Pulmonary Hypertension

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2. Pulmonary Hypertension

3. What is pulmonary hypertensionand what causes it? • Pulmonary hypertension (PH) • Mean pulmonary arterial pressure (PAP): >25 mm Hg • Elevated PAP burdens normally thin-walled right ventricle • Without treatment, right heart dysfunction = progressive symptoms, often eventually death • PH classified into 5 categories, each with a different… • Mechanism for the elevated PAP • Natural history • Approach to treatment

4. What causes pulmonary hypertension? • PH due to left heart disease (most common cause) • Systolic or diastolic left heart dysfunction • Mitral or aortic valve disorders • PH due to chronic hypoxemic lung disease • Obstructive lung disorders; interstitial lung disease • Sleep-disordered breathing • PH due to embolic disease • Miscellaneous causes • Pulmonary arterial hypertension (PAH) • Heritable / genetic abnormalities; idiopathic • Risk factors: collagen vascular diseases, HIV infection, liver disease, anorectic agent use

5. Who should be screened? Patients with… • Systemic sclerosis • Family history of a heritable form of PAH • Portal hypertension considered for organ transplant • Increased perioperative mortality with elevated mean PAP • Therapy may be needed prior to transplantation • Annual screening recommended in this setting

6. What are the symptoms? • Progressive dyspnea (most common symptom) • Fatigue • Chest pain • Presyncope / syncope • Lower extremity edema • Palpitations • Hoarseness from Ortner syndrome (rare)

7. What are the physical exam findings? • Accentuated intensity of pulmonary second heart sound • Tricuspid regurgitant murmur • Pulmonary insufficiency murmur • Right ventricular S3 or S4 • Parasternal heave or subxiphoid thrust • Jugular venous distension • Peripheral edema • Hepatomegaly • Ascites

8. What is the role of echocardiography patients with suspected PH? • One of best tests to evaluate for possible PH • May report an estimate of systolic PAP • May provide information on cause of PH and symptoms • ECHO evaluating dyspnea or cardiac murmur may find PH • Indicators of more severe disease • Right atrial or ventricular enlargement • Hypertrophy; decreased right ventricular function • Severe elevations in right ventricular pressure may cause leftward deviation of interventricular septalum • Pericardial effusion

9. What other tests should be ordered in the evaluation of PH? • Autoantibody testing for collagen vascular disease • Brain natriuretic peptide or N-terminal BNP • Chest radiography; ECHO; EKG • CBC; electrolytes / creatinine measurement • HIV serologic testing • Liver function testing • Pulmonary function testing • Oxyhemoglobin saturation at rest and with exertion • Polysomnography • Radionuclide ventilation-perfusion imaging • Right heart catheterization • Six-minute walking distance

10. Which patients require cardiac catheterization? • Right heart catheterization • Required if PAH suspected (before advanced medical Rx) • Also helps identify unrecognized left heart dysfunction and pulmonary venous hypertension • Left heart catheterization • Often done concurrently, particularly if risk for CAD • No right heart catheterization needed… • PH known to be due to left heart or chronic pulmonary disease AND and cath not needed to guide management • Presence of PH itself doesn’t usually alter therapy

11. How should right heart catheterization be done when PH is a consideration? • Assess for possible left-to-right shunts • Measure oxygen saturation in central veins, right atrium, right ventricle, pulmonary artery • Increased oxyhemoglobin saturation suggests oxygenated blood being shunted to right-sided circulation • Measure hemodynamics accurately • At end of exhalation + level equipment at mid-thoracic line • Unsure of wedge?  Measure left ventricular end-diastolic pressure simultaneously • Test pulmonary vasoreactivity in PAH

12. What are the requirements for the diagnosis of PAH? • Presence of PH • Mean PAP > 25 mm Hg • Absence of pulmonary venous hypertension • Left atrial or “wedge” pressure < 15 mm Hg • Elevated pulmonary vascular resistance • > 3 Wood units • Exclusion of significant chronic hypoxemic lung disease • Exclusion of chronic thromboembolic disease

13. When should a clinician consider consultation with a specialist in diagnosing pulmonary hypertension? • Uncertainty regarding the diagnosis • Multiple comorbid conditions that may complicate diagnosis or treatment • High-risk features or NYHA functional class III or IV • Refer to a specialized center for evaluation

14. CLINICAL BOTTOM LINE: Diagnosis and Screening… • Confirm elevated pulmonary pressures • Evaluate for potential causes • ECHO; chest X-ray • Ventilation-perfusion scanning • Pulmonary function and blood testing • Assess disease severity • Measure oxyhemoglobin saturation • 6-minute walking distance • Blood BNP • Right heart catheterization • Mandatory if therapy directed at PH itself (e.g., PAH)

15. What is the approach to treatment of PH? • Identify the cause • Chronic cardiac or pulmonary disease • Treat the underlying condition • Oxygen therapy • Maintain oxygen saturation ≥90% • Right heart dysfunction • Minimize fluid overload and dyspnea • Use diuretics • Restrict salt • Monitor weight

16. How should patients with PH due to left heart disease be treated? • Systolic HF • Use: ACE inhibitors; β-blockers; diuretics • Possibly: cardiac resynchronization, implantable cardioverter defibrillator placement, digitalis • HFpEF • Control BP + heart rate with β-blockers, diuretics • Left HFrEF • Differentiate from PAH • Don’t use prostacyclin analogues, endothelin antagonists • PH secondary to left valvular heart disease • Evaluation for correction of valvular disease

17. How should patients with PH due to lung disease be treated? • Optimize treatment of underlying cause • Sleep apnea (minimize nocturnal desaturation) • COPD • Idiopathic lung disease • Use supplemental oxygen to avoid hypoxia • Enroll in pulmonary rehabilitation • Don’t use PAH therapy in PH due to lung disease

18. How should patients with chronic thromboembolic PH be treated? • Prevention of recurrent clot / embolism (anticoagulation) • Pulmonary thromboendarterectomy (PTE) • Refer to center experienced in procedure and postop • Improves symptoms in most patients • Surgical mortality at experienced centers: <5% • Use medical therapy directed at PH only when… • PTE impossible due to distal location of disease within pulmonary vasculature • Therapeutic bridge needed until PTE performed • Patient decides not to have PTE

19. What drugs are available for the treatment of PAH? • Diuretics • Supplemental oxygen • Calcium-channel blockers (trial only if demonstrated vasoreactivity) • Anticoagulants • Digoxin • Advanced therapies • Endothelin Antagonists (for lower risk patients) • Phosphodiesterase-5 Inhibitors (for lower risk patients) • Prostacyclins (for higher risk patients)

20. Is there a role for combination therapy in PAH? • Sequential addition of advanced therapies: current model • Initial therapy: based on illness severity + functional class • If PAH worsens: add therapies until treatment goals met • Approach shown to improve 6-min walking distance and delay clinical worsening • Combination therapy • Optimum and safest approaches not yet established • Investigations ongoing

21. What is the role of lung transplantation? • Treats underlying pulmonary condition in patients with… • Late-stage respiratory disease: severe PH + COPD or ILD • PAH: consider transplant when progressive disease requires parenteral therapy • Defer until deterioration occurs despite max medical Rx • Double lung transplantation: preferred procedure for PAH • Reduces right ventricular afterload: right heart often recovers • PAH: 23% unadjusted 3-mo mortality after procedure • Heart-lung transplantation • For uncorrectable congenital heart lesions • For concomitant primary cardiac failure

22. What is the role of exercise? • Exercise improves exercise capacity and QOL • Important adjunct to medical therapy • Avoids deconditioning from being sedentary • PH isn’t a contraindication to judicious exercise • Encourage patients to remain active within symptom limits • Mild breathlessness is acceptable • Avoid severe breathlessness, exertional dizziness, near syncope, or chest pain • Isometric exercises discouraged due to exertional syncope

23. CLINICAL BOTTOM LINE: Treatment… • Right HF • Use diuretics, salt restriction • PH due to left heart or chronic hypoxemic lung disease • Treat underlying disorders (not PH per se) • Chronic thromboembolic PH • Use anticoagulants + possible thromboendarterectomy • PAH • Perform right heart catheterization with vasodilator test • Don’t treat empirically with calcium-channel blockers • Advanced therapies: prostacyclins, endothelin-receptor antagonists, PDE5 inhibitors • Evaluate need for supplemental oxygen • Lung transplantation: in nonresponsive advanced disease

24. What is the prognosis of PH? • Negative prognostic sign in many conditions (HF, COPD) • In particular, PAH prognosis • Worse in presence of advanced NYHA/WHO functional class, shorter 6-min walking distance • Worse with connective tissue disease • Worse with portopulmonary hypertension • Untreated PAH: median survival 2.8 years

25. What should patients be taught about pulmonary hypertension? • How PH is distinct from systemic hypertension • How PH often connotes significant systemic disease • Regardless of the cause • Requires ongoing closely coordinated medical care • Requires monitoring salt intake, fluid balance, weight • That patients with PAH may need to… • Self-administer medications • Monitor themselves for AEs or progression of disease • Know how other health issues might compromise PAH care • Where to find information and peer support

26. CLINICAL BOTTOM LINE: Prognosis… • PH a negative prognostic sign in many conditions (HF, COPD) • Treatment can delay complications • Ongoing closely coordinated medical care is important • Monitor salt intake, fluid balance, weight • PH almost always connotes significant systemic disease