Peds test 2

Cardiac catheterization: provides information requarding oxygen saturation of blood within the chambers and great vessels, pressure changes, cardiac output or stroke volume, and anatomic abnormities. Pre-op: complete assessment is to be done including height and weight, allergies (such as iodine), S&S of infection (if they appear procedure will be canceled), pedal pulses (assess and mark pulse locations Post-op: monitor for o Pulses: especially below the catheterization site for equal and symmetry o Temperature and color of the affected extremity o Vital signs o Blood pressure especially for hypotension which may indicate hemorrhage or bleeding o Dressing for evidence of bleeding o Fluid intake both IV and oral to ensure adequate hydration o Hypoglycemia- they should receive dextrose containing IV fluids Pt will have to remain in bed with the affected extremity maintained straight for 4-6 hours after VENOUS catheterization and 6-8 hours after ARTERIAL catheterization to facilitate healing

Arterial Septal defects: abnormal opening between the atria, allowing blood from the higher pressure left atrium to flow into the lower pressure right atrium Increased pulmonary blood flow Pts are usually asymptomatic but may develop heart failure later on in life, there is usually a murmur Treatment: surgery to close the opening pening between the atria Because L atrial pressure is slightly higher than R atrial pressure, blood flows from L to R. Causes increased flow of oxygenated blood into the R atria. R atria becomes distended. Because there is low pulmonary vascular resistance, blood backs up into the pulmonary vessels and the R ventricle becomes distended as well. But because it is under low pressure, this is often tolerated very well and the child may be asymptomatic. Rarely see CHF in uncomplicated ASD. Minimal symptoms of Pulmonary vascular changes until several decades of unrepaired ASD. Risk for atrial dysrhythmias and emboli formation in later life if unrepaired. TX: may be closed in cardiac cath procedure; surgical repair w/ patchusually before age 6

Ventricular septal defect: abnormal opening between the right and left ventricles Usually associated with other defects Many close spontaneously during the first year of life Increased pulmonary blood flow The increased blood volume is pumped into the lungs which may eventually result in increased pulmonary vascular resistance and may cause the muscle to atrophy Heart failure is common in these pts and there is a characteristic murmur Treatment: surgical **Most common abnormal opening between R and L ventrical. Blood is flowing from L to R. Why? Increased pulmonary artery flow? Defect in ventricular septumError in early fetal development Can occur anywhere in muscle or membranous ventricular septum 20-25% of all CHDs are VSD ManifestationsDepends on size of VSD and degree of shunting. Can be pinhole size to absence of entire septum. Sm to moderated defects may close spontaneously within first yr of life. Patho-phys: Pressure is higher in L ventricle than in the R ventricle and systemic arterial circulation offers more resistance than the pulmonary circulation; blood flows through the defect and into the pulmonary artery. R Ventricle becomes enlarged (hypertrophied); over time the R atria may also become distended. Symptoms: Characteristic Murmur; failure to thrive, excissive sweating, and [Link] is common; risk of Bact endocarditis; risk of pulmonary vascular obstructive disease. Severe cases: Eisenmenger syndrome: very severe; resistance in pulm blood flow is >> than systemic circulation. Reversal of blood flow through ventricles. REPAIRS: o Cath repairs in clinical trials o Surgical repair w/ bypass; Pulmonary artery banding (if not too large) or patch

Patent ductus Arteriosus: failure of the fetal ductus arteriosus to close during the first weeks of life Increased pulmonary blood flow The continued patency of this vessel allows blood to flow from higher pressure aorta to the lower pressure pulmonary artery which causes a left to right shunt S&S: pts may be asymptomatic or show signs of heart failure, there is a characteristic machine like murmur, widened pulse pressure and bounding pulses Pts are at risk for infective endocarditis and pulmonary vascular obstructive disease in later life Administration of indomethacin has been proven to successfully close the patent ductus in premature infants and some newborns

Surgery may be needed Ductus SHOULD close by about age 15 hours after birth. Some shunting of blood may occur up to 24 hrs of life. DUCTUS closes because increase in arterial oxygen concentration that follows initiation of pulmonary function. ALSO--Y in Prostaglandin E leads to closure of PDA. 5-10% of all CHDs are PDA. More common in females (abt 3:1) Patent DA allows blood to flow from left to right and X pulmonary blood flow Manifestations: Small PDA may be asymptomatic Large PDA may be CHF w/ tachypnea, dyspnea, and hoarse cry. o Symptoms:  BOUNDING Peripheral pulses  widened Pulse Pressure (>25)  Murmur (machinery murmur) at upper left sternal border or in L infraclavicular area. Murmur audible throughout cardiac cycle DEFINITIVE DX: ECHO Management of PDA: Medical>> Preterm kids= INDOMETHACIN to close PDAs; surgical ligation if meds fail prophylactic antibx to prevent bacterial endocarditis Surgery>> between age 1-2 yrs

Coarctation of aorta: localized narrowing near the insertion of the ductus arteriosus, which results in increased pressure proximal to the defect (head and upper extremities) and decreased pressure distal to the obstruction (body and lower extremities) Obstructive defect S&S: high blood pressure and bounding pulses in the arms, weak or absent femoral pulses, and cool lower extremities with lower blood pressure o There are signs of heart failure in infants o In infants with critical coarctation the condition may deteriorate rapidly with severe , acidosis and hypotension o Older children may experience dizziness, headaches, fainting and epitaxis resulting from hypertension. o Pts are at risk for hypertensioni, ruptured aorta, aortic aneurysm, and stroke. o Mechanical ventilation and inotropic support are necessary before surgery o Treatment: surgery, balloon angioplasty, stents The aorta is narrowed near the insertion of the ductus arteriosus. Increased pressure proximal to the defect. Causes high BP & bounding pulses in arms; weak or absent femoral pulses, and cool lower extremities with low BP. Classic symptoms Different BP or pulses. Possible murmur.

Signs of CHF in infants. Condition can deteriorate rapidly. Older kids may c/o dizziness, h/a, fainting and epistaxis from hypertension. Pt at risk for ruptured aorta, aortic aneurysm, or stroke TX: Nonsurgical = balloon angioplasty. Usually effective Surgical: Does not require bypass because defect is outside pericardium. Postop complication is hypertension. Usually done before age 2 yrs. Risk of recurrence.

Aortic stenosis: narrowing or stricture of the aortic valve causing resistence to blood flow in the left ventricle, decreased cardiac output, left ventricle hypertrophy, and pulmonary vascular congestion. Obstructive defect A stricture in the aortic outflow tract causes resistance to ejection of blood from the left ventricle S&S: signs of decreased cardiac output with faint pulses, hypotension, tachycardia, and poor feeding, exercise intolerance, chest pain, and dizziness when standing for to long Treatment: surgery, balloon angioplasty Narrowing of aortic valve Usually malformed in BI- rather than TRI-cuspid valve. Causes increased resistance in left ventricle, decreased CO, L ventricular hypertrophy and pulmonary vascular congestion. L Ventricular wall is hypertrophied>>increased pulmonary vascular resistance and pulm HTN. L Ventricular hypertrophy >> decreased coronary artery perfusion & increased risk of MI Clinical manifestations: Infants w/ severe defects demonstrate signs of decreased CO. Faint pulses, hypotension, poor feeding, tachycardia. Have a murmur. Exercise intolerance. Chest pain, dizziness w/ standing. TX: Balloon Angioplasty to dilate the valve; or Surg: Konno procedure [valve replacement]. May require repeat procedures.

Pulmonic stenosis: narrowing of the entrance to the pulmonary artery Obstructive defect Pulmonary atresia is the extreme form of this in that there is total fusion of the comissures and no blood flows to the lungs Resistance to blood flow causes right ventricular hypertrophy and if the right ventricular failure develops right atrial pressure increases and this may result in reopening of the foramen ovale, shunting of unoxygenated blood into the left atrium and systemic cyanosis occurs Treatment: surgery, balloon angioplasty Pulmonary valve is stenosed. Narrowing at entrance to pulmonary artery. >>R Ventricular hypertrophy and decreased pulm blood flow. Extreme form of PS is Pulmonary atresia (total fusion of the commissures and no blood flow to lungs)

PS>>R vent hypertrophy, R ventricular failure>> R atrial pressure increases and may reopen foramen ovale. Shunts unoxygenaeted blood to L atrium>>systemic cyanosis. May lead to CHF. Often have PDA as well. Cardiomegaly on CXR; TX: Balloon angioplasty to dilate the valve. SURG TXBrock procedure (Bypass to do valvotomy). Usually can repair w/ catheterization.

Tetralogy of fallot: includes 4 defects: ventricular septal defect, pulmonic stenosis, overriding aorta, right ventricular hypertrophy Decreased pulmonary blood flow S&S: infants may be acutely cyanotic at birth, others have mild cyanosis that progresses over the first year of life, there may be acute episodes of cyanosis and hypoxia called blue spells or tet spells PLACE IN KNEE CHEST POSITION TO PROTECT VITAL SIGNS AND BRAIN IN CASE OF TET SPELL Treatment: surgery Tetralogy of Fallot: Tetra means 4. 4 Defects are: VSD Pulmonic stenosis Overriding aorta R Ventricular hypertrophy. Hemodynamics vary widely; depends on extent of pulmonic valve stenosis & size of VSD. IF VSD is large pressures are = in R and L ventricles. Blood is shunted in the direction of the least resistance (pulm or systemic vascular resistance). If PVR is > than Systemic Vasc resistance, shunt with be right to left. Clinical manifestations: Vary with types of defect. TET SPELLS or blue spells with acute episodes of cyanosis and hypoxia. May be anoxic after feeding or w/ crying. RISK of emboli, LOC, Sudden death, Seizures. REPAIRS: usually indicated when tet spells and hypercyanotic spells increase. Stage 1: Blalock or Modified Blalock shunt>>blood to pulm arteries from L or R subclavian artery Complete repair: Usually in 1st yr of life. Repair of VSD, resect stenosed area, and patch R ventricular outflow.

Tricuspid atresia: the tricuspid valves fails to develop, consequently there is no communication fron the right atrium to the right ventricles Decreased pulmonary blood flow S&S: cyanosis is usually seen in the newborn period, there may be tachycardia and dyspnea, older kids have signs of chronic hypoxemia with clubbing. CLUBBING OF FINGERS INDICATES ARTERIES AND VEINS FIGHTING FOR OXYGENATION. Treatment: shunt placement

Failure of tricuspid valve to develop. No opening between R atria and R ventricle. Blood flows through ASD or patent foramen ovale to L side of heart and through VSD to R ventricle and out to lungs. Mixing of oxygenated and unoxygenated blood in L side of heart. Systemic desaturation and pulmonary obstruction. TX: Need to keep open the PDA or VSD. Kept open with Prostaglandin E (continuous infusion) until surgery Surgery: #1 Shunt to increase blood flow to lungs. (Create Atrial septotomy if ASD is small) #2 Bi-directional Glenn Shunt (Cavopulmonary anastomosis) at 6-9 mos age #3 Fontan (or Modified Fontan) Pt must have nl ventricular function and a low pulm vascular resistance for the procedure to be +. Separates mixed blood, but does not restore normal anatomy or hemodynamics. High surgical mortality rate. (< 10% survival in some centers).

Transposition of great vessels: the pulmonary artery leaves the left ventricle and the aorta exits from the right ventricle with no communication between the systemic and pulmonary circulations Mixed defect Newborns with minimum communication are severly cyanotic and have depressed function at birth, those with large septal defects or a patent ductus arteriosus may be less cyanotic but have symptoms of HF, cradiomegaly Treatment: IV prostaglandin E and surgery Pulmonary artery leaves the L ventricle and the aorta exits from the R ventricle. No communication between the systemic and pulmonary circulations. Must have PDA or Septal defect to permit blood flow. Mixed blood flow: Clinically: Cyanoisis with severe respiratory depression, evident quickly after birth, NO MURMER. Surgical TX of choice is Arterial switch procedure to resect and reanastomose great vessels. Coronary arteries have to be reimplanted to supply myocardial circulation. Other procedures possible, depending on defect.

Total anomalous pulmonary venous connection: rare defect characterized by failure of the pulmonary veins to join the left atrium, instead pulmonary veins are abnormally connected to the systemic venous circuit via the right atrium or various veins draining toward the right atrium such as the superior vena cava Results in mixed blood being returned to the right atrium and shunted from the right to left through an atrial septal defect Mixed defect S&S: cyanosis Treatment: surgery Rare Defect. Pulmonary veins fail to join L atrium. Pulm veins drain into L atrium. Results in mixed blood.

Clinical manifestations: Usually cyanotic early on. Condition rapidly deteriorates as pulmonary blood flow increases and causes CHF. SURG TX: Common pulmonary vein is anastomosed to the L atrium, ASD is closed and anomalous venous connections ligated. Success depends on specifics of anomalies.

Surgical interventions: Open heart Closed heart procedures Staged procedures Prepare child and family for procedures -Impaired bonding Surgical complications: Cardiac changes CHF due to excessive pulmonary blood flow or fluid overload Hypoxia--due to resp difficulties or inadequate pulmonary blood flow Decreased CO &/or Decreased peripheral perfusion--due to hypothermia or LV unable to maintain circulation Tx w/ inotropic drugs: Dopamine, dobutamine, milrinone Assistive divices: ECMO if meds fail>>>then poss transplant Dysrhythmias Due to electrolyte imbalance (Low K+) + surgical intervention to septum or myocardium Cardiac tamponade, cardiac effusion (blood in Pericardium) [restricted function of heart] Pulmonary complications: pneumothorax, pulmonary edema, pleural effusion Neuro complications: cerebral edema, brain damage due to hypoxia, seizures Infection Hematologic changes RBC hemolysis, renal tubular necrosis; clotting abnormalities after prolonged heparinization, anemia PROGNOSIS Best statistical outcomes if recovery of ventricular function in 2-3 days and short period of support (meds, vent, etc.) Post op care: o Monitor vital signs and A/V pressures o Intra-arterial monitoring of BP o Intracardiac monitoring o Respiratory needs o Rest, comfort, and pain management

o Fluid management o Progression of activity Post op complications: o CHF o Dysrhythmias o Decreased cardiac output syndrome o Decreased peripheral perfusion o Pulmonary changes o Neurologic changes Postpericardiotomy syndrome: o Symptoms: fever, WBCs, pericardial friction rub, pericardial and pleural effusion o Occurs in immediate postoperative period o Also can occur later (days 7 to 21 postop) o Etiology unknown o Theories of etiology  Viral infection, autoimmune response, reaction to blood in pericardium  May require pericardiocentesis or pleurocentesis

Congestive Heart Failure: the inability of the heart to pump an adequate amount of blood to the systemic circulation at normal filling pressures to meet the bodys metabolic demands. Causes: volume overload, pressure overload, decreased contractility, and high cardiac output demands, in which the bodys need for oxygenated blood exceeds the hearts cardiac output. Most frequently occurs secondary to congenital heart defects in which structural abnormalities result in an increased volume load or increased pressure load on the ventricles. S&S: o Impaired myocardial function: tachycardia, fatigue, weakness, restless, pale, cool extremities, decreased BP, decreased urinary output, gallop rythum (extra heart sound), sweating, anorexia, weak peripheral pulses o Pulmonary congestion: tachypnea, dyspnea, retractions, flaring nares, exercise intolerance, orthopnea, cough, hoarseness, cyanosis, wheezing, grunting o Systemic venous congestion: weight gain, hepatomegaly, peripheral edema, ascites, neck vein distention Treatment: o digoxin (safe range: 0.8-2) which improves contractility, increases force of contraction, decreases heart rate and slows the conduction of impulses through the AV node and it indirectly enhances dieresis by increasing renal perfusion  S&S of digoxin toxicity: bradycardia, anorexia, nausea, vomiting  Digoxin immune fab fragments are used as an antidote to digoxin toxicity o ACE inhibitors which reduce the afterload on the heart making it easier for it to pump, and also causes vasodilation. It also reduces the preload by preventing volume expansion.

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Beta blockers: (new medication for treatment) such as metoproplol and coreg which decrease heart rate, BP, and vasodilation Cardiac resynchronization therapy Diuretics: to remove fluid Fluid restriction Salt restricted diets Oxygen therapy Bedrest Monitor potassium levels NURSING ALERT: Chest tube drainage of more than 3ml/kg/hr for more than 3 consecutive hours or 5 to 10 ml/kg in any one 1 hour is excessive and may indicate postoperative hemorrhage. Notify the surgeon immediately, since cardiac tempnade can develop rapidly and is life threatening. NURSING ALERT: During suctioning observe for signs and symptoms of respiratory distress, such as tachypnea, use of accessory muscles for breathing. And restlessness. Chest tube removal: intravenous analgesics such as morphine sulfate (0.1 mg/kg) often in combination with (versed) may be given before the procedure. Oral analgesics and sedatives have also been used.

Bacterial (infective) Endocarditis: an infection of the valves and inner lining of the heart Most common causes are streptococci and staphylococci and fungus infections S&S: unexplained low grade intermittent fever, malaise, myalgias, arthralgias, headache, diaphoresis, weight loss, a new murmur, splenomegaly, other signs that result from embolus formation elsewhere in the body include splinter hemorrhages (thin black lines) under the nails o Neonates have feeding difficulties, respiratory distress, tachycardia, HF or symptoms of septicemia Diagnostic evaluation: ECG changes, anemia, elevated erythrocyte sedimentation rate, leukocytosis, microscopic hematuria, cardiomegaly o Definitive dx can be made after the growth of the organism and identification of the causative agent in the blood  Three blood specimens are drawn Treatment: high doses of antibiotics IV for 2-8 weeks Prevention: prophylaxsis antibiotics before dental procedures or invasive procedures of the reapiratory tract or on infected skin or musculoskeletal tissue o Regular dental appts should be made Bacterial Endocarditis or BE, or IE (infective endocarditis), or SBE (subacute bacterial endocarditis) Infection in valves and endocardium Usually sequelae of sepsis in child w/ cardiac disease or congenital anomaly Staph, Strep, Candida, gram neg bacteria

Hi dose ANTBX: Penicillin, amipicillin, methicillin, cloxacillin, streptomycin, or gentamicin Amophotericin for flucytosine for fungal infections Treat 2-8 weeks. If antbx unsuccessful>>CHF develops, valvular damage Prophylax before dental procedures, bronchoscopy, T&A, SURGERIES

Rheumatic fever: a poorly understood inflammatory disease that occurs after pharyngitis cause by group A B-hemolytic streptococci Involves the joints, skin, brain, serous surfaces and heart Cardiac valve damage (referred to as rheumatic heart disease) is the most significant complication which causes damage to the valves S&S: o carditis involving the endocardium, pericardium, and myocardium o polyarthritis: affected joint is swollen, hot, red and painful o erythema marginatum: nonpuritic rash found on the trunk and proximal portion of the extremities o subcutaneous nodules: found over bony prominenecs o chorea (referred to as ST Vitus dance) which is sudden aimless irregular movements of the extremities, involuntary facial grimaces, speech disturbances, emotional liability, and muscle weakness o low grade fever that often spikes in the late afternoon treatment: Treatment of streptococcal tonsillitis/pharyngitis o Penicillin GIM x 1 o Penicillin Voral x 10 days o Sulfaoral x 10 days o Erythromycin (if allergic to above)oral x 10 days o Salicylates used to reduce fever and discomfort and to control the inflammation in the joints Treatment of recurrent RF o Same as above

Kawasaki Disease: an acute systemic vasculitis of unknown cause Self limiting and usually resolves in 6-8 weeks Pts develop cardiac sequelae in which there is damage to the blood vessels that supply the heart muscle and causes damage to it. o Ectasia: dilation of the coronary arteries or coronary artery aneurysm formation Autoimmune disease Affects vascular bed Only affects kids S&S: in acute phase begins with an abrupt onset f high fever that is unresponsive to antibiotics and antipyretics. bulbar conjunctivae of the eyes become reddened with clearing

around the iris, eyes are dry, inflammation of the pharynx and the oral mucosa develops with red cracked lips and the characteristic :strawberry tougue, rash on the perineum, hand s and feet become edematous, irritable and unconsolable, Treatment: o IV IgG o ASA 80-100 mg/kg/dayfever o Then 3-5 mg/kg/dayantiplatelet Nursing care: monitor cardiac status, I&O and daily weight, fluids, minimize skin discomfort by applying cool cloths, unscented lotions, loose fitting clothes, provide mouth care, clear liquids and soft foods, monitor temp, IV Immune globulinhigh dose to reduce duration of fever and incidence of coronary artery abnormalities. Given within the 1st 10 days of the illness. aspirininitially as antiinflammatory 80-100 mg/kg/day in divided doses q6h for fever and inflammation. After fever gone, cont to use as an antiplatelet dose @ 3-5 mg/kg/day. Continue till platelet count retn to nl If coronary abnormalitiescontinue ASA indefinitely. Coumadin (warfarin) possible for children w/ giant aneurysms (> 8 mm)

Systemic Hypertension: Prehypertension in young people is defined as a BP that falls above the 90th percentile for age and height, but does not meet the criteria for true hypertension. Stage 1 hypertension is classified as a BP that falls persistently between the 95th and 99th percentile. Etiology- is secondary to a structural abnormality or an underlying pathologic process, although the results of screening programs of relatively healthy children have challenged this view. S&S- depend on underlying cause. Older and adolescent children complain of headaches, dizziness, or changes in vision, infant and small children- irritable and often indulge in an abnormal degree of head banging or rubbing. DX: Measure Bp in children of any age if they are diagnosed as having or are suspected of having coarctation of the aorta, unexplained heart failure, unexplained heart murmurs, prematurity, unexpected seisures or toher neurologic signs an abonormal mass or masses, edema, ascites, evidence of renal failure, failure to thrive possible obstructive sleep apnea, respiratory distress, hyperlipidemia, and unexplained headaches, check BMI because of associated to obesity with hypertension. o Measure BP in sitting position on 3 different occasions. o 24 hours BP o Urinalysis o Urine culture o Renal function studies Creatinine and BUN o Lipid profile o Fasting glucose level

o CBC o Electrolyte level o Urine and blood catecholamine renin, and aldosterone levels. o *** Due to structural abnormality, renal disease, Pt no angiotension 1 or 2 Therapeutic Management- Surgical repair o Dietary practices and lifestyle changes o Weight reduction o Lessen sodium intake o Regular exercise o Drug therapy-ACE inhibitors  Lisinopril, captopril, and enalapril o Beta blockers- amlodipine, angiotensin receptor blockers, losartan and diuretics o DRUG ALERT= beta blockers cause lipid abnormalities or mood disturbances such as depression in some children. In addition, ACE inhibitors and angiotensin receptor blockers are teratogenic and therefore should not be used by teenage girls who are at risk of getting pregnany.

HYPERLIPIDEMIA: is a general term for excessive lipids. High lipid or cholesterol levels play important part in producing atherosclerosis.

Risk factors: positive family history of elevated cholesterol or early heart disease. Cigarrette smoking, diabetes type 1 and 2, obesity, hypertension. Diagnostic evaluation and screening: blood analysis drawn after 12 hour fast. NOTE: lipid values can be affected by febrile illnesses and therefore lipids should not be drawn within 3 weeks of a febrile illness. Full fasting profile is recommended for intital screen in children if family risk factors are present. Treatement- begins with lifestyle modification, if doesnt work will give Cholestyramine and colestipol which are powders that can be mixed into liquids Treatment: heart healthy diet, low fat dairy products, avoid trans fat, reduce sweetened beverages, and lots of fruit and veggies and whole grain Increase exercise Medication: lipid lowering drugs such as questran, colestid, HMG-CoA Reductase inhibitors such as atorvastatin, simvastatin, pravastatin

CARDIOMYOPATHY: refers to abonomalites of the myocardium in which the cardiac muscles ability to contract is impaired. Relatively rare in children Possible causes: family or genetic factors, infection, deficiency states, metabolic abnormalities, and collagen vascular diseases. TX: Correct underlying problem if possible And treatment is aimed at managing HF and dysrhythmias

Administration of digoxin, diureticsBeta blockers, calcium channel blockers, dobutamine, nitroprusside which can be dangerous. Amrinone,  Careful monitoring and treatment of dysrhymias are essential. Placement of an implantable defibrillator should be considered for patients at high risk of sudden death due to the ventricular arrhythmias,  For worsening heart failure and signs or poor perfusuion, severely ill children may benefit from mechanical ventilation, oxygen administration, IV inotropic support and IV administration of afterload reduction agents such as milrinone.

Cardiac dysrythmias: abnormal heart rhythums Diagnostic evaluation ECG Holter monitoring Electrophysiologic cardiac catheter Transesophageal recording Bradydysrhythmias o AV block o May use pacemaker Tachydysrhythmias o SVT most common tachydysrhythmia o Treatments: vagal maneuvers such as applying ice to the face, massaging the carotid artery, valsalva maneuver  IV adenosine for emergency situation: must be given by rapid IV push with a saline bolus immediately following. Incrementally increasing doses given 2 minutes apart may be needed. Digoxin Esophageal overdrive pacing or synchronized cardioversion can be done in an intensive care setting Radiofrequency, cryablation

Heart transplant: indication are cardiomyopathy and end stage CHD and is an option for pts with some forms of complex congenital cardiac defects such as hypoplastic left heart syndrome for whom conventional surgery approaches have a mortality. Orthotopic: removal of the recipients own heart and implantation of anew heart from a donor who has experienced brain death but whose heart is healthy Heterotopic: the recipients own heart is left in place and a new heart is implanted to act as an additional pump or piggyback heart Leading cause of death in the first 3 years after heart transplant is rejection with the greatest risk in the first 6 months Immunosuppressents must be taken for life

Triple drug therapy for immunosuppression with a calcineurin inhibitor (cyclosporine and tacrolimus), steroids and azathioprine is most commonly used.

Constiaption: An alteration in the frequency, consistency, or ease of passage of stool May be secondary to other disorders Idiopathic (functional) constipationno known cause Obstipation: having extremely long intervals between defecation Encopresis: constipation with fecal soiling Chronic constipationmay be due to environmental or psychosocial factors First meconium should be passed within 24 to 36 hours of life; if not assess for: o Hirschsprung disease, hypothyroidism o Meconium plug, meconium ileus (CF) Infancy o Often related to diet o Constipation in exclusively breastfed infant almost unknown o Infrequent stool may occur because of minimal residue from digested breast milk o Formula-fed infants may develop constipation o Interventions - adding cereals, fruits and vegetables may help after 4 months Childhood o Often due to environmental changes or control over body functions o Encopresis: inappropriate passage of feces, often with soiling o May result from stress o Management History of bowel patterns, medications, diet Educate parents and child Dietary modifications (age appropriate) increase fiber

Hirschsprung disease: congenital anomally that results in mechanical obstruction from inadequate motility of the part of the intestine Mutations in the RET protooncogene have been found in these pts Also called congenital aganglionic megacolon Mechanical obstruction from inadequate motility of intestine Incidence: 1 in 5000 live births; more common in males and in Down syndrome Absence of ganglion cells in colon Aganglionic segment usually includes the rectum and proximal colon Accumulation of stool with distention Failure of internal anal sphincter to relax

Enterocolitis may occur S&S: o Newborn period: failure to pass meconium in 24-48 hours after birth, refusal to eat, bilious vomiting, abdominal distention o Infancy: failure to thrive, constipation, abdominal distention, episodes of diarrhea and vomiting, signs of enterocolitis such as explosive diarrhea, fever, appears very ill o Childhood: constipation, ribbonlike foul smelling stool, abdominal diastention, visible peristalsis, easily palpable fecal mass, undernourished To confirm diagnosis rectal biopsy is performed either surgically to obtain full thickness biopsy specimen or by suction biopsy for histologic evidence of the absence of ganglion cells Aganglionic segment usually includes the rectum and proximal colon Accumulation of stool with distention Failure of internal anal sphincter to relax Enterocolitis may occur A noninvasive procedure that may be used is anorectal manometry Treatment: frequent enemas, surgery, possible colostomy o Pre-op pt needs to empty the bowel by repeated saline enemas, and decrease bacterial flora with oral or systemic antibiotics and colonic irrigation by using antibiotic solution o Enterocolitis is most frequent complication o Post op measure abdominal circumference Surgery Two stages Temporary ostomy Second stage pull-through procedure

Gastroesophageal reflux: the transfer of gastric contents into the esophagus Defined as transfer of gastric contents into the esophagus Occurs in everyone Frequency and persistency may make it abnormal May occur without GERD GERD may occur without regurgitation o GERD represents symptoms or tissue damage that result from GER o Causes that predispose pt to GERD: neurologic impairment, hiatal hernia, repaired esophageal atresia, morbid obesity Infants who are prone to develop GER are premature or who have bronchopulmonary dysplasia Children who have had tracheoesophageal or esophageal atresia repairs, neuologic disorders, scoliosis, asthma, cystic fibrosis, or celebral palsy are also prone to GER S&S: o Infancy: passive regurgitation or emesis, recurrent vomiting in the first three months of life

o Children: intermittent vomiting, heartburn, regurgitation, reswallowing Diagnosis: Gi series, 24 hour intraesophageal pH monitoring study is the gold standard, endoscopy with biopsy, scintigraphy (detects radioactice substances in the esophagus after a feeding of the compound and assess gastric emptying. Treatment: avoidance of caffine, citrus, tomatoes, alcohol, spicy or fried food, small frequent feedings, feedings thickened with rice ceral Treatment of GERD: tagament, zantac, pepcid, PPIs, nexium, prevacid, prilosec, protonix and aciphex Surgery is reserved for pts with severe complications o Most common procedure is nissen fundoplication

Irritable bowel syndrome: Identified as cause of recurrent abdominal pain in children Classified as a functional GI disorder Alternating diarrhea and constipation Cause is unclear Pts complain of alternating diarrhea and constipation, flatulence, bloating or a feeling of abdominal distention, lower abdominal pain, a feeling of urgency when needing to defecate, and a feeling of incomplete evacuation of bowel.

Acute Appendicitis: inflammation of the vermiform appendix Most common cause of emergency abdominal surgery in children Classically the first symptom is periumbilical pain followed by nausea, RLQ pain, and later vomiting with [Link] also has rebound tenderness, rovsing sign (tenderness that occurs in the RLQ that occurs during palpation and percussion of other quadrants, tachycardia, rapid shallow breathing, rigid abdomen, pallor, lethargy, stooped posture Cause is obstruction of the lumen of the appendix usually by hardened fecal material, swollen lymphoid tissue frequently occurring after a viral infection can also obstruct appendix, a parasite such as enterobius vermicularis, or pinworms, which can obstruct the lumen Diagnosis: CT scan, palpation, pain is cardinal feature, rebound tenderness Treatment: surgical removal of appendix, antibiotic pre op, IV fuids and electrolytes pre op, pain meds Management of a pt with ruptured appendix begins eith IV fluids and electrolytes, systemic antibiotics, and NG suction, o Post-op; comtinue with fluids and antibiotics, NG suction for abdominal decompression

Meckel Diverticulum: failure of obliteration of the omphalomesenteric duct resulting in omphalomesentic fistula

Most common congenital malformation of the GI tract Occurs in 1% to 3% of population S&S: imflammation, bleeding or intestinal obstruction, painless bright red rectal bleeding, jelly like stools, Diagnosis: xray, CT scan, wireless capsule endoscopy, mesenteric angiography Treatment: surgical removal o In diverticulitis antibiotics may be given pre op o Post op: pt needs IV fluids and NG tube

Ulcerative Colitis: inflammation of the colon and rectum with the distal colon and rectum the most severely affected Clinical manifestations ulceration, bleeding, anorexia, anemia, severe diarrhea Suppository and enema are used for left sided colitis

Crohns Disease: involves any part of the GItract from the mouth to the anus but most often affects the terminal ileum Clinical manifestations-abdominal pain, severe diarrhea and even malnutrition Extraintestinal manifestations-arthritis, skin problems, fever, anemia, severe anorexia, weight loss and growth retardation, moderate to severe diarrhea, mild rash Therapeutic management Medical- corticosteriods, Remicade for remission, 6-MP Surgical Nursing considerations nutritional support, education

For both UC and CD: Corticosteroids such as prednisone and prednisolone are given Immunomodulators such as azathioprine are used to induce and maintain remission Antibiotics such as metronidazole and cipro Growth failure is common Treatment: surgery

Peptic ulcer disease: Etiology and pathophysiology -Loss of tissue of mucosal, submucosal, and even muscular layer Significant relationship between H-pylori and ulcers, alcohol and smoking Gastric ulcers involves the mucosa of the stomach Duodenal ulcers involves the pylorus and the duodenum Most likely cause is from an imbalance between the destructive and defensive factors in the GI tract Zollinger-ellison syndrome may occur in pts who have multiple large or recurrent ulcers

S&S: chronic abdominal pain, especially when stomach is empty such as during the night or early moring, recurrent vomiting, hematemesis, melena, chronic anemia, abdominal tenderness Diagnostic evaluation upper GI, endoscopy Therapeutic management Medical treat increased [Link] PPI, amoxicillin, flagyl Surgical Nursing considerations -stress