Depending on the cause of a patient's pulmonary hypertension, she/he would typically receive care from pulmonologists, cardiologists, rheumatologists, and/or gastroenterologists. In addition, the PVDP Program has dovetailed its expertise with members of the Adult Congenital Heart Disease Program, the Hereditary Hemorrhagic Telangiectasia Program (HHT), and Pramod Mistry, MD, a renowned expert in Gaucher's Disease. In addition, the Yale Liver Transplant Program relies on the Program's expertise in evaluating and treating transplant candidates with cirrhosis and portopulmonary hypertension. The pulmonary hypertension experts at Yale also closely collaborate with their connective tissue disease specialists that focus on diseases associated with pulmonary hypertension, such as Scleroderma (systemic sclerosis), lupus, Sjogren's disease, myositis, dermatomyositis, or mixed connective tissue diseases. Patients seen in the PVDP program in New Haven, whether they are local (from within Connecticut, on the shoreline, north of Hartford, or east and west of the state) or regional from surrounding states in the northeastern United States (New York, Massachusetts, or Rhode Island) also benefit from the expertise of the Heart Failure team within the Cardiology section at Yale.
We are fully committed to defeating pulmonary hypertension and providing world class services to our patients suffering from pulmonary hypertension, regardless of the cause of their pulmonary hypertension, be it pulmonary arterial hypertension (PAH), pulmonary venous hypertension, chronic thromboembolic pulmonary hypertension, or any other cause of pulmonary hypertension. We provide high quality patient care in our outpatient, inpatient, and emergency services settings. The hospital pharmacists, nurses, and emergency department personnel are well trained and qualified to handle the complex treatments and evaluations of patients who have pulmonary hypertension. Once the anticipated Pulmonary Hypertension Association's Center of Excellence criteria come out, we expect that the PVDP program at Yale to be one of the Pulmonary Hypertension Centers that are designated as a Center of Excellence.
Patients evaluated in the PVDP program at Yale benefit from the availability of multiple clinical trials within the PVDP program that focus on research that advance our understanding of pulmonary hypertension, at the basic and translational level (including for example the role of endothelial cells, inflammatory cells and cytokines), clinical research, and at the advanced clinical management level. The pulmonary hypertension physicians within the PVDP program at Yale, in addition to the program's nurse, Janice Napoletano, are heavily involved in educational activities at the local level in Connecticut and also at the national level, in education and training of patients, their families/care providers, and physicians to help combat pulmonary hypertension.
The PVDP program includes physicians specifically trained and experienced to manage patients with pulmonary hypertension, nurses, coordinators, and researchers. The services provided include:
- Outpatient specialized clinic.
- Designated floors in the hospital for pulmonary hypertension patients.
- State of the art heart catheterization laboratory that is equipped to do elaborate and highly specialized right heart catheterizations (that include vasodilatory challenge tests most commonly using inhaled Nitric Oxide, fluid challenges, and exercise challenges during the right heart catheterization).
- Highly trained and qualified cardiologists that read echocardiograms focusing on the right side of the heart (which is a key clinical factor in patients with pulmonary hypertension).
- Physicians, nurses, pharmacists, and quality-tested systems in place that provide effective complex therapies to patients in a safe environment. Such FDA-approved specific therapies for pulmonary hypertension include:
- Oral therapies: Sildenafil (Revatio®), Tadalafil (Adcirca®), Ambrisentan (Letairis®), Bosentan (Tracleer®), Macitentan (Opsumit®), and Riociguat (Adempas®).
- Inhaled therapies: Treprostinil (Tyvaso®) and Iloprost (Ventavis®).
- Infused therapies (using a pump): Epoprostenol (Flolan® and Veletri®), Treprostinil (Remodulin®).
- Blood tests
- Chest X-ray
- Pulmonary function tests
- Sleep study and/or overnight oximetry study
- Echocardiogram (ultrasound of the heart)
- Six-minute walk test
- Heart catheterization (which is the gold standard for confirming pulmonary hypertension and quantifying its severity)
- Ventilation/Perfusion scan (also called V/Q scan) (looking for blood clots in the lungs)