PEA) Thrombectomy Endarterectomy 
  18. 18. Principles of pulmonary endarterectomy – Bilateral procedure via median sternotomy • To achieve complete relief of obstruction – Intrapericardial surgery • To avoid pleural adhesions • To have bilateral access • To use cardiopulmonary bypass – Deep hypothermic circulatory arrest (18°-20°C) • To stop the bronchial back bleeding and obtain good visualization of the distal vessels – Video-assistance • To provide light and visualization of distal vessels
  19. 19. Opitz, de Perrot. Op Technique in Cardiothoracic Surg 2012;17:168-80
  20. 20. Endarterectomy specimen Right heart cath preop and 3 months postop: preop: PA 96/14, mean 46 mmHg, CO 6.7 L/min, TPR 549 postop: PA 28/9, mean 15 mmHg, CO 8.4 L/min, TPR 143 -5
  21. 21. Mortality after pulmonary endarterectomy 0 2 4 6 8 10 12 Mortality (%) Hospital mortality 1 year mortality Overall 1-10 cases /year 11-50 cases /year >50 cases /year p=n.s. Hospital mortality and 1 year mortality tended to be higher in centers doing 1-10 cases per year Mayer et al. J Thorac Cardiovasc Surg 2011;141:702-10
  22. 22. Outcomes following PEA • Improvement of WHO Class from III/IV to I/II • Immediate and sustained HD improvement with ↑ CI, significant ↓ in PVR and PAP • RV remodelling - Tricuspid valve function and geometry return to normal - Immediate decrease in right-sided size - Decreased leftward shift of the interventricular septum - Increased LV end-diastolic area  Clinical improvement is related to removal of pulmonary arterial obstruction and reversal of pulmonary vascular remodeling.
  23. 23. ICU management of post-op PEA patient  All patients are transported to the ICU  Kept fully sedated and ventilated overnight  POD #1-2 – extubated POD #2-3 – discharged from the ICU POD #7-14 – discharged from the hospital Some complications are similar to other cardiothoracic surgical procedures: atelectasis, pleural effusion, pericardial effusion, diaphragmatic dysfunction, and arrhythmias Unique complications in PEA: reperfusion pulmonary edema (RPA) and residual PH/RV failure
  24. 24. ICU management of post-op PEA patient Neuro Keep fully sedated overnight with – Propofol 200-300 mg/hr – Fentanyl 100-200 mcg/hr Allows for decreased CO and prevention of pulmonary edema! Start weaning the sedation on POD#1 with the goal to extubate, if pt tolerates
  25. 25. ICU management of post-op PEA patient Cardiovascular and Respiratory Standard postoperative care • Levophed to target MAP ≥ 75 mmHg (up to 0.3 mcg/kg/min) • Other HD targets SvO2 ≥ 60% CI 1.5-2.5 L/min/m² (avoid high CI initially to prevent RPA) CVP 8-14 mmHg • Lines: Cordis and PA catheter, triple lumen CVC, 2 artlines (radial & femoral), epicardial pacer wires connected to pacemaker • HD monitoring on an hourly basis via cardiac and continuous SvO2/Swan Ganz monitors (BP, HR, CI/CO, PAP, CVP). Expect a decrease of PAP by 25 mmHg from pre-op value, improved RV function and PVR reduction
  26. 26. ICU management of post-op PEA patient Review of Basic Hemodynamics Cardiac Output = Stroke Volume x Heart Rate Preload Afterload Contractility Fluid overload from RV failure pre-op  Lasix to target 5-15 L loss/3-5days Risk for pulmonary edema from reduced PVR, CPB inflammation, removal of PEEP with extubation  keep sedated & ventilated overnight with lower CI/CO Atrial pacing at 80-90 beats/min to help with RV remodeling Goal to maintain negative fluid balance and avoid IV fluid bolus. Keep pt dry!!!
  27. 27. ICU management of post-op PEA patient Cardiovascular and Respiratory Cont’d • Mechanical ventilation overnight to observe for RPA • 3 CTs to -20 cm H2O (2 large bore CTs - retrosternal and pericardial above the diaphragm; 1 small bore – mediastinal) • Monitor CT output for bleeding: output ≥ 50ml/hr • Target PaCO2 35-45 mmHg PaO2 ≥ 75 mmHg • CBC, lytes, extended lytes, creatinine, lactate, LFTs, trop, q4h X 24 hrs • Ancef 1g q8h IV x 48 hrs • TED for venous thrombosis prophylaxis, NEVER use SCD • Anticoagulation Post-op: Hold Heparin IV drip until R/A in 2-3 hours. When re-started (if no bleeding), UFH level in 6 hours. Dr. de Perrot will initially manage rate until patient is back on Heparin nomogram.
  28. 28. What to watch for? PEA-specific complications Hypoxemia • Related to atelectasis, postoperative pain, V/Q mismatch d/t loss of hypoxic vasoconstriction, pulmonary edema • Vascular “steal” phenomenon in PEA: Blood flow taking the path of least resistance resulting in relative “hyperperfusion” of newly endarterectomised lung with “steal” from previously normal perfused lung region. Low V/Q regions contribute to hypoxemia. Resolves within several months & O2 is weaned off.
  29. 29. What to watch for? PEA-specific complications Reperfusion pulmonary edema • Occurs in < 5-10% of patients - 60% immediately post-op - 30% within the first 48 hours - 10% later during hospitalization (> 48hours) • Definition: localized form of high-permeability edema seen as opacity on CXR in the areas of the lung that have been endarterectomised & reperfused. • Associated with ↑ in duration of MV and ICU stay • Most severe form – severe alveolar hemorrhage with profound hypoxemia. High morbidity secondary to deconditioning and infections.
  30. 30. What to watch for? PEA-specific complications Reperfusion pulmonary edema Treatment is supportive: • +++ diuresis to reduce lung water and avoidance of high cardiac output • Oxygenation is better in one lateral decubitus position versus the contralateral • Inhaled NO may improve oxygenation • If RV and LV function is preserved, VV ECMO for severe cases is lifesaving. Natural repair process takes 4-5 days, allowing weaning from ECMO.
  31. 31. What to watch for? PEA-specific complications Residual pulmonary hypertension • Significant problem in 5% to 35% of patients undergoing PEA • Most common cause of perioperative mortality • Etiology: distal chronic thromboembolic disease or superimposed small- vessel vasculopathy that is not cured by endarterectomy Treatment of residual PH in immediate post-op period is challenging 1. Inhaled nitric oxide to reduce PVR and does not carry risk for systemic hypotension 2. Optimization of RV preload 3. Inotropic support (i.e. Milrinone)  NO systemic vasodilators d/t risk for systemic hypotension
  32. 32. What to watch for? General complications Postoperative pericardial effusion • Suspect with hypoxia and HD compromise (i.e. hypotension) • Immediate ECHO to confirm the diagnosis • Significant pericardial effusions should be drained Atrial arrhythmias • Atrial flutter / fibrillation are common following PEA secondary to cardiac manipulation intra-op • Careful electrolytes management to target Mg ≥ 1.1 & K ≥ 4 • Amiodarone is the drug of choice in CTEPH patients
  33. 33. ICU management of post-op PEA patient GI/GU • Keep NPO until extubated then CF • NG tube – to straight drainage. For medication administration while intubated and NPO • Famotidine 20 mg IV BID before and after extubation • Once oral intake initiated, switch to Pantoloc 40 mg PO OD until hospital discharge to prevent stress ulcer and GI bleeding • Foley catheter - aggressive diuresis with target fluid loss of 10 L/2-3 days post-op. Increase Levophed if needed to maintain MAP ≥ 75 mmHg. Avoid fluid boluses. • Aim at negative 24 hr fluid balance
  34. 34. ICU management of post-op PEA patient • Extubated POD#1 • PA catheter removed POD#2-3 • 2 out of 3 CTs and epicardial pacer wires removed on POD #3-6. - Heparin drip held x 4 hours for the procedure - Bed rest x 1 hr - Resume Heparin drip 1 hr post CT removal - CXR 2 hrs post CT removal • Mediastinal, substernal CT tube removed 24 hrs later to prevent substernal hematoma • Ambulate after extubation • PCA for pain management to promote deep breathing/coughing and active physiotherapy
  35. 35. Upon discharge from the hospital • Patients remain on anticoagulation for life with Warfarin and INR target 2-3 • If residual PH (mean PAP ≥ 35 mmHg) and clinically symptomatic, consider resuming PAH vasodilators • Regular outpatient follow up with the CTEPH Team at TGH or PH Team locally for monitoring Riociguat (Adempas) • Member of a new class of medications called soluble guanylate cyclase stimulators - Vasorelaxation in the pulmonary arteries - Antiproliferative and antifibrotic effects - Results in the increased exercise capacity (↑ 39m on 6MWT) & improved HD (↓ PVR by 226 ) • The only agent approved for non operable CTEPH patients or for recurrent PH following PEA
  36. 36. Acknowledgement • Dr. de Perrot
  37. 37. Thank you for your attention!