PowerPoint Presentation

New York, New YorkOctober 21, 2013Suspecting Pulmonary Hypertension in the Dyspneic Patient: Who, When, and HowFor HostWelcome attendeesIntroduce yourself

Suspecting Pulmonary Hypertension in the Dyspneic Patient: Who, When, and HowHost: Kenneth J. Steier, DO, FACOI, FCCP, MBA, MPH, MHA, MGH

Guest Presenter: Arunabh Talwar, MD, FCCPFor Host

Welcome attendeesIntroduce yourself

Host: Kenneth J. Steier, DO, FACOI, FCCP, MBA, MPH, MHA, MGH Dean, TouroCOM-Middletown CampusClinical Dean, TouroCOM-Harlem CampusInternal Medicine, Pulmonary and Critical CareTouro College of Osteopathic MedicineNew York, New YorkSuspecting Pulmonary Hypertension in the Dyspneic Patient: Who, When, and How3HousekeepingPlease mute your cellphones/pagers now

Syllabus folder contains slides, agenda, CME information, and faculty disclosuresPHA pamphlet contains valuable professional and patient resource informationPlease hand in CME evaluation form before leaving; CME certificates will be mailedFor HostWe thank our CME partner, Washington University School of Medicine, for their careful review of all meeting materials to ensure that this program has been planned and conducted according to ACCME guidelines.Todays program provides 1.5 hours of full category 1 AMA credits for any doctors, nurses, PAs and NPs attending.Please be sure to complete the CME evaluation form in your meeting folder and hand it in to the coordinator at the end of the program; your input is invaluable.You will receive your CME certificate in the mail.

4Supported in part by educational grants from:

This live activity is jointly sponsored by Washington University School of Medicine, Continuing Medical Education and PHADiamondPlatinumGoldSilverActelionGileadUnitedBayerTherapeuticsFor HostPlease acknowledge partnership with Washington University School of Medicine CME.Please acknowledge and thank the Corporate Sponsors of the Med Ed Fund.

5This live activity has been planned and implemented in accordance with the Essential Areas and Policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Washington University School of Medicine, Continuing Medical Education and the Pulmonary Hypertension Association. Washington University is accredited by the ACCME to provide continuing medical education for physicians.Accreditation and Credit Designation

Washington University designates this live activity for a maximum of 1.5 AMA PRA Category 1 Credits. Physicians should claim only the credit commensurate with the extent of their participation in the activity.For HostWe thank our CME partner, Washington University School of Medicine, for their careful review of all meeting materials to ensure that this program has been planned and conducted according to ACCME guidelines.Todays program provides 1.5 hours of full category 1 AMA credits for any doctors, nurses, PAs and NPs attending.Please be sure to complete the CME evaluation form in your meeting folder and hand it in to the coordinator at the end of the program; your input is invaluable.You will receive your CME certificate in the mail.

6Faculty DisclosureDr. Steier has [to be added]

Dr. Talwar has no relevant financial relationships to disclose.

For HostNote that full CME information, including these disclosures, is available in the hand-out, and that the program has gone through an extensive CME review process.

Syllabus materials. Including 6-ups of the slides, are in the meeting folder.

7Lets get started8Guest Presenter: Arunabh Talwar, MD, FCCPAssociate Professor of Clinical MedicineAlbert Einstein College of MedicineDirector, Pulmonary Hypertension and Interventional Bronchoscopy Program North Shore University HospitalManhasset, New YorkSuspecting Pulmonary Hypertension in the Dyspneic Patient: Who, When, and How9The Pulmonary Hypertension Association (PHA) is the leading non-profit organization for PH research, public awareness, and services.The organization has over 12,000 members, including patients, family members, and medical professionals. www.PHAssociation.org10Case Presentations2 Women With Dyspnea112 Women With DyspneaPatient 2

Patient 1

Patient 212Age: 57 yearsPatient 1Comorbidities:HTNDiabetesCKDAtrial fibrillation

2 Women With DyspneaPatient 2

Age: 48 yearsComorbidities:HTNCKDSystemic sclerosis13Patient 2NYHA Class IIIBP: 120/84 mm HgJVP elevatedRegular rate, rhythmLoud P23/6 murmur (holosystolic, left sternal border)2+ leg edema

NYHA Class III

Patient 1BP: 172/65 mm HgJVP elevatedIrregularly irregularLoud P22/6 murmur (holosystolic, left sternal border)2+ leg edema2 Women With Dyspnea14Pulmonary Arterial Hypertension (PAH): Key PointsAverage 14-mo delay from initial presentation to diagnosis: need to diagnose earlyEvaluation must be methodical and include echocardiography and right heart catheterizationTo treat effectively and avoid harm, PAH must be differentiated from pulmonary venous hypertensionPrognosis improves with therapy, but PAH remains a progressive fatal diseaseTherapies and management strategies continue to evolveWe hope that by the end of this talk the audience will recognize that:The non-specific nature of symptoms of PAH contributes to significant delays in diagnosis, and that Such delays likely have an impact on prognosis, which is poor in the absence of therapy and close follow-up.While the diagnostic workup is not difficult, it must be performed methodically so as to be certain of the diagnosis. RHC is required to make a diagnosis of PAHDifferent etiologies of PH have very different treatment approaches and prognosesNotably, while PAH remains a life-threatening disease, therapies have evolved rapidly.We now know that better diagnosis and treatment of PAH can make a substantive differenceEffective therapies now exist Hemodynamic Definition of PH/PAHPHPAHMean PAP 25 mm Hg plusPCWP/LVEDP 15 mm HgMean PAP 25 mm HgACCF/AHA includes PVR >3 Wood UnitsBadesch D et al. J Am Coll Cardiol. 2009;54:S55-S66.Gali N et al. Eur Heart J. 2009;30:2493-2537.McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.The hemodynamic working definition of PAH listed here is derived from the 2009 Proceedings of the 4th World Symposium on PH. In the new recommendations, exercise and PVR criteria have been eliminated. An accurate PCWP can be difficult to obtain in patients with PH and enlarged pulmonary arteries. If PCWP is elevated despite multiple attempts, especially if blood obtained in the wedge position is not fully saturated, direct measurement of LVEDP should strongly be considered so as not to misdiagnose patients who have PAH. In a recent retrospective study of 4320 patients undergoing simultaneous right and left catheterization, 54% meeting criteria for PAH on the basis of a PCWP 15 had a LVEDP >15 (even among patients being evaluated specifically for PH).

ACCF = American College of Cardiology FoundationAHA = American Heart AssociationLVEDP = left ventricular end-diastolic pressurePAP = pulmonary arterial pressurePCWP = pulmonary capillary wedge pressurePVR = pulmonary vascular resistance

Refs: Badesch D et al. J Am Coll Cardiol. 2009;54:S55-S66.Gali N et al. Eur Heart J. 2009;30:2493-2537.McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.

Clinical Classification of Pulmonary Hypertension (Dana Point) PAHIdiopathic PAHHeritableDrug- and toxin-inducedPersistent PH of newbornAssociated with:CTDHIV infectionportal hypertensionCHDschistosomiasischronic hemolytic anemia

1. PVOD and/or PCH

PH Owing to Left Heart DiseaseSystolic dysfunctionDiastolic dysfunctionValvular disease3. PH Owing to Lung Diseases and/or HypoxiaCOPDILDOther pulmonary diseases with mixedrestrictive and obstructive patternSleep-disordered breathingAlveolar hypoventilation disordersChronic exposure to high altitudeDevelopmental abnormalities

4. CTEPH

5. PH With Unclear Multifactorial MechanismsHematologic disordersSystemic disordersMetabolic disordersOthersSimonneau G et al. J Am Coll Cardiol. 2009;54;S43-S54.The clinical classification of pulmonary hypertension, updated at the 4th World Symposium, is represented here.

PAH is represented in the first subgroup. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are housed within this classification, but in a separate group, distinct from but very close to Group 1 (now called Group 1-prime).

Of note, left heart disease (Category 2) probably represents the most frequent cause of PH. Therefore, it is critically important in the diagnostic workup to distinguish right heart from left heart disease.

The predominant cause of PH in Category 3 is alveolar hypoxia as a result of lung disease, impaired control of breathing, or residence at high altitude.

Patients with suspected or conrmed CTEPH (Category 4) should be referred to a center with expertise in the management of this disease.

Group 5 comprises several forms of PH for which the etiology is unclear or multifactorial.

CHD = congenital heart diseaseCOPD = chronic obstructive pulmonary diseaseCTEPH = chronic thromboembolic pulmonary hypertensionHIV = human immunodeficiency virusILD = interstitial lung diseasePCH = pulmonary capillary hemangiomatosisPVOD = pulmonary veno-occlusive disease

Simonneau G et al. J Am Coll Cardiol. 2009;54:S43-S54.PH LessonsPulmonary Hypertension Is CommonWHO Group I PAH Is Rare but DeadlyMake the Diagnosis EarlyKnow the PH Clinical Clues in the Dyspneic PatientLook Beyond the PA Pressure on EchocardiographyDefinitive Diagnosis of PAH Requires Invasive Hemodynamic Testing18PH Lessons (contd)Always Look for the Underlying Cause of PHTreatment of PHGet the Diagnosis Correct and Determine Functional StatusLack of Response to Acute Vasodilator Challenge in PAH UntreatableFirst Do No HarmLearn to Differentiate WHO Group I PAH From Other Forms of PHAppropriate, Timely, and Collaborative Care: Key to Early and Effective Treatment of PH in the Dyspneic Patient19Lesson 1Pulmonary Hypertension Is Common20

Wheres Waldo?Among all these people, who has shortness of breath?Who has PAH?21

Wheres Waldo? (This is PAH.)22

In the real world there are lots of Waldos but the majority have PH, not PAH.23PH in the Community: PASP and SurvivalAll Participants(N=1413)Overall Log Rank p