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Pulmonary Hypertension: Overview

Pulmonary Hypertension: Overview. Pulmonary Hypertension (PH) is an under-recognized disease PH is estimated to be the third most common cardiovascular syndrome Is the result of pulmonary, cardiac, infectious, and connective tissue disease Treatment varies based on etiology.

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Pulmonary Hypertension: Overview

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  1. Pulmonary Hypertension: Overview • Pulmonary Hypertension (PH) is an under-recognized disease • PH is estimated to be the third most common cardiovascular syndrome • Is the result of pulmonary, cardiac, infectious, and connective tissue disease • Treatment varies based on etiology

  2. Pulmonary Arterial Hypertension: Definition • Pulmonary arterial hypertension (PAH) is a progressive, incurable disease of the small pulmonary arteries characterized by vascular cell proliferation, aberrant remodeling, and thrombosis in situ • PH is defined as a combination of: • Mean pulmonary artery pressure > or =25mmHg at rest • Mean pulmonary artery pressure > or =30mmHg with exercise

  3. Vascular Pressure in Systemic and Pulmonary Circulations (mm Hg) 120/80, mean 90 25/10, mean 15 Systemic Circulation Pulmonary Circulation Arteries Arteries Right Atrium Mean >6 Left Atrium Mean 5 Body Lung SVR= 17.6PVR= 1.8 Right Ventricle 25/5 Left Ventricle 120/5 Veins Veins

  4. WHO Clinical Classification of PH • Group 1. Pulmonary arterial hypertension • Group 2. Pulmonary venous hypertension • Group 3. PH associated with disorders of the respiratory system and/or hypoxemia • Group 4. PH due to chronic thromboembolic disease • Group 5. Miscellaneous

  5. KDMC Statistics FY 08 415.0-415.9 (Acute Pulm Heart Disease – includes Cor Pulmonale, Pulm Embolism) 416.8 (PH, Secondary) 416.0 – 416.9 (Chronic Pulm Heart Disease – includes all types of PH, Cor Pulmonale)

  6. KDMC Statistics FY07 – FY08 Outpatient Inpatient Emergency Department

  7. Appropriate Patient for Referral • Echo revealing: • Pulmonary Artery (PA) Systolic Pressure > 40mmHg • PA Systolic pressures between 35-40 mmHg when patient is symptomatic • Right Heart Cath revealing: • Sustained Mean PA Pressure >25mmHg at rest or >30 mmHg with exercise • Mean pulmonary-capillary wedge pressure and left ventricular end-diastolic pressure <15 mmHg

  8. Appropriate Patient for Referral • CXR revealing pulmonary artery enlargement or pruning • EKG showing Right Ventricular strain

  9. Required Documentation/Testing • Detailed Physician H&P • Complete PFT • VQ Scan • CXR • Echo

  10. Initial Clinic Visit • Assessment • 12 lead EKG • 6 Minute Walk Test • Patient Education • Additional Testing ordered as indicated • Testing will be coordinated for minimal patient inconvenience

  11. Radiology HRCT of Thorax Pulmonary Angiogram Labs: CBC with platelets BMP LFTs BNPEP Thyroid Function Panel ANA screen RF Sed Rate CRP Scl-70 PT/INR Hypercoagulability Panel HIV Antiphospholipid Antibodies Sleep Study Right Heart Cath Possible Additional Testing

  12. Follow up Clinic Visit • All testing will be evaluated • Patient follow up evaluation • Diagnosis including WHO Group and Functional Class • Treatment Plan initiated • Continual patient education • Letter to referring physician detailing findings and treatment plan

  13. Multi-Departmental Support Echo Lab • Things to obtain and focus on when performing and interpreting ALL Echocardiograms • A global evaluation of right heart structure and function • Right Ventricular systolic pressures • Right Atrial and/or Ventricular enlargement • Diastolic Dysfunction • Valvular Heart Disease, e.g. TR • Ejection fraction • Pericardial disease / Pericardial effusion

  14. Multi-Departmental Support • Cath Lab • Right Heart Cath protocols must be followed in order to diagnose and obtain treatment

  15. Right Heart Catheterization in PAH: Indications • Confirm presence of PH • Aid to determining etiology • Prognostication • Guide therapeutic choices, e.g. CCBs • Follow up therapeutic interventions

  16. RHC in PAH:Key Findings • PH: mean PAP ≥ 25mmHg at rest or ≥ 30 mmHg with exercise • PAWP <15 mmHg • PVR >3 Wood Units • Evidence of valvular heart disease • Evidence of left-sided dysfunction • Evidence of congenital heart disease

  17. RHC in PAHSpecial Procedures • Vasodilator administration • Fluid challenge • Exercise • Shunt evaluation

  18. Multi-Departmental Support • All Departments need to provide the best possible testing and timely reporting • These two aspects are crucial to treating these patients quickly and effectively

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