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Primary pulmonary hypertension (PPH) is increased pressure in the pulmonary arteries, the arteries carry blood from your heart to your lungs to pick up oxygen. Your right ventricle, the lower right chamber of your heart, pumps blood to your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen and then is pumped throughout of your body.
Pulmonary hypertension (PH) is caused by changes in the cells that line your lungs’ arteries. This process includes three types of changes that affect the pulmonary arteries:
• The walls of the arteries tighten.
• The walls of the arteries are stiff (at birth or
become stiff from an overgrowth of cells).
• Blood clots form in the arteries.
Any or all of these changes make it hard for the heart to push blood through the arteries and into the lungs. As a result, the blood pressure in the arteries rises and PPH occurs. Eventually, your heart may become so weak that it can’t pump enough blood to the lungs. This leads to heart failure.
Symptoms of Pulmonary Hypertension
The symptoms of pulmonary hypertension may not be apparent until the illness is in advanced stages. It is vitally important to identify them once they appear, the sooner treatment is started the better the outcome. Symptoms of PH include:
• Shortness of breath
• Chest pain (also called angina pectoris)
• Dizziness
• Fainting (also called syncope)
• Loss of energy
• Swelling of the arms, legs, ankles or abdomen
(also called edema)
• Dry cough
• Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold)
Causes of Pulmonary Hypertension
Many different conditions can cause pulmonary hypertension. In different ways, each of these illnesses may lead to high blood pressure in the pulmonary arteries:
• Congestive heart failure
• Autoimmune diseases (lupus, scleroderma, and rheumatoid arthritis)
• Venous thromboembolic disease (blood clots in the lungs)
• Human immunodeficiency virus infection – HIV
• Illegal drug use (cocaine, methamphetamine)
• Cirrhosis of the liver
• Appetite suppressant medications (fenfluramine, dexfenfluramine, diethylpropion), these are no longer available in the U.S.
• Heart shunts (abnormal blood flow between heart chambers)
• Chronic lung disease (emphysema, chronic bronchitis, or pulmonary fibrosis)
• Obstructive sleep apnea
When no cause can be identified after testing, the condition is called idiopathic pulmonary arterial hypertension. This condition was formerly known as primary pulmonary hypertension.
Diagnosis of Pulmonary Hypertension
When people seek medical attention for shortness of breath with undiagnosed pulmonary hypertension the doctor will often seek the source of the symptom through a detailed medical history and diagnostic testing. Diagnostic tests that help in the diagnosis of Pulmonary Hypertension are:
Echocardiogram: An ultrasound picture of the beating heart. An echocardiogram can estimate pulmonary artery pressures and check the function of the heart’s right and left ventricles.
Computed tomography (CT Scan): Detailed X-ray films of the chest provided by a CT scan may show enlarged pulmonary arteries and presence of clots. A CT scan may also identify other problems in the lungs that could cause shortness of breath.
Ventilation-perfusion scan (V/Q scan): A nuclear medicine test that can help identify blood clots in the lungs (pulmonary emboli), one cause of pulmonary hypertension.
Electrocardiogram (ECG/EKG): An electrical tracing of the heart’s activity. ECG may show strain on the right side of the heart, which suggests pulmonary hypertension.
These tests can suggest pulmonary hypertension, but to make the final diagnosis, the blood pressure in the pulmonary arteries must be measured directly. This is done through a procedure called right heart catheterization. While right heart catheterization is an invasive test it holds a low risk of complications and is routinely performed.
Treatment of Pulmonary Hypertension
In most patients, pulmonary hypertension has an identifiable cause. In such cases, finding and treating the underlying cause is the best option and leads to a promising prognosis.
Those requiring medication to directly lower the blood pressure in the pulmonary arteries often find symptom improvement and these medication may prevent the progression of pulmonary hypertension. Medications commonly used:
• Prostanoids, including epoprostenol, treprostinil or iloprost
• Endothelin receptor antagonists, such as bosentan and ambrisentan
• Phosphodiesterase inhibitors, like sildenafil
In people with severe advanced pulmonary hypertension, for whom drug therapy hasn’t worked, other treatment options are available:
• Lung transplantation: An organ donor’s lungs and pulmonary arteries are transplanted into someone with severe pulmonary hypertension.
• Atrial septostomy: A surgeon creates an opening between chambers on the right and left sides of the heart. Atrial septostomy improves pulmonary hypertension, but can cause side effects.
What to Expect With Pulmonary Hypertension
The severity, treatment and prognosis of pulmonary hypertension varies significantly depending of its cause. From an incidental finding on a test done for other reasons to a life threatening disease with very limited treatment options and a poor survival rate. Therefore, it is crucial to obtain an accurate diagnosis of the underlying conditions responsible for the pulmonary hypertension. Some conditions that cause pulmonary hypertension may improve over time with treatment of the underlying cause.
Idiopathic pulmonary arterial hypertension (primary pulmonary hypertension) is a progressive condition. Symptoms of this form of pulmonary hypertension steadily worsen with time. While there is no cure, there have been advances in treatment options which may reduce symptoms and slow down the progression of the disease.
While Pulmonary Hypertension is a scary disease, treatment options have advanced making it possible for a person coping with this disease to control symptoms, extend life and dramatically improve the quality of life. It is important to seek treatment if you suspect you may have symptoms of Pulmonary Hypertension. The sooner treatment is begun the more promising the prognosis. Although there’s not a cure, treatments can extend life, reduce symptoms and improve the quality of life for those experiencing primary pulmonary hypertension.
Dr. Andres F. Ruiz is a Fellowship-trained and Board Certified Interventional Cardiologist who specializes in the diagnosis and treatment of cardiovascular disease. In addition to a comprehensive
evaluation, full diagnostic imaging is done in his office, followed by an office meeting to carefully structure a treatment plan unique to each patient’s pathology.
To schedule a consultation, call 561-336-3031.
Andres F. Ruiz, MD
709 South Federal Highway, Suite 3
Boynton Beach, FL 33435
