RYAN O’GOWAN, MBA, PA-C FAPACVS FCCM Pulmonary Hypertension
Disclosures I have no financial relationships with any drug or device manufacturers to disclose.
Objectives At the conclusion of this lecture the participant should be able to: Define Pulmonary Arterial Hypertension (PAH) and discuss its clinical relevance, including various modes of evaluation and diagnosis. Discuss and define the various subgroups of PAH according to the World Health Organization (WHO) classification. Discuss available treatment strategies and their mechanisms for selected WHO subgroups.
Definition Pulmonary Arterial Hypertension: “is a syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance and ultimately in right heart failure. ” ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
Clinical Diagnosis Clinical Definition of pulmonary hypertension: Mean PA pressure>25 mm Hg PCWP <15mm Hg Cardiac output may be normal or reduced Galie N, et al. Eur Heart J. 2009;30(20):2493-2537.
Causes of Pulmonary Hypertension ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
Diseases Associated with Pulmonary Hypertension ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
Presentation Shortness of breath with exertion Dry cough Lower extremity edema Weakness & malaise No Physical Exam findings other than those associated with Right Heart failure can reliably diagnose pulmonary hypertension American Thoracic Society source document on Pulmonary Hypertension
Epidemiology of Pulmonary Hypertension Average age at presentation- 36 years Prevalence women>men by 2:1 Three year survival after diagnosis ~50% Incidence: 10-15 people per million of population Number of U.S. patients: 10,000-20,000. Patients who survive go on to be candidates for total lung transplant. American Thoracic Society source document on Pulmonary Hypertension
SWAN-GANZ CATHETERIZATION SELECTIVE RIGHT HEART CATHETERIZATION ECHOCARDIOGRAPHY CT SCANNING FOR PULMONARY EMBOLI Evaluation and Diagnosis
Swan Ganz Catheterization Clinical Procedures in Anesthesia and Intensive Care, p 416. JB Lippincott, 1994
Selective Right Heart Catheterization Uses the Swan to gather: right atrial pressure (RAP) right ventricular pressure (RVP) pulmonary arterial pressure (PAP) pulmonary capillary wedge pressure (PCWP) systemic arterial pressure (BP) and heart rate cardiac output (CO) pulmonary arterial vasoreactivity pulmonary arterial (PA) ("mixed venous") saturation superior vena cava (SVC) saturation inferior vena cava (IVC) saturation right atrial (RA) saturation right ventricular (RV) saturation
CLASS IIDIOPATHIC PAH (IPAH) CLASS IIPAH WITH LEFT HEART DISEASE CLASS IIIPAH SECONDARY TO HYPOXIA CLASS IV CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION (CTEPH) CLASS VMISCELLANEOUS WHO Classification
Class I Pulmonary Hypertension Idiopathic (IPAH) Heritable (HPAH) Bone morphogenetic protein receptor type 2 (BMPR2) Activin receptor-like kinase 1 gene (ALK1), endoglin Drug- and toxin-induced Cocaine,Amphetamines Fenfluramine (Taken off the market) Associated with (APAH): Connective tissue diseases (SLE) Human immunodeficiency virus (HIV) infection Portal hypertension Congenital heart disease (CHD) Schistosomiasis Chronic hemolytic anemia Persistent pulmonary hypertension of the newborn (PPHN) J Am Coll Cardiol. 2009;54:S43–54.
Class II Pulmonary Hypertension PAH with Left Heart Disease: Mitral Valve Disease (Stenosis/Regurgitation) Aortic Valve Disease (Stenosis/Regurgitation) Systolic Dysfunction Diastolic Dysfunction J Am Coll Cardiol. 2009;54:S43–54.
Class III Pulmonary Hypertension PAH secondary to hypoxia: COPD Interstitial Lung Disease (ILD) Pulmonary Diseases with Mixed Restrictive and Obstructive Patterns Obstructive Sleep Apnea (OSA) Alveolar Hypoventilation Disorders Chronic Exposure to High Altitudes J Am Coll Cardiol. 2009;54:S43–54.
Class IV Pulmonary Hypertension Chronic thromboembolic pulmonary hypertension (CTEPH) Patients may have presentations consistent with hypercoagulable states. Common heritable sources of thrombosis include: Factor V Leiden Prothrombin G 20101A Mutation Proteins C & S Deficiency Lupus Procoagulant J Am Coll Cardiol. 2009;54:S43–54.
Pharmacologic Treatment Group 3 Pulmonary Hypertension Ambrisentan Bosentan Epoprostenol IV Iloprost Inhaled Sildenafil
Group 4 Pulmonary Hypertension Epoprostenol IV (Class A) Iloprost Inhaled(Class B) Pulmonary Thromboendarterectomy (Performed in selected referral centers-UC-San Diego*) TPA for active clot from “fresh” PE