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| Looking at PAH |
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What happens in the Pulmonary Arteries and Vascular Bed
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Cardiologists View of Heart and Lungs
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What happens from a Hemodynamic level |
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What happens to the Right Heart |
| Questions to Address |
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When and why to do a heart catheterization
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What about echocardiograms
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Congenital Heart Disease and PAH |
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Right heart failure and its treatment |
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| Heart vs Lung in Pulmonary Hypertension from a Cardiologist Viewpoint! |
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The right heart determines the outcome in PAH
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When pulmonary hypertension gets severe the right heart fails, the cardiac output falls. |
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Saving the patient involves protecting the right heart and treating it |
| Why Do a Cardiac Catheterization? |
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To make sure the pulmonary pressures are not due to a problem in the left heart |
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To look for shunt lesions |
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To measure cardiac output |
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To confirm the echo derived pressures |
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Clearance for Transplant |
| When to do a Cardiac Catheterization |
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At least once!!!! |
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To confirm the diagnosis, When there is a significant deterioration or change particularly if high output from too much prostacyclin is being considered |
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To confirm that therapy is working? |
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Not necessary frequently |
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Transplant evaluation |
| Hemodynamic Perspectives |
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In the NIH multivariate analysis of hemodynamic predictors of mortality at baseline, three factors were identified |
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 mPAP |
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mRAP |
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CI |
| D'Alonzo et al. Annals Int Med 1991;115:343-349 |
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When not to do a Cardiac Catheterization? |
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When everything is going along well |
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Just because..... |
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Its time..... |
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Controversial- when adding intravenous medication (we do not routinely!) |
Work-Up Echo
The Entry Point for PAH Diagnosis |
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Assess left-sided disease |
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Define the congenital heart disease |
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TEE when indicated particularly to evaluate pulmonary veins |
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Confirm pulmonary hypertension and measure pressure |
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Follow pressures over time |
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Contrast echoes to assess shunt |
Left-Sided Heart Disease
Associated with Pulmonary Hypertension
Left Sided Diseases |
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These usually cause reversible pulmonary hypertension with minimal arteriopathy |
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Mitral, Aortic valve diseases and LV dysfunction |
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Treatment of the left-sided problem results in resolution or significant reduction in the pressures |
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Mitral valve disease may cause disproportionate PH and patients may be left with higher pressures especially immediately post-op |
| Echoes are not Perfect |
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Pulmonary pressures can be underestimated by Doppler |
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Estimates of Right Atrial Pressure may result in overestimating the pulmonary pressure |
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Only the Pulmonary Systolic pressure is measured |
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May not tell you why- could be from the left side |
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Stress Echoes estimates of pulmonary pressures are highly variable and very misleading |
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Congenital Heart Disease:
Shunt Lesions |
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Atrial Septal Defects, |
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Atrial Septal Defects with anomalous veins |
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VSD, Double outlet right ventricles etc. |
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PDA and Aorto-Pulmonary Windows |
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Complex Congenital defects |
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| What is Eisenmengers? |
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Congenital heart disease associated with large systemic to pulmonary shunts exposing the pulmonary bed to arterial pressures and leads to pulmonary vascular disease characterized by progressive cyanosis, polycythemia and functional limitation. |
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Should ASD or patient with anomalous veins be called Eisenmengers? Are they the same? |
| Assessment of Congenital Heart Disease and PH |
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Cardiac Catheterization |
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Vasoreactivity testing includes using: |
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100% Oxygen |
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Prostacyclins |
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Nitric Oxide |
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Adenosine infusion |
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| Our Preliminary Experience with ASD Closures in PAH |
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In patients with vasoreactivitiy demonstrated by reversal of shunting or increase in L to R shunting patients survived surgery and improved clinically |
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Patients didn't require oxygen and had improved 6 min walks |
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All patients remained on PH meds and had persistent PH |
| Regulation of Pulmonary Resistance |
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Hypoxia increase vasoconstriction |
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Chemical neural and hormonal regulation occurs |
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ERDF (Nitric Oxide) vasodilates |
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Endothelin vasoconstricts |
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Prostocyclin vasodilates |
| Treatment relates to pathogensis |
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PAH is a disease characterized by vascular proliferation, hypertrophy, and fibrosis, leading to hemodynamic and right ventricular abnormalities |
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Endothelin, prostacyclin and nitric oxide are thought to be key mediators in PAH |
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Treatment models are based on these 3 pathways |
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| Prostacyclins |
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Flolan is the gold standard of therapy for PAH |
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By reducing pulmonary pressures and affecting vascular remodeling in the pulmonary arteries prostacyclins improve right heart function |
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Other prostacyclins have a role and are easier to use |
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| Sildenafil Data |
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Presented at Chest in October 2004, 12 week placebo controlled double-blind doses ranging from 20-80 mg TID |
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Results Comparable to Other Agents, I.e. 40+ increase in 6 minute walk, mild reductions in PAP. 80 mg slightly better. |
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Changes sustained over longer term follow-up although almost all patients titrated up to 80 TID |
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Side effect profile excellent |
| Treatment Options in PPH |
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Calcium Channel Blockers in responders not in right heart failure- very limited use |
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Prostacyclins- IV Flolan, Remodulin, Subcutaneous Remodulin, Inhaled Ilaprost |
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Endothelin Inhibitors - Bosentan |
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Sildenafil- data now available |
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Experimental Therapy
Myogen, Sitaxsentan, Sildenafil, Inhaled Prostacyclins Arginine |
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Atrial Septostomy- of limited value compared other therapies |
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Lung/Heart Lung Transplantation |
| Be Scared! Be Very Scared |
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Calcium Channel Blockers are Dangerous |
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In the absence of vasoreactivity they can cause profound RIGHT heart failure |
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Only a small percentage of patients with vasoreactivity will respond to them anyway |
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If patients are responders their outcomes are excellent |
| Treatment From a Cardiologists Perspective |
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Protect the Right Side of the Heart and Improve Right Ventricular |
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Function Digoxin |
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Oxygen to maximize transport |
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Control Volume status with diuretics and fluid restriction |
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DRY IS GOOD!!! |
| Right Heart Failure |
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Dilatation in response to pressure load on RV results in increased wall stress and decompensation |
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Hypertrophy is an advantageous response to pressure load and preserves function |
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? Genetic differences in response or is it related to rapidity of pressure rise |
| Right Heart Failure - Treatment |
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Increase myocardial contractility- digoxin |
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Reduce afterload on RV |
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Reduce volume load- this is key to RV decompensation- |
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Lower volume = less wall stress, less TR and less compression of the LV |
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Less liver congestion and dyspnea and better oxygenation |
| Conclusion |
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Cardiac catheterization has a place in diagnosis and treatment |
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The right heart is key to treatment of PAH |
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Echoes provide a way to identify PAH |
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Understanding hemodynamics in PAH allows us to improve therapy in congenital heart disease and IPAH |
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DRY is GOOD |
