Understanding Pulmonary Hypertension

What is Pulmonary Hypertension?

Your heart is a pumping system with two primary chambers (known as ventricles). The right side of your heart or right ventricle pumps blood through the lungs via small blood vessels known as arterioles. The lungs add oxygen to the blood which returns to the left side of your heart (left ventricle) where the oxygenated blood is pumped around your body. If the small blood vessels become narrowed or reduced in number it becomes more difficult to get blood from the right ventricle to the left ventricle. As a result, the pressure increases in the lungs and in the right side of your heart. This may cause the side of your right heart to enlarge, as it works harder. This syndrome is called pulmonary hypertension (PH). Pulmonary Hypertension simply refers to elevation of blood pressure in your lungs.

Types

  • Pulmonary arterial hypertension (PAH): This type of PH is caused by the changes in the walls of the small arteries of the lungs which makes the vessels smaller and stiffer
  • Pulmonary venous hypertension (PVH): This type of PH is usually due to problems related to the left side of the heart such as valve disease, congestive heart failure, and cardiomyopathy

Regardless of whether the problem is PAH or PVH the right side of the heart has to work harder to push blood through narrowed arteries in the lungs. Eventually, the extra stress causes the right ventricle to dilate (enlarge) and become less flexible, compromising the heart’s ability to push blood out of the heart, through the lungs, and into the rest of the body.

Causes

Pulmonary hypertension (PAH and PVH) may be caused by many conditions, and in some cases, the case is unknown. The following are some known causes of pulmonary hypertension:

  • Non-modifiable risk factors: Those that are irreversible and cannot be changed
    • Family history/Genetics
  • Modifiable risk factors: Those that you can control or treat
    • Use of cocaine
    • The diet drug fenfluramine/phentermine, also known as "fen-phen"
    • Living in high altitudes
  • Other causes
    • Aortic valve disease
    • Chronic obstructive pulmonary disease “COPD” is a group of lung diseases that block airflow and make it difficult to breathe
    • Congenital heart disease
    • Liver cirrhosis is a disease that occurs when healthy cells in the liver are damaged and replaced by scar tissue, usually as a result of alcohol abuse or chronic hepatitis
    • Connective tissue disorders such as lupus and scleroderma
    • Mitral valve disease
    • Pulmonary fibrosis is a type of lung disease that occurs when lung tissue becomes damaged and scarred
    • Sickle cell disease is a condition in which there aren't enough healthy red blood cells to carry adequate oxygen throughout your body
    • Sleep apnea is a serious sleep disorder in which breathing repeatedly stops and starts

Symptoms

The signs and symptoms of pulmonary hypertension in its early stages might not be noticeable for months or even years. As the disease progresses, symptoms become worse. Pulmonary hypertension symptoms include:

  • Abdominal bloating
  • Shortness of breath during routine activity
  • Fatigue
  • Heart palpitations which may feel like your heart is racing, pounding or fluttering
  • Heart arrhythmias
  • Chest pain
  • Decreased appetite
  • Pain in your right side of the abdomen
  • Rapid heart rate (tachycardia) of more than 100 beats per minute
  • Lightheadedness/Fainting
  • Shortness of breath, initially while exercising or climbing stairs
  • Swelling in your ankles, legs and abdomen
  • Bluish lips or skin (cyanosis)

Diagnosis

Pulmonary hypertension is hard to diagnose early because it's not often detected in a routine physical exam. Even when the condition is more advanced, its signs and symptoms may be attributed to asthma or other lung or heart conditions.

A normal average pulmonary artery pressure is 10-20 mm Hg at rest. If the pressure in the pulmonary artery is greater than 25 mmHg at rest or 30 mmHg during physical activity, it is abnormally high. This is considered “pulmonary hypertension.”

To diagnose your condition, your doctor may review your medical and family history, discuss your signs and symptoms, and conduct a physical examination. Doctors may order several tests to diagnose pulmonary hypertension. These tests include:

  • Diagnostic tests and procedures
    • Blood tests
    • Cardiac magnetic resonance imaging (MRI)
    • Chest x-rays
    • Computed tomography (CT scan)
    • Echocardiogram (ECHO)
    • Electrocardiogram (EKG/ECG)
    • Exercise stress test
    • Genetic testing
    • Lung biopsy
    • Pulmonary function tests
    • Right-heart catherization
    • Sleep study
    • Ventilation/Perfusion (V/Q) scan

Classification

The cause of pulmonary hypertension is classified by the World Health Organization into five groups. The WHO Group classes are:

  • Group 1- pulmonary arterial hypertension: This grouping is caused by:
    • Certain drugs
    • Conditions that affect veins and small blood vessels of the lungs
    • Congenital heart disease
    • Connective tissue disorders such as scleroderma, lupus and others
    • Genetic tests
    • HIV infection
    • Liver disease
    • Sickle cell disease
    • Unknown cause
  • Group 2- pulmonary hypertension caused by left-sided heart disease: This grouping is caused by:
    • Aortic valve disease
    • Cardiomyopathy
    • Congestive heart failure
    • Mitral valve disease
  • Group 3- pulmonary hypertension caused by lung disease: This grouping is caused by:
    • Chronic obstructive pulmonary disease (COPD)
    • Interstitial lung disease
    • Long-term exposure to high altitudes
    • Sleep apnea and other sleep disorders
  • Group 4- pulmonary hypertension caused by chronic blood clots: This grouping is caused by:
    • Chronic blood clots in the lungs or general clotting disorders
  • Group 5- pulmonary hypertension associated with other conditions that have unclear reasons why the pulmonary hypertension occurs: This grouping is caused by:
    • Blood disorders such as polycythemia vera and essential thrombocythemia
    • Metabolic disorders such as thyroid and glycogen storage diseases
    • Systemic disorders such as sarcoidosis and vasculitis
    • Tumors pressing against pulmonary arteries

Patients with pulmonary hypertension are normally classified into 4 symptom-based (functional) classes also described by the World Health Organization. The classes are:

  • Class I: Patients in this category show no limitation of physical activity. Ordinary physical activity does not cause fatigue, palpitation or shortness of breath
  • Class II: Patients in this category show slight limitation of physical activity. No symptoms at rest. Ordinary physical activity results in fatigue, palpitation or shortness of breath
  • Class III: Patients in this category show great limitation of physical activity. No symptoms at rest. Less than ordinary activity causes fatigue, palpitation or shortness of breath
  • Class IV: Patients in this category are unable to carry on any physical activity without discomfort. There are symptoms at rest. If any physical activity is undertaken, discomfort increases

The WHO Group classes and the WHO Functional classifications are not independent of each other. Your doctor will use them together to help decide your most appropriate treatment options.

Treatment

Although medical treatment can't cure pulmonary hypertension, it can lessen symptoms. Lifestyle changes also can help improve your condition. Consider these tips:

  • Lifestyle changes
    • Avoid sitting in a hot tub or sauna or taking long hot baths or showers
    • Avoid smoking
    • Avoid traveling to or living at high altitudes
    • Exercise under the directions of your doctor
    • Eat a heart-healthy diet
    • No more than 7-8 hours of sleep per day
    • If you’re overweight, talk to your doctor about weight-loss options
    • Make and keep appointments to see your doctor for routine check-ups and follow-up tests
  • Medications
    • Antiarrhythmic medications will help control your heart’s rhythm
    • Beta blockers will help reduce your blood pressure
    • Diuretics “water pills” will help reduce the amount of fluid retention in your body
    • Endothelin receptor antagonists will help lower pulmonary pressures
    • Phosphodiesterase inhibitors will help the vessels produce natural vasodilators
    • Prostacyclins will serve as another type of vasodilator to help with the widening of the blood vessels
    • Soluble guanylate cyclase stimulators will help the vessels relax
  • Medical and Surgical procedures
    • Heart transplantation
    • Heart-lung transplantation
    • Pulmonary thromboendarterectomy
    • Repair of mitral and aortic valve disease

Which of our locations treat this disease?

Pulmonary hypertension care is delivered at each of our medical centers, with centers of excellence being provided at Newark Beth Israel Medical Center, Newark and Robert Wood Johnson University Hospital, New Brunswick.

To request an appointment with one of New Jersey’s top cardiac specialists, visit rwjbh.org/heart.

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