What is 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.
What are the causes of PAH in children?
Depending on the cause, PAH in children is classified as:
- Persistent Pulmonary Hypertension in the Newborn: Because the pulmonary vascular resistance of the newborn remains high after birth due to an associated disease such as neonatal respiratory distress syndrome, immaturity, lack of surfactant in premature newborns, neonatal infection or suffocation.
- PAH associated with congenital heart disease
- Idiopathic PAH: No known cause
- hereditary PAH
- PAH associated with respiratory diseases
Signos there sintomas
Depending on the age of the child and the evolution of the disease, it will manifest itself in one way or another:
- Infants:
- Fatigue during shots.
- Frequent vomiting.
- Fast breathing.
- Tachycardia.
- Irritability.
- Delay in weight and height.
- Syncope that may resemble seizures.
- Chest pain that can manifest as crying spells.
- Cyanosis: bluish coloration of the skin, mucous membranes and fingers.
- Preschoolers:
- Fatigue.
- syncope
- Older children:
- Dyspnea with exertion.
- Chest pain.
- Dizziness
- Blackouts.
- heart murmurs
- Children over 10 years of age and advanced cases with a long evolution:
- Swollen ankles and legs.
- Hepatomegaly: Enlargement of the liver.
- Increased size of the neck veins.
- Acropaquia or drum fingers: Thickening of the distal phalanges of the fingers.
Diagnosis
To make the diagnosis in children, tests similar to those used in adults will be used (see the Pulmonary Arterial Hypertension section of this website), with some qualifications:
- The 6-minute walk test is performed from the age of 4.
- Respiratory function tests require the child's collaboration, so if this does not occur they cannot be performed.
- Cardiac catheterization with a vasodilator test will be performed with the child sedated or anesthetized, as appropriate.
Treatment
Children affected by PAH, like adults, must have an individualized treatment plan.
Oral, inhaled, subcutaneous and intravenous medications will be used.
Children's PAH on a day-to-day basis
All teachers, monitors, caregivers... who are in contact with the child must know what the disease consists of, the medication schedules, the special care the child needs, what they can and cannot do in relation to their general activity. .
When going on excursions, playing or exercising, the physical limitation of the child must be taken into account and compliance with the prescribed medical treatment.
All children have to be in contact with other children. Parents have to help the child with PAH to know her limits and educate her to avoid risky behaviors and teach her to take responsibility for her behavior.
As in other patients with PAH, annual influenza and pneumococcal vaccination is recommended.
The rest of the recommendations are common to those of adults (See section HAP of this website).
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
