An Introduction to the Westchester Medical Center Pulmonary Hypertension Program iii Westchester Medical Center (WMC) in Valhalla, Contents New York, and the WMCHealth Heart and Vascular The Pulmonary Hypertension Program Leadership 1 Institute, are proud to showcase the Pulmonary Hypertension Program, serving Westchester County Overview of Pulmonary Hypertension 2 and beyond. Its goal is to provide state-of-the-art advanced diagnosis and treatment for this rare Diagnostic Evaluation of PH 3 condition. As a large tertiary center, WMC currently offers cutting-edge, personalized treatment for Testing: Non-invasive and Invasive 4 cardiovascular disease including advanced heart failure, valvular, coronary and vascular disease. Treatment: Outpatient and Inpatient Care 5 “In continuing its mission to Pulmonary Embolism and Chronic provide a high level of care to Thromboembolic Pulmonary Hypertension 6 the region, WMCHealth now introduces its multidisciplinary Extending the Impact of the team dedicated to treating WMC PH Program through Research 7 pulmonary hypertension. An Julio A. Panza, MD Chief of Cardiology overview of the components Team Members 8 Westchester Medical Center and WMCHealth of the Pulmonary Hypertension Program is detailed in the following pages.” iv Director Cardiothoracic Surgery Gregg M. Lanier, MD Ramin Malekan, MD The Pulmonary Hypertension Program Leadership Gregg M. Lanier, MD, Director of Pulmonary Ramin Malekan, MD, Hypertension at Westchester Medical Center is a cardiothoracic surgeon at WMC who (WMC), is a heart failure specialist with training trained previously at Mount Sinai Hospital in in pulmonary hypertension (PH). He completed New York City. He sought additional training his medicine residency and fellowship at Mount and experience in performing pulmonary Sinai Hospital in New York City, and spent an thromboendarterectomy, a highly skilled surgical additional year in advanced heart failure training treatment of PH from chronic pulmonary blood at New York-Presbyterian Columbia Hospital. clots. Working together, Dr. Lanier and Dr. He has more than eight years of hands-on Malekan, along with many other consultative experience with the diagnosis and treatment of services at WMC, are able to provide PH, including expertise with all drugs currently comprehensive and highly effective care for approved for this condition. He has published this condition. several articles on PH, and is a current principal investigator at WMC of a multicenter clinical trial in PH. Additionally, he participates in patient support groups, is a member of the Pulmonary Hypertension Association, and is active in the region promoting awareness of this disease. 1 Overview of Pulmonary Hypertension Pulmonary Hypertension (PH) is a general than two years after the onset of symptoms, working in consultation with a centralized PH term for a multitude of conditions that result and this delay is thought to lead to overall program. After the diagnosis is established, the in elevated pressures in the lung vasculature. poor outcomes. Within group 1 PAH, there PH team determines the need and monitors While common diseases such as heart are several conditions such as familial the initiation of oral, inhaled, intravenous and dysfunction and emphysema may result in PAH, and PAH secondary to: liver disease subcutaneous medications as appropriate. mildly elevated pressures incidentally found (portopulmonary hypertension), scleroderma, In the case of WHO group 4 PH, surgical in routine testing, some patients develop HIV, diet drugs, congenital heart disease, pulmonary thromboendarterectomy may result very specific disease of the pulmonary methamphetamine use and schistosomiasis. in a “cure” of the disease with excellent clinical arterioles, which causes pulmonary arterial More common causes of PH include non-PAH outcomes. hypertension (PAH). Untreated, this rarer conditions such as WHO group 2 PH from left form of PH can result in significant symptoms heart disease (heart failure), WHO group 3 This coordination of multiple disciplines in and a very poor prognosis. PH from low oxygen levels, and WHO group 4 the diagnostic evaluation and treatment of PH from chronic pulmonary emboli (chronic patients with PH is the central purpose of an The World Health Organization (WHO) has thromboembolic pulmonary hypertension or interdisciplinary PH program and is essential categorized the difference causes of PH CTEPH). Other miscellaneous causes of PH in ensuring quality outcomes such as improved into five groups. PAH, or WHO group 1 PH, are included in group 5, as shown in the quality and duration of life. The WMCHealth is an often misunderstood disease that can table below. Heart and Vascular Institute’s Pulmonary cause significant morbidity and mortality. Hypertension Program has a prominent and Idiopathic PAH is rare, with an incidence of The most important mission of a comprehensive growing role in the highly specialized care of about 12 per 1 million people. This disease PH program is to first establish the correct these patients who are referred from a large predominantly affects younger women, usually diagnosis, as the treatment depends on the geographical and sometimes underserved in their child-bearing years. Unfortunately, cause. The diagnostic evaluation of a patient region of New York, northern New Jersey, the diagnosis of PAH is made often more with PH requires multiple tests and specialists and western Connecticut. World Health Organization Classification (WHO) of Pulmonary Hypertension (PH) Group 1 Group 2 Group 3 Group 4 Group 5 Pulmonary Arterial Hypertension (PAH) PH with PH from hypoxia Chronic Thromboembolic Multifactorial • Idiopathic/Familia Left Heart Disease • COPD/emphysema PH (CTEPH) • Hemolytic Anemias • Connective Tissue Disease (Ex. Scleroderma) • Systolic, Diastolic and • Asthma • Sarcoidosis Valvular Heart Failure • Portopulmonary Hypertension • Obstructive Sleep Apnea • Sickle Cell • HIV, Drugs and Toxins • Pulmonary Fibrosis • Systemic Disorders • Congenital Heart Disease 2 Diagnostic Evaluation of PH Patients with possible PH are referred to the Heart failure specialists may be consulted This test, as well as pulmonary function tests, PH program at WMC at varying points in their in cases of WHO group 2 PH from systolic, are performed in the Pulmonary Department evaluation and disease. A thorough assessment diastolic or valvular heart failure. Clear lines and Laboratory at WMC. Sleep studies and includes performing tests to screen for all of referral and communication are necessary overnight oximetry are indicated in patients possible risk factors for PH. Each of these for efficient evaluation and care in a with a history of snoring or hypoxia. This study tests requires coordination with several other comprehensive PH program. may be done at WMC or sometimes in a specialties and departments. Not all of the patient’s home. Clearly defining a possible suggested tests are indicated, and some may Functional assessment with a cardiopulmonary WHO 3 aspect of PH is important before PAH have been performed prior to referral. In the exercise tolerance test is used to stratify risk for medications are started, as inappropriate PAH initial comprehensive consultation, prior test adverse outcomes and to distinguish between therapy may worsen shortness of breath. results are obtained and reviewed, and a plan cardiac and pulmonary limitations of exercise. for further testing is devised. The initial consultation of a patient referred for PH usually starts in the outpatient cardiology office. The initial visit includes resting oximetry, ECG and a six-minute walk test. At subsequent visits, if familial PAH is suspected, genetic counseling and testing may be done to evaluate for mutations such as BMPR2 and ALK-1 PAH. Several other specialists familiar with PH are essential in the initial evaluation and subsequent treatment. Referrals are often made to different departments at WMC, including: pulmonary (WHO group 3 PH from COPD, asthma, OSA, lung disease), infectious disease in PH-HIV, adult congenital heart disease, and hepatology in portopulmonary HTN (liver transplant may improve outcomes). 3 Testing Non-invasive Imaging and Testing Invasive Testing Perhaps the most important non-invasive test in PH is echocardiography. When the suspicion for PAH is very high, or when the non-invasive testing The non-invasive cardiology laboratory department, under the direction of is completed and suggests WHO group 1 PAH, then the next essential test Tanya Dutta, MD, has a dedicated PH protocol for estimating pulmonary is a right heart catheterization (RHC). This is performed in the cardiac artery pressure, quantifying right ventricular function, and using bubble catheterization laboratory by invasive cardiologists who follow an estab- contrast echocardiography when indicated to detect intra-cardiac shunts. lished diagnostic protocol. This procedure may involve vasodilator testing Transesophageal echocardiography is sometimes employed in the with nitric oxide, volume loading in cases of suspected WHO 2 PH, and evaluation of possible congenital heart disease. exercise testing. A thorough RHC is important to establish the correct diagnosis and understand the physiology prior to starting treatment. Other cardiac imaging includes cardiac CT and cardiac MRI, both performed to elucidate congenital heart disease, including ASD, VSD, In cases of CTEPH, a pulmonary angiogram may be performed. This test is anomalous pulmonary veins, and other structural heart