Congestive Heart Failure in Children

Congestive Heart Failure

-occurs when a cardiac output is inadequate to meet the demands of the body and results in the accumulation of excessive blood volume in the pulmonary and/ or systemic venous system.

or Congestive heart failure (CHF) is a condition in which the heart cannot effectively meet the oxygen needs of the body's other organs. It actually is not as much a specific diagnosis but rather a collection of symptoms that can be the result of a variety of different heart problems

CHF usually results from:

Congenital hearts defects Post cardiac surgery Rheumatic Fever Severe anemia Hypocalcaemia Myocarditis

CAUSES:
Congenital heart disease (primary cause of first 3 years of life) Acquired heart disease- rheumatic heart disease (caused by damage to the heart from group A strep infections), endocarditis, myocarditis Noncardiovascular causes- acidosis, pulmonary disease, various metabolic disease cardiac malformations, such as tetralogy of Fallot abnormalities of the heart valves underdevelopment of one or both ventricles coarctation of the aorta, which is a narrowing of the vessel bringing blood to the heart

Non cardiac causes:

1. 2. 3. 4. 5. Volume overload HPN Anemia Sepsis Renal Failure

SIGNS AND SYMPTOMS

Tachycardia Venous congestion Right-sided Hepatomegaly Ascites Edema Increase venous pressure Left-sided Tachypnea Dyspnea Orthopnea Cyanosis Rales/ crackles Pulmonary edema Low cardiac output Fatigue or low energy, Pallor Difficulty of feeding Sweating/ diaphoresis Poor growth Dizziness Altered consciousness Generalized edema Abrupt gain weight

LEFT SIDE CHF

Mitral valve stenosis Aortic stenosis Ischemic Heart Disease

Overworking of the left side of the heart

Pulmonary Congestion

Failure of the left side to contract properly

Dyspnea, wheezing, Rales/crackles, Productive cough, Salivation, cyanosis

 RIGHT SIDE CHF

Tricuspid valve stenosis Pulmonary stenosis, COPD, Pulmonary embolism, Left sided heart failure

Overworking of the right side of the heart

Venous Congestion

Failure of the right side to contract properly

Jugular vein distention Weight gain, dependent pitting edema, ascites, Hepato-splenomegaly Esophageal varices, jaundice

VENTRICULAR SEPTAL DEFECT, or holes in the walls that separate the left and right sides of the heart

A PATENT DUCTUS ARTERIOSUS is a blood vessel between the aorta and main pulmonary artery that all babies require in fetal life but which usually closes within the first couple of days of life.

Some babies are born with other connections between the two main arteries leaving the heart, i.e., aortopulmonary window or truncus arteriosus. These babies are also at risk for having too much blood flow to the lungs. Holes between the two upper chambers of the heart (atrial septal defects) rarely cause problems with congestive heart failure no matter how large.

In older children where the structure of the heart is normal, it is usually due to a weakening of the heart muscle, or cardiomyopathy, or Kawasaki Disease, which all can lead to congestive heart failure.

CARDIOMYOPATHY can also be seen in babies and can be due to a number of problems such as rhythm disturbances or infections.

KAWASAKI's disease-also known as motorcycle syndrome, lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart.

Other CAUSES:
complications of open heart surgery chronic anemia, which results in a low red blood cell count poor nutrition drug toxicity

Pathophysiology:
Congenital Heart Disorder Acquired Heart Diseases Noncardiovascular causes cardiac malformations abnormalities of the heart valves underdevelopment of one or both ventricles coarctation of the aorta ventricular septal defects patent ductus arteriosus complications of open heart surgery
ENLARGEMENT OF VENTRICLES

BLOOD CANNOT BE PUSHED FORWARD EFFECTIVELY

BLOOD CANNOT BE PUSHED FORWARD EFFECTIVELY

RIGHT VENTRICLE Back up of blood into systemic circulation Venous pressure Cardiac Output to lungs Edema of extremities & other body organs including brain, distended neck veins, flushed face, headache, shortness of breath, dyspne LEFT VENTRICLE Back up of blood to pulmonary veins High pressure in pulmonary capillaries, cardiac output to system Pulmonary congestion, rales, cough, pallor, weakness, fatigue DECREASE RENAL BLOOD FLOE

Stimulation of reninangiotensin system and aldosterone secretion

Stimulation of thirsts center and sodium retention

Tachycardia, edema

Clinical Manifestations:
Dyspnea and tachypnea Tachycardia Orthopnea Peripheral edema Feeding difficulties, anorexia Easy fatigability Restlessness Pallor or grayish tint to the skin Weight gain

Diaphoresis Growth Failure or failure to thrive, meaning that the child's growth and weight gain are slower than expected Non productive, irritative cough Neck vein distention Hepatomegaly pain and tenderness of the abdomen coolness of extremities to the touch

Diagnostic Evaluation:
Congestive heart failure is diagnosed on the basis of the child's medical history and physical exam. Identification of the underlying disease may require special tests, including:

Palpation
May have weak peripheral pulses Hepatomegaly ( feature of right heart failure) Abnormal precordial activity may occur

Auscultation Gallop rhythm ( frequent) Cardiac murmurs may or may not be present Crackles (infrequent in infants)

Chest X-rays- cardiomegaly; pulmonary congestion Electrocardiogram, or ECG, which graphs the electrical activity of the heart Echocardiography, which uses ultrasound waves to provide information about the structure, function, and motion of the

Cardiac catheterization, which involves injection of a contrast agent to allow the doctor to watch the blood flow through the heart and its arteries Laboratory data Dilutional Hyponatremia Hypochloremia Hyperkalemia

Pharmacological Treatment:

digoxin - a medication that helps strengthen the heart muscle, enabling it to pump more efficiently. - Side effects: loss of appetite, nausea or vomiting, headaches, irregular heartbeat or skipped heart diuretics - helps the kidneys remove excess fluid from the body. - Side effect: fatigue, decrease blood pressure, kidney complications, excessive loss of potassium

potassium-sparing diuretics - helps the body retain potassium, an important mineral that is often lost when taking diuretics. -Side Effect: On their own this group of drugs may raise potassium levels beyond the normal range, termed hyperkalemia, which risks potentially fatal arrhythmias. potassium supplements - replaces the potassium lost when taking diuretics.

ACE (angiotensin-converting enzyme) inhibitors - dilates the blood vessels, making it easier for the heart to pump blood forward into the body. -Dizziness, headache, diarrhea, constipation, loss of appetite, nausea, loss of taste, flushing, fatigue, cough or increased urination may occur beta blockers - decrease the heart rate and blood pressure, and improve heart function by blocking the stress hormone adrenalin. - You may experience dizziness, lightheadedness, drowsiness, and blurred vision as your body adjusts to the medication.

SURGICAL MANAGEMENT:

ARTIFICIAL PACEMAKER -used for a small battery-operated device that helps the heart beat in a regular rhythm. Some are permanent (internal) and some are temporary (external). An artificial pacemaker can replace a defective natural pacemaker or blocked pathway.

CARDIAC ABLATION

- In cardiac ablation, a form of energy renders a small section of damaged tissue inactive. This puts an end to arrhythmias that originated at the problematic site.

Left ventricular assist devices, or LVADs

-used to mechanically pump blood through the hearts of individuals with heart failure as they await transplantation, can reverse reduced heart muscle performance.
Extracorporeal membrane oxygenation (ECMO) -is an extracorporeal technique of providing both cardiac and respiratory support oxygen to patients whose heart and lungs are so severely diseased that they can no longer serve their function.

Heart transplantation or cardiac transplantation -is a surgical transplant procedure performed on patients with end-stage heart failure or severe coronary artery disease -is an open-heart surgery in which a severely diseased or damaged heart is replaced with a healthy heart from a recently deceased person

NURSING MANAGEMENT:

Improved myocardial efficiency

Administer Digoxin as prescribed by the physician Carefully calculate dosage; it is given in very small amount in children and infant Count apical pulse in 1 full minute before administering Observe for vomiting and report to the physician Observe for the development of premature ventricular contraction when digoxin is initially started and report to the physician Be aware of signs of digitalis intoxication altered emotional status digitalis blues decreased appetite Bradycardia Arrythmias gastrointestinal symptoms

 Reduce energy requirements Avoid necessary activities such as frequent complete baths and clothing changes Prevent excessive crying Use pacifier Hold baby Eliminate sources of distress (hunger, wet diapers, etc.) Remove accumulated sodium and fluid Administer diuretics as prescribed by the physician.

 Hypokalemia may cause weakened myocardial contractions and may precipitate digoxin toxicity. Oral potassium supplements may be indicated when a child is on diuretics for an extended period of time. Restrict sodium intake The child may be placed on a low-sodium diet. Be aware of the prescribed diet and the amount of sodium in foods and fluids offered to the child. Question the child about his likes and dislikes so that the diet can be made as appealing as possible. Interpret the diet and its purpose to the child and his parents. Infants may require low-sodium formulas.

Be aware of the sidE effects of the prescribed medication. Weigh the child at least daily to observe response. Maintain an accurate record of intake and output. Record urine specific gravity. Encourage foods such as bananas and orange juice that have a high potassium content to prevent potassium depletion associated with many diuretics.

 Relieve the respiratory distress associated with pulmonary engorgement Improve tissue oxygenation Administer oxygen therapy Maintain the infant in a neutral thermal environment. Provide adequate nutrition to meet the caloric requirements of the child Provide foods that the child enjoys in small amount, because he may have a poor appetite due to liver enlargement. Infant feeding Feed frequently in small amounts. Feed slowly in sitting position, allowing frequent rest periods. Supplement oral feedings with gavage feeding if the infant is unable to take an adequate amount of formula by mouth.

Obstructive Defect
An obstruction defect is a type of defect where one of the valves or ventricles is narrowed to such a degree that it partially or completely blocks the flow of blood. There are several types of obstruction defects, depending upon where the malformation occurs.

Types of Obstruction Defects

Pulmonary stenosis Aortic stenosis Coarctation of the aorta

Symptoms of Obstruction Defects

Cyanosis (a bluish color to the skin due to lack of blood oxygen) Chest pain Unusual fatigue Dizziness More serious symptoms include congestive heart failure or high blood pressure.

Pulmonary Stenosis

In children, these problems can include:

a valve that has leaflets that are partially fused together. a valve that has thick leaflets that do not open all the way. the area above or below the pulmonary valve is narrowed.

different types of pulmonary stenosis:

valvar pulmonary stenosis supravalvar pulmonary stenosis subvalvar (infundibular) pulmonary stenosis branch peripheral pulmonic stenosis

Causes of pulmonary stenosis

improper development of the pulmonary valve in the first 8 weeks of fetal growth a number of factors, though most of the time this heart defect occurs sporadically (by chance) with no clear reason evident for its development.

Symptoms of pulmonary stenosis

heavy or rapid breathing shortness of breath fatigue rapid heart rate swelling in the feet, ankles, face, eyelids, and/or abdomen fewer wet diapers or trips to the bathroom

Diagnosis:

chest x-ray electrocardiogram (ECG or EKG) echocardiogram (echo) cardiac catheterization

Treatment
Specific treatment for pulmonary stenosis will be determined by your child's physician based on: your child's age, overall health, and medical history extent of the condition your child's tolerance for specific medications, procedures, or therapies expectations for the course of the condition your opinion or preference

 balloon dilation or valvuloplasty

Valvotomy Valvectomy (with or without transannular patch) pulmonary valve replacement

Post-procedure care for your child:

cath lab interventional procedure surgical repair
Ventilator intravenous (IV) catheters arterial line nasogastric (NG) tube urinary catheter chest tube heart monitor

Long-term outlook after pulmonary stenosis repair:

recommends antibiotics be given to prevent bacterial endocarditis after discharge from the hospital. repeat interventional cath lab procedures may be necessary during infancy and childhood to stretch the valve open. Replacement of the pulmonary valve. Regular follow-up care.

AORTIC STENOSIS

TYPES: VALVULAR STENOSIS SUBVALVULAR STENOSIS SUPRAVALVULAR STENOSIS

Aortic Stenosis

Aortic Valve
found between the left ventricle and the aorta. It has three leaflets that function like a oneway door, allowing blood to flow forward into the aorta, but not backward into the left ventricle.

Aortic Valve

In children, these problems can include a valve that: bicuspid aortic valve leaflets that are partially fused together. has thick leaflets that do not open all the way. becomes damaged by rheumatic fever or bacterial endocarditis. area above or below the valve is narrowed (supravalvar or subvalvar).

Bicuspid Aortic Valve

Causes
improper development of the aortic genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Acquired aortic stenosis may occur after a strep infection that progresses to rheumatic heart fever.

DEGENERATION AND CALCIFICATION

BICUSPID AORTIC VALVE

BICUSPID AORTIC VALVE

AORTIC VALVE BEGINS TO DEGENERATE

CALCIUM ACCUMULATES IN THE VALVE

IRREGULAR ROCK LIKE DEPOSITS

DEPOSIT INFRINGE ON THE VALVULAR OPENING

NARROWING OF AORTIC VALVE

CAUSES: UNKNOWN(CONGENITAL) DEGENERATION OR CALCIFICATION BICUSPID AORTIC VALVE RHD

NARROWING OR STRICTURE OF AORTIC VALVE

RESISTANCE TO BLOOD FLOW

DECREASED CARDIAC OUTPUT

LEFT VENTRICULAR HYPERTROPHY

PULMONARY VASCULAR CONAGESTION and POSSIBLE PULMONARY EDEMA

SIGNS AND SYMPTOMS

A characteristic murmur is present Infant severe defects demonstrates signs of: decreased cardiac output faint pulses Hypotension tachycardia poor feeding(too sleepy to feed)

 Children show signs of : -exercise in tolerance - chest pain - dizziness when standing long period of time

DIAGNOSIS:
Ultrasound Scan oxygen saturation monitor chest x-ray EKG (electrocardiogram) Echocardiography Cardiac catheterization

Treatment
Non Surgical treatment for valvular aortic stenosis: Balloon angioplasty during cardiac catheterization to dilate narrowed valve Surgical treatment for valvular aortic atenosis: Aortic valvotomy under inflow occlusion (palliative);valve replacement may be required a second procedure Surgical treatment for Subvalvular Aortic Stenosis: may involve incising a membrane if one exists or cutting the fibromuscular ring; patch may be required

SURGICAL TREATMENT FOR SUBVALVULAR STENOSIS

In cases where the narrowing in the aorta is above the aortic valve (supravalvular stenosis), the obstructing portion is removed and the remaining parts of the vessel are stitched together

SURGICAL TREATMENT FOR SUPRAVALVULAR STENOSIS

VALVOTOMY aortic valve replacement aortic homograft pulmonary homograft (Ross procedure)

Post Operative Care

Ventilator intravenous (IV) catheters arterial line nasogastric (NG) tube urinary catheter chest tube heart monitor

COARCTATION OF THE AORTA

Types

Preductal coarctation Ductal coarctation Postductal coarctation

PATHOPHYSIOLOGY
CoA imposes significant afterload on the left ventricle (LV), which results in increased wall stress and compensatory ventricular hypertrophy. The afterload may be imposed acutely, as occurs following closure of the ductus arteriosus in neonates with severe coarctation. These infants may rapidly develop CHF and shock. Rapid constriction of the ductus arteriosus, producing sudden severe aortic obstruction, seems to be the most likely explanation.

As the ductus (aortic end) constricts, the left ventricular afterload rapidly increases, with a resultant increase in left ventricular pressures (systolic and diastolic). This causes elevation of the left atrial pressure, which may open the foramen ovale, causing left-to-right shunt and dilatation of the right atrium and right ventricle. If the foramen ovale does not open, pulmonary venous pressures and pulmonary artery pressures increase, and right ventricular dilatation develops. Cardiomegaly revealed by chest roentgenography and right ventricular hypertrophy seen on ECG and echocardiography are related to the indirect effects of rapid development of severe aortic obstruction.

The stroke volume, ejected into the limited aortic receptacle, produces a higher pressure proximal to coarctation. However, this theory does not explain the following:

The lack of relationship between the degree of elevation of blood pressure and the magnitude of obstruction The increased peripheral vascular resistance distal to the site of obstruction The delayed or lack of reduction of blood pressure immediately following relief of obstruction

ASSESSMENT

If the coarctation is slight:

absence of palpable femoral pulses Children who have an obstruction proximal to the left subclavian artery:

absent brachial pulses leg pain obvious nodules on the ribs

DIAGNOSIS:

History
Physical examination Lab Studies Imaging Studies

Lab Studies:

Laboratory studies in neonatal patients who present in shock include the following:
Septic workup includes blood, urine, and cerebral spinal fluid (CSF) cultures. Electrolyte levels, BUN, creatinine, and glucose concentrations should be tested. Measure arterial blood gases and serum lactate levels.

Laboratory studies in older patients who present with hypertension include:

Urinalysis Electrolyte levels

BUN
Creatinine Glucose concentrations.

Imaging Studies

Chest radiography Echocardiography

Instantaneous peak pressure

MRI and CT

Other Tests:

Electrocardiography

Preductal and postductal pulse oximetry

MEDICAL CARE
Early presentation Treatment in patients with congestive heart failure (CHF) includes the use of diuretics and inotropic drugs. Prostaglandin E1 (0.05-0.15 mcg/kg/min) is infused intravenously to open the ductus arteriosus. Ventilatory assistance is provided to patients with markedly increased work of breathing.

 Infusion of inotropic drugs (dopamine, dobutamine, epinephrine) is useful when ventricular dysfunction is present, especially with hypotension.
A Foley catheter is inserted to monitor renal perfusion and urine output. Arterial blood gases are tested to monitor acidosis.

 An umbilical artery catheter may be placed in neonates to assess the response to prostaglandin infusion with regard to improving lower-body blood flow. Patients stabilized by the above interventions are better candidates for surgical or catheter intervention. In the presence of associated defects, the significance of coarctation on the clinical course of the patient should be assessed with echo-Doppler and/or catheterization and angiographic studies.

Late presentation

Treatment of hypertension
Preoperative hypertension can be effectively treated using beta-blockers. Postoperative hypertension can be treated short-term with vasodilators, such as sodium nitroprusside, and intravenous beta-blockers, such as esmolol.

Evaluate associated abnormalities, such as aortic stenosis, subaortic stenosis, or mitral valve disease.

Evaluate adequacy of collateral blood vessels to assess the safety of surgical intervention

SURGICAL CARE

balloon angioplasty Stents Resection and end-to-end anastomosis patch aortoplasty left subclavian flap aortoplasty tubular bypass grafts

Balloon Angioplasty

NURSING MANAGEMENT
Diet Persistent hypertension has been shown to increase the incidence of coronary artery disease (CAD); therefore, periodically examine patients who have undergone CoA repair for hypertension and recommend a healthy low-fat diet. Measure cholesterol levels and intervene pharmacologically in older patients as indicated, with a total cholesterol goal of less than 200 g/dL. Patients with persistent hypertension may require varying degrees of salt restriction. Emphasize dietary counseling and avoidance of obesity and smoking.

Activity

Patients with CoA and hypertension who are awaiting surgical repair should limit heavy isometric exercises to a degree commensurate with the degree of hypertension. Generally, the duration of hypertension after CoA repair is related in part to the duration of hypertension prior to diagnosis and repair of coarctation. Patients who undergo repair of coarctation in infancy usually remain normotensive in the absence of significant residual arch obstruction and require no specific activity restrictions or limitations. With growth, coarctation may recur, and some patients may be normotensive at rest but have significant upper extremity hypertension provoked by exercise. Such patients who desire to participate in competitive athletics should undergo exercise stress testing prior to clearance.

 Patients who undergo repair later in life and who have had a significant period of preoperative hypertension are at particular risk for sustained postoperative hypertension, which may be permanent. Restrict heavy isometric exercise and other activities in these patients, commensurate with the degree of hypertension and BP control. Use exercise testing to assess BP response to exercise as a means of delineating reasonable exercise limitations.

Cardiac Surgery
Surgery on the heart and or the blood vessels

Closed Heart Surgery

Cardiac catheterization

Open Heart surgery

Operation under hypothermia

2. Temperature and notify the physician if a fever occurs.

 Maintain aseptic technique Monitor for sign of sepsis Fever chills Diaporesis lethargy Altered level of consciousness

Monitors lines, tubes, or catheters that are in place and remove promptly as prescribed when no longer needed, to prevent infection. Assess for signs of discomfort: Irritability Change in heart rate, respiratory rate, BP Inability to sleep

 Administer pain medication as prescribed noting effectiveness Administered antibiotic and antipyretic as prescribed. Encouraged rest period. Facilitate parent child contact as soon as possible.

Defects with decreased Pulmonary Blood Flow

TETRALOGY OF FALLOT

Tetralogy of Fallot
is a congenital heart defect which is classically understood to involve four anatomical abnormalities It is the most common cyanotic heart defect, representing 55-70%, and the most common cause of blue baby syndrome. It was described in 1672 by Niels Stensen, in 1673 by Edward Sandifort, and in 1888 by the French physician tienne-Louis Arthur Fallot , for whom it is named.

"Tetralogy" - refers to four heart problems -four cardiac abnormalities characteristic of TOF 1. 2. 3. 4. A ventricular septal defect(VSD) Pulmonary stenosis Overriding aorta Right ventricular hypertrophy

Epidemiology
TOF occurs in approximately 3 to 6 per 10,000 births and represents 5-7% of congenital heart defects. Its cause is thought to be due to environmental or genetic factors or a combination. It is associated with chromosome 22 deletions and diGeorge syndrome It occurs slightly more often in males than in females. Embryology studies show that it is a result of anterior malalignment of the septum , resulting in the clinical combination of a VSD, pulmonary stenosis, and an overriding aorta. Right ventricular hypertrophy results from this combination, which causes resistance to blood flow from the right ventricle.

Risk factors
Genetic factors Maternal abuse of alcohol during pregnancy, leading to fetal alcohol syndrome (FAS) Mothers who take medications to control seizures Mothers with phenylketonuria (PKU) Most of the time, this heart defect occurs sporadically (by chance), with no clear reason evident for its development.

Pathophysiology
Decreased pulmonary blood flow
VSD and overriding aorta Mixing of oxygenated and unoxygenated blood Blood shunt from right to left ventricle and vice versa Reduces blood flow to the lungs Diluted and O2 poor blood reaches the body Low O2 saturation Tet or pink spells
Hypertrophy of the right ventricle

Pressure builds up in the right side of the heart

A Healthy Heart Cross-Section

Tetralogy of Fallot

Tet spells Infrequently, babies with tetralogy of Fallot will suddenly develop the ff. deep blue skin, nails and lips after crying, feeding or upon awakening. these episodes are called "Tet spells" and result from a rapid drop in the amount of oxygen in the blood. Toddlers or older children may instinctively squat when they are short of breath. Squatting increases blood flow to the lungs.

A baby experiencing tet spell

Signs and symptoms

A bluish coloration of the skin caused by blood low in oxygen (cyanosis) Shortness of breath and rapid breathing Loss of consciousness (fainting) Clubbing of fingers and toes (an abnormal, rounded shape of the nail bed) Lack of appetite Poor weight gain Tiring easily during play Irritability

Diagnostic tests
Echocardiography

Chest x-ray

Cardiac catherization

Complete blood count

Electrocardiogram

Treatment
Specific treatment for tetralogy of Fallot will be determined by your child's physician based on: your child's age, overall health, and medical history extent of the condition your child's tolerance for specific medications, procedures, or therapies expectations for the course of the condition your opinion or preference

Medical Management
Emergency management of tet spells (acute hypoxia) Beta blockers Propanolol acute episode Morphine, Phenylephrine O2 therapy not effective Squatting in the knee chest position

Surgical Management
Palliative surgery Will redirect a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month old Eileen Saxon on November 29, 1944 with dramatic results.

The Pott shunt Waterson procedure - are other shunt procedures which were developed for the same purpose. 2. Total Surgical Repair - first total surgical repair was performed in 1954 - This first total repair was performed by C. Walton Lillehei at the University of Minnesota in 1954 on a 10-month boy

Total surgical Repair

The surgery generally involves making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle, and repairing the VSD using a Gore-Tex patch or a homograft. Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.

Nursing Management

PRE-OPERATIVE CARE
If at all possible, it is important that the patient be free of infection prior to going to surgery. If the patient is taking aspirin, contact the cardiologist to ask when to discontinue taking the aspirin. Early cross-matching of the blood prior to operation If the patient is taking blood thinners such as Coumadin or Lovenox, . These patients may need to discontinue these medications and convert to Heparin prior to surgery.

 The patient must undergo pre-operative work-out in order to attain the very best surgical outcome for the patient NPO post midnight the night before the operation. Nursing Management

POST-OPERATIVE CARE
patient will continue to be monitored closely.(Neurologic and vital signs) the patient must be free of infection continually assess the patients comfort level

Tricuspid Atresia

a heart defect present at birth (congenital) in which one of the valves (tricuspid valve) between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers. If your baby is born with tricuspid atresia, blood cannot flow through the heart and into the lungs to pick up oxygen as it normally would. The result is the lungs can't supply the rest of your baby's body with the oxygen it needs. Babies with tricuspid atresia tire easily, often short of breath and have blue tinged skin

Signs and symptoms

Blue tinge to the skin and lips (cyanosis) Difficulty breathing (dyspnea) Tiring easily, especially during feedings Slow growth Most babies who have tricuspid atresia begin to show these signs and symptoms within the first two months of life. Some babies with tricuspid atresia may also develop signs and symptoms of congestive heart failure, including: Fatigue and weakness Persistent cough or wheezing with white or pink blood-tinged phlegm Swelling (edema) in the legs, ankles and feet Swelling of the abdomen (ascites) Sudden weight gain from fluid retention Decreased alertness

Difference between normal or triscuspid valve with defect

Causes

Heredity Factors Down syndrome, may increase your baby's risk of Tricuspid Atresia

The exact cause of tricuspid atresia is unknown, but several factors may increase the risk of a baby being born with this condition:

Risk factor

A mother who had German measles (rubella) or another viral illness during early pregnancy A parent who has a congenital heart defect Excessive alcohol consumption during pregnancy A mother who has diabetes Use of some types of medications during pregnancy, such as the acne drug isotretinoin (Accutane) and lithium (Eskalith), which is used to treat bipolar disorder Babies who have Down syndrome, a genetic condition that results from an extra 21st chromosome, also often have a congenital heart defect.

complications

Lack of oxygen to tissues (hypoxemia)

Increased red blood cell count (polycythemia).

Possible complications later in life

Although treatment greatly improves the outcome for babies with tricuspid atresia, they may still have the following complications later in life, even after surgery:

Formation of blood clots that may lead to a clot blocking an artery in the lungs (pulmonary embolism) or to a stroke Easily tiring when participating in sports or other exercise Heart rhythm abnormalities (arrhythmias) Abnormal loss of protein from the digestive tract (protein-losing enteropathy) Infection of the heart valves (endocarditis) For this reason, your child will need lifelong care from a heart specialist (cardiologist) to monitor for complications and treat them as necessary.

Heart rhythm abnormalities (arrhythmias) Abnormal loss of protein from the digestive tract (protein-losing enteropathy) Infection of the heart valves (endocarditis) For this reason, your child will need lifelong care from a heart specialist (cardiologist) to monitor for complications and treat them as necessary.

Diagnostic procedure
Before birth Ultrasound technology It's possible for a baby to be diagnosed with tricuspid atresia before he or she is born. Doctors can identify the condition on a routine ultrasound exam during pregnancy.

After birth
Echocardiogram

This test uses high-pitched sound waves that bounce off your baby's heart to produce moving images your baby's doctor can view on a video screen. It reveals the absence of a tricuspid valve and a smaller than normal right ventricle. It tracks blood flow, it can also measure the amount of blood moving through holes in the walls between the right and left sides of the heart.

It can identify associated heart defects, such as an atrial septal defect or a ventricular septal defect.

Prostaglandin drug
used to help widen (dilate) the blood vessels and keep the ductus arteriosus and the foramen ovale open.

Preventive antibiotics
Used to prevent bacteria from entering the bloodstream and infecting the inner lining of the heart (infective endocarditis). Practicing good oral hygiene brushing and flossing teeth, getting regular dental checkups is another good way of preventing infection.

Fontan procedure
a palliative surgical procedure used in children with complex congenital heart defects. It involves diverting the venous blood from the right atrium to the pulmonary arteries without passing through the morphologic right ventricle. It was initially described in 1971 as a surgical treatment for tricuspid atresia.

Fontan Procedure

Fontan procedure

Fontan procedure
Used in tricuspid atresia The right atrium is connected to the pulmonary artery This means that blood no longer has to flow through the right ventricle (which is blocked anyway) If an ASD is present, it is repaired.

Atrial septostomy
This procedure creates or enlarges the opening between the heart's upper chambers (atria) to allow more blood to flow from the right atrium to the left atrium. A balloon is inflated in the right atrial opening, dilating it and thus increasing blood flow through it Often used whilst awaiting surgery to ensure adequate oxygen supply Used in transposition of the great arteries to reduce cyanosis

Atrial septostomy

Shunting
The shunts are used to increase blood flow to the lungs Used in tricuspid atresia and severe tetralogy of Fallot.

Creating a bypass (shunt) from the main blood vessel leading out of the heart (aorta) to the pulmonary arteries allows for adequate blood flow to the lungs.

 Shunting

Glenn Procedure
It connects one of the large veins that return blood to the heart (superior vena cava) to the pulmonary artery.

This allows oxygen-poor blood to flow directly to the lungs.

The procedure reduces the workload on the left ventricle, decreasing the risk of damage to it.

Mixed Defects
TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION (TAPVC)

- TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE (TAPVD) - TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR)

 the pulmonary veins that bring oxygen-rich (red) blood from the lungs back to the heart aren't connected to the left atrium Instead, the pulmonary veins drain through abnormal connections to the right atrium. In the right atrium, oxygen-rich (red) blood from the pulmonary veins mixes with venous (bluish) blood from the body Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium From there it goes into the left ventricle, to the aorta and out to the body The rest of the poorly oxygenated mixture flows through the right ventricle, into the pulmonary artery and on to the lungs The blood passing through the aorta to the body doesn't have enough oxygen, which causes the

 Symptoms may develop soon after birth This defect must be surgically repaired in early infancy In surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed When surgical repair is done in early infancy, the longterm outlook is very good Still, lifelong follow-up is needed to make certain that any remaining problems, such as an obstruction in the pulmonary veins or irregularities in heart rhythm, are treated properly Lifelong follow-up is important to make certain that a blockage doesn't develop in the pulmonary veins or where they're attached to the left atrium Heart rhythm irregularities also may occur at any time after surgery.

Rheumatic fever
An inflammatory autoimmune disease that affects the connective tissue of the heart, joints, subcutaneous tissue, and or blood vessels of the CNS The most serious complication is rheumatic heart disease, which affect the cardiac valves Present 2-6 weeks following an untreated or partially treated group A beta hemolytic streptococcal infection of upper respiratory tract. Jones criteria are utilized in determining diagnosis

Assessment
Aschoff bodies (lesion); found in the heart, blood vessels, brain and serous surfaces of the joints and pleura Signs of carditis; shortness of breath, edema of the face, abdomen or ankles, and precordial pain Polyarthritis; inflammation of large joints, and joints pain Erythema marginatum; erythematous macular rash on the trunk and extremities

 Subcutaneous nodules found in crops over the bony prominences Chorea; sudden aimless irregular movement of the extremities, involuntary facial grimaces, speech disturbances, emotional liability and muscle weakness Low grade fever that spikes in the late afternoon Elevated antistreptolysin O titer, sedimentation rate and C-reactive protein

management
Assess vital signs Assess for the clinical manifestation Control joint pain and inflammation with massage and alternating hot and cold application as prescribed Provide bed rest during acute febrile phase Limit physical exercise in the child with carditis Administer antibiotics (penicillin) as prescribed

 Administer salicylates and antiinflammatory agent as prescribed Initiate seizure precaution if the child is experiencing chorea Instruct the parent about the importance of follow up and the need for antibiotic prophylaxis for dental work, infection and invasive procedure Advice the child to inform the parents if anyone in school develops a streptococcal throat infection

Kawasaki disease
Known as mucocutaneous lymp node syndrome and an acute systemic inflammatory disease Cause is unknown but may be associated with an infection from an organism or toxin Cardiac involvement is the most serious complication; aneurysm can develop

assessment
Acute stage
Fever Conjuctivalhyperemia Red throat Swollen hands, rash and enlargement of the cervical lymph nodes

Assess heart sound Assess extremities for edema, redness and desquamation

 Examine eyes for conjuctivitis Monitor mucous membranes for inflammation Monitor I&O

GASTRO INTESTINAL DISORDER

RENAL DISORDER

NEPHROTIC SYNDROME
A set of clinical manifestation arising from protein wasting secondary to diffuse glomerular damage Defined as massive proteinuria, hypoalbuminemia, hyperlipemia and edema

assessment
Pale, irritable and fatigued child Child gains weight Decreased urine output Dark frothy urine; hematuria may be present Ascites Waxy pallor skin Hypertension Anorexia, anemia

management