Pulmonary heart disease (Cor pulmonale)
Pulmonary heart disease, also known as cor pulmonale, is a serious condition characterized by the failure of the right side of the heart due to high blood pressure in the pulmonary arteries, a condition referred to as pulmonary hypertension. This increased pressure places significant strain on the right ventricle, ultimately weakening the heart and leading to its failure if left untreated. Symptoms often mimic those experienced after physical exertion, including shortness of breath, elevated heart rate, and fatigue, but may also escalate to more severe signs such as chest pain, coughing, and swelling in the extremities.
Common underlying causes of pulmonary hypertension include chronic obstructive pulmonary disease (COPD), autoimmune disorders, cystic fibrosis, and interstitial lung diseases. Diagnosis typically involves a thorough medical examination, blood tests, and imaging studies to assess heart function and rule out other conditions. Management focuses on treating the underlying causes of pulmonary hypertension and may involve medications, lifestyle changes, and, in advanced cases, oxygen therapy or surgery. It is crucial for individuals with pulmonary heart disease to avoid strenuous activities and adopt a heart-healthy lifestyle to mitigate risks and improve overall health.
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Pulmonary heart disease (Cor pulmonale)
Pulmonary heart disease, also called cor pulmonale or right-sided heart failure, is a type of heart disease, or cardiovascular disease. Heart disease encompasses a range of medical issues affecting the heart and blood vessels; some of these can be very serious. Pulmonary heart disease is caused by high blood pressure, also called pulmonary hypertension or pulmonary arterial hypertension. High blood pressure puts a strain on a chamber of the heart known as the right ventricle, which causes the heart to weaken and eventually fail. If left untreated, pulmonary heart disease can be life-threatening. Treatment for the condition includes managing pulmonary hypertension with medication and adopting healthy lifestyle changes.


Background
Pulmonary hypertension is the most common cause of pulmonary heart disease. Pulmonary hypertension causes the blood vessels of the lungs to constrict or become blocked, which in turn causes increased pressure in the right ventricle of the heart. This pressure makes it difficult for the right side of the heart to pump and transport blood to the lungs. As this condition continues, it strains the heart and eventually causes pulmonary heart disease.
People may not recognize the signs indicative of pulmonary heart disease because they sometimes mimic the symptoms felt after exercise. Signs include elevated heart rate, light-headedness, tiredness, and shortness of breath. These usually occur during physical activity, however, they may be present when a person is at rest. Over time, individuals with pulmonary heart disease may have more severe symptoms, which include coughing; fainting; pain in the chest or chest wall; fatigue; wheezing; swelling of the ankles, feet, or legs; and cyanosis (bluish discoloration) of the fingers and lips.
Several other medical conditions can cause high blood pressure, which in turn can trigger pulmonary heart disease. While most of these do not have a cure, they can be managed with specific treatments to lessen a person's chances of developing pulmonary heart disease related to these disorders.
Chronic obstructive pulmonary disease (COPD) is an incurable lung disease that makes it difficult for a person to breathe. Two forms of COPD exist: chronic bronchitis and emphysema. Chronic bronchitis is the constant inflammation of the lining of the bronchial tubes that lead to the lungs, while emphysema causes the lungs to become stretched. No cure exists for COPD, but it can be managed with lifestyle changes, medications, and supplemental oxygen, all of which can help prevent pulmonary heart disease.
Autoimmune disorders are conditions in which the immune system mistakenly attacks and destroys healthy cells and tissues in the body. Numerous autoimmune disorders exist, but no known cause or cure has been found for them. Symptoms can be managed with medications, supplements, blood transfusions and physical therapy. By managing symptoms, individuals can protect their hearts and lungs, prevent pulmonary hypertension, and in turn lessen their chances of developing pulmonary heart disease.
Cystic fibrosis (CF) is an inherited disorder that affects the lungs and digestive tract. No cure exists for the serious condition. It is life-threatening, and most people with the condition do not survive to age forty. Medications, lung treatments, and lung transplantation can prevent other serious medical conditions associated with CF such as pulmonary heart disease; these may even improve the outlook for some individuals living with CF.
Lung diseases, known collectively as interstitial lung disease, can affect a person's ability to get enough oxygen, which can cause pulmonary hypertension. These lung conditions affect the tissues and area around the lung's air sacs, called interstitium, and lead to inflammation and scarring, which stiffens the lungs and causes breathing difficulty. Management of the disease includes medication, breathing treatments, and in severe cases, lung transplantation.
Obstructive sleep apnea occurs when an individual stops breathing during sleep. This is usually caused by airway inflammation and can lead to heart disease. Kyphoscoliosis, a type of scoliosis that causes a severe curvature of the upper spine, can cause breathing issues and lead to high blood pressure, among other issues. Blood clots of the lungs also may trigger pulmonary hypertension. Sometimes the blood vessels of the lungs constrict for no medical reason (called idiopathic) and lead to pulmonary heart disease.
Diagnosis and Treatment
A physician can diagnose pulmonary heart disease by performing a physical exam and through testing. The heart is checked for abnormal rhythms and fluid retention, and the neck is examined for visible neck veins. Blood tests are performed to check antibody levels and to determine the presence of brain natriuretic peptide, an amino acid secreted by the heart when it is not functioning properly or is overstressed. Other tests may include chest X-ray, lung scan, echocardiogram (an ultrasound to examine heart function), computed tomography (CT) scan, and lung biopsy to test for damaged tissue.
Pulmonary heart disease is managed by treating pulmonary hypertension. Medications can help regulate blood pressure and increase oxygen flow to the lungs. Diuretics can decrease the amount of fluid retained by the heart and lungs and can reduce sodium levels in the blood. Blood thinners can help reduce the chance of developing blood clots. Advanced cases of pulmonary heart disease that do not respond to medication may require oxygen therapy and, in severe cases, heart or lung transplant.
People with pulmonary heart disease should avoid any activities that stress the heart and lungs, such as heavy lifting and rigorous physical activity. Women should refrain from getting pregnant unless under the supervision of a physician. Individuals should not travel to high altitudes, which can make breathing more difficult. They should avoid smoking, drinking alcohol or caffeine, and taking drugs. In addition, individuals should adopt a lifestyle that includes exercise, stress reduction, weight loss, and a healthy diet.
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