Pulmonary Hypertension


Pulmonary Hypertension is a rare but serious condition characterized by high blood pressure in the arteries that supply the lungs (the pulmonary arteries). Over time, this increased pressure can strain the heart, especially the right ventricle, leading to heart failure and other complications. It can affect people of all ages and is often associated with other heart or lung diseases, but in some cases, it occurs without a known cause.


Symptoms

Symptoms of Pulmonary Hypertension include:

  • Shortness of breath during physical activity
  • Fatigue
  • Chest pain or tightness
  • Dizziness or fainting (especially during exertion)
  • Swelling in the legs, ankles, or abdomen (edema)
  • Blue lips or skin (in severe cases)
  • Rapid heartbeat or palpitations

Types of Pulmonary Hypertension

Pulmonary Hypertension is classified into five groups based on its underlying causes:

  • Group 1 (PAH - Pulmonary Arterial Hypertension): Caused by narrowing of the small pulmonary arteries, may be idiopathic or genetic.
  • Group 2: Due to left heart disease (e.g., heart failure or valve problems).
  • Group 3: Due to lung diseases (e.g., COPD, interstitial lung disease) or low oxygen levels.
  • Group 4: Caused by chronic blood clots in the lungs (chronic thromboembolic pulmonary hypertension, CTEPH).
  • Group 5: Due to other systemic diseases (e.g., sarcoidosis, blood disorders).

Causes

Hypertension can be caused by:

  • Genetic mutations (especially in idiopathic PAH)
  • Heart diseases that affect the left side of the heart
  • Chronic lung diseases or sleep apnea
  • Blood clots in the lungs
  • Autoimmune diseases (e.g., lupus, scleroderma)

Prevention and Care

some forms of Pulmonary Hypertension cannot be prevented, early diagnosis and treatment can improve outcomes. Care strategies include:

  • Medications to relax blood vessels, reduce blood pressure, and prevent blood clots
  • Oxygen therapy for those with low oxygen levels
  • Lifestyle changes (e.g., low-sodium diet, avoiding high altitudes, light exercise)
  • Regular monitoring by a cardiologist or pulmonologist
  • In severe cases, lung or heart-lung transplantation may be considered






Articles


Read the latest articles on Hereditary Epidermolysis Bullosa and related topics

Pulmonary Hypertension

Pulmonary hypertension encompasses a heterogeneous group of disorders with the common feature of elevated pulmonary artery pressures. Patients often present with worsening dyspnea on exertion, with signs of right heart strain or failure. Diagnosis requires a high suspicion index and extensive testing to confirm the condition and determine the underlying etiology. Management is based on treating the underlying cause and alleviating the symptoms. Interventional and surgical options are available for chronic thromboembolic pulmonary hypertension. Lung transplantation may be considered for patients not responding to medical therapy.

Pulmonary Hypertension: Pharmacological and Non-Pharmacological Therapies

Pulmonary hypertension (PH) is a severe and chronic disease characterized by increased pulmonary vascular resistance and remodeling, often precipitating right-sided heart dysfunction and death. Although the condition is progressive and incurable, current therapies for the disease focus on multiple different drugs and general supportive therapies to manage symptoms and prolong survival, ranging from medications more specific to pulmonary arterial hypertension (PAH) to exercise training.

Pulmonary Hypertension: A Contemporary Review

Major advances in pulmonary arterial hypertension, pulmonary hypertension (PH) associated with lung disease, and chronic thromboembolic PH cast new light on the pathogenetic mechanisms, epidemiology, diagnostic approach, and therapeutic armamentarium for pulmonary vascular disease. Here, we summarize key basic, translational, and clinical PH reports, emphasizing findings that build on current state-of-the-art research.



Revised Definition of Pulmonary Hypertension and Approach to Management: A Clinical Primer

The definition of pulmonary hypertension (PH) has changed recently based, in part, on contemporary outcome data and to focus on early disease detection. Now, PH includes patients with mean pulmonary artery pressure >20 mm Hg measured by right heart catheterization. In contrast to the classical era, pulmonary vascular resistance >2.0 Wood units is also used for diagnosis and prognostication. These lowered thresholds aim to identify patients early in the disease course, which is important because delay to diagnosis of PH is common and linked to elevated morbidity and shortened lifespan.

Pulmonary Hypertension

Pulmonary hypertension encompasses a heterogeneous group of disorders with the common feature of elevated pulmonary artery pressures. Patients often present with worsening dyspnea on exertion, with signs of right heart strain or failure. Diagnosis requires a high suspicion index and extensive testing to confirm the condition and determine the underlying etiology. Management is based on treating the underlying cause and alleviating the symptoms.

Exercise Capacity and Quality of Life in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease with a high mortality rate that can be divided into different groups according to etiology and prognosis. Few studies have investigated differences in the exercise capacity and quality of life (QOL) among the different groups of PAH patients. Therefore, we aimed to (1) compare the hemodynamic exercise responses between patients with idiopathic pulmonary arterial hypertension (IPAH) and PAH associated with other diseases (APAH), and (2) determine the factors associated with exercise capacity in patients with PAH.








Hospital and Specialists


Dr. Sean P. Pinney, MD

Director of the Pulmonary Hypertension Program at Mount Sinai Medical Center; Associate Professor of Cardiology with numerous publications in cardiovascular disease.

Mount Sinai Medical Center


Past Experiences:

  • Professor of Medicine (Cardiology)
  • The Philip J. and Harriet L. Goodhart Chair of Cardiology
  • Associate Director of the Cardiovascular Clinical Institute at Mount Sinai Morningside

Harrison Farber, MD

Professor of Medicine and Director of the Pulmonary Hypertension Center at Boston University; extensive research in pulmonary vascular biology.

Boston Medical Center


Past Experiences:

  • Medical College of Virginia - Internal Medicine
  • Medical College of Virginia - Internal Medicine Chief Resident
  • Medical College of Virginia - Internal Medicine (Internship)

Dr. Vallerie V. McLaughlin, MD

Director of the Pulmonary Hypertension Program at the University of Michigan; involved in clinical trials for new PAH treatments.

University of Michigan Health System


Past Experiences:

  • Fellow of the American College of Chest Physicians
  • Fellow, American College of Cardiology
  • Fellow, American Heart Association
  • University of Michigan Health System, Internal Medicine, 1992 (Residency)





Patient Groups & Foundations


EB Research Network


The EB Research Network is a collaborative global network of organizations, researchers, and advocates working together to advance research and find treatments for Epidermolysis Bullosa.

Debra of America


Debra of America is a nonprofit organization dedicated to supporting people affected by Epidermolysis Bullosa (EB) through advocacy, research funding, and resources for individuals and families.

Taiwan EB Association


The EB Association Taiwan is dedicated to improving the lives of individuals affected by Epidermolysis Bullosa (EB) in Taiwan through advocacy, support services, and raising awareness of this rare genetic condition.












《餘命十年》 The Last 10 Years


The heroine of The Last 10 Years, Matsuri Takabayashi (played by Nana Komatsu), is diagnosed with Pulmonary Hypertension. At the age of 20, she learns that she only has ten years left to live. Within the limits of what's possible and imaginable, she makes a firm decision not to fall in love and convinces herself to let go of her desire for an ordinary life. However, an invitation to a junior high school reunion leads her to meet Kazuto Manabe (played by Kentaro Sakaguchi), whose life is in disarray. As these two people, now living very different lives, cross paths again—what kind of spark or change will they bring to each other's world?


Main Character
Love Interest
Scenic Moment




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