CONGENITAL HEART DISEASE

Prepared by:

Genorga, Christian Marcos NCF-LBSN2A

CONGENITAL HEART DISEASE

Congenital heart defects are abnormalities in the heart's structure that are present at birth. -defect in the structure of the heart and great vessels. -Happens because of incomplete or abnormal development of the fetus' heart during the very early weeks of pregnancy. Some are known to be associated with genetic disorders, such as Down syndrome, but the cause of most congenital heart defects is unknown. -While they can't be prevented, there are many treatments for the defects and any related health problems. -Approximately 8 out of every 1,000 newborns have congenital heart defects, ranging from mild to severe.

How a Healthy Heart Works

The heart, lungs, and blood vessels make up the circulatory system of the human body. The heart is the central pump of the circulatory system, and consists of four chambers the left atrium and left ventricle and the right atrium and right ventricle.

The heart also has four valves that direct the flow of blood through the heart:

The left atrium of the heart receives oxygen-rich blood from the lungs and then empties into the left ventricle through the mitral valve.

The left ventricle pumps oxygen-rich blood out to the rest of the body. Blood leaves the left ventricle through the aortic valve and enters the aorta, the largest artery (a blood vessel that carries oxygenated blood) in the body. Blood then flows from the aorta into the branches of many smaller arteries, providing the body's organs and tissues with the oxygen and nutrients they need.

After oxygen in the blood is released to the tissues, the now deoxygenated (oxygenpoor) blood returns to the heart through veins, the blood vessels that carry deoxygenated blood. This blood, which appears blue, enters the right atrium of the heart and then travels across the tricuspid valve into the right ventricle.

The right ventricle then pumps deoxygenated blood through the pulmonic valve into the lungs. The oxygen in the air we breathe binds to cells within this blood that is being pumped through the lungs. The oxygen-rich blood, which appears red, then returns to the left atrium and enters the left ventricle, where it is pumped out to the body once again.

This is the normal pathway that blood travels through the heart and the body. However, abnormalities in the heart's structure such as congenital heart defects can affect its ability to function properly.

CONGENITAL HEART DISEASE

Related Factors:
1.Increased or decreased ventricular filling (preload) 2.Alteration in afterload 3.Impaired contractility 4.Alteration in heart rate, rhythm, and conduction 5.Decreased oxygenation 6.Cardiac muscle disease

Types: CONGENITAL HEART DISEASE

1.Aortic Stenosis

2.Atrial Septal Defect (ASD) 3.Atrioventricular Canal Defect 4.Coarctation of the Aorta (COA) 5. Hypoplastic Left Heart Syndrome 6. Patent Ductus Arteriosus (PDA) 7. Pulmonary Atresia

8.Pulmonary Stenosis 9. Tetralogy of Fallot (TOF) 10. Total Anomalous Pulmonary Venous Connection 11. Transposition of the Great Arteries

Types: CONGENITAL HEART DISEASE

Aortic Stenosis

In aortic stenosis, the aortic valve is stiffened and has a narrowed opening (a condition called stenosis). It does not open properly, which increases strain on the heart because the left ventricle has to pump harder to send blood out to the body. Sometimes the aortic valve also does not close properly, causing it to leak, a condition called aortic regurgitation.

Atrial Septal Defect (ASD)

ASD is a hole in the wall (called the septum) that separates the left atrium and the right atrium.

Atrioventricular Canal Defect

This defect also known as endocardial cushion defect or atrioventricular septal defect is caused by a poorly formed central area of the heart. Typically there is a large hole between the upper chambers of the heart (the atria) and, often, an additional hole between the lower chambers of the heart (the ventricles). Instead of two separate valves allowing flow into the heart (tricuspid on the right and mitral valve on the left), there is one large common valve, which may be quite malformed. Atrioventricular canal defect is commonly seen in children with Down syndrome.

Types: CONGENITAL HEART DISEASE

Coarctation of the Aorta (COA)

COA is a narrowing of a portion of the aorta, and often seriously decreases the blood flow from the heart out to the lower portion of the body.

Hypoplastic Left Heart Syndrome

When the structures of the left side of the heart (the left ventricle, the mitral valve, and the aortic valve) are underdeveloped, they're unable to pump blood adequately to the entire body. This condition is usually diagnosed within the first few days of life, at which point the baby may be critically ill. Fortunately, many of these infants are recognized to have serious heart disease even before birth on ultrasound tests. A fetal echocardiogram is a specialized ultrasound that allows doctors to see the baby's heart in great detail and plan the best care for the baby while still in utero.

Types: CONGENITAL HEART DISEASE

Patent Ductus Arteriosus (PDA)

The ductus arteriosus (DA) is a normal blood vessel in the developing fetus that diverts circulation away from the lungs and sends it directly to the body. (The lungs are not used while the unborn fetus is in amniotic fluid the fetus gets oxygen directly from the mother's placenta.) The DA usually closes on its own shortly after birth; it is no longer needed once a newborn breathes on his own. If the DA doesn't close, then a condition called patent ductus arteriosus (PDA) results, which can result in too much blood flow to a newborn's lungs. PDA is common in premature babies.

Pulmonary Atresia

In this defect the pulmonic valve does not open at all and may indeed be completely absent. The main blood vessel that runs between the right ventricle and the lungs also may be malformed and the right ventricle can be abnormally small.

Pulmonary Stenosis

In pulmonary stenosis, the pulmonic valve is stiffened and has a narrowed opening (a condition called stenosis). It does not open properly, which increases strain on the right side of the heart because the right ventricle has to pump harder to send blood out to the lungs.

Types: CONGENITAL HEART DISEASE

Tetralogy of Fallot (TOF)

Tetralogy of Fallot is actually a combination of four heart defects. It includes pulmonary stenosis, a thickened right ventricle (known as ventricular hypertrophy), a hole between the lower chambers (known as a ventricular septal defect), and an aorta that can receive blood from both the left and right ventricles, instead of draining just the left. Because deoxygenated (blue) blood can flow out to the body, children with this defect often appear bluish.

Total Anomalous Pulmonary Venous Connection

The pulmonary veins normally are the blood vessels that deliver oxygenated blood from the lungs to the left atrium. Sometimes these vessels don't join the left atrium during development. Instead they deliver blood to the heart by other pathways, which may be narrowed. Pressure builds up in this pathway and in the pulmonary veins, pushing fluid into the lungs, decreasing the amount of oxygenated blood that reaches the body. These infants often have difficulty breathing and appear bluish.

Types: CONGENITAL HEART DISEASE

Transposition of the Great Arteries

In this condition, the pulmonary artery and the aorta (the major blood vessels leaving the heart) are switched so that the aorta arises from the right side of the heart and receives blue blood, which is sent right back out to the body without becoming oxygen-rich. The pulmonary artery arises from the left side of the heart, receives red blood and sends it back to the lungs again. The result is that babies with this condition often appear very blue and have low oxygen levels in the bloodstream. They usually come to medical attention within the first days of life.

Tricuspid Atresia

Blood normally flows from the right atrium to the right ventricle through the tricuspid valve. In tricuspid atresia, the valve is replaced by a plate or membrane that does not open. The right ventricle therefore does not receive blood normally and is often small.

Types: CONGENITAL HEART DISEASE

Truncus Arteriosus

In an embryo, the aorta and the pulmonary artery are initially a single vessel. During normal development, that vessel splits to form the two major arteries. If that split does not occur, the child is born with a single common great blood vessel called the truncus arteriosus. There is usually a hole between the ventricles associated with this defect.

Ventricular Septal Defect (VSD)

One of the most common congenital heart defects, VSD is a hole in the wall (septum) between the heart's left and right ventricles. These can occur at different locations and vary in size from very small to very large. Some of the smaller defects may gradually close on their own.

Signs and Symptoms of Heart Defects

Because congenital defects often compromise the heart's ability to pump blood and to deliver oxygen to the tissues of the body, they often produce telltale signs such as:

a bluish tinge or color (cyanosis) to the lips, tongue and/or nailbeds an increased rate of breathing or difficulty breathing poor appetite or difficulty feeding (which may be associated with color change) failure to thrive (failure to gain weight or weight loss) abnormal heart murmur sweating, especially during feedings diminished strength of the baby's pulse

Diagnosing a Heart Defect

An electrocardiogram (EKG) Echocardiogram Cardiac catheterization

CONGENITAL HEART DISEASE

Ongoing Assessment

* Assess mentation.--Restlessness is noted in the early stages; severe anxiety and confusion are seen in later stages. * Assess heart rate and blood pressure.--Sinus tachycardia and increased arterial blood pressure are seen in the early stages; BP drops as the condition deteriorates. Elderly patients have reduced response to catecholamines, thus their response to reduced cardiac output may be blunted, with less rise in heart rate. Pulsus alternans (alternating strongthen-weak pulse) is often seen in heart failure patients. * Assess skin color and temperature.--Cold, clammy skin is secondary to compensatory increase in sympathetic nervous system stimulation and low cardiac output and desaturation. * Assess peripheral pulses.--Pulses are weak with reduced cardiac output.

CONGENITAL HEART DISEASE

Ongoing Assessment: (cont.)

* Assess fluid balance and weight gain.--Compromised regulatory mechanisms may result in fluid and sodium retention. Body weight is a more sensitive indicator of fluid or sodium retention than intake and output. * Assess heart sounds, noting gallops, S3, S4.--S3 denotes reduced left ventricular ejection and is a classic sign of left ventricular failure. S4 occurs with reduced compliance of the left ventricle, which impairs diastolic filling. * Assess lung sounds. Determine any occurrence of paroxysmal nocturnal dyspnea (PND) or orthopnea.--Crackles reflect accumulation of fluid secondary to impaired left ventricular emptying. They are more evident in the dependent areas of the lung. Orthopnea is difficulty breathing when supine. PND is difficulty breathing that occurs at night. * Monitor continuous ECG as appropriate.

CONGENITAL HEART DISEASE

Ongoing Assessment: (cont.)

* Monitor ECG for rate; rhythm; ectopy; and change in PR, QRS, and QT intervals.--Tachycardia, bradycardia, and ectopic beats can compromise cardiac output. Elderly patients are especially sensitive to the loss of atrial kick in atrial fibrillation. * Assess response to increased activity.--Physical activity increases the demands placed on the heart; fatigue and exertional dyspnea are common problems with low cardiac output states. Close monitoring of patients response serves as a guide for optimal progression of activity. * Assess urine output. Determine how often the patient urinates.-Oliguria can reflect decreased renal perfusion. Diuresis is expected with diuretic therapy. * Assess for chest pain.--This indicates an imbalance between oxygen supply and demand. * Assess contributing factors so appropriate plan of care can be initiated.

CONGENITAL HEART DISEASE

Therapeutic Interventions

* Administer medication as prescribed, noting response and watching for side effects and toxicity. Clarify with physician parameters for withholding medications.--Depending on etiological factors, common medications include digitalis therapy, diuretics, vasodilator therapy, antidysrhythmics, ACE inhibitors, and inotropic agents. * Maintain optimal fluid balance. For patients with decreased preload, administer fluid challenge as prescribed, closely monitoring effects.--Administration of fluid increases extracellular fluid volume to raise cardiac output.

CONGENITAL HEART DISEASE

Therapeutic Interventions (cont.)

* Maintain adequate ventilation and perfusion, as in the following: o Place patient in semi- to high-Fowlers position. This reduces preload and ventricular filling. o Place in supine position. This increases venous return, promotes diuresis. o Administer humidified oxygen as ordered. The failing heart may not be able to respond to increased oxygen demands. * Maintain physical and emotional rest, as in the following: o Restrict activity. This reduces oxygen demands. o Provide quiet, relaxed environment. Emotional stress increases cardiac demands. o Organize nursing and medical care. This allows rest periods. - Monitor progressive activity within limits of cardiac function.

CONGENITAL HEART DISEASE

Therapeutic Interventions (cont.)

* Administer stool softeners as needed.--Straining for a bowel movement further impairs cardiac output. * Monitor sleep patterns; administer sedative.--Rest is important for conserving energy. * If arrhythmia occurs, determine patient response, document, and report if significant or symptomatic. o Have antiarrhythmic drugs readily available. o Treat arrhythmias according to medical orders or protocol and evaluate response.--Both tachyarrhythmias and bradyarrhythmias can reduce cardiac output and myocardial tissue perfusion.

CONGENITAL HEART DISEASE

Education/Continuity of Care
* Explain drug regimen, purpose, dose, and side effects.

* Explain symptoms and interventions for decreased cardiac output related to etiological factors.

* Explain progressive activity schedule and signs of overexertion.

* Explain diet restrictions (fluid, sodium).

Fin..