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Pulmonary Arterial Hypertension 

What is Pulmonary Arterial Hypertension (PAH)?

It is the excessive and abnormal increase in pressure in the pulmonary arteries (blood vessels that carry blood from the heart to the lungs). PAH is considered to exist when the mean pulmonary artery pressure at rest is greater than 25 mmHg or 30 mmHg during exercise.

How is it produced?

 

The pulmonary artery is the blood vessel responsible for carrying blood from the heart to the lungs for oxygenation. Throughout its trajectory, this artery divides into smaller and smaller vessels called arterioles and capillaries, depending on their diameter. Due to an alteration of the endothelial cells that form the walls of the pulmonary arterioles, a thickening of the same occurs and, therefore, a narrowing of its lumen. This narrowing makes it difficult for blood to reach the pulmonary capillaries for gas exchange. The blood will not be sufficiently oxygenated and therefore the amount of oxygen distributed by the body will be deficient, causing dyspnea (suffocation sensation) with effort, among other things.

This increase in pressure within the pulmonary artery will also have its effects at the cardiac level. The right ventricle, to try to adapt to this high pressure of the pulmonary artery, will thicken its walls and will dilate progressively.

types of PAHs

According to the cause it is divided into:

  • Idiopathic PAH: unknown cause
  • Hereditary PAH: genetically transmitted within the same family by mutation of a certain gene
  • Drug-induced or toxic PAH: due to the consumption of toxic substances (denatured rapeseed oil), drugs (Amphetamines, Methamphetamines, Cocaine, Chemotherapeutic Agents) or certain weight-loss medications (Aminorex, Fenfluramine, Desfenfluramine)
  • PAH associated with other diseases: connective tissue, viral (HIV) or liver diseases
  • Persistent PAH of the newborn: due to problems during pregnancy or birth
Symptom

 

They are related to the difficulty that the heart presents to provide sufficient blood flow to the different organs.

In the initial phases of the disease there will be:

  • Dyspnea: feeling of shortness of breath, especially during exertion, although it can occur at rest. The severity of dyspnea is related to the prognosis of the disease; if the sensation of choking occurs at rest or with little movement, the prognosis is worse.
  • Fatigue: due to the lack of oxygenation of the different tissues of the organism
  • Chest pain: feeling of oppression in the center of the chest. It does not move to other areas.
  • Syncope: temporary loss of consciousness occurs with exertion or standing up
  • Cough: unknown cause

As the disease progresses, signs and symptoms characteristic of right heart failure may appear.

 

  • Edema in the lower limbs: as the heart cannot pump blood normally, fluid retention occurs in the legs.
  • Ascites: this accumulation of fluid can also occur in the abdomen
  • Jugular venous venous enlargement: the veins in the neck (jugulares) become enlarged
  • Cyanosis: due to lack of oxygen the lips and skin turn bluish
  • Clubbing: thickened fingers and toes

 

Until the PAH does not take a time of evolution neither the signs nor the symptoms appear. As these are common to other cardiac and respiratory pathologies, their diagnosis is considerably delayed and difficult.

suffocation
Who is affected?

The incidence is higher in people between the ages of 30 and 50 and, for an unknown cause, in women of childbearing age, although it can affect people of any age, race, or ethnicity.

How many people suffer from it?

It is a serious disease included in the group of rare diseases. In Spain about 800 people suffer from it. In Europe, for every 100.000 inhabitants there are between 1.5 and 5.2 cases of PAH.

Diagnosis

The diagnosis, in the presence of signs and symptoms that are not due to other pathologies and that may lead to suspicion of PAH, should be made by a specialist (pulmonologist, internist or cardiologist). In several Spanish hospitals there are specific units for the diagnosis and treatment of this pathology.

To do this, a detailed medical history must be prepared in which all the necessary information on symptoms and previous diseases and family history is collected. A thorough physical examination will be performed where a cardiopulmonary auscultation will be performed and specific signs of PAH will be sought. Different diagnostic tests can be performed such as:

  • Electrocardiogram: Measures the electrical activity of the heart.
  • Chest x-ray: To look for morphological and size changes of the heart, lungs and internal structures of the thoracic cavity.
  • Echocardiogram: Detects if there is increased pressure in the pulmonary artery
  • Blood tests: To obtain data on the state and functioning of the body and detect diseases that can lead to PAH.
  • Pulmonary hemodynamic study and acute vasodilator test (right heart catheterization): It is used to confirm the diagnosis and verify the effectiveness of treatment. It allows to know and analyze the real pressures of the pulmonary circulation and the cardiac output (volume of blood that the right ventricle ejects every minute).
  • Pulmonary angiography: Same as catheterization but with contrast.
  • Ventilation/perfusion scintigraphy: It is used to check what problems exist in the respiratory tract and the defects present in the pulmonary circulation.
  • High-resolution Computed Axial Tomography (TACAR): Radiological test with contrast that allows to study the lungs and pulmonary arteries with precision.
  • Magnetic resonance: To assess the morphology, dimensions and function of the right ventricle and pulmonary arteries.
  • Pulmonary function tests:
  • Arterial blood gases: Extraction of arterial blood to know exactly what the oxygen supply to the body is.
  • pulse oximetry: Measure oxygen saturation.
  • Forced Spirometry: Measures the volume of air that the lungs can move in a given time.
  • Diffusing Capacity of Carbon Monoxide (DLCO): Allows us to know how gas exchange is taking place in the pulmonary alveoli.
  • plethymography: Measures lung volumes that are not mobilized during inspiration.
  • Tests to assess exercise capacity:
  • 6 minute walk test
  • Stress Test

Functional classification of PAH

According to the WHO and the NYHA, the functional classification of PAH is:

  • Class I: Without limitation to physical activity. Ordinary physical activity does not cause any symptoms
  • Class II: Slight limitation to physical activity. Ordinary physical activity produces symptoms. At rest there is no limitation.
  • Class III: Marked limitation to physical activity. Ordinary physical activity produces symptoms. At rest there is no limitation.
  • Class IV: Inability to perform any physical activity without presenting symptoms. Appearance of signs of Right Heart Failure and syncope. Dyspnea appears at rest. All symptoms worsen and increase with any physical activity.

Treatment

Although there is still no known cure, there are different treatment options that help control the disease.

The goals of treatment in PAH are:

  • Treat the cause, if known.
  • Reduce symptoms and improve quality of life
  • Decrease the development of blood clots and reduce coronary work by increasing the supply of blood and oxygen to the heart.

PAH treatment is divided into:

  • Pharmacological
  • support medications
  • Anticoagulants: Prevent the appearance of clots
  • Diuretics: Help reduce edema by facilitating the elimination of liquids
  • Digitalis: Improve heart contraction
  • Specific medications:
  • Calcium antagonists: Help dilate the pulmonary veins and arteries
  • Endothelin receptor antagonists: Produce dilation of the pulmonary vessels and decreases pulmonary pressure.
  • Phosphodiesterase type 5 inhibitors: Relax the pulmonary arteries and lower lung pressure.
  • Prostacyclins: Vasodilator.
  • Oxygen
  • Surgical:
  • Atrial septostomy: It consists of opening a hole in the septum that separates both atria in the heart to relieve symptoms, but does not cure the disease
  • Lung or cardiopulmonary transplant

All people with PAH must have an individualized treatment plan. The treatment must be followed meticulously since if it is not done in this way it will worsen.

Life habits

Patients with PAH should follow healthy lifestyle habits such as those indicated below:

  • Perform physical exercise: It must be started gradually, with a frequency of 2 times a week until you achieve your daily tolerance. It will begin with about 30 minutes a day, which can be divided into different sessions, which will increase depending on tolerance. The ideal intensity to work at is between 4-6 fatigue on a scale of 0 to 10, where 0 is no fatigue and 10 is maximum fatigue. The exercises to perform are:
  • Global aerobic exercises: Low to medium intensity but long lasting where all the muscles of the body are put into operation. Their intensity will be considered based on the functional class to which the patient belongs due to his PAH.
  • Muscle strength exercises: They serve to gain strength in specific muscles. They complement the previous ones.
  • Muscle stretching: They are essential, they must be done before and after any exercise.
  • Avoid salt: To reduce and prevent fluid retention.
  • Follow a balanced diet: Both obesity and malnutrition impair the treatment of the disease.
  • constipation prevention
  • Protection against infections:
  • Regular hand washing
  • optimal oral hygiene
  • Influenza and pneumococcal vaccination

When to go to the emergency room?

If chest pain suddenly appears, palpitations, fainting, high fever, swollen feet and/or difficulty breathing, you should go to the nearest hospital center without hesitation, with your own up-to-date therapeutic plan and the corresponding medication. Many hospitals do not have it due to its specificity.

More Information

SEPARATE. Controlling the HPA: http://www.separ.es/biblioteca-1/bibliotecaparatodos


Associations and Foundations in Spain

Pulmonary Hypertension Spain – C/ Riera de la Salut, 7 – CC Les Tovalloles 08980 Sant Feliu de Llobregat (Barcelona) Telephones: 688 931 858 / 688 941 858 Contact: treasury@hpe-org.com Website: www.hypertension-pulmonary.com

Foundation Against Pulmonary Hypertension (FCHP) – C/ Pablo Neruda, 3928981 Parla (Madrid) Telephone: 91 128 05 02/ 91 128 88 57 Contact: elena@fchp.es Website: www.fchp.es

National Association of Pulmonary Hypertension – Av. de las Artes, 728300 Aranjuez (Madrid) Telephones: 685 454 351/ 685 454 349 Contact: information@hypertensionpulmonary.es Website: www.hypertensionpulmonar.es

Pulmonary hypertension blog of the AAHP (Associació d'afectats per Hipertensió Pulmonar) – Telephone: 608 440 044 Contact: acumeres@gmail.com; hipertensio_pulmonary@yahoo.es Website: www.aahp.es