The Child with Cardiovascular Dysfunction

The Child with Cardiovascular Dysfunction
Chapter 34

Changes at Birth Fetal: Blood enters from umbilical vein To liver
To inferior vena cava via ductus venosus A, Prenatal circulation. B, Postnatal circulation. Arrows indicate direction of blood flow. Although four pulmonary veins enter the LA, for simplicity this diagram shows only two. RA, Right atrium; LA, left atrium; RV, right ventricle; LV, left ventricle. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Pediatric Indicators of Cardiac Dysfunction
Poor feeding Tachypnea/tachycardia Failure to thrive/poor weight gain/activity intolerance Developmental delays Prenatal history Family history of cardiac disease Cardiac dysfunction>>hypoxia Maslow’s hierarchy: Hypoxia>>poor feeding, poor growth, poor development Prenatal Hx: Preterm birth; IDM; IUGR more likely to have congenital anomalies than general population Family Hx: Frequent fetal loss; sudden infant deaths; sudden deaths in adults>>Heart disease Hx of congenital heart defects Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

“Innocent Murmurs” Murmurs = heart sounds that reflect flow of blood within the heart May occur in systole or diastole, or both Can occur in a normal heart in periods of stress: anemia, fever, or rapid growth Can reflect abnormalities in heart or vessels “Innocent murmurs” = normal cardiac anatomy and cardiac function Occur in up to 50% of all kids at some time Murmurs = heart sounds that reflect flow of blood within the heart Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Thrills The sound of a thrill is a soft vibration over the heart that reflects the transmitted sound of a heart murmur Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Conduction System of the Heart
Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Electrode Placement for Standard Chest Lead II

Tests of Cardiac Function
Chest x-ray ECG Echocardiography Cardiac catheterization Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Hypoplastic Left Heart
Left side of heart is underdeveloped. Left ventricle is small and aortic atresia. Most blood flows across patent foramen ovale to Right atrium..to Right ventricle and out the pulmonary artery. Descending aorta receives blood from the PDA to supply the systemic circulation. PDA closure >>rapid deterioration and CHF. TX: Keep ductus open w/ Prostaglandin E infusion. SURG TX: #1 is Norwood procedure to create a new aorta using the main pulmonary artery and creation of large ASD. #2 is Bidrectional Glenn Shunt at 6-9 mos age to reduce volume load on the R ventricle. #3 is modified Fontan procedure, similar to Tricuspid atresia repair. Transplant may be option for some pts. Mortality rate very high (30-50% mortality rates). Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Interventional Cardiac Catheterization Procedures in Children
Transposition of great vessels Some complex single-ventricle defects ASD Pulmonary artery stenosis Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Interventional Cardiac Catheterization Procedures in Children (cont.)
DIAGNOSIS INTERVENTION Valvular pulmonic stenosis Balloon dilation Recurrent coarctation of aorta Congenital mitral stenosis Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Two Types of Cardiac Defects
Congenital Anatomic → abnormal function Acquired Disease process Infection Autoimmune response Environmental factors Familial tendencies Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Causes of CHD Maternal or environmental = 1% to 2% Maternal drug use
Fetal alcohol syndrome—50% have CHD Maternal illness Rubella in first 7 weeks of pregnancy → 50% risk of defects including PDA and pulmonary branch stenosis CMV, toxoplasmosis, other viral illnesses → cardiac defects IDMs = 10% risk of CHD (VSD, cardiomyopathy, TGA most common) Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Causes of CHD (cont.) Chromosomal/genetic = 10% to 12%
Multifactorial = 85% Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

CHD Incidence: 5 to 8 per 1000 live births
About 2 or 3 of these are symptomatic in first year of life Major cause of death in first year of life (after prematurity) Most common anomaly is VSD 28% of kids with CHD have another recognized anomaly (trisomy 21, 13, 18, ) 5-8/ 1000 live births [excluding PDAs in Pretermers] Trisomy 21 = Down 30%-50% have structural heart defect Most common defect w/Down is endocardial cushion defects (ECD) = ASD and VSD Syndromes commonly associated w/ CHD Noonan syndrome Williams syndrome Holt-Oram syndrome DiGeorge syndrome Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Older Classifications of CHD
Acyanotic May become cyanotic Cyanotic May be pink May develop CHF Very confusing; newer diagnostic categories now Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Newer Classifications of CHD
Hemodynamic characteristics Increased pulmonary blood flow Decreased pulmonary blood flow Obstruction of blood flow out of the heart Mixed blood flow Table on pg 1476 in blue; 1595 in orange Increase Pulmonary blood flow>>CHF Decreased Pulmonary blood flow >>cyanosis Obstructive defects decrease blood flow out of the ventricles Obstructed Left heart >>CHF Obstructed Right heart>>Cyanosis Mixed blood flow Depends on the degree of mixing May have hypoxemia with or without cyanosis May have CHF also Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Comparison of CHD Classification Systems

Increased Pulmonary Blood Flow Defects
Abnormal connection between two sides of heart Either the septum or the great vessels Increased blood volume on right side of heart Increased pulmonary blood flow Decreased systemic blood flow Patients present w/symptoms of congestive failure Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Hemodynamics in Defects with Increased Pulmonary Blood Flow

Increased Pulmonary Blood Flow Defects
Atrial septal defect Ventricular septal defect Patent ductus arteriosus Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

ASD Opening 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/ patch—usually before age 6. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

VSD Defect in ventricular septum—Error in early fetal development
Can occur anywhere in muscle or membranous ventricular septum 20-25% of all CHDs are VSD Manifestations—Depends 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; CHF 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: Cath repairs in clinical trials Surgical repair w/ bypass; Pulmonary artery banding (if not too large) or patch Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

PDA 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-- 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  pulmonary blood flow Manifestations: Small PDA may be asymptomatic Large PDA may be CHF w/ tachypnea, dyspnea, and hoarse cry. 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 Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Obstructive Defects Coarctation of the aorta Aortic stenosis
Pulmonic stenosis Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Sites of Obstruction to Ventricular Ejection

COA 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. 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. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

AS 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. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

PS and Catheter Placement
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 TX—Brock procedure (Bypass to do valvotomy). Usually can repair w/ catheterization. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Decreased Pulmonary Blood Flow Defects
Tetralogy of Fallot Tricuspid atresia Pulmonary blood flow is obstructed + have defect of ASD or VSD.>>Blood backs up in R side of heart. Causes desaturated blood to shunt to the left, and into systemic circulation. Usually hypoxemic and usually cyanotic. Most common defects are TET & tricuspid atresia. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

TOF 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. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Tricuspid Atresia 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). Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Mixed Defects Transposition of great vessels
Total anomalous pulmonary venous connection Hypoplastic heart syndrome Right Left Blood is mixed from pulmonary and systemic circulations within the heart chambers. >>Relative desaturation of blood in systemic blood flow. Cardiac Output decreases because of volume load on ventricle. Signs of desats, cyanosis, and CHF, but variable depending on anatomy. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Transposition of Great Vessels
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. 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. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Normal Heart Remember what normal heart looks like

Total Anomalous Pulmonary Venous Connection
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. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

CHF in Children Impaired myocardial function Pulmonary congestion
Tachycardia; fatigue; weakness; restless, pale, cool extremities; decreased BP; decreased urine output Pulmonary congestion Tachypnea, dyspnea, respiratory distress, exercise intolerance, cyanosis Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

CHF in Children (cont.) Systemic venous congestion
Peripheral and periorbital edema, weight gain, ascites, hepatomegaly, neck vein distention Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Care of the Family and Child with Congenital Heart Disease
Help family adjust to the disorder Educate family Help family cope with effects of the disorder Prepare child and family for surgery Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Surgical Interventions
Open heart Closed heart procedures Staged procedures Prepare child and family for procedures 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.) Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Postoperative Care for the Child
Monitor vital signs and A/V pressures Intra-arterial monitoring of BP Intracardiac monitoring Respiratory needs Rest, comfort, and pain management Fluid management Progression of activity Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Postoperative Complications
CHF Dysrhythmias Decreased cardiac output syndrome Decreased peripheral perfusion Pulmonary changes Neurologic changes Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Postpericardiotomy Syndrome
Symptoms: fever,  WBCs, pericardial friction rub, pericardial and pleural effusion Occurs in immediate postoperative period Also can occur later (days 7 to 21 postop) Etiology unknown Theories of etiology Viral infection, autoimmune response, reaction to blood in pericardium May require pericardiocentesis or pleurocentesis Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Acquired Cardiovascular Disorders
Infectious and inflammatory cardiac disorders These are things that can occur in the normal heart or in addition to congenital heart defects. Infection, autoimmune response, environmental factors, familial tendencies. Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Endocarditis BE, IE, or SBE Streptococci Staphylococci
Fungal infections Prophylaxis: 1 hour before procedures (IV) or may use PO in some cases 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 Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Rheumatic Fever (RF) and Rheumatic Heart Disease
Inflammatory disease occurs after group A β-hemolytic streptococcal pharyngitis Infrequently seen in U.S.; big problem in Third World Self-limiting Affects joints, skin, brain, serous surfaces, and heart Rheumatic heart disease Most common complication of RF Damage to valves as result of RF Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Clinical Manifestations of RF
Carditis Polyarthritis Erythema marginatum Subcutaneous nodules Carditis involves endocardium, pericardium, and myocardium; most commonly the mitral valve Arthritis is reversible and migrates, especially in large joints (knees, elbows, hips, shoulders, wrists) Erythema marginatum—Rash; usually on trunk and proximal portion of extremities. Red macule w/ clear center and wavy, well-demarcated border Sub-q nodules: small nontender nodules appear over bony prominences (hands, feet, elbows, scalp, scapulae, vertebrae) persist indefinitely after onset of the disease and resolve w/ no resulting damage. Aschoff bodies: Inflammed Hemorrhagic bullous lesions that cause swelling, fragmentation and alterations in connective tissue. Found in the heart, blood vessels, brain and on serous surfaces of joints and pleura Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

St. Vitus Dance: The Fifth Manifestation
St. Vitus dance (aka, chorea) reflects CNS involvement Definition: Chorea refers to sudden, aimless movements of extremities, involuntary facial grimaces, speech disturbances, emotional lability and muscle weakness Worse with anxiety and relieved by rest Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Prevention of RHD Treatment of streptococcal tonsillitis/pharyngitis
Penicillin G—IM x 1 Penicillin V—oral x 10 days Sulfa—oral x 10 days Erythromycin (if allergic to above)—oral x 10 days Treatment of recurrent RF Same as above Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Kawasaki Disease Treatment
IV IgG ASA mg/kg/day—fever Then 3-5 mg/kg/day—antiplatelet IV Immune globulin—high dose to reduce duration of fever and incidence of coronary artery abnormalities. Given within the 1st 10 days of the illness. ASA—initially as antiinflammatory mg/kg/day in divided doses q6h for fever and inflammation. After fever gone, cont to use as an antiplatelet 3-5 mg/kg/day. Continue till platelet count retn to nl If coronary abnormalities—continue ASA indefinitely. Coumadin (warfarin) possible for children w/ giant aneurysms (> 8 mm) Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Systemic Hypertension
Primary = no known cause Secondary = identifiable cause Pediatrics: HTN generally secondary to structural abnormality or underlying pathology Renal disease CV disease Endocrine or neurologic disorders Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

BP Screenings for Children
Routine w/ annual checkups; With any illness Dx or suspicion of CV disease, Sz, neuro disease, abd mass, edema, ascites, failure to thrivive, unexplained h/a Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Cardiac Dysrhythmias (cont.)
Bradydysrhythmias AV block May use pacemaker Tachydysrhythmias SVT most common tachydysrhythmia Treatments Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

Heart Transplantation
Orthotopic transplant Heterotopic transplant (piggyback) Organ donation issues Nursing considerations Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.

The Child with Gastrointestinal Dysfunction
Chapter 33

Absorption Principally from small intestine Large intestine Osmosis
Carrier-mediated diffusion Active energy-driven transport (“pump”) Large intestine Absorption of water Absorption of sodium Role of colonic bacteria

Ingestion of Foreign Substances
Pica Food picas Nonfood picas Foreign bodies Nursing considerations

DISORDERS OF MOTILITY

Constipation An alteration in the frequency, consistency, or ease of passage of stool May be secondary to other disorders Idiopathic (functional) constipation—no known cause Chronic constipation—may be due to environmental or psychosocial factors Pg 891

Newborn Period First meconium should be passed within 24 to 36 hours of life; if not assess for: Hirschsprung disease, hypothyroidism Meconium plug, meconium ileus (CF)

Constipation in Childhood
Often due to environmental changes or control over body functions Encopresis: inappropriate passage of feces, often with soiling May result from stress Management

Nursing Considerations
History of bowel patterns, medications, diet Educate parents and child Dietary modifications (age appropriate)

Hirschsprung Disease 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

Hirschsprung Disease

Clinical Manifestations of Hirschprung Disease
Aganglionic segment usually includes the rectum and proximal colon Accumulation of stool with distention Failure of internal anal sphincter to relax Enterocolitis may occur

Diagnostic Evaluation
X-ray, barium enema Anorectal manometric exam Confirm diagnosis with rectal biopsy

Therapeutic Management
Surgery Two stages Temporary ostomy Second stage “pull-through” procedure

Nursing Considerations
Preoperative care Postoperative care Discharge care

Gastroesophageal Reflux (GER)
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

GER Diagnostics Therapeutic management Nursing considerations

Irritable Bowel Syndrome (IBS)
Identified as cause of recurrent abdominal pain in children (Chapter 18) Classified as a functional GI disorder Alternating diarrhea and constipation Therapeutic management Nursing considerations

Acute Appendicitis Etiology and pathophysiology Diagnostic evaluation
Therapeutic management Ruptured appendix Prognosis Nursing considerations

Inflammatory Bowel Disease (IBD)
Includes ulcerative colitis (UC) and Crohn disease (CD) Etiology and pathophysiology Diagnostic evaluation Therapeutic management Medical treatment Nursing considerations

Ulcerative Colitis (UC)
Pathophysiology Clinical manifestations Extraintestinal manifestations

Crohn Disease Pathophysiology Clinical manifestations
Extraintestinal manifestations Therapeutic management Medical Surgical Nursing considerations

Effects of UC or Crohn Disease

OBSTRUCTIVE DISORDERS

Hypertrophic Pyloric Stenosis (HPS)
Constriction of the pyloric sphincter with obstruction of the gastric outlet

Pyloric Stenosis Pathophysiology Diagnostic evaluation
Therapeutic management Prognosis Nursing considerations

Hypertrophic Pyloric Stenosis

Intussusception Telescoping or invagination of one portion of intestine into another Occasionally due to intestinal lesions Often cause is unknown Diagnostic evaluation Therapeutic management Prognosis Nursing considerations

Ileocolic Intussusception

Malrotation and Volvulus
Malrotation is due to abnormal rotation around the superior mesenteric artery during embryonic development Volvulus occurs when intestine is twisted around itself and compromises blood supply to intestines May cause intestinal perforation, peritonitis, necrosis, and death

Malabsorption Syndromes
Characterized by chronic diarrhea and malabsorption of nutrients May result in failure to thrive Digestive defects Absorptive defects Anatomic defects

Celiac Disease Also called gluten-induced enteropathy and celiac sprue
Four characteristics Steatorrhea General malnutrition Abdominal distention Secondary vitamin deficiencies

Celiac Disease (cont.) Pathophysiology Diagnostic evaluation
Therapeutic management Nursing considerations

Short Bowel Syndrome (SBS)
A malabsorptive disorder Results from decreased mucosal surface area, usually as result of small bowel resection Etiology and pathophysiology Result of decreased mucosal surface area, usually due to extensive resection of small intestine Other causes NEC, volvulus, gastroschisis, Crohn disease in

Therapeutic Management of SBS
Nutritional support—first phase: TPN Associated risks and complications Second phase: enteral feeding Long-term maintenance Medical therapies Surgical therapies Nursing considerations

GI Bleeding Upper GI bleeding Lower GI bleeding Esophagus Stomach
“Coffee grounds” emesis Hematemesis Lower GI bleeding Bright red (rectal bleeding): hematochezia Tarry stools: melena

GI Bleeding (cont.) Diagnostic evaluation Therapeutic management
Assess blood loss Establish hemodynamic stability Nursing considerations

Types of Diarrhea Acute Acute infectious/infectious gastroenteritis
Chronic Intractable diarrhea of infancy Chronic nonspecific diarrhea (CNSD)

Anorectal Malformations
Imperforate anus Persistent cloaca Cloacal exstrophy Genitalia may be indefinite Diagnostic evaluation Management

Dehydration Types of dehydration Diagnostic evaluation
Therapeutic management Nursing considerations

Daily Maintenance Fluid Requirements
Calculate child’s weight in kg Allow 100 ml/kg for first 10 kg body weight Allow 50 ml/kg for second 10 kg body weight Allow 20 ml/kg for remaining body weight

Example 1: Daily Fluid Calculation
Child weighs 32 kg 100 x 10 for first 10 kg of body weight = 1000 50 x 10 for second 10 kg of body weight = 500 20 x 12 for remaining body weight = 240 = 1740 ml/24 hr

Child weighs 8.5 kg 100 x 8.5 for first 10 kg of body weight = 850 No further calculations 850 ml/24 hr

Child weighs 14 kg 100 x 10 for first 10 kg of body weight = 1000 50 x 4 for second 10 kg of body weight = 200 No further calculations = 1200 ml/24 hr

The Child with Cardiovascular Dysfunction

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