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Pulmonary Hypertension, Right Heart Failure and CTEPH Program

When it comes to identifying and treating the complex causes of pulmonary hypertension and its and affiliated conditions, the Temple Heart & Vascular Institute is the clear choice for your patients. PH patients deserve to have access to the best experts in this condition.

  • The top experts in the field, treating only pulmonary hypertension
  • Pioneering echocardiogram use
  • Pinpoint patient assessment can eliminate unnecessary procedures, tests
  • Physicians whose expertise and thought leadership are recognized worldwide

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TEMPLE MANAGES THE TOUGHEST PH CASES

Temple Cardiology’s pulmonary hypertension team is 100% devoted to PH. This makes an undeniable difference in patient outcomes because:

  • PH has many potential underlying causes — and diagnosis can be complex.
  • We excel in pinpointing the cause, stage, complications, and comorbidities of PH.
  • By zeroing in on one disease area, Temple provides faster and better diagnosis.
  • Temple’s expert diagnoses result in optimum treatment plans and better outcomes.
  • Our team’s proven expertise has earned national designation as a Pulmonary Hypertension Association Center of Comprehensive Care.

Pregnancy and Pulmonary Arterial Hypertension

Our team is the only program in published literature with 100% maternal and fetal survival rate with multidisciplinary care focused on optimized RV physiologic reserve to survive pregnancy and delivery.

IMMEDIATE INSIGHTS FROM THE ECHOCARDIOGRAM

Paul R. Forfia, MD and Anjali Vaidya, MD, Professors of Medicine and Co-Directors of the Pulmonary Hypertension, Right Heart Failure and CTEPH Program at Temple, are recognized worldwide for proving that the basic echocardiogram can be used not only to diagnose PH but also to accurately differentiate various forms of the disease. Temple’s dedicated PH team uses enhanced echo interpretation to tailor diagnostic workups and therapies. Early insight into the nature of PH means many Temple patients are provided with rapid, accurate, non-invasive insights into their PH, which means faster diagnosis and more streamlined use of testing.

< PAH /CTEPH versus non-PAH /CTEPH>

The Temple team can quickly distinguish between PH with origins in the lung vasculature (i.e., pulmonary arterial hypertension or CTEPH) and PH caused by forces outside the lung (e.g., left-sided heart disease). This distinction is critical in selecting appropriate therapy. For example, newer therapies, such as prostacyclins and endothelin-receptor blockers approved for PAH might actually worsen PH due to other conditions, such as left heart disease or hypoxemia associated with COPD.

Surgical Room

 

We employ state-of-the-art right heart catheterization combined with cardiopulmonary exercise testing (so-called invasive CPET) to uncover complex cases of dyspnea. This approach can pinpoint the cause of a patient’s dyspnea that has eluded diagnosis for years. We are one of the only programs in the United States that have the expertise in conducting and interpreting such studies.

OUR TEAM’S INTENSIVE METHOD DRIVES EXCELLENT PATIENT OUTCOMES

We employ state-of-the-art right heart catheterization combined with cardiopulmonary exercise testing (so-called invasive CPET) to uncover complex cases of dyspnea. This approach can pinpoint the cause of a patient’s dyspnea that has eluded diagnosis for years. We are one of the only programs in the United States that have the expertise in conducting and interpreting such studies.

PH is a complex disease with many possible underlying causes and affiliated conditions. Optimal treatment depends on understanding the exact cause, consideration of potential contributing factors, and an ability to distinguish between cause and effect when confronting overlapping pathologies.

  • The Temple Cardiology team begins with a thorough history, physical examination, and routine tests.
  • Noninvasive testing with echocardiography is used to confirm a problem (with novel and innovative echo-Doppler techniques) AND distinguish between PH related to pulmonary arterial vascular disease and PH related to other problems. This enhanced up-front assessment based on an in-depth reading of the echocardiogram leads to a tailored diagnostic and therapeutic workup for each patient.
  • Cardiac catheterization is required in some cases to confirm the diagnosis and gauge other aspects of the condition. The catheterization/electrophysiology laboratories at Temple are among the newest and best equipped in the region. We perform about 4,000 procedures every year.
  • Our program offers state-of-the-art, highly specialized exercise physiology testing to help diagnose pulmonary hypertension as well as assist in the diagnosis of more complex causes of shortness of breath. Our exercise physiology testing combines noninvasive assessments and/or right heart catheterization tests with supine bicycle exercise +/- gas exchange testing to elucidate the origin and associated risks of the cardiopulmonary problem.
  • Our unique capabilities provide deep insights to the cause of patients’ shortness of breath. This is critical to confirming a diagnosis and determining a personalized treatment plan.
  • Other tests we might conduct to pinpoint diagnosis, rule out other conditions, assess patient function, or fully characterize the disease include: ventilation-perfusion (V/Q) scan, CT scan, polysomnography (sleep tests with oximetry), and pulmonary angiography.

WE TAILOR OPTIONS TO YOUR PATIENTS

Over the past 25 years, a variety of pharmacologic, molecular, and surgical therapies have improved outcomes for PH patients. Decisions on when and how to use specific therapies—for which PH type, at what severity, with which comorbidities or risk factors, and in what sequence or combination—are complex. Our clinicians in the Temple Pulmonary Hypertension, Right Heart Failure and CTEPH Program have extensive experience in managing therapy for diverse PH patients—even those with a complex or borderline disease that does not fit easily into any standard treatment algorithm or that requires a novel or investigational treatment.

MANAGEMENT OF PH MEDICATIONS

Medications for PH are complex. The logistical and clinical hurdles include: phone calls to insurance companies, preauthorizations, specialty pharmacy policies, black box warnings, modern nebulizers, IV and subcutaneous infusion requirements, catheter care, constantly changing guidelines, and the need for appropriate follow-up monitoring. Temple Cardiology PH specialists have extensive experience in all the steps required to initiate and monitor PH medical therapy.

• Our nurse coordinators and administrators help to ensure that patients begin appropriate therapy without delay and with insurer approvals.

• We get patients started on the right track with the proper preparation and administration of their inhaled or infused anti-PH agents.

Our whole Temple Cardiology PH team works in conjunction with referring physicians to monitor therapy results over the long-term, making adjustments as needed.

 

MEDICAL THERAPIES

Basic medical therapies for PH support and symptom relief

  • Diuretics, anticoagulation, supplemental oxygen
  • Lifestyle counseling, including diet and exercise

Agents targeted for underlying disease states

  • Optimization of medical management of underlying heart or lung disease

PH-specific therapies

  • Oral agents: endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulator, prostacylin analogues
  • Inhaled therapies: prostacyclin analogues
  • Parenteral (intravenous or subcutaneous) pump-infused therapies: prostacyclin analogues

Combination therapies

  • • Multidrug treatment drawing from approved classes

SURGICAL & PROCEDURAL THERAPIES

Medications can reduce symptoms or slow progression of PH but won’t always halt disease progression. If the condition worsens, Temple Cardiology PH specialists can advise on cardiac or lung surgery or plan for transplantation.

Pulmonary thromboendarterectomy (PTE)
Temple has performed more than 350 PTEs with 100% survival rate in the last year (2021-2022), driven by the unique experience and specialized training of our multidisciplinary CTEPH team.


Balloon pulmonary angioplasty (BPA)
Temple has performed more than 250 BPAs with improvement in hemodynamics, functional capacity, and oxygenation.


Transplants
Temple provides access to heart, lung, or heart-lung transplantation for patients with severe PH, having performed over 1000 heart transplants since 1984 and 300 lung transplants since 1994.


Other advanced options

  • Percutaneous RVAD and LVAD mechanical circulatory support
  • Durable LVAD mechanical circulatory support
  • Mechanical circulatory assist
  • Extracorporeal Membranous Oxygenation (VA or VV ECMO)

Please call 215-707-8484 or email PHapptRequest@tuhs.temple.edu to arrange for an immediate virtual or in-office consultation with the Temple Pulmonary Hypertension, Right Heart Failure and CTEPH program.

 

WHEN TO REFER

Temple Cardiology specializes in prompt recognition and appropriate treatment to markedly improve PH prognosis. Certain patients with long-standing or advanced disease often benefit from a re-evaluation and change of therapy. Patients ideal for referral to Temple Cardiology include:

PATIENTS WITH KNOWN OR SUSPECTED PH AND SYMPTOMS (E.G., SHORTNESS OF BREATH, CHEST DISCOMFORT, EXERTIONAL SYNCOPE), ESPECIALLY THOSE WITH:

  • Conditions associated with pulmonary arterial hypertension (e.g., HIV infection, connective tissue disease, portal hypertension)
  • A history of acute or chronic pulmonary embolism
  • A history of dasatinib or methamphetamine use
  • Past or present congenital heart disease (e.g., ASD, VSD)
  • Past or present valvular heart disease
  • Out-of-proportion’ PH in left heart disease or chronic respiratory disease
  • Dyspnea of unclear or multifactorial cause

PATIENTS WITH KNOWN PH WHO REQUIRE ASSISTANCE WITH NEW OR ONGOING MEDICAL MANAGEMENT, INCLUDING THOSE WITH:

  • Newly diagnosed disease
  • Progressive disease
  • Disease unresponsive to current therapy

PATIENTS REQUIRING CONSULTATION ON APPROPRIATENESS AND TIMING OF POSSIBLE SURGERY OR PROCEDURES FOR PH OR RELATED HEART AND/OR LUNG DISEASES, INCLUDING:

  • Acute pulmonary embolism and chronic thromboembolic PH (CTEPH) (e.g., Pulmonary Embolectomy or Pulmonary Thromboendarterectomy [PTE])
  • Heart failure (e.g., transplantation, mechanical circulatory devices, pacemakers)
  • Congenital heart defects (e.g., closure of ASD, VSD)
  • Valvular heart disease complicated by PH (e.g., repair or replacement), including TAVR, MitraClip, and other advanced valvular interventions

 

OUR COMMITMENT TO PHYSICIANS

After referral, the Temple team can manage all aspects of PH patient care. We also keep physicians in the loop with clear and timely patient assessments. We routinely call physicians and send detailed status reports to ensure that they remain part of the patient’s care team. Easy accessibility and open communication are key to good long-term outcomes. Our group also reaches out to the region’s cardiopulmonary community with regular PH educational sessions and guideline updates.


A solid partnership between Temple’s PH specialists and the patient’s main cardiac and pulmonary providers is required for optimal care. Our program has a clear track record of fostering such team-based care.

TEMPLE CARDIOLOGY PULMONARY HYPERTENSION, RIGHT HEART FAILURE AND CTEPH PROGRAM

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