INTERRUPTED AORTIC ARCH

The primary blood channel that transports oxygen-rich blood from the heart to the body, the aorta, is not continuous in individuals with an interrupted aortic arch (IAA), a rare congenital cardiac abnormality. Instead, the aortic arch is interrupted or has a gap in it. This disorder is frequently identified in infancy or early childhood and typically occurs during fetal development.

IAA symptoms might change based on the condition’s severity and any related cardiac abnormalities. IAA-affected newborns may exhibit heart failure symptoms include poor feeding, fast breathing, weak pulses, and pale or chilly skin. IAA may cause serious complications and life-threatening diseases if left untreated.

TYPES

IAA is classified into three types based on the location of the interruption:

1. Type A: This is the most typical kind, when the aortic arch and ascending aorta are interrupted. A patent ductus arteriosus, a foetal blood channel that joins the pulmonary artery and the descending aorta, often maintains blood flow to the lower body.
2. Type B: The descending aorta and the left subclavian artery are where the obstruction develops. A patent ductus arteriosus or ventricular septal defect (a hole in the wall between the heart’s ventricles) is often used to provide blood flow to the lower body.
3. Type C: The pulmonary artery and ascending aorta are where the obstruction develops. This kind often coexists with more complicated cardiac abnormalities like tetralogy of Fallot or ventricular septal defect.

SYMPTOMS

Symptoms of interrupted aortic arch (IAA) can vary depending on the severity of the condition and associated heart defects. In newborns and infants, common symptoms may include:

1. Poor feeding: Infants with IAA may have difficulty feeding or may tire easily during feeding due to inadequate blood flow to the body.

2. Rapid breathing: Rapid or labored breathing, also known as tachypnea, can be a sign of heart failure and inadequate oxygenation.

3. Weak pulses: Weak or absent pulses, particularly in the lower extremities, may indicate reduced blood flow to the body.

4. Pale or cool skin: Inadequate oxygen supply can cause the skin to appear pale or have a bluish tint, especially in the lips and extremities.

5. Cyanosis: Cyanosis, a bluish discoloration of the skin and mucous membranes, can occur when there is inadequate oxygenation of the blood.

6. Heart murmur: A heart murmur, an abnormal sound heard during a physical examination, may be present due to associated heart defects such as ventricular septal defect.

DIAGNOSIS

Clinical assessment, imaging studies, and diagnostic techniques are often used in conjunction to make the diagnosis of interrupted aortic arch (IAA). Here are a few typical techniques for diagnosing IAA:

1. Physical examination: To determine your child’s general health, a medical specialist will do a complete physical examination, usually a paediatric cardiologist. They will examine any symptoms of poor blood circulation and listen to the heart for any abnormal sounds, such as heart murmurs.

2. Echocardiography: Also referred to as an echo, echocardiography is a non-invasive imaging procedure that employs ultrasonic waves to produce fine-grained pictures of the internal organs of the heart. As MRI can see the aortic arch and see any breaks or irregularities, it is the main tool for diagnosing IAA.

3. Cardiac MRI: To acquire more precise pictures of the heart and blood arteries, a cardiac MRI may sometimes be carried out. This imaging method generates high-resolution pictures using strong magnets and radio waves, enabling a thorough analysis of the structure of the heart.

4. Cardiac catheterization: During a cardiac catheterization, a thin, flexible tube called a catheter is inserted into a blood artery, usually one that is in the groyne. After that, the catheter is directed to the heart where pressures are measured, blood samples are taken, and angiography is done to see the blood arteries. This method is sometimes performed to evaluate the architecture of the heart and major vessels, as well as to confirm the diagnosis of IAA.

5. Genetic testing: This may be advised in certain circumstances to find any underlying genetic disorders or syndromes linked to IAA. This may aid in figuring out the condition’s source and directing therapy choices.

TREATMENT

The most common course of action for interrupted aortic arch (IAA) is surgery to correct the abnormality and bring back normal blood flow. The severity of the illness, any accompanying cardiac problems, and the patient’s general health will all influence the precise therapy strategy. Here are a few typical IAA treatments:

1. Surgical correction: This is the cornerstone of therapy for IAA. The treatment seeks to rebuild a healthy aortic arch and join the aorta’s broken portions. In most instances, a prosthetic graft is used to close the space between the ascending and descending aortas. Any accompanying cardiac problems, such as a ventricular septal defect or a patent ductus arteriosus, may also be treated during surgery.

2. Scheduled procedures: A planned surgical approach may be used in certain circumstances, notably when there are many severe cardiac abnormalities or if the patient is too unstable for a full repair. This entails doing the repair in stages, giving the patient time to recuperate and stabilise in between operations.

3. Medications: During or after surgery, medications may be recommended to assist control symptoms and improve heart function. Diuretics to lessen fluid retention, inotropic medicines to enhance cardiac function, and blood pressure meds are only a few examples of these drugs.

4. Follow-up care: After surgical repair, a paediatric cardiologist’s frequent follow-up visits are crucial to track the patient’s development, evaluate heart function, and guarantee healthy growth and development.

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