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Prepared by:

Genorga, Christian Marcos NCF-LBSN2A

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.

Blood then flows from the aorta into the branches of many smaller arteries. Blood leaves the left ventricle through the aortic valve and enters the aorta.  After oxygen in the blood is released to the tissues. and blood vessels make up the circulatory system of the human body.   .  The right ventricle then pumps deoxygenated blood through the pulmonic valve into the lungs.  The left ventricle pumps oxygen-rich blood out to the rest of the body. which appears blue. The oxygen-rich blood. then returns to the left atrium and enters the left ventricle. and consists of four chambers — the left atrium and left ventricle and the right atrium and right ventricle. the largest artery (a blood vessel that carries oxygenated blood) in the body. The heart is the central pump of the circulatory system. the now deoxygenated (oxygenpoor) blood returns to the heart through veins.How a Healthy Heart Works The heart. lungs. enters the right atrium of the heart and then travels across the tricuspid valve into the right ventricle. This blood. providing the body's organs and tissues with the oxygen and nutrients they need. which appears red. the blood vessels that carry deoxygenated blood. The oxygen in the air we breathe binds to cells within this blood that is being pumped through the lungs. where it is pumped out to the body once again.   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.

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

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

Pulmonary Atresia         8. Hypoplastic Left Heart Syndrome 6.Coarctation of the Aorta (COA) 5. Tetralogy of Fallot (TOF) 10.Aortic Stenosis 2. Patent Ductus Arteriosus (PDA) 7. Total Anomalous Pulmonary Venous Connection 11.Pulmonary Stenosis 9.Types: CONGENITAL HEART DISEASE   1.Atrioventricular Canal Defect 4. Transposition of the Great Arteries    .Atrial Septal Defect (ASD) 3.

often. .   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. a condition called aortic regurgitation. an additional hole between the lower chambers of the heart (the ventricles). which increases strain on the heart because the left ventricle has to pump harder to send blood out to the body.   Atrial Septal Defect (ASD) ASD is a hole in the wall (called the septum) that separates the left atrium and the right atrium. the aortic valve is stiffened and has a narrowed opening (a condition called stenosis). Instead of two separate valves allowing flow into the heart (tricuspid on the right and mitral valve on the left). It does not open properly. Typically there is a large hole between the upper chambers of the heart (the atria) and.Types: CONGENITAL HEART DISEASE   Aortic Stenosis In aortic stenosis. Sometimes the aortic valve also does not close properly. there is one large common valve. which may be quite malformed. causing it to leak. Atrioventricular canal defect is commonly seen in children with Down syndrome.

at which point the baby may be critically ill. and the aortic valve) are underdeveloped.    Hypoplastic Left Heart Syndrome When the structures of the left side of the heart (the left ventricle. the mitral valve. Fortunately.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. 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. This condition is usually diagnosed within the first few days of life.   . they're unable to pump blood adequately to the entire body. many of these infants are recognized to have serious heart disease even before birth on ultrasound tests.

 . If the DA doesn't close.) The DA usually closes on its own shortly after birth. 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. which can result in too much blood flow to a newborn's lungs. It does not open properly.    Pulmonary Atresia In this defect the pulmonic valve does not open at all and may indeed be completely absent.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 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. then a condition called patent ductus arteriosus (PDA) results. PDA is common in premature babies. it is no longer needed once a newborn breathes on his own. the pulmonic valve is stiffened and has a narrowed opening (a condition called stenosis).

Instead they deliver blood to the heart by other pathways. Pressure builds up in this pathway and in the pulmonary veins. It includes pulmonary stenosis. children with this defect often appear bluish. a thickened right ventricle (known as ventricular hypertrophy).    Total Anomalous Pulmonary Venous Connection The pulmonary veins normally are the blood vessels that deliver oxygenated blood from the lungs to the left atrium. These infants often have difficulty breathing and appear bluish.Types: CONGENITAL HEART DISEASE  Tetralogy of Fallot (TOF) Tetralogy of Fallot is actually a combination of four heart defects. pushing fluid into the lungs. Because deoxygenated (blue) blood can flow out to the body. . Sometimes these vessels don't join the left atrium during development. instead of draining just the left. which may be narrowed. 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. decreasing the amount of oxygenated blood that reaches the body.

The right ventricle therefore does not receive blood normally and is often small. . In tricuspid atresia.    Tricuspid Atresia Blood normally flows from the right atrium to the right ventricle through the tricuspid valve. The result is that babies with this condition often appear very blue and have low oxygen levels in the bloodstream. the valve is replaced by a plate or membrane that does not open. receives red blood and sends it back to the lungs again. The pulmonary artery arises from the left side of the heart.Types: CONGENITAL HEART DISEASE  Transposition of the Great Arteries In this condition. They usually come to medical attention within the first days of life. which is sent right back out to the body without becoming oxygen-rich. 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.

that vessel splits to form the two major arteries.    Ventricular Septal Defect (VSD) One of the most common congenital heart defects.Types: CONGENITAL HEART DISEASE  Truncus Arteriosus In an embryo. These can occur at different locations and vary in size from very small to very large. During normal development. the child is born with a single common great blood vessel called the truncus arteriosus. Some of the smaller defects may gradually close on their own. . VSD is a hole in the wall (septum) between the heart's left and right ventricles. If that split does not occur. the aorta and the pulmonary artery are initially a single vessel. There is usually a hole between the ventricles associated with this defect.

especially during feedings diminished strength of the baby's pulse  . they often produce telltale signs such as:  a bluish tinge or color (cyanosis) to the lips.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. 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.

Diagnosing a Heart Defect An electrocardiogram (EKG) Echocardiogram Cardiac catheterization  .

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

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

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

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

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

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

 * Explain progressive activity schedule and signs of overexertion.  . * Explain symptoms and interventions for decreased cardiac output related to etiological factors. purpose.CONGENITAL HEART DISEASE   Education/Continuity of Care * Explain drug regimen.   * Explain diet restrictions (fluid. dose. and side effects. sodium).

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