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Course: Pediatric Nursing

Topic: Nursing care of Child with Cardiovascular Disorder Part II

The Nurses International Community

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Module Goals

Learners will be able to:

Identify common cardiovascular disorders in children (congenital heart defects).
Describe common medical treatments for management of children with congenital heart defects.
Discuss the nursing management of a child with congenital heart defect.

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Types of Congenital Heart Defects

Septal defects: Refers to a hole (opening) between 2 of the heart's chambers.
Coarctation of the aorta: The aorta, is narrower than normal.
Pulmonary valve stenosis: Narrower pulmonary valve.
Transposition of the great arteries: Pulmonary and aortic valves and the arteries are connected and swapped.
Underdeveloped heart: A part of the heart doesn’t develop properly.

CDC, 2022a

Contact info: info@nursesinternational.org

Septal defects – where there's a hole between 2 of the heart's chambers (commonly referred to as a "hole in the heart"). Examples of septal defects include Atrial Septal Defects, Ventricular Septal Defects, Arto-ventricular septal defects.
Coarctation of the aorta – where the main large artery of the body, called the aorta, is narrower than normal
Pulmonary valve stenosis – where the pulmonary valve, which controls the flow of blood out of the lower right chamber of the heart to the lungs, is narrower than normal
Transposition of the great arteries – where the pulmonary and aortic valves and the arteries they're connected to have swapped positions (example: Dextro-TGA, levo-TGA)
Underdeveloped heart – where part of the heart doesn’t develop properly making it difficult for it to pump enough blood around the body or lungs. E.g. Hypoplastic Left Heart Syndrome

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Septal Defects

Atrial Septal Defect

A hole between the two atria i.e left and right atrium
Extra blood flows through the defect into the right atrium which stretches and enlarges the right side atrium

CDC, 2022a

Ventricular Septal Defects

A Common form of CHD
A hole between two ventricles (left and right)
Extra blood flows through the hole into the right ventricle; extra blood flows to the lungs, pressurizes the lungs and left side stretches

Contact info: info@nursesinternational.org

Septal defects

A septal defect is where there's an abnormality in the wall (septum) between the main chambers of the heart.

There are 2 main types of septal defect.

Atrial septal defects

An atrial septal defect (ASD) is where there's a hole between the 2 collecting chambers of the heart (the left and right atria). When there's an ASD, extra blood flows through the defect into the right side of the heart, causing it to stretch and enlarge.

Ventricular septal defects

A ventricular septal defect (VSD) is a common form of congenital heart disease. It occurs when there's a hole between the 2 pumping chambers of the heart (the left and right ventricles).

This means that extra blood flows through the hole from the left to the right ventricle, due to the pressure difference between them. The extra blood goes to the lungs, causing high pressure in the lungs and a stretch on the left- sided pumping chamber.

Small holes often eventually close by themselves, but larger holes need to be closed using surgery.

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Atrial Septal Defects: Causes, Risk Factors, and Diagnosis

Cause (unknown)

Might develop due to changes in genes or chromosomes
Are thought to be caused by a combination of genes and other risk factors (environmental, medications)

Diagnosis:

May be diagnosed during Pregnancy (ultrasound or prenatal screening tests)
Or after the birth (signs and symptoms)

CDC, 2022a

Contact info: info@nursesinternational.org

The causes of heart defects such as atrial septal defect among most infants are unknown. Some infants have heart defects because of changes in their genes or chromosomes. These types of heart defects also are thought to be caused by a combination of genes and other risk factors, such as things the mother comes in contact with in the environment or what the mother eats or drinks or the medicines the mother uses.

Diagnosis

An atrial septal defect may be diagnosed during pregnancy or after the infant is born. In many cases, it may not be diagnosed until adulthood.

During Pregnancy

During pregnancy, there are screening tests (prenatal tests) to check for birth defects and other conditions. An atrial septal defect might be seen during an ultrasound (which creates pictures of the body), but it depends on the size of the hole and its location. If an atrial septal defect is suspected, a specialist will need to confirm the diagnosis.

After the infant is Born

An atrial septal defect is present at birth, but many infants do not have any signs or symptoms.

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Atrial Septal Defects: Signs and Symptoms

Frequent respiratory or lung infections
Difficulty breathing
Tires during feeding (infants)
Shortness of breath when active or exercising
Skipped heartbeats
A heart murmur, or a whooshing sound that may be heard with a stethoscope
Swelling of legs, feet, or stomach area
Stroke

CDC, 2022b

Contact info: info@nursesinternational.org

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Atrial Septal Defects: Treatment

Depends on the age of diagnosis, the number of or seriousness of symptoms, size of the hole, and presence of other conditions.

Medications to treat symptoms
No known medication to treat the hole
Sometimes surgery to repair the hole: Cardiac catheterization or open-heart surgery

CDC, 2022b

Contact info: info@nursesinternational.org

Treatment for an atrial septal defect depends on the age of diagnosis, the number of or seriousness of symptoms, size of the hole, and presence of other conditions. Sometimes surgery is needed to repair the hole. Sometimes medications are prescribed to help treat symptoms. There are no known medications that may repair the hole.

If a child is diagnosed with an atrial septal defect, the health care provider may want to monitor it for a while to see if the hole closes on its own. During this period of time, the health care provider might treat symptoms with medicine. A health care provider may recommend the atrial septal defect be closed for a child with a large atrial septal defect, even if there are few symptoms, to prevent problems later in life. Closure may also be recommended for an adult who has many or severe symptoms. Closure of the hole may be done during cardiac catheterization or open-heart surgery. After these procedures, follow-up care will depend on the size of the defect, individual’s age, and whether the individual has other birth defects.

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Ventricular Septal Defect (VSD)

Blood flows from the left ventricle (oxygenated blood) through the septal defect to the right ventricle (deoxygenated blood)
The extra blood being pumped forces heart and lungs to work harder
If not repaired, complications arises

Heart Failure
Pulmonary hypertension
Arrhythmias
Stroke

CDC, 2022b

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Ventricular Septal Defect: Causes, Risk Factors and Diagnosis

Causes and risk factors same as for Atrial Septal Defects

Diagnosis:

Usually diagnosed after the birth of the child
May be diagnosed upon physical examination (heart murmur)

CDC, 2022b

Contact info: info@nursesinternational.org

A ventricular septal defect happens during pregnancy if the wall that forms between the two ventricles does not fully develop, leaving a hole. A ventricular septal defect is one type of congenital heart defect. Congenital means present at birth.

In a child without a congenital heart defect, the right side of the heart pumps oxygen-poor blood from the heart to the lungs, and the left side of the heart pumps oxygen-rich blood to the rest of the body.

In infants with a ventricular septal defect, blood often flows from the left ventricle through the ventricular septal defect to the right ventricle and into the lungs. This extra blood being pumped into the lungs forces the heart and lungs to work harder. Over time, if not repaired, this defect may increase the risk for other complications, including heart failure, high blood pressure in the lungs (called pulmonary hypertension), irregular heart rhythms (called arrhythmia), or stroke.

A ventricular septal defect usually is diagnosed after an infant is born.

The size of the ventricular septal defect will influence what symptoms, if any, are present, and whether a doctor hears a heart murmur during a physical examination. Signs of a ventricular septal defect might be present at birth or might not appear until well after birth. If the hole is small, it usually will close on its own and the infant might not show any signs of the defect

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Ventricular Septal Defect: Signs and Symptoms

Shortness of breath,
Fast or heavy breathing
Sweating
Feeding fatigue
Poor weight gain
Murmur

CDC, 2022b

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Ventricular Septal Defect: Treatment

Depends on size of the hole and degree of problem
Cardiac catheterization or open-heart surgery
May need medication to strengthen heart muscle, lower blood pressure and remove extra fluid
Proper nutrition to ensure healthy growth

May need tube feedings

CDC, 2022b

Contact info: info@nursesinternational.org

Treatments

Treatments for a ventricular septal defect depend on the size of the hole and the problems it might cause. Many ventricular septal defects are small and close on their own; if the hole is small and not causing any symptoms, the doctor will check the infant regularly to ensure there are no signs of heart failure and that the hole closes on its own. If the hole does not close on its own or if it is large, further actions might need to be taken.

Depending on the size of the hole, symptoms, and general health of the child, the doctor might recommend either cardiac catheterization or open-heart surgery to close the hole and restore normal blood flow. After surgery, the doctor will set up regular follow-up visits to make sure that the ventricular septal defect remains closed. Most children who have a ventricular septal defect that closes (either on its own or with surgery) live healthy lives.

Medicines

Some children will need medicines to help strengthen the heart muscle, lower their blood pressure, and help the body get rid of extra fluid.

Nutrition

Some infants with a ventricular septal defect become tired while feeding and do not eat enough to gain weight. To make sure infants have a healthy weight gain, a special high-calorie formula might be prescribed. Some infants become extremely tired while feeding and might need to be fed through a feeding tube.

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Coarctation of the aorta

The main artery (the aorta) has a narrowing resulting in less blood flow through it.
May occur by itself or in combination with other types of heart defects, such as a ventricular septal defect or a type of defect known as a patent ductus arteriosus.
The narrowing may be severe and will often require treatment shortly after birth.

CDC, 2022c

Contact info: info@nursesinternational.org

If the narrowing is severe enough and if it is not diagnosed, the infant may have serious problems and may need surgery or other procedures soon after birth. For this reason, coarctation of the aorta is often considered a critical congenital heart defect. The defect occurs when a baby’s aorta does not form correctly as the infant grows and develops during pregnancy. The narrowing of the aorta usually happens in the part of the blood vessel just after the arteries branch off to take blood to the head and arms, near the patent ductus arteriosus, although sometimes the narrowing occurs before or after the ductus arteriosus. In some infants with coarctation, it is thought that some tissue from the wall of ductus arteriosus blends into the tissue of the aorta. When the tissue tightens and allows the ductus arteriosus to close normally after birth, this extra tissue may also tighten and narrow the aorta.

The narrowing, or coarctation, blocks normal blood flow to the body. This may back up flow into the left ventricle of the heart, making the muscles in this ventricle work harder to get blood out of the heart. Since the narrowing of the aorta is usually located after arteries branch to the upper body, coarctation in this region may lead to normal or high blood pressure and pulsing of blood in the head and arms and low blood pressure and weak pulses in the legs and lower body.

If the condition is very severe, enough blood may not be able to get through to the lower body. The extra work on the heart may cause the walls of the heart to become thicker in order to pump harder. This eventually weakens the heart muscle. If the aorta is not widened, the heart may weaken enough that it leads to heart failure. Coarctation of the aorta often occurs with other congenital heart defects.

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Coarctation of the Aorta: Causes, Risk Factors and Diagnosis

Causes and risk factors (same like ASD and VSD)

Diagnosis:

Usually diagnosed at birth
Newborn screening using pulse oximetry during the first few days of life may or may not detect coarctation of the aorta.
Physical examination: weaker pulse in legs and groins, heart murmur, high blood pressure in arms.
Confirmatory tests: Echocardiogram, chest X-ray, EKG, MRI, cardiac catheterization.

CDC, 2022c

Contact info: info@nursesinternational.org

Causes and Risk Factors

The causes of heart defects, including coarctation of the aorta, among most infants are unknown. Some infants have heart defects because of changes in their genes or chromosomes. Heart defects, like coarctation of the aorta, are also thought to be caused by a combination of genes and other risk factors, such as things the mother comes in contact with in the environment, what the mother eats or drinks, or medicines the mother uses.

Diagnosis

Coarctation of the aorta is usually diagnosed after the child is born. How early in life the defect is diagnosed usually depends on how mild or severe the symptoms are. Newborn screening using pulse oximetry during the first few days of life may or may not detect coarctation of the aorta.

Detection of the defect is often made during a physical exam. In infants and older individuals, the pulse will be noticeably weaker in the legs or groin than it is in the arms or neck, and a heart murmur—an abnormal whooshing sound caused by disrupted blood flow—may be heard through a doctor’s stethoscope. Older children and adults with coarctation of the aorta often have high blood pressure in the arms.

Once suspected, an echocardiogram is the most commonly used test to confirm the diagnosis. An echocardiogram is an ultrasound of the heart that may show problems with the structure of the heart and the blood flow through it, and how well the heart is working. It will show the location and severity of the coarctation and whether any other heart defects are present. Other tests to measure the function of the heart may be used including chest x-ray, electrocardiogram (EKG), magnetic resonance imaging (MRI), and cardiac catheterization.

It also may be detected with newborn pulse oximetry screening. Pulse oximetry is a simple bedside test to determine the amount of oxygen in a baby’s blood. Low levels of oxygen in the blood may be a sign of it.

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Coarctation of the Aorta: Signs and Symptoms

In infants with a more serious condition, early signs usually include:

Pale skin
Irritability
Heavy sweating
Difficulty breathing

CDC, 2022c

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Coarctation of the Aorta: Treatment

Cardiac Catheterization: Balloon Angioplasty
Surgery
Medications to control high blood pressure after surgery
Regular follow up and monitoring of progress

CDC, 2022c

Contact info: info@nursesinternational.org

Treatments

No matter what age the defect is diagnosed, the narrow aorta will need to be widened once symptoms are present. This may be done with surgery or a procedure called balloon angioplasty, which is done during a cardiac catheterization. A balloon angioplasty is a procedure that uses a thin, flexible tube, called a catheter, which is inserted into a blood vessel and directed to the aorta. When the catheter reaches the narrow area of the aorta, a balloon at the tip is inflated to expand the blood vessel. Sometimes a mesh-covered tube (stent) is inserted to keep the vessel open. The stent is used more often to initially widen the aorta or re-widen it if the aorta narrows again after surgery has been performed. During surgery to correct a coarctation, the narrow portion is removed and the aorta is reconstructed or patched to allow blood to flow normally through the aorta.

Even after surgery, children with a coarctation of the aorta often have high blood pressure that is treated with medicine. It is important for children and adults with coarctation of the aorta to follow up regularly with a cardiologist (a heart doctor) to monitor their progress and check for other health conditions that might develop as they get older.

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Pulmonary Valve Stenosis

Type of congenital heart defect
Narrowed pulmonary valve allows less blood to enter the heart
Heart has to work harder to push blood to the lungs

Diagnosis:

Before birth:

Fetal ultrasound

After Birth:

Heart murmur on auscultation

Confirmatory tests: Echocardiogram, ECG, Chest X-ray

MyHealth.Alberta.ca., 2021

Contact info: info@nursesinternational.org

Pulmonary valve stenosis is a type of congenital heart defect. Congenital heart defects are heart problems a chil is born with. These heart problems are usually diagnosed at or before birth.

"Pulmonary" means "of the lungs." The pulmonary artery takes the blood from the heart to the lungs to pick up new oxygen. The pulmonary valve is the gate through which the heart pumps blood into the pulmonary artery.

"Stenosis" means "narrowed." In pulmonary valve stenosis, that gate is too narrow. The heart has to work harder to push blood through it. It also means less blood gets to the lungs. In more severe cases, this may mean that the blood going out to the body doesn't have enough oxygen.

An infant with pulmonary valve stenosis may also have other heart defects.

It may be scary to the parents to learn that there is something wrong with their baby's heart. The hospital staff understands this. They will explain what happens and will answer their questions.

Diagnosis:

The doctor may hear abnormal heart sounds, such as a heart murmur, when he or she examines the newborn.

The doctor will order tests to find the cause of abnormal heart sounds or of symptoms. The most common test is an echocardiogram, or "echo" for short. It uses sound waves to make an image of the baby's heart.

The infant may have other tests to find the problem, such as an EKG (electrocardiogram) or a chest X-ray. Another test may look at the amount of oxygen in the blood.

A fetal ultrasound, which looks at the baby's heart, may find this defect before birth.

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Pulmonary Valve Stenosis: Symptoms and Treatment

Symptoms:

Mild or no symptoms
Severe case: Cyanosis, rapid breathing, tires easily with physical exertion and feeding

Treatment:

Mild cases do not require treatment
Regular check ups
In serious cases, surgery to stretch, repair, or replace valve

MyHealth.Alberta.ca., 2021

Contact info: info@nursesinternational.org

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Transposition of Great Arteries (TGA)

Aorta and pulmonary artery swap their positions (i.e. aorta arises from right ventricle and pulmonary artery arises from left ventricle).
The aorta is in the front of the pulmonary artery and is either primarily to the right or to the left.
The most common type of TGA is Dextro-TGA.

Szymansk et al, 2021; CDC, 2021d

Contact info: info@nursesinternational.org

The aorta arises from the right ventricle and the pulmonary trunk arises from the left ventricle. The most common form of TGA is referred to as dextro-TGA (D-TGA) which is characterized by the right ventricle being positioned to the right of the left ventricle and the aorta arising anterior and rightward to the pulmonary artery thus forming two parallel circuits.

In transposition of the great arteries, the aorta is in front of the pulmonary artery and is either primarily to the right (dextro) or to the left (levo) of the pulmonary artery. Levo-TGA is rarer than dextro-TGA. Dextro-TGA is often simply called “TGA.” However, “TGA” is a broader term that includes both dextro-TGA (d-TGA) and levo-TGA (l-TGA).

In a infant without a congenital heart defect, the right side of the heart pumps oxygen-poor blood from the heart to the lungs through the pulmonary artery. The left side of the heart pumps oxygen-rich blood to the rest of the body through the aorta. The aorta is usually behind the pulmonary artery.

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Dextro-Transposition of Great Arteries (D-TGA)

Deoxygenated blood from the body enters the right chamber of heart.
The blood is directly pumped out to the rest of the body through the aorta.
Oxygenated blood is pumped back to the lungs through main pulmonary artery.
Infants with D-TGA are born with other additional heart defects (like ASD or VSD).
The patent ductus arteriosus allows some oxygenated blood to be pumped to the rest of the body.

CDC, 2021d

Contact info: info@nursesinternational.org

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D-TGA: Causes, Risks and Diagnosis

Causes and Risks

Unknown cause might be due to genetic or chromosomal changes.
Are thought to be caused by the combination of genes and other risk factors like: environment and medication intakes.

Diagnosis

During Pregnancy: Prenatal Tests, ultrasounds, echocardiogram.
After Birth: Monitoring signs and symptoms, EKG, Pulse oximeter test.

CDC, 2021d

Contact info: info@nursesinternational.org

Causes and Risk Factors

The causes of congenital heart defects, such as d-TGA, among most infants are unknown. Some infants have congenital heart defects because of changes in their genes or chromosomes. Heart defects are also thought to be caused by the combination of genes and other risk factors such as things the mother comes in contact with in her environment, or what the mother eats or drinks, or certain medications she uses.

Diagnosis

This defect may be diagnosed during pregnancy or soon after the child is born.

During Pregnancy

During pregnancy, there are screening tests that the mother may have (also called prenatal tests) to check for birth defects and other conditions. D-TGA may be diagnosed during pregnancy with an ultrasound test (which creates pictures of the baby). Some findings from the ultrasound may make the health care provider suspect a child could have d-TGA. If so, the health care provider may request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby’s heart. This test may show problems with the structure of the heart and how the heart is working with this defect.

After the child is Born

Symptoms occur at birth or very soon afterwards. How severe the symptoms are will depend on whether there is a way for blood to mix and for oxygen-rich blood to get out to the rest of the body. For example, if an infant with d-TGA has another defect, like an atrial septal defect (ASD), the ASD forms a passageway for some oxygen-rich blood to be pumped to the rest of the body. This infant with both d-TGA and an ASD may not have as severe symptoms as infants whose hearts don’t have any mixing of blood. Infants with d-TGA may have a bluish looking skin color—called cyanosis—because their blood doesn’t carry enough oxygen. Because the infant might be bluish in color and have trouble breathing, d-TGA is usually diagnosed within the first week of life. The health care provider may request one or more tests to confirm the diagnosis. The most common test is an echocardiogram. An echocardiogram is an ultrasound of the heart that may show problems with the structure of the heart, like incorrect positioning of the two large arteries, and any irregular blood flow. An electrocardiogram (EKG), which measures the electrical activity of the heart, chest x-rays, and other medical tests may also be used to make the diagnosis.

D-TGA may also be detected with newborn pulse oximetry screening. Pulse oximetry is a simple bedside test to determine the amount of oxygen in a baby’s blood. Low levels of oxygen in the blood may be a sign of a CCHD. Newborn screening using pulse oximetry may identify some infants with a CCHD, like d-TGA, before they show any symptoms.

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D-TGA: Signs and Symptoms

Infants with d-TGA or other conditions causing cyanosis may have symptoms such as:

Problems breathing
Pounding heart
Weak pulse
Ashen or bluish skin color (cyanosis)
Poor feeding

CDC, 2021d

Contact info: info@nursesinternational.org

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Treatments:

Surgery to repair, maintain or create opening

Arterial Switch Surgery
Atrial Switch Operation

Medications to control blood pressure, remove extra fluids, improve pumping action
Pacemaker if the heart beats too slowly
Regular follow up to monitor progress and avoid complications

CDC, 2021d

Contact info: info@nursesinternational.org

Treatments

Surgery is required for all infants born with d-TGA. Other procedures may be done before surgery in order to maintain, enlarge or create openings that will allow oxygen-rich blood to get out to the body.

There are two types of surgery to repair d-TGA:

Arterial Switch Operation: This is the most common procedure and it is usually done in the first month of life. It restores usual blood flow through the heart and out to the rest of the body. During this surgery, the arteries are switched to their usual positions—the pulmonary artery arising from the right ventricle and the aorta from the left ventricle. The coronary arteries (small arteries that provide blood to the heart muscle) also must be moved and reattached to the aorta.
Atrial Switch Operation: This procedure is less commonly performed. During this surgery, the arteries are left in place, but a tunnel (baffle) is created between the top chambers (atria) of the heart. This tunnel allows oxygen-poor blood to move from the right atrium to the left ventricle and out the pulmonary artery to the lungs. Returning oxygen-rich blood moves through the tunnel from the left atrium to the right ventricle and out the aorta to the body. Although this repair helps blood to go to the lungs and then out to the body, it also makes extra work for the right ventricle to pump blood to the entire body. Therefore, this repair may lead to difficulties later in life.

After surgery, medications may be needed to help the heart pump better, control blood pressure, help get rid of extra fluid in the body, and slow down the heart if it is beating too fast. If the heart is beating too slowly, a pacemaker may be used.

Infants who have these surgeries are not cured; they may have lifelong complications. A child or adult with d-TGA will need regular follow-up visits with a cardiologist (a heart doctor) to monitor their progress and avoid complications or other health problems. With proper treatment, most infants with d-TGA grow up to lead healthy, productive lives.

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Underdeveloped Heart: Hypoplastic Heart Syndrome

A critical congenital heart defect where left side of the heart does not form correctly.
The left side of the heart maynot pump oxygenated blood to the body properly.
Oxygenated blood bypasses the poorly functioning left chambers through patent ductus arteriosus and patent foramen ovale.
Therefore right side pump blood both to the lungs and the body.

CDC, 2021e

Contact info: info@nursesinternational.org

Hypoplastic left heart syndrome (HLHS) is a birth defect that affects normal blood flow through the heart. As the infant develops during pregnancy, the left side of the heart does not form correctly. Hypoplastic left heart syndrome is one type of congenital heart defect. Congenital means present at birth. Because an infant with this defect needs surgery or other procedures soon after birth, HLHS is considered a critical congenital heart defect (CCHD).

In an infant without a congenital heart defect, the right side of the heart pumps oxygen-poor blood from the heart to the lungs. The left side of the heart pumps oxygen-rich blood to the rest of the body. When an infant is growing in a mother’s womb during pregnancy, there are two small openings between the left and right sides of the heart: the patent ductus arteriosus and the patent foramen ovale. Normally, these openings will close a few days after birth.

In infants with hypoplastic left heart syndrome, the left side of the heart maynot pump oxygen-rich blood to the body properly. During the first few days of life for an infant with hypoplastic left heart syndrome, the oxygen-rich blood bypasses the poorly functioning left side of the heart through the patent ductus arteriosus and the patent foramen ovale. The right side of the heart then pumps blood to both the lungs and the rest of the body. However, among infants with hypoplastic left heart syndrome, when these openings close, it becomes hard for oxygen-rich blood to get to the rest of the body.

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Underdeveloped Heart: Hypoplastic Heart Syndrome

Affects number of structures:

The left ventricle is underdeveloped and too small
The mitral valve is not formed or is very small
The aortic valve is not formed or is very small
The ascending aorta is underdeveloped or is too small
Often, with atrial septal defect

CDC, 2021e

Contact info: info@nursesinternational.org

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Hypoplastic Heart Syndrome: Causes, Risk Factors and Diagnosis

Causes and Risks

Unknown cause, may be due to genetic or chromosomal changes
Thought to be caused by the combination of genes and other risk factors like: Environment and medication

Diagnosis

During Pregnancy: Prenatal Tests, ultrasounds, Echocardiogram.
After Birth: Monitoring signs and symptoms, EKG, Pulse oximeter test.

CDC, 2021e

Contact info: info@nursesinternational.org

Causes and Risk Factors

The causes of heart defects such as hypoplastic left heart syndrome among most infants are unknown. Some infants have heart defects because of changes in their genes or chromosomes. These types of heart defects also are thought to be caused by a combination of genes and other risk factors, such as things the mother comes in contact with in the environment or what the mother eats or drinks or the medicines the mother uses.

Diagnosis

Hypoplastic left heart syndrome may be diagnosed during pregnancy or soon after the child is born.

During Pregnancy

During pregnancy, there are screening tests (also called prenatal tests,) to check for birth defects and other conditions. Hypoplastic left heart syndrome may be diagnosed during pregnancy with an ultrasound, (which creates pictures of the body). Some findings from the ultrasound may make the health care provider suspect an infant may have hypoplastic left heart syndrome. If so, the health care provider may request a fetal echocardiogram, an ultrasound of the baby’s heart, to confirm the diagnosis. This test may show problems with the structure of the heart and how the heart is working with this defect.

During a physical examination, a doctor may see these signs or might hear a heart murmur (an abnormal whooshing sound caused by blood not flowing properly). If a murmur is heard or other signs are present, the health care provider might request one or more tests to make a diagnosis, the most common being an echocardiogram. Echocardiography also is useful for helping the health care provider follow the child’s health over time.

HLHS is a defect that also may be detected with newborn pulse oximetry screening. Pulse oximetry is a simple bedside test to determine the amount of oxygen in a baby’s blood. Low levels of oxygen in the blood may be a sign of a CCHD. Newborn screening using pulse oximetry may identify some infants with a CCHD, like HLHS, before they show any symptoms.

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Hypoplastic Heart Syndrome: Signs and Symptoms

Problems breathing,
Pounding heart
Weak pulse
Ashen or bluish skin color

CDC, 2021e

Contact info: info@nursesinternational.org

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Hypoplastic Heart Syndrome: Treatment

Medication to strengthen heart muscle, lower blood pressure and remove extra fluid.
Nutrition: High calorie formula, may need feeding tube.
Surgery: Includes multiple surgeries (Norwood procedure, Fontan procedure, Bi-directional Glenn Shunt Procedure) to increase blood flow.

Note: Infants with hypoplastic left heart syndrome may have lifelong complications, therefore, will need regular monitoring with a cardiologist

CDC, 2021e

Contact info: info@nursesinternational.org

Treatments

Treatments for some health problems associated with hypoplastic left heart syndrome might include:

Medicines

Some infants and children will need medicines to help strengthen the heart muscle, lower their blood pressure, and help the body get rid of extra fluid.

Nutrition

Some infants with hypoplastic left heart syndrome become tired while feeding and do not eat enough to gain weight. To make sure infants have a healthy weight gain, a special high-calorie formula might be prescribed. Some infants become extremely tired while feeding and might need to be fed through a feeding tube.

Surgery

Soon after a infant with hypoplastic left heart syndrome is born, multiple surgeries done in a particular order are needed to increase blood flow to the body and bypass the poorly functioning left side of the heart. The right ventricle becomes the main pumping chamber to the body. These surgeries do not cure hypoplastic left heart syndrome, but help restore heart function. Sometimes medicines are given to help treat symptoms of the defect before or after surgery. Surgery for hypoplastic left heart syndrome usually is done in three separate stages:

Norwood Procedure�This surgery usually is done within the first 2 weeks of a baby’s life. Surgeons create a “new” aorta and connect it to the right ventricle. They also place a tube from either the aorta or the right ventricle to the vessels supplying the lungs (pulmonary arteries). Thus, the right ventricle may pump blood to both the lungs and the rest of the body. This may be a very challenging surgery. After this procedure, an infant’s skin still might look bluish because oxygen-rich and oxygen-poor blood still mix in the heart.
Bi-directional Glenn Shunt Procedure�This usually is performed when an infant is 4 to 6 months of age. This procedure creates a direct connection between the pulmonary artery and the vessel (the superior vena cava) returning oxygen-poor blood from the upper part of the body to the heart. This reduces the work the right ventricle has to do by allowing blood returning from the body to flow directly to the lungs.
Fontan Procedure�This procedure usually is done sometime during the period when an infant is 18 months to 3 years of age. Doctors connect the pulmonary artery and the vessel (the inferior vena cava) returning oxygen-poor blood from the lower part of the body to the heart, allowing the rest of the blood coming back from the body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart and an infant’s skin will no longer look bluish.

Infants who have these surgeries are not cured

Infants with hypoplastic left heart syndrome may have lifelong complications. They will need regular follow-up visits with a cardiologist (a heart doctor) to monitor their progress. If the hypoplastic left heart syndrome defect is very complex, or the heart becomes weak after the surgeries, a heart transplant may be needed. Infants who receive a heart transplant will need to take medicines for the rest of their lives to prevent their body from rejecting the new heart.

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Underdeveloped Heart: Tetralogy of Fallot (ToF)

Baby’s heart does not form correctly as the infant grows and develops in the mother’s womb during pregnancy:

Made up of 4 defects:

Ventricular Septal Defect
Pulmonary Stenosis
Ventricular Hypertrophy
Overriding Aorta

CDC, 2021f

Contact info: info@nursesinternational.org

Tetralogy of Fallot is made up of the following four defects of the heart and its blood vessels:

A hole in the wall between the two lower chambers―or ventricles―of the heart. This condition also is called a ventricular septal defect.
A narrowing of the pulmonary valve and main pulmonary artery. This condition also is called pulmonary stenosis.
The aortic valve, which opens to the aorta, is enlarged and seems to open from both ventricles, rather than from the left ventricle only, as in a normal heart. In this defect, the aortic valve sits directly on top of the ventricular septal defect.
The muscular wall of the lower right chamber of the heart (right ventricle) is thicker than normal. This also is called ventricular hypertrophy.

Because an infant with tetralogy of Fallot may need surgery or other procedures soon after birth, this birth defect is considered a critical congenital heart defect. Congenital means present at birth.

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Tetralogy of Fallot: Causes, Risk Factors and Diagnosis

Causes and Risks:

Unknown cause, could be due to genetic or chromosomal changes.
Are thought to be caused by the combination of genes and other risk factors like: Environment and medication intakes.

Diagnosis:

During Pregnancy: Prenatal Tests, ultrasounds, Echocardiogram.
After Birth: Monitoring signs and symptoms, EKG, Pulse oximeter test.

CDC, 2021f

Contact info: info@nursesinternational.org

Causes and Risk Factors

The causes of heart defects (such as tetralogy of Fallot) among most infants are unknown. Some infants have heart defects because of changes in their genes or chromosomes. Heart defects such as tetralogy of Fallot also are thought to be caused by a combination of genes and other risk factors, such as the things the mother or fetus come in contact with in the environment or what the mother eats or drinks or the medicines she uses.

Diagnosis

Tetralogy of Fallot may be diagnosed during pregnancy or soon after an infant is born.

During Pregnancy

During pregnancy, there are screening tests (also called prenatal tests) to check for birth defects and other conditions. Tetralogy of Fallot might be seen during an ultrasound (which creates pictures of the body). Some findings from the ultrasound may make the health care provider suspect an infant may have tetralogy of Fallot. If so, the health care provider may request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is an ultrasound of the heart of the fetus. This test may show problems with the structure of the heart and how the heart is working with this defect.

After a Infant Is Born

Tetralogy of Fallot usually is diagnosed after an infant is born, often after the infant has an episode of turning blue during crying or feeding (a tet spell). Some findings on a physical exam may make the health care provider think an infant may have tetralogy of Fallot, including bluish-looking skin or a heart murmur (a “whooshing” sound caused by blood not flowing properly through the heart). However, it is not uncommon for a heart murmur to be absent right at birth.

The health care provider may request one or more tests to confirm the diagnosis. The most common test is an echocardiogram. An echocardiogram is an ultrasound of the heart that may show problems with the structure of the heart and how the heart is working (or not) with this defect. Echocardiography also is useful for helping the doctor follow the child’s health over time.

Tetralogy of Fallot is a critical congenital heart defect (critical CHD) that may be detected with newborn screening using pulse oximetry (also known as pulse ox). Pulse oximetry is a simple bedside test to estimate the amount of oxygen in a baby’s blood. Low levels of oxygen in the blood may be a sign of a critical CHD. Newborn screening using pulse oximetry may identify some infants with a critical CHD, like tetralogy of Fallot, before they show any symptoms.

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Tetralogy of Fallot: Signs and Symptoms

Signs and Symptoms

Bluish looking skin color
Tet Spells*

Infants with TOF are at risk for:

Endocarditis.
Arrhythmia.
Dizziness, fainting, or seizures
Delayed growth and development

CDC, 2021f

Contact info: info@nursesinternational.org

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Tetralogy of Fallot: Treatment

Surgery

To widen or replace the pulmonary valve
Enlarge the passage of pulmonary artery
Close the hole between two lower chambers of the heart

Note: Even after a surgery, regular follow up with cardiologist to monitor progress and heart condition is required

CDC, 2021f

Contact info: info@nursesinternational.org

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Total Anomalous Pulmonary Venous Return or TAPVR

Birth defect of the heart in which the veins bringing blood back from the lungs (pulmonary veins) don’t connect to the left atrium instead they go to the heart by way of an abnormal (anomalous) connection.
Not all veins have abnormal connections; some have normal connections.
Causes, risk factors and diagnosis are same like that of other heart defects.

CDC, 2021g

Contact info: info@nursesinternational.org

In an infant with TAPVR, oxygen-rich blood does not return from the lungs to the left atrium. Instead, the oxygen-rich blood returns to the right side of the heart. Here, oxygen-rich blood mixes with oxygen-poor blood. This causes the infant to get less oxygen than is needed to the body. To survive with this defect, infants with TAPVR usually have a hole between the right atrium and the left atrium (an atrial septal defect) that allows the mixed blood to get to the left side of the heart and pumped out to the rest of the body. Some children may have other heart defects along with TAPVR, aside from the atrial septal defect. Because an infant with this defect may need surgery or other procedures soon after birth, TAPVR is considered a critical congenital heart defect. Congenital means present at birth.

In a related defect, partial anomalous pulmonary venous return (PAPVR), not all of the veins have an abnormal connection. There are some abnormal connections, but one or more of the veins return normally to the left atrium. Therefore, PAPVR is not as critical as TAPVR.

In ann infant without a congenital heart defect, the right side of the heart pumps oxygen-poor blood from the heart to the lungs through the pulmonary artery. The blood that comes back from the lungs is oxygen-rich, and it moves through the pulmonary veins to the left atrium. The left side of the heart pumps oxygen-rich blood to the rest of the body through the aorta.

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Types of TAPVR

Supracardiac: Pulmonary veins form an abnormal connection above the heart to the superior vena cava.

Cardiac: Pulmonary veins meet behind the heart and connect to right atrium.

Infracardiac: The pulmonary vein comes together and forms an abnormal connection below the heart.

CDC, 2021g

Contact info: info@nursesinternational.org

Types of TAPVR

There are different types of TAPVR, based on where the pulmonary veins connect:

Supracardiac– In supracardiac TAPVR, the pulmonary veins come together and form an abnormal connection above the heart to the superior vena cava, which is a main blood vessel that brings oxygen-poor blood from the upper part of the body to the heart. In this type of TAPVR, a mixture of oxygen-poor and oxygen-rich blood returns to the right atrium through the superior vena cava.
Cardiac – In cardiac TAPVR, the pulmonary veins meet behind the heart and connect to the right atrium. The coronary sinus, which is a vein that helps bring oxygen-poor blood from the heart muscle back to the heart, helps connect the pulmonary veins to the right atrium in this type of TAPVR.
Infracardiac – In infracardiac TAPVR, the pulmonary veins come together and form abnormal connections below the heart. A mixture of oxygen-poor blood and oxygen-rich blood returns to the right atrium from the veins of the liver and the inferior vena cava, which is the main blood vessel that brings oxygen-poor blood from the lower part of the body to the heart.

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TAPVR: Treatment

Surgery

To repair defects
To restore normal blood flow through heart
May have lifelong complications even after surgically
Need regular follow-up with a cardiologist to monitor progress and avoid complications

CDC, 2021g

Contact info: info@nursesinternational.org

Treatment

Infants with TAPVR will need surgery to repair the defect. The age at which the surgery is done depends on how sick the child is and the specific structure of the abnormal connections between the pulmonary veins and the right atrium. The goal of the surgical repair of TAPVR is to restore normal blood flow through the heart. To repair this defect, doctors usually connect the pulmonary veins to the left atrium, close off any abnormal connections between blood vessels, and close the atrial septal defect.

Infants whose defects are surgically repaired are not cured; they may have lifelong complications. A child or adult with TAPVR will need regular follow-up visits with a cardiologist (a heart doctor) to monitor their progress, avoid complications, and check for other health conditions that might develop as they get older.

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Nursing Interventions for a Child With Congenital Heart Disease

Assess and monitor vital signs; set baseline and identify changes
Auscultate heart for murmur, gallop location and severity
Auscultate lung sounds; rule out pulmonary edema
Assess skin for cyanosis or pale color
Assess peripheral pulse and capillary refill; assesses cardiac output
Obtain weight daily; monitor fluid balance
Provide comfort; semi-fowler/upright makes breathing easier

Nursing.com, n.d.

Contact info: info@nursesinternational.org

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Nursing Interventions for a Child With Congenital Heart Disease (Continued)

Administer oxygen; prevents hypoxia
Administer prescribed medications
Plan rest periods along with activities
Prepare child and parent for surgery (if indicated); NPO, Catheterization, medications, education
Educate child and family members regarding

Exercise restrictions
Importance of oral care and overall hygiene
Healthy diet and hydration

Nursing.com, n.d.

Contact info: info@nursesinternational.org

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Critical Thinking Question

Which four (4) congenital defects characterize Tetralogy of Fallot?

Pulmonary stenosis, atrial septal defect, overriding aorta, ventricular hypertrophy
Pulmonary stenosis, overriding aorta, murmur, atrial hypertrophy
Ventricular septal defect, pulmonary stenosis, ventricular hypertrophy, overriding aorta
Murmur, atrial septal defect, ventricular hypertrophy, pulmonary stenosis

Contact info: info@nursesinternational.org

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Red Flags

Recurrent syncope with exertion
Failure to thrive
Decreased femoral pulses
Murmur
Recurrent wheezing
Chest pain
Trouble breathing
Swelling (edema)
Feeling tired
Not eating well

Contact info: info@nursesinternational.org

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Cultural Considerations

Religion, culture, beliefs, and ethnic customs may influence how families understand and use health concepts:

Health beliefs: In some cultures talking about a possible poor health outcome will cause that outcome to occur.
Health customs: In some cultures family members play a large role in health care decision-making.
Ethnic customs: Differing gender roles may determine who makes decisions about accepting & following treatment recommendations.

AHRQ, 2020

Contact info: info@nursesinternational.org

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Cultural Considerations (Continued)

Religion, culture, beliefs, and ethnic customs may influence how families understand and use health concepts:

Religious beliefs: Faith and spiritual beliefs may affect health seeking behavior and willingness to accept treatment.
Dietary customs: Dietary advice may be difficult to follow if it does not fit the foods or cooking methods of the family.
Interpersonal customs: Eye contact or physical touch may be ok in some cultures but inappropriate or offensive in others.

AHRQ, 2020

Contact info: info@nursesinternational.org

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References:

Andrews, M.M., Boyle, J.S. & Collings, J. W. (2020). Transcultural Concepts in Nursing Care (8th Ed.). Wolters Kluwer, Philadelphia, PA.

CDC- Center for Disease Control and Prevention. (2022a, Jan 24). Congenital Heart Defects: Facts about Atrial Septal Defect. https://www.cdc.gov/ncbddd/heartdefects/atrialseptaldefect.html

CDC- Center for Disease Control and Prevention. (2022b, Jan 24). Congenital Heart Defects: Facts about Ventricular Septal Defect. https://www.cdc.gov/ncbddd/heartdefects/ventricularseptaldefect.html

CDC- Center for Disease Control and Prevention. (2022c, Jan 24). Congenital Heart Defects: Facts about Coarctation of the Aorta. https://www.cdc.gov/ncbddd/heartdefects/coarctationofaorta.html

Contact info: info@nursesinternational.org

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References:

CDC- Center for Disease Control and Prevention. (2022d, Jan 24). Congenital Heart Defects: Facts about dextro-Transposition of the Great Arteries (d-TGA). https://www.cdc.gov/ncbddd/heartdefects/d-tga.html

CDC- Center for Disease Control and Prevention. (2022e, Jan 24). Congenital Heart Defects: Facts about Hypoplastic Left Heart Syndrome. https://www.cdc.gov/ncbddd/heartdefects/hlhs.html

CDC- Center for Disease Control and Prevention. (2022f, Jan 24). Congenital Heart Defects: Facts about Tetralogy of Fallot. https://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html

Contact info: info@nursesinternational.org

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References:

CDC- Center for Disease Control and Prevention. (2022g, Jan 24). Congenital Heart Defects: Facts about Total Anomalous Pulmonary Venous Return or TAPVR. https://www.cdc.gov/ncbddd/heartdefects/tapvr.html

CDC- Center for Disease Control and Prevention. (2022h, Jan 24). Congenital Heart Defects: Living with a Congenital Heart Defect. https://www.cdc.gov/ncbddd/heartdefects/living.html

MyHealth.Alberta.ca. (2021, April 29). Health Information and Tools: Learning About Mild Pulmonary Valve Stenosis in Newborns. https://myhealth.alberta.ca/Health/aftercareinformation/pages/conditions.aspx?hwid=abs1110#:~:text=Pulmonary%20valve%20stenosis%20is%20a,to%20pick%20up%20new%20oxygen.

Contact info: info@nursesinternational.org

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References:

Nursing Care Plan (NCP) for Congenital Heart Defects. https://nursing.com/lesson/nursing-care-plan-for-congenital-heart-defects/

Szymanski, M. W., Moore S.M., Kritzmire, S.M. (2021, Nov 8). Transposition Of The Great Arteries. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK538434/

Contact info: info@nursesinternational.org

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