Presenter Disclosure Information Evaluation and Treatment of Evaluation and Treatment of Pulmonary Arterial Hypertension Pulmonary Arterial Hypertension � I will not discuss off label use or investigational Advanced Lung Disease Course, May 2015 use in my presentation. Teresa De Marco, MD, FACC Professor of Medicine & Surgery � I have financial relationships to disclose: Director, Heart Failure and Pulmonary Hypertension Medical Director, Heart Transplantation • Consultant for: Actelion, Gilead, United Therapeutics, Bayer • Research support from: United Therapeutics, Reata • Honoraria from: Actelion, Gilead Pulmonary Hypertension Objectives (PH) Review : • Sustained elevation of mean • Definition and classification of pulmonary pulmonary artery pressure: hypertension (PH) and pulmonary arterial hypertension (PAH) > 25 mmHg • Diagnostic approach MPAP= 1/3(PAs-PAd) + PAd • Treatment Normal: 8 - 20 mmHg Simonneau G, et al. J Am Coll Cardiol . 2004:43:5S-12 Simonneau G, et al, J Am Coll Cardiol . 2009;54:S43-54. 1
Updated Clinical Classification Etiology of PH on Echocardiogram of PH (5 th WSPH Nice, 2013) • Single Center Study from Australia – 4579 patients screened � 483 patients (10.5%) with PH on echocardiogram (defined as PASP >40 mm Hg) • Group 1: Pulmonary arterial hypertension (PAH) 1’ Pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) 1” Persistent PH of newborn • Group 2: PH due to left heart disease • Group 3: PH due to lung diseases and/or hypoxia 78.7% • Group 4: Chronic thromboembolic PH (CTEPH) • Group 5: PH with unclear multifactorial mechanisms Gabbay E . Am J Respir Crit Care Med . 2007;175:A713. The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the ESC and ERS, endorsed by ISHLT. Eur Heart J. 2009;30:2493-2537. Simonneau et al, J Am Coll Cardiol . 20013;62:D34-41. 5 th WSPH Clinical Classification of PAH Group 1: Pulmonary Arterial Hypertension (PAH) (WHO Group 1) • Subset of PH (15 cases/ million) Group 1 � Pulmonary Arterial Hypertension (PAH) • Vasoconstriction, remodeling, Idiopathic PAH thrombosis in situ Heritable • Progressive cardiopulmonary BMPR2 deterioration ALK-1, endoglin, SMAD9,CAAV1,KCNK3 • Leads to RH failure and death Unknown Drug and toxin-induced (67% 5-yr survival) PAH associated with: Humbert M et al. Am J Respir Crit Care Med . Connective tissue disease 2006;173:1023-30 Thenappan T, et al. Eur Respir J . 2010;35:1079-1087. HIV infection • Characterized by progressive and sustained elevation of Portal hypertension pulmonary artery pressure and vascular resistance : Congenital heart disease – PA mean Schistosomiasis > 25 mmHg (nl 8-20 mmHg) 1’ – Pulmonary veno-occlusive disease or pulmonary capillary – PAWP/LVEDP < 15 mmHg (nl 4-12 mmHg) hemangiomatosis – PVR > 3 Wood units (240 dyn/sec/cm-5) 1’’ – Persistent PH of the newborn Hoeper MM, et al. J Am Coll Cardiol . 2013;62:D42-50 . Simonneau et al, J Am Coll Cardiol . 20013;62:D34-41. 2
PAH Determinants of Patient Risk and Prognosis ACC/AHA Expert Consensus Evaluation Low Risk Determinants of Risk High Risk No Yes Clinical evidence of RV failure Gradual Rapid Disease progression II, III IV WHO functional class Longer (> 400 meters) Shorter (< 300 meters) 6-MWD Peak VO 2 > 10.4 mL/kg/min Peak VO 2 < 10.4 mL/kg/min Cardiopulmonary exercise testing Minimally elevated and stable Significantly elevated BNP/NT-proBNP PaCO 2 > 34 mm Hg PaCO 2 < 32 mm Hg Blood gasses Pericardial effusion, RV Minimal RV dysfunction ECHO findings dysfunction, RA enlargement RAP < 10 mm Hg; RAP > 20 mm Hg; Hemodynamics CI > 2.5 L/min/m 2 CI < 2 L/min/m 2 McLaughlin, et al. J Am Coll Cardiol 2009;53:1573 Provencher, et al. E Heart J 2006: 27:589 D ’ Alonzo, et al. Ann Int Med 1991;115:343 Nagaya, et al. Circ 2000;102:865 Raymond, et al. J Am Coll Cardiol 2002;39:1214 Blyth, et al. Eur Respir J. 2007;29:737 REVEAL Database: PAH Diagnostic Guidelines: Most Frequent Symptoms at Diagnosis Decision Analysis Dyspnea at rest ��� IPAH Unexplained Symptoms of Dyspnea on ��� APAH Cough ��� Exertion, Syncope/Near Syncope, Fatigue ����� Dizzy/lightheaded ��� ����� Presyncope/syncope ��� ����� Edema ��� ����� Chest pain/discomfort ��� ��� Other ��� Clinical History, Examination, ����� Fatigue ��� ECG, Chest X-Ray ����� Dyspnea on exertion ��� ����� 0 25 50 75 100 Incidence (%) N=1479. Elliott EG, et al. Chest. 2007;132(4 suppl):631S. McGoon M, et al. Chest . 2004;126:14S-34S. 3
Electrocardiogram Signs on Physical Examination • Insufficiently sensitive as screening tool for PH • Loud pulmonic valve closure (P 2 ) (93%) •Prognosis: � � p-wave in II, qR V1, RVH � � � � � � risk of death � • TR murmur (40%) • RAD, RAE, RBBB,RVH • PR murmur (13%) • Right-sided fourth heart sound • Right ventricular lift Signs of RHF • Jugular venous distention • RV third heart sound (23%) • Peripheral edema (32%) • Ascites • Low BP, low PP, cool extremities (low CO, peripheral vasoconstriction, hypoperfusion) • Stigmata of secondary causes PAH McLaughlin VV et al. J ACC. 2009;53:1573-1619 Image courtesy of Vallerie McLaughlin, MD McGoon M, et al. Chest ��������������������� Bossone E, et al. Chest 2002;121:513 Rich S, et al. Ann Intern Med. 1987;107:216-223. PAH Diagnostic Guidelines: Chest Radiograph in PAH Decision Analysis � • Cardiac enlargement � • No evidence of pulmonary edema � • Prominent proximal PA s � • Lungs appear normal � • “ Pruning ” of distal PA s Clinical History, Examination, Peripheral Prominent Central Chest X-Ray, ECG Hypovascularity Pulmonary Artery RV Enlargement Is There a Reason to Suspect PH? Yes No Echocardiography Work-Up Right Descending for Other Pulmonary Artery Conditions McGoon M, et al. Chest 2004;126:14S-34S McGoon M, et al. Chest . 2004;126:14S-34S. McLaughlin VV et al. JACC 2009;53:1573-1619. 4
Apical Four Chamber Signs of PAH with Echo/Doppler • Increased sPAP or TR jet IVS • Right atrial and ventricular RV hypertrophy/enlargement LV • Flattening of intraventricular RV LV septum • Tricuspid regurgitation RA RA LA LA • Small LV dimension Traditional ECHO does Parasternal Short Axis not accurately measure: •Mean PA pressure •PAWP RV •Cardiac output (blood flow) • Cannot calculate PVR LV • Other limitations- 15% no TR jet, not all congenital lesions obvious, small errors in TRV tracing can alter results ePAsP = 4V 2 + eRAP Normal PAH McGoon M, et al. Chest ������������������ PAH Diagnostic Guidelines PAH Diagnostic Guidelines Echocardiography Indicates PH Echocardiography Indicates PH Evaluate for PFTs Evaluate for PFTs V/Q scan V/Q scan Associated Causes Arterial Saturation Associated Causes Arterial Saturation HIV Infection, Scleroderma, HIV Infection, Scleroderma, Suspected Parenchymal Lung Suspected Parenchymal Lung SLE, Other CTD, Liver Disease, Hypoxemia, SLE, Other CTD, Liver Disease, Hypoxemia, Chronic PE Chronic PE Disease, CHD, Drug- or Sleep Disorder Disease, CHD, Drug- or Sleep Disorder Associated Associated McGoon M, et al. Chest . 2004;126:14S-34S. McGoon M, et al. Chest . 2004;126:14S-34S. 5
Ventilation Perfusion Lung Scan PAH Diagnostic Guidelines Idiopathic PAH Chronic PE Echocardiography Indicates PH Evaluate for PFTs V/Q scan Perfusion Ventilation Perfusion Ventilation Associated Causes Arterial Saturation � 3 – 4% of acute PE do not entirely resolve � 50% of those with CTEPH do not have hx of acute PE HIV Infection, Scleroderma, Parenchymal Lung Suspected SLE, Other CTD, Liver Chronic PE Disease, Hypoxemia, � V/Q scan should be performed to exclude CTEPH even Disease, CHD, Drug- or Sleep Disorder when another explanation for PH is present Associated � CTEPH: >1 segmental-sized or larger mismatched perfusion defects � Normal or very low probability V/Q scan excludes CTEPH McGoon M, et al. Chest . 2004;126:14S-34S. PAH Diagnostic Guidelines: Pulmonary Function Tests, Arterial Blood Confirmation of PAH Gases, and Oxygen Saturation • Findings suggestive • Findings suggestive of Echocardiography Indicates PH of PAH alternate PH diagnoses �� DLCO 40% - 80% of – Hypoxic PH due to COPD expected • Irreversible airway obstruction + increased – Mild to moderate � of residual volumes lung volumes Right Heart Catheterization • reduced DLCO + normal or – Peripheral airway increased CO 2 tension • Establish diagnosis obstruction – Interstitial lung disease • Ascertain etiology – Arterial O 2 tension normal • Decrease in lung volume + • Establish severity & prognosis or slightly � at rest • Decreased DLCO • Verify presence and severity of shunts – Arterial CO 2 is � • Evaluate vasoreactivity – SpO 2 preserved at rest, • Guide treatment may be � with Adapted from McGoon M, et al. Chest . 2004;126:14S-34S. exercise/ambulation Galie N, et al. Eur Heart J. 2009;30(20):2493-2537. 6
Recommend
More recommend
