PULMONARY ATRESIA SIGNS AND SYMPTOMS
Pulmonary atresia is a congenital heart defect in Cyanosis
which the pulmonary valve, the valve that controls
Rapid Breathing (Tachypnea)
blood flow from the right ventricle of the heart to
Difficulty Feeding
the pulmonary artery, doesn't form properly. This
Fatigue and Weakness
condition obstructs blood flow from the heart to the
Poor Growth
lungs.
Heart Murmur
Clubbing of Fingers and Toes
ATRESIA - is a medical term that means absence of
Syncope (Fainting)
a normal opening.
DIAGNOSTIC TEST:
In some cases, the pulmonary valve is completely
absent, while in others, it's only partially formed.
Prenatal Screening: Anomalies in fetal heart
structure and blood flow patterns may raise
TYPES OF PULMONARY ATRESIA:
suspicion for congenital heart defects like
pulmonary atresia.
Pulmonary atresia with an intact ventricular
septum: In this form of pulmonary atresia, the wall,
Physical Examination: A thorough physical
or septum, between the ventricles remains
examination by a healthcare provider may reveal
complete and intact.
signs such as cyanosis (bluish discoloration of the
skin), abnormal heart sounds (murmurs), and signs
Pulmonary atresia with a ventricular septal defect:
of heart failure.
Is a pulmonary atresia in which there is a hole in the
wall (septum) that separates the two lower
Echocardiography (2D Echo): It can help visualize
chambers (ventricles) of the heart.
the pulmonary valve, pulmonary artery, and other
cardiac structures to identify abnormalities
FACTORS:
associated with pulmonary atresia.
Genetic Factors: There may be a genetic component
Electrocardiogram (ECG or EKG): An
to pulmonary atresia, as it sometimes occurs in
electrocardiogram records the electrical activity of
families with a history of congenital heart defects.
the heart and can help identify abnormal rhythms
However, in many cases, there's no identifiable
or patterns that may be indicative of heart defects.
genetic cause.
Chest X-ray: A chest X-ray may be performed to
Environmental Factors: These factors can include
assess the size and shape of the heart and detect
exposure to certain medications, chemicals, or
any abnormalities, such as enlargement of the heart
toxins, as well as maternal illnesses such as rubella
or fluid accumulation in the lungs.
(German measles) during pregnancy.
Cardiac MRI (Magnetic Resonance Imaging):
Chromosomal Abnormalities: Some cases of
provides detailed images of the heart and blood
pulmonary atresia are associated with chromosomal
vessels and can help evaluate the anatomy and
abnormalities, such as Down syndrome (trisomy 21)
function of the heart in individuals with complex
congenital heart defects like pulmonary atresia.
In many cases, the exact cause of pulmonary atresia
remains unknown. – Idiopathic
MANAGEMENT:
Monitoring and Follow-Up: Regular monitoring by a
pediatric cardiologist is essential to assess the
individual's heart function, growth, and overall
health.
Medications: Certain medications may be Lifestyle Modifications: Maintaining a healthy diet,
prescribed to help manage symptoms and improve staying physically active within recommended
heart function. These may include medications to limits, and avoiding activities or substances that
help the heart pump more effectively (inotropic could strain the heart.
agents)- pang increase ug contractility sa heart.
diuretics to reduce fluid buildup, and medications to Psychosocial Support: Psychosocial support
prevent blood clots (anticoagulants). services, such as counseling and support groups,
can provide emotional support and resources for
Oxygen Therapy: to improve oxygenation and managing the condition.
relieve cyanosis. They monitor oxygen saturation
levels and adjust oxygen therapy as needed to
maintain optimal oxygen levels.
Surgical Repair: Many individuals with pulmonary
atresia require surgical intervention to improve
blood flow to the lungs. The specific surgical
approach depends on the individual's anatomy and
may involve procedures such as:
Shunt Placement: A surgical shunt may be placed to
create a pathway for blood to flow from the heart
to the lungs. This helps increase oxygen levels in the
blood.
Pulmonary Valve Repair or Replacement: In some
cases, surgical repair or replacement of the
pulmonary valve may be necessary to improve
blood flow to the lungs.
Ventricular Septal Defect Closure: If there's a hole
in the wall between the heart's lower chambers
(ventricular septal defect), surgical closure may be
performed to prevent the mixing of oxygen-rich and
oxygen-poor blood.
Heart Transplant: In severe cases where other
treatment options are not effective, heart
transplantation may be considered as a last resort.
