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Pulmonary Hypertension

Pulmonary Hypertension. Ghulam Saydain MD Assistant Professor of Medicine Pulmonary Critical Care Allergy and Sleep Division Wayne State University School of Medicine 2010. Pulmonary Hypertension: the other blood pressure. Idiopathic/ Familial. Connective Tissue Disease Cong HD.

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Pulmonary Hypertension

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  1. Pulmonary Hypertension Ghulam Saydain MD Assistant Professor of Medicine Pulmonary Critical Care Allergy and Sleep Division Wayne State University School of Medicine 2010

  2. Pulmonary Hypertension:the other blood pressure Idiopathic/ Familial Connective Tissue Disease Cong HD Thrombo -emolism Left Ventricular Failure Lung Diseases Other Conditions High Pulmonary Vascular Pressure/ Resistance Asymptomatic Appropriate Therapy Non-Specific Symptoms Poor Survival Improved Survival

  3. PCWP < 15 Mean > 25 S V C PA LA RA PC IVC LV RV

  4. Pulmonary Hypertension • Etiology & Classification

  5. Pulmonary Hypertension: Classification • Group I. Pulmonary arterial hypertension Idiopathic (primary) IPAH Familial (FPAH) Related conditions: Collagen vascular disease, congenital systemicto- pulmonary shunts, Portal hypertension, HIV infection, drugs and toxins (e.g., anorexigens, rapeseed oil, l -tryptophan, methamphetamine, and cocaine); hemoglobinopathies, Associated with significant venous or capillary involvement Pulmonary veno-occlusive disease Pulmonary-capillary hemangiomatosis Persistent pulmonary hypertension of the newborn Simonneau G et al. JACC 2004;43(Suppl):5S-12S

  6. Pulmonary Hypertension: Classification • Group II. Pulmonary venous hypertension Left-sided atrial or ventricular heart disease Left-sided valvular heart disease Simonneau G et al. JACC 2004;43(Suppl):5S-12S

  7. Pulmonary Hypertension: Classification • Group III. Pulmonary hypertension associated with hypoxemia Chronic obstructive pulmonary disease Interstitial lung disease Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental abnormalities Simonneau G et al. JACC 2004;43(Suppl):5S-12S

  8. Pulmonary Hypertension: Classification • Group IV. Pulmonary hypertension due to chronic thrombotic disease, embolic disease, or both • Thromboembolic obstruction of proximal pulmonary arteries • Thromboembolic obstruction of distal pulmonary arteries • Pulmonary embolism (tumor, parasites, foreign material) Surgical therapy Potential Cure Simonneau G et al. JACC 2004;43(Suppl):5S-12S Am J Respir Crit Care Med Vol 172. pp 1072–1077, 2005

  9. Pulmonary Hypertension: Classification • Group V. Miscellaneous Sarcoidosis (norweigan, african americans), Pulmonary Langerhans’-cell histiocytosis, lymphangiomatosis, Compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) Simonneau G et al. JACC 2004;43(Suppl):5S-12S

  10. LVEDP/LAP 4 Mean 16 S V C PA LA RA PC IVC LV RV

  11. Clinical Presentation : Symptoms • Asymtomatic • Fatigue • Dyspnea • Chest Pain • Syncope • Edema

  12. Physical Examination • JVD – Jugular Venous Distention • Loud P2 • Systolic murmur – Rush of blood pulmonary valve • Pulmonary regurgitation murmur - Backflow • Murmur of Tricusped regurgitation • Hepatomegaly (Pulsatile) • Ascites – b/c accumulation of H2O in belly • Peripheral edema + Signs of underlying disease (SLE, Clots, etc…)

  13. EKG

  14. CXR

  15. Pulmonary Function Tests • Mildly reduced TLC and FVC • Markedly reduced DLCO • FVC%/DLCO% ≥ 1.8 * (Scleroderma) • Used to exclude restrictive and obstructive disease • Not used to make a diagnosis

  16. Echocardiogram • LV should be normal • Normal EF, no LVH • LA not dilated • RVH, RAD • IV septum flattened • Tricuspid regurgitation • Systolic PA = 4v2 + RA

  17. Work up & management of suspected PAH • Establish Diagnosis

  18. Diagnosis of Pulmonary Arterial Hypertension Right Heart Catheterization • Pulmonary Hypertension (PH) • Mean PA pressure ≥ 25 mmHg • Mean PA pressure ≥ 30 mmHg with exercise • Systolic PA pressure ≥ 40 mmHg • Pulmonary ArterialHypertension (PAH) • Mean PA pressure ≥ 25 mmHg or ≥ 30 with exercise • PCWP ≤ 15 mmHg • PVR ≥ 3 mmHg•min/L (Wood) or 240 dyn•sec•cm-5 Exclude Pulmonary Venous Hypertension

  19. Normal Normal Normal Class I-IV Abnormal Class III-IV Normal Abnormal Abnormal Class I-II The sequence and the extent of testing depend on the clinical scenario Diagnostic Algorithm Sirithanakul, Mubarak, J Fam Prac 53(12):963, Dec 2004

  20. Idiopathic Pulmonary Arterial Hypertension“Primary Pulmonary Hypertension” • Prototypical PAH, NO other underlying disease. • Rare (1-2 per million per year) • Female preponderance (M:F=1:3) • Age 30-50 • Untreated survival 2-3 years • Inherited disorder (BMPR2) • Familial 6% Ann Intern Med, 1987; 107:216-23, Ann Intern Med, 1991; 115:343-49).

  21. PAH Due To Collagen Vascular Disorders • Scleroderma incidence: 5 to 38% (mean 16%) • long-standing limited scleroderma (CREST syndrome), • Autoantibodies (including anti-centromere antibodies), • Disease onset after menopause • Anti-nucleolar antibodies including U3RNP, B23 and Th/To,64–66 and U1RNP • SLE • Mixed Connective Tissue disease • Rheumatoid arthritis CHEST 2004; 126:14S–34S

  22. PAH in HIV Infected Patients • Incidence 0.5 % • The interval between the diagnosis of HIV and the diagnosis of PAH about 33 months. • 82% related to HIV • Plexogenic pulmonary arteriopathy • The median length of time from diagnosis to death was 6 months. Mehta CHEST 2000; 118:1133–1141

  23. Survival in PAH CHEST 2004; 126:78S–92S)

  24. Assessment of Severity Predictors of Survival

  25. WHO Functional Classification Class I: Ordinary physical activity does not cause symptoms With exercise, become SOB Class II:Slight limitation of physical activity. Ordinary physical activity causes symptoms Class III:Marked limitation of physical activity. Less than ordinary activity causes systems Brushing teeth, dressing, etc… Class IV: Inability to carry out any physical activity without symptoms. Dyspnea and/or fatigue may even be present at rest. (Symptomaic at rest doing nothing) Symptoms: Dyspnea, Chest pain Syncope

  26. Six-Minute Walk Miyamoto, AJRCCM, 161:487-492, 2000

  27. Pathology & Pathogenesis

  28. Histologic Subtypes of Pulmonary Hypertension COPD Sever PAH Scleroderma Normal Cool CHEST 2005; 128:565S–571S

  29. Pathogenesis of PAH • Imbalance of Mediators Vasoconstriction Proliferation Thrombosis • TX A2 • ET 1 • 5 HT • PGI2 • NO • VIP

  30. Conventional Therapy for IPAH • Oxygen supplementation • Oxygen is a vasoconstrictor • Diuretics for edema in lungs and legs • Anti-coagulation • Heparin given to reduce PE and to undercoagulate them preventitavely

  31. Oral Therapies

  32. Prostocyclin Derivatives • Continuous IV infusion Epoprostenol (FLOLAN) • Subcutneous or IV infusion Treprostinil sodium (REMODULIN) • Inhalation Therapy Iloprost (VENTAVIS)

  33. Drawbacks (Epoprostenol) • Hickman catheter • Exit site infection: 0.24 per person-year • Sepsis: 0.14 per person-year • Thrombosis • Very short half life • 3-5 minutes • Continuous pump • Very unstable • Ice pack McLaughlin, Circulation, 106:1477-82, 2002

  34. Prostacyclin Therapy : Side effects • Flushing, Dizzines, Hypotension • Jaw Pain • Dairrhea, Nausea, Vomiting • Muscle Aches

  35. ProDose AAD Systemfor Vnetavis inhalation

  36. Endothelin Receptor Antagonist • Bosentan (Tracleer) • Ambrisentan ( Letairis)

  37. Bosentan Side Effects • Jaw pain , Flushing , Headache , • Can’t take when pregnant • Nasal Congestion • Anemia • Elevated liver enzymes • Edema Placebo Bosentan Abnormal hepatic function 2 (3) 13 (9)

  38. Ambrisentan • Side effects Edema Muscle Aches Nasal Congestion Liver function abnormalities

  39. Phosphodiasterase Type 5 inhibitors Sildenafil (Revatio) Tadalafil (Adcirca) Side effects Headache Muscle aches Visual disturbances

  40. Pulmonary Hypertension General Treatment Measures Diuretics, O2 supp, Anticoagulation Yes Vasoreactivity Class II Class III Class IV Calcium Channel Blockers No Bosentan Epoprostenol Ambrisentan* Sildiniafil Trepoprostinil Iloprost Epoprostenol Bosentan Sildiniafil Trepoprostinil Iloprost Ambrisentan* Sildinafil Ambrisentan* Treprostinil SC/IV Sustained Response Class I -II Yes Continue CCB Combination Therapy Worsening Lack of response Atrial Septostomy Lung Transplant CHEST 2007; 131:1917–1928

  41. Pulmonary Arterial HypertensionPromising Therapies • Newer therapies based on known pathophysiologic mechanism are available • These therapies have shown significant improvement in symptoms as well as quality of life • Newer treatments have made, improvement in survival possible • Combination therapy is likely to be beneficial

  42. Early Diagnosis Saves Lives • Screening echo for high risk patients • Family history of IPAH • Connective tissue disease • Liver disease, cirrhosis, portal hypertension • HIV infection • Sickle cell disease • Evaluate unexplained dyspnea carefully • Echo performed by experienced cardiologist • Early referral to a PH Center

  43. Thank You • Questions ? gsaydain@med.wayne.edu

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