caring for a child with a cardiovascular condition - chapter 27

Kawasaki Disease Treatment  Intravenous immunoglobulin  Aspirin  Anti-inflammatory dose initially  Anti-platelet dose after fever decreases  Heart issues  Monitor for coronary artery aneurisms

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with hypertension, A young child may be irritable where an older child may complain of changes in vision, dizziness, or headaches.

A child is hypertensive if the blood pressure is above the 95th percentile for age, height, and gender. As a screening tool, the nurse should consider any reading greater than 20 mm Hg above normal blood pressure for the child's age as high.

Most of the cardiac tumors are benign but significant because they occupy space in the heart and may restrict the normal FLow of blood. .

A long-term consequence of cardiac tumors includes scar tissue formation, which in turn may lead to arrhythmias

Aortic stenosis or aortic valve stenosis (AS or AVS) is a malformation and narrowing in the aorta or around the aortic valve

A narrowing in this area causes an increased workload on the left ventricle and eventually leads to hypertrophy [increase in size] and heart failure.

treatment goals of hyperlipidemia are similar to those for adults and include diet modiFIcation, exercise, and medication.

A recommended balanced nutrition is one that provides less than 10% of total calories from saturated fatty acids, 30% or less of total calories from fat, and less than 300 mg of cholesterol each day.

pulse pressure; which is the difference between the systolic and diastolic blood pressure. A normal pulse pressure range is < 40 mm Hg.

A sudden widening of the pulse pressure postprocedure may indicate a dislodged or embolized device.

Carvedilol is indicated for the treatment of mild-to-severe heart failure of ischemic or cardiomyopathic origin.

Action: Beta blockers slow tachycardia and vasodilation; Carvedilol decreases peripheral vascular resistance, decreases renal vascular resistance,reduces plasma renin levels, and increases atrial natriuretic peptide levels.

Therapeutic effects: Increases stroke volume, decreases blood pressure and improves renal FLow, decreases heart rate.

Adverse Reactions and Side Effects: Chest pain, dizziness, hyperglycemia, bradycardia, nausea

If tachycardia is identiFIed postcatheterization, the nurse should suspect pain or dehydration.

After a cardiac catheterization, a child is required to lie FLat for up to 6 hours depending on the insertion site of the catheter. The supine position minimizes the risk of bleeding through the insertion site.

An ECG may show left ventricular hypertrophy. Cardiac catheterization is the definitive test, also measuring the exact gradient.

An angioplasty or valvuloplasty is performed to open the narrow area with a balloon. Another treatment mode is surgical intervention to repair or replace the valve. Aortic stenosis requires life-long monitoring.

ModiFIed Norwood:• The main pulmonary artery is divided from the branch pulmonary arteries and patched to the hypoplastic aortic arch, creating a hybrid systemic arterial outflow from the RV.

An atrial septectomy is created via catheterization or surgical incision to allow for left to right shunting, so that oxygenated pulmonary venous blood is directed toward systemic outFLow.

Initially, vague symptoms may include a low-grade fever, malaise, loss of appetite, and muscle aches. A high fever, chills, sweating, stiff joints, or back pain can indicate that acute illness has occurred.

As the condition worsens, symptoms of heart failure occur. A physical exam may reveal eye hemorrhage petechiae, FLuid in the lungs, or signs of a stroke. Blood cultures to identify bacteria or fungi in the bloodstream may detect endocarditis.

A ventricular septal defect (VSD) is the most common congenital heart defect. Fortunately, it is one of the mildest.

At the worst case, the child suffers from right ventricular overload from left-to-right [acyanotic] shunting of blood due to the high-pressure gradient from the left to the right side.

a simple defect of the atria that results when the two septae fail to form properly

Atrial septal defect [ASD]

atrial septal defects and an opening called a patent foramen ovale are closed with transseptal closure devices. In the case of a PDA closure, a plug type device is used.

By approximately 6 weeks after the closure device is placed, the child's own tissue grows over the device [endothelialization] and by 6 months, a permanent seal is created.

TOF, truncus arteriosus, vascular ring, interrupted aortic arch Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of Growth and development, and Ear abnormalities and deafness

CHARGE

the inability of the cardiac muscle to perform its proper function of moving blood forward.

CHF, or called simply heart failure

the amount of blood discharged from the left or right ventricle per minute .

Cardiac output

Often these children undergo adobutamine [Dobutrex] or exercise stress test to assess the vascular response to exercise.

Cardiomyopathy (CM) is a condition in which the cardiac muscle becomes dilated, hypertrophied, stiff, or inFLamed.

Pharmacological treatment is recommended for children older than 8 ears whose LDL, cholesterol is high

Cholesterol Levels in Children Desirable: Total cholesterol Less than 170; LDL cholesterol Less than 110 Borderline: Total cholesterol 170-199; LDL cholesterol 110-129 Associated with Higher Risk 200 or more; LDL cholesterol 130 or more

the aorta and pulmonary arteries (PAs) are combined, with full mixing of blood

Clinical signs and symptoms of truncus arteriosus include cyanosis, congestive heart failure, and low cardiac output.

Diagnosis of TOF is based on signs and symptoms such as cyanosis, breathing difficulties, fainting, fatigue and weakness, slow growth, or developmental delay.

Complex single ventricle defects are most often cyanotic conditions with full mixing of oxygenated and deoxygenated blood. The main problem in these defects is that there is only one physiological pumping chamber or ventricle.

a defect in the heart, great vessels, or a noted disease pattern after birth.

Congenital heart disease

Increased Pulmonary Blood Flow Defects  Patent Ductus Arteriosus (PDA)  Atrial Septal Defect (ASD)  Ventricular Septal Defect (VSD)  Atrioventricular Canal Defect (AV Canal)

Decreased Pulmonary Blood Flow Defects  Pulmonic Stenosis (PS)  Tetralogy of Fallot (TOF)  Pulmonary or tricuspid atresia

Interrupted aortic arch, truncus arteriosus, VSD, PDA, TOF; Decreased immune response, low set ears, palate problems, hypoparathyroidism and hypocalcemia

DiGeorge or Velo-cardio-facial Chromosome

The initial signs are dramatic; as the PDA starts to close in the hours after birth, the child becomes severely cyanotic, tachycardic, and dyspneic. A heart murmur is generally present.

Diagnostic testing includes ECG, echocardiogram, chest radiograph, and cardiac catheterization.

Dilated (DCM) or congestive cardiomyopathy is the most common form and is due to weakened contractions leading to dilation of all four chambers of the heart.

Dilated cardiomyopathy is caused by myocardial damage from chemotherapy, microbes, bacteria, viruses, immunological defects, or nutritional disorders.

Cardiomyopathy; Generalized weakness and muscle wasting FIrst affecting the muscles of the hips, pelvic area, thighs, and shoulders; calves are often enlarged

Duchenne's Muscular Dystrophy

If the valve does not close properly, there may be leakage called regurgitation or insufFIciency when the valve is stiff or stenotic.

During periods of increased blood pressure, such as with excitement, exercise, and crying, the child may experience dyspnea [breathlessness] or in the most severe cases, brief moments of cyanosis when the blood FLow does not reach the pulmonary bed.

Elevating the head of the bed enhances lung expansion, decreases venous return to the heart, redistributes blood to dependent areas, and relieves pressure on the diaphragm.

Electrolyte measurement, particularly potassium, is perhaps the most critical lab test in the initial postoperative period because the cardiac bypass machine hemolyzes the cells, thus creating a high concentration of extracellular potassium.

Initially, the body tries to compensate by increasing the contractile force, thus increasing the stroke volume (SV).

Eventually, this will plateau or the cardiac muscle may not be strong enough to compensate as in congestive heart failure (CHF) or cardiomyopathy.

VSD, PDA, ASD, TOF; Growth deficiencies, skeletal deformities, facial abnormalities, organ deformities: genital malformations; kidney and urinary defects, central nervous system handicaps

Fetal Alcohol Syndrome

As a consequence of ASD, mixing of venous blood with arterial blood may occur, causing a cyanotic condition. One problem for a child with TAPVR is that a high volume of blood returns to the right side of the heart, causing right-sided hypertrophy and enlargement.

If an ASD is not present, one needs to be created with a balloon septostomy. A variation of this condition is partial anomalous pulmonary venous return (PAPVR), in which two of the veins return blood to the left side and two of the veins return blood to the right side.

If the tilt test is positive, the nurse will see a remarkable drop in blood pressure or heart rate and the child will experience syncope or pre-syncope [dizziness or lightheadedness]..

If the child does not exhibit any symptoms, such as syncope or lightheadedness, the table is laid FLat again and isoproterenol [Isuprel] is administered to stimulate a fast heart rate

The PDA may also be "forced"closed using the medication indomethacin [Indocin].

If the child has a high risk for bacterial endocarditis, the physician may prescribe antibiotics before dental work and surgery.

An ECG is a graphic display of electrical activity produced by changes in the intraBcellular charge of the cardiac muscles.

If the heart rate is too fast, the diastolic phase shortens in relation to the length of the full cardiac cycle. The ventricle is not allowed enough FIlling time; therefore the stroke volume is less, decreasing the cardiac output.

Idiopathic pulmonary arterial hypertension (IPAH) is a condition of high blood pressure in the lungs. It may result from cardiac defects or may be idiopathic [without recognizable cause].

If the pressure in the lungs is high, as with IPAH, the right ventricle must pump harder to force the blood to the lungs.

essential nursing care includes bedrest. Activity restrictions must also be enforced until the cardiac muscle heals.

In addition, antiarrhythmics, inotropic agents, and pericardiocentesis [aspiration of fluid from the pericardiacsac] may be required.

Children with cardiac transplant have a high incidence of hyperlipidemia because of the immunosuppressant drug cyclosporine-A [Neoral].

In addition, children with cardiac transplant form atherosclerotic lesions at a very rapid rate and the child risks facing coronary artery bypass graft (CABG), or worse, a second transplant surgery.

Balloon atrial septostomy is an emergent palliative procedure necessary to keep the child alive when the heart has no means of blood FLow to the pulmonary system or body such as in tricuspid atresia or hypoplastic left heart.

Infusion of prostaglandin is a palliative treatment until septostomy occurs to keep the PDA open and blood FLowing to the pulmonary system or body.

Initially prostaglandin (PGE 1) is infused to maintain patency [free FLow] of the PDA. A balloon atrial septostomy [formation of an opening in aseptum] is performed to create an ASD.

Initial surgical repair includes a shunt [diversion] or a conduit [channel]. Later, a Fontan procedure [a procedure used to repair complex single ventricle type congenital heart defects] may be needed.

Kawasaki Disease Clinical manifestations Three stages of illness  1st phase = Fever  Conjunctiva hyperemia  Cervical lymph node enlargement  2nd phase = Peeling rash  Cracking skin [lips, fingers, toes]  3rd phase = Vasculitis  Coronary artery aneurisms

Kawasaki Disease Diagnostics  Chest x-ray  Echocardiogram  Electrocardiogram  Urinalysis - may show pus in the urine or protein in the urine  Complete blood count  C-reactive protein (CRP)  ESR (Sed Rate)  Serum albumin  Serum transaminase

Decreased Pulmonary Pressure Tetralogy of Fallot 1. Hole between the right and left ventricles 2. Narrowing of the pulmonary outflow tract 3. Aorta shifted to R ventricle 4. Right ventricular hypertrophy

Lesions Which Obstruct Systemic Blood Flow  Hypoplastic Left Heart  Coarctation of the Aorta  Aortic Stenosis

Blocks are disruptions in the FLow of electrical current throughout the heart.

Lethal rhythms are those producing little or no cardiac output and include ventricular tachycardia, ventricular FIbrillation, torsades de pointes, pulseless electrical activity [PEA; formerly known as electromechanical dissociation [EMD]],or asystole.

If a teen with syncope is of driving age, he or she must be syncope free for 6 months in some states before the teen may drive again.

Long Q-T syndrome (LQTS) is an electrophysiological condition predisposing the child to fatal arrhythmias such as ventricular tachycardia (VT), torsade de pointes, and ventricular fibrillation (VF)

Thrombotic agents, such as streptokinase [Streptase], urokinase [Abbokinase], and alteplase [Activase], have been used with some success in thrombus formation.

Long-term use of anti-coagulants such as warfarin [Coumadin] or clopidogrel [Plavix] may be used to prevent thrombus formation in the engorged or aneurysmal vessels.

Aortic aneurism, aortic and/or mitral regurgitation; Arms disproportionately long, tall thin with laxity of joints, dislocation of lenses, spinal problems, stretch marks, hernia, pectus abnormalities, restrictive lung disease

Marfan Syndrome

Diagnosis includes a complete physical examination that may reveal a murmur, gallop, and venous congestion as demonstrated by hepatomegaly or distended neck veins.

Medications in the angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers have positive inotropic properties influencing the force of muscular contractility.

Embolization occurs when the device becomes dislodged from its intended location, migrating to the atria, pulmonary artery, the ventricle, or aorta.

Narrow vessels or valves may be opened or dilated with a balloon angioplasty or valvuloplasty as an initial treatment or a stent may be placed in a vessel as a long-term treatment

Pulmonic valve stenosis, LVH; Elfin facies, pectus deformity, joint laxity, undescended testes, spine abnormalities,hypotonia, seizures

Noonan Syndrome

Coarctation of the aorta (CoA) is a narrowing or stricture of the descending aorta distal to the carotid arteries.

Normally, blood pressure in the legs should be higher or equal to that in the arms. When a BP in the lower extremities measures > 10 mm Hg less than that in the upper extremities, the practitioner suspects a CoA

high blood pressure or hypertension may indicate a coarctation of the aorta, kidney disease, left ventricular hypertrophy(LVH), or early-onset familial hypertension.

Nursing care for the child with hypertension is based on education about the condition, diet, exercise, lifestyle modiFIcation, and medication. Medications for high blood pressure or hypertension include beta-blockers and ACE inhibitors.

A nursing assessment includes a history of fainting triggered by intense emotions, vigorous physical activity, swimming, auditory stimuli [such as a school bell], and upon awakening.

Other signs and symptoms of LQTS are palpitations, seizure,or sudden death.

The PDA murmur is distinctive in sound and location. This murmur is heard best at the left subclavicular margin and is described as machine-like, to and from or continuous.

Other symptoms include frequent colds and susceptibility to RSV, fatigue, poor feeding, and poor growth pattern. The blood pressure reveals a wide pulse pressure

Nursing care begins with the administration of I V immunoglobulin (IVIG) and aspirin (ASA), which are used primarily for their anti-inFLammatory effects.

Other treatments such as steroids, plasma exchange, or cytotoxic agents may be used if this initial therapy is ineffective

P wave = depolarization of the atria

PR interval = time it takes for the impulse to get to the AV node. Repolarization of atria is "buried" in the QRS complex

these drugs are used when poor contractility is the cause of CHF.

Positive inotropes such as digoxin [Lanoxin] or even the stronger dopamine drugs

high pressure in the right ventricle may indicate a ventricular septal defect (VSD) with left-to-right shunting or pulmonary artery stenosis or pulmonary hypertension

Postcatheterization The nurse must monitor pressure dressing in the groin, heart rate, respirations and blood pressure.

Hypoplastic left heart syndrome is a life-threatening defect that must be treated shortly after birth in order to sustain life.The defect is one in which the left ventricle is extremely small or hypoplastic and unable to maintain an adequate cardiac output

Prostaglandin (PGE 1 ) is given to keep the PDA open.

Treatments include balloon angioplasty or valvuloplasty [a procedure that reopens narrowed blood vessels and restores forward blood FLow] or open heart surgical intervention.

Pulmonary atresia is absence of the pulmonary valve, pulmonary artery, or both

QRS complex = depolarization of the ventricle

QT interval = time from start of depolarization to end of repolarization of the ventricle. Also represents relative refractory period.

Rheumatic Fever  Definition: toxin in strep attacks multiple systems  Etiology: Group A beta-hemolytic streptococci  Clinical manifestations  Heart  Joints  Brain  Skin

Rheumatic Fever Treatment  Antibiotics to eradicate strep infection  Aspirin to treat cardiac, inflammation  Steroids

A primary cause of hypercholesterolemia is hereditary predisposition and children may develop atherosclerotic lesions as early as infancy.

Secondary causes are exogenous [originating outside an organ], endocrine, or metabolic disorders; liver diseases; renal diseases; or other miscellaneous reasons such as anorexia nervosa or collagen diseases.

Although the blood is mixed through this large open space, the shunt is left to right and the child is not cyanotic.

Signs and symptoms of cardiac failure such as shortness of breath, respiratory distress,periorbital edema, failure to thrive, increased respiratory infections, and distended liver may be noted.

the amount of blood ejected by the left ventricle with each heartbeat

Stroke volume

aortic murmur. This type of murmur is detected as an abnormal, soft blowing sound heard during the systolic phase of the cardiac cycle.

Subacute bacterial endocarditis (SBE) occurs subsequent to a bacterial infection or introduction of an infective agent into the child's blood stream.

If a child is hypertensive, the nurse should suspect CoA. The child may have pain in the legs or cyanotic lower extremities.

Surgery is always indicated for this condition. Nonsurgical treatment for older infants and adolescents involves a balloon angioplasty and stent placement.

S T segment represents early ventricular repolarization

T wave is the repolarization of the ventricle

Symptoms appear at birth or soon afterwards and include cyanosis, shortness of breath, poor feeding, and clubbing of the FIngers and toes.

TGA in all cases must be surgically corrected, typically with an arterial switch operation

located at the junction of the left ventricle and the ascending aorta, also prevents regurgitation

The aortic valve

for any devices left in the body such as closure devices or stents, the child will be on a baby aspirin (ASA) for 6 months after the procedure.

The aspirin prevents clot formation while endothelialization [growth of new tissue] occurs around the artificial device. Subacute bacterial endocarditis prophylaxis (SBE) precautions should be followed for 6 months for device placement patients.

The infection may be caused by an invasive procedure such as surgery, urological procedures, or most often dental cleaning.

The bacterium in the blood stream adheres to a rough area in the heart such as a stenotic valve or an area of turbulent FLOw.The bacterium colonizes and causes tissue destruction.

Neurally mediated syncope (NMS) is a condition caused by an exaggerated response to a normal bodily function. It is also called vasovagal syncope.

The baroreceptors in the carotid artery act as a regulator of blood pressure during certain situations. If the system is stressed such as during extreme heat, pain, fright, or prolonged standing, the initial response is a release of epinephrine, stimulating the sympathetic nervous system.

Generally, the child with CM has a variety of sometimes vague symptoms such as weakness, excessive tiredness, shortness of breath, exercise intolerance, heart palpitations, chest pain, poor feeding, and slow weight gain.

The child may also experience fainting or lightheadedness.

In children with aortic stenosis, a murmur may be audible during the systolic phase of the cardiac cycle. A click may be heard and a thrill maybe noted.

The child may have chest pain or fatigue and syncope on exertion. Critical AS causes heart failure in neonates.

Prior to surgery, nursing care is geared toward optimizing the cardiac output and ensuring adequate weight gain.

The child should always be monitored for signs of CHF and measures taken to prevent fatigue, FLuid overload, or infections.

The main sign is a temporary loss of consciousness [a fainting spell]. Occasionally, the response is so dramatic that the child may experience a seizure.

The definitive diagnosis of neurally mediated syncope is made via the tilt test but can be made on history alone

An atrioventricular canal defect (AVC) is also known as complete AVC or endocardial cushion defect (ECD). an AVC is a large hole in the center of the heart.

The endocardial cushion fails to form properly. There are openings on the atrial wall and the ventricular wall, and the tricuspid and mitral valve come together to form one large valve

lies below the heart and carries blood from the legs, abdominal organs, and lower part of the body.

The inferior vena cava

receives fully oxygenated blood from the lungs through the pulmonary veins, with an approximate oxygen saturation of 100%

The left atrium

typically is the thicker ventricle with a smooth interior. It receives blood from the left atrium and pumps it into the systemic circulation via the aorta

The left ventricle

has two cusps, connects the left atrium to the left ventricle.

The mitral valve,called the bicuspid valve

Nursing care for NMS may be as simple as increasing the child's sodium and water intake. Anadrenocorticosteroid such as FLudrocortisone [Florinef] may be given to retain FLuid.

The next level of treatment is a beta-blocker, which regulates the exaggerated response.

Initially, the neonate is given PGE 1 to keep the PDA from closing. In addition, if there is no ASD or PFO, an emergent balloon atrial septostomy is performed to ensure survival.

The next step is to perform a systemic-pulmonary (SP) shunt if the pulmonary blood flow is deficient or a PA band if the pulmonary blood FLow is excessive. Eventually a Glenn procedure may be done and Fontan procedure is the definitive surgery.

The purpose of the cardiac catheterization is to determine the pressures within the child's heart vessels and to provide a radiographic picture of the anatomy by measuring the size and shape of vessels, valves, and ventricles.

The normal pressure of the left ventricle correlates with the normal blood pressure for age. The normal pressures in the right ventricle and right and left atrium are similar to those of an adult patient.

the only named artery in the body that carries deoxygenated blood.

The pulmonary artery

located at the junction of the right ventricle and pulmonary artery. It prevents regurgitation of blood from the pulmonary artery to the right ventricle.

The pulmonary valve

delivers oxygen via the blood to the cells of the body

The pulmonary veins, left atrium, mitral valve, left ventricle, aortic valve,aorta, and arteriole system

a reservoir or collecting chamber for the peripheral venous return. it receives deoxygenated blood from the entire body [except lungs] through the superior and inferior venae cavae and coronary sinus with an approximate oxygen saturation of 70%.

The right atrium

this ventricle has smaller muscle mass with trabeculated surfaces. it receives blood from the right atrium and pumps it into the lungs via the pulmonary artery.

The right ventricle

Restrictive (RCM) is the least occurring type of cardiomyopathy and is characterized by unusually noncompliant ventricular walls that fail to relax.

The size of the ventricle is normal, but the atria are enlarged because of the impaired diastolic filling due to stiffness in the ventricle

lies above the heart and carries blood from the head, arms, and upper body.

The superior vena cava

The child has cyanosis, respiratory distress, lethargy, poor and rapid breathing, poor feeding, frequent respiratory infections, and signs of heart failure.

The treatment for TAPVR is complete surgical repair. Post-surgery, the child can live a full normal life, but should be followed routinely [every 1 to 2 years] for any reoccurring stenosis.

connects the right atrium to the right ventricle and is so named because it consists of three cusps or "doors"

The tricuspid valve

In TOF, a stenotic vessel and high pulmonary vascular resistance (PVR) cause the right ventricle to hypertrophy. The hallmark sign of TOF is cyanosis with crying or playing, which is relieved by squatting or drawing up the legs.

These episodes are called "TET" spells—cyanotic events exacerbated by excitement and crying, then relieved by a decrease in pulmonary vascular resistance.

The narrowing of the valve causes an increased workload on the right ventricle, which in turn leads to congestive heart failure (CHF) with symptoms such as hepatomegaly.

This condition is frequently associated with Noonan syndrome and is part of the combination condition called tetralogy of Fallot.

A systemic-pulmonary shunt [Sano or Blalock-Taussig] is created to allow pulmonary arterial blood FLow, as the creation of single ventricle physiology only has a direct connection to systemic outFLow.

This stage creates a single ventricle that is responsible for pulmonary and systemic circulation. is used for Stage 1 repair for HLHS.

Epstein's malformation occurs when the tricuspid valve is displaced into the right ventricle

Total anomalous pulmonary venous return (TAPVR) is a condition in which the pulmonary blood FLow returns to the heart through the right atrium rather than the left

Conal-truncal defects develop during the formation of the trunk dissection.

Transposition of the great arteries or vessels (TGA orTGV) occurs in utero when the signals cross and instead of twisting there is simply a septation and the aorta arises from the right side of the heart and the pulmonary artery arises from the left

VSD, PDA, dextrocardia; Omphalocele, holoprosencephaly [an anatomic defect of the brain involving failure of the forebrain to divide properly], kidney defects, skin defects of the scalp

Trisomy 13

VSD, PDA, PS; Multiple joint contractures, spina bifida, hearing loss, radial aplasia [underdevelopment or missing radial bone of forearm], cleft lip, birth defects of the eye

Trisomy 18

arterial switch; In surgery the position of the aorta and the PA are permanently switched.

Truncus arteriosus is a complicated cyanotic lesion with a poor prognosis if not treated surgically. In truncus arteriosus, the trunk has not twisted or septated.

If the VSD is large and there is a heavy left to right shunt, the patient develops signs of right ventricular failure, such as shortness of breath, feeding difficulties, poor growth, easy fatigability, and recurrent pulmonary infections.

Typically, a harsh murmur [along with a thrill at the lower left sternal border] is detected at the well-baby checkups.

Lanoxin Toxicity  Bradycardia  Dysrhythmias  Nausea  Vomiting  Anorexia

Unusual Symptom of infective endocarditis:  splinter-like hemorrage discoloration under the fingernails

VSD and others; Vertebral anomalies, vascular anomalies, Anal atresia, Cardiac anomalies, Tracheo-esophageal (T-E) fistula, Esophageal atresia, Renal anomalies, radialdysplasia, Limb anomalies, Single umbilical artery

VATER [VACTERLS]

these drugs are used if increased afterload is the causative factor of CHF

Vasodilators such as captopril [Capoten] or enalapril [Vasotec]

picks up oxygen

Venous system, venae cavae, right atrium, tricuspid valve, right ventricle, pulmonic valve,pulmonary artery, and lungs

Supravalvular aortic stenosis, PA stenosis; a small upturned nose, long philtrum [upper lip length], wide mouth, full lips, small chin, and pufFIness around the eyes; hypercalcemia, dental abnormalities, renal problems, sensitive hearing, hypotonia, joint laxity, overly friendly personality

Williams Syndrome

signs and symptoms of ASD may or may not include

a murmur, a right ventricle (RV) heave or a thrill [abnormal tremor accompanying a vascularor cardiac murmur felt on palpation]

In advanced stages of CHF these develop

an enlarged liver or edema

In the presence of normal hemoglobin, a decrease in the oxygen saturation to 85% will cause

an outward sign of cyanosis [bluish coloration] that appears around the lips, nose, and mouth of babies and toddlers and in the nail beds of older children.

Symptoms of IPAH include shortness of breath [dyspnea], especially during exercise, chest pain, weakness, fatigue, dizziness, leg swelling, and fainting episodes.

as the condition progresses, a physical exam shows a heart murmur, enlargement of the neck veins, liver and spleen enlargement, leg swelling,parasternal heave, ascites, and clubbing.

two chambers of the heart which act as reservoirs or collecting chambers

atria

The components of cardiac output [CO] are

cardiac output and stroke volume.

ASD is conFIrmed by

chest x-ray exam, echocardiography, cardiac catheterization, and ECG.

If the decreased oxygen state is chronic, the child eventually will develop

clubbing of the fingernails. The longer and lower the oxygen saturation the more evident the clubbing. The physiological explanation for this change is that the capillaries enlarge to accommodate the low saturation in an attempt to deliver more blood to the periphery.

Hypoplastic Left Heart  Absence of mitral and aortic valves HLHS  Abnormally small aorta  Severe heart defect  Prostaglandin E1 to maintain PDA  Treatment options  Comfort care  Norwood procedure (three-staged palliation)  Heart transplantation

digoxin (Lanoxin) Alert  Infants rarely receive more than 1ml

these drugs are used If increased preload is the cause of CHF

diuretics such as furosemide [Lasix] or hydrochlorothiazide [Aquazide]

AV canal, VSD; down's facies, developmental delay

down syndrome

The four main complications of cardiac transplantation are: •Rejection •Infection •Posttransplant lymphoproliferative disorder (PTLD) •Transplant coronary artery disease (TCAD)

every year coronary angiography is performed with cardiac catheterization to assess for coronary atherosclerosis, a side effect of the immunosuppressant drugs.

Postoperative hemorrhage is considered when there is

excessive chest tube drainage greater 5-10 mL/kg in 1 hour or more than 3 mL/kg per hour in 3 consecutive hours.

Pacemakers are used to treat cardiac conditions such as heart block or severe bradycardia, sick sinus syndrome,and junctional or idioventricular rhythms or lethal arrhythmias.

external pacing is always temporary. pacemakers may be placed in the abdomen in younger children and in the subclavicular area in older children. Pacemaker generators must be replaced every 5-10 years. do not recommend pacemaker placement for neurally mediated syncope.

the primum and secundum, stretch around the center of the common atrium. Eventually these septae overlap and form an area called the

foramen ovale.

CoA, ASD, A S; Kidney problems, high blood pressure, overweight, hearing difficulties,diabetes, cataracts, and thyroid problems, lack of sexual development, a"webbed" neck, a low hairline at the back of the neck, drooping of the eyelids, dysmorphic,

low set ears, abnormal bone development, multiple moles; Turner Syndrome

Kawasaki disease, also known as mucocutaneous lymphnode syndrome, is a multisystem disease affecting the cardiovascular system.

one long-term sequel of Kawasaki disease is aneurysm formation in arterial vessels. Other sequelae are myocarditis or rhythm disturbances.

Postoperative arrhythmias may be caused by electrolyte disturbances or trauma caused by surgery.

one of the most important aspects of medication administration is proper dosing.

signs and symptoms of CHF in an infant include

poor feeding, poor growth, irritability, shortness of breath [SOB], or excessive sweating.

Older children with CHF present with

poor growth, shortness of breath, and exercise intolerance.

physical growth can be stunted because of frequent prednisone dosing.

postoperative vital signs: The vital signs should be taken every q 15 minutes for the FIrst few hours then q 30 min, then q 1hr until the child is stable. All changes in vital signs,no matter how subtle, should be documented and reported to the physician or practitioner.

• ArtiFIcial valve replacement. • A faulty or damaged native valve is replaced with a valve made of either human or animal tissue or a mechanical valve. for defects Endocarditis, failed Ross,valvular stenosis.

potential long term implcations and sequelae; Need for a lifetime of replacement prosthetic valve procedures; need for long-term oral anticoagulation; thromboemboli formation, aortic or pulmonary insufFIciency.

Stroke volume is the product of

preload, afterload, and contractility [inotropy] [CO = SV x HR].

Babies and toddlers with ascites exhibit

puffy eyelids, swelling of hands and feet, and the fontanel may be bulging.

from the lungs, the blood returns to the heart through these, the only veins that carry oxygenated blood, into the left atrium

pulmonary veins

Angiography visualizes the structure and function of the ventricles, the vessels, and the valves. It is also useful to determine size and location of septal defects.

radio-opaque contrast medium is injected into the chambers and vessels of the heart. If there is a defect, the contrast highlights septal openings, narrow vessels, and extra vessels.

At worst case, the ASD in the child is the source of .

right atrial enlargement caused by FLuid overload from left to right shunting through the opening in the atrial wall. This FLuid overload can extend to the right ventricle, which is the source of the RV heave, and show an axis change on an ECG. It also produces a fixed split second heart sound

Cardiac defects are those of

septum or septae [pl] [chamber walls], vessels and valves, conaltruncal defects, as well as combination defects.

Tricuspid atresia (TA) occurs when there is an error of the formation of the tricuspid valve. As a single defect, this condition is incompatible with life,

since no blood from the RA reaches the RV and thus the right ventricular outflow tract [RVOT] leading to the PA and the lungs. The deoxygenated blood must reach the pulmonary bed to sustain life

due to the tendency of blood pooling and risk of thrombus formation, An ASD, along with atrial fibrillation, may predispose the child to

stroke

Cardiac output is the product of

stroke volume [SV] and heart rate [HR]

Treatment for children includes aggressive medical regimen with inotropic medications along with preload and afterload reduction.

tetralogy of fallot: There are always four associated conditions: VSD, overriding aorta, hypertrophic RV, and pulmonary stenosis or atresia.

Other treatment includes beta-blocker therapy and nutritional supplementation particularly with carnitine (Carnitor or L -carnitine). Diuretic and inotropic therapy is also recommended, except in the case of hypertrophic CM.

the child is placed on activity restrictions to prevent overstimulation of the heart muscle. The ultimate treatment quite often is cardiac transplantation.

Another long-term effect of low oxygenation is polycythemia which is

the increase in the red blood cell production in response to the low oxygen output. The patient has hemoglobin [Hgb] levels greater than 15 [g/dL].The condition also causes thickening of the blood and predisposes the child to thrombi and stroke.

also as a result of this fluid overload with ASD this becomes engorged

the liver. Other signs and symptoms of cardiac failure such as shortness of breath, respiratory distress, periorbital edema, failure to thrive, and increased respiratory infections may be noted.

The most frequently used medications are beta-blockers. Treatment also includes pacemaker-deFIbrillator [insertion] or left cardiac sympathetic denervation.

the most common cause of treatment failure is noncompliance with medication

Renal failure is considered when

the output is less than 1 mL/kg per hour along with an elevation in serum creatinine and blood urea nitrogen.

This stimulation in turn raises the blood pressure. The baroreceptors send messages to control the blood pressure. In neurally mediated syncope (NMS),

the response is exaggerated and the blood pressure, and often the heart rate, drop significantly and suddenly.

potential long term implications and sequelae: Coronary ischemia due to diastolic "steal" of aortopulmonary shunt if BT shunt is used, pulmonary artery distortion, cyanosis, aortic outflow obstruction,

tricuspid insufFIciency,potential stenosis of systemic-pulmonary shunt, FTT, feeding difficulties, arrhythmia.

two chambers of the heart that act as pumping chambers

ventricles

Rheumatic fever (RF) is a group-A hemolytic streptococcal infection affecting multiple bodily systems such as the heart, the joints, subcutaneous tissue, and at times the nervous system.

when a valve is replaced in infancy or childhood, it must be replaced every 5 years to accommodate the child's growth. Valves replaced during adolescence can last up to 10 years.

Innovative treatment strategies that are producing promising results for idiopathic pulmonary arterial hypertension include:• Prostacyclin [Flolan] dilates blood vessels and decreases pulmonary vascular resistance.

• Inhaled nitric oxide relaxes pulmonary, but not systemic, blood vessels. • SildenaFIl [Revatio] decreases pulmonary artery pressures and bosentan [Tracleer] blocks endothelin-1 [hormone that causes vasoconstriction].

A normal irregular rhythm in which the rhythm varies with respiration is called sinus arrhythmia. Sinus arrhythmia has no adverse effect on the cardiac output.

• The heart rate increases with inspiration. [Remember:"Inspiration" and "Increase" both start with an "I".] • The heart rate decreases with expiration.

if the patient has four of the FIve signs listed below a Kawasaki diagnosis may be made:

•Skin rash •Cervical lymphadenopathy, typically unilateral, greater than 1.5 mm in diameter •Edema and erythema of hands and feet with eventual peeling of skin •Irritation and inFLammation of the mouth with "strawberry tongue," erythema, and cracking lips •Conjunctivitis without exudate