ALTERATIONS IN OXYGENATION: CARDIAC AND TISSUE PERFUSION

HOMOGRAFT CONDUIT without tx, prognosis is poor with 80% mortality during 1st year of life.

(TRUNCUS ARTERIOSUS - TREATMENT) a chemiclly created human aorta or pulmonary artery obtained form a cadaver that is placed like a tube to conduct blood from the RV to the PA.

DIAGNOSIS - TRUNCUS ARTERIOSUS

- CXR ; moderate cardiomegaly - ECHOCARDIOGRAM - CARDIAC CATHERIZATION to determine anatomic variations and hemodynamic

CLINICAL MANIFESTATION - TETRALOGY OF FALLOT

- Cyanotic or Acyanotic - systolic murmur - Hyper-cyanotic episodes "tet spells" tet spells - hyperpnea, deep, rapid respirations

INCIDENCE AND ETIOLOGY - CONGESTIVE HEART FAILURE

- congenital heart defects are the most common causes of CHF in infants. - acquired disease is most common in OLDER CHILDREN AND ADOLESCENTS.

ATRIOVENTRICULAR SEPTAL DEFECT - POST OPERATIVE COMPLICATIONS

- heart block - arrhythmias - bleeding from the multiple intracardiac suture lines - pulmonary HPN - poor cardiac output ST poorly functioning or incompetent AV valves

VENTRICULAR SEPTAL DEFECT: DIAGNOSIS

- loud holosystolic murmur is hard - CXR - small ; normal moderate to large; cardiomegaly with an increase pulmonary blood flow. - ECHOCARDIOGRAM

TREATMENT VSD: infants with small VSD require no tx as 75-8-% of these defects will close spontaneously large VSD:

1 Digoxin 2 ACE inhibitors 3 high caloric formula to promote growth 4 nasal gastric feeding for infants who tire with feedings 5 Surgical Repair between 3 to 12 months of age ; necessary to prevent development of pulmonary vascular disease. 6 the defect is closed with a stitch or with synthetic material while on CBP.

POSTOPERATIVE COMPLICATION - TETRALOGY OF FALLOT

1. Low cardiac output and Arrhythmias LONG TERM COMPLICATIONS - Residual VSD - Pulmonary regurgitation

DEFECTS WITH INCREASED PULMONARY BLOOD FLOW

1. are caused by a shunting of the blood from the left side of the heart to the right side through an abnormal connection (L to R shunting) 2. clinally manifest CHF

TETRALOGY OF FALLOT "baby blue" - MADE UP OF 4 COMPONENTS

1.VENTRICULAR SEPTAL DEFECT 2.PULMONARY STENOSIS 3.RIGHT VENTRICULAR HYPERTROPHY OVERRIDING AORTA the degree of PS is most important as it's severity will determine clinical presentation, timing of surgery, and preop management

ATRIAL SEPTAL DEFECT

AN ABNORMAL CONNECTION BETWWEN THE RIGHT AND LEFT ATRIA. - ACYANOTIC DEFECT

evaluate feeding behaviors - during feeding does the infant exhibit diaphoresis, SOB, fatigue, cyanosis. INFANT HYPERCYANOTIC SPELLS cyanosis of the lips and tongue irritability inconsolability poor weight gain poor feeding OLDER CHILD height weight exercise intolerance Syncope frequent respiratory infections palpitations

ASSESSMENT OF THE CHILD WIHT CARDIOVASCULAR ALTERATION

DIURETICS * Surgical repair at preschool age * OPEN HEART SURGERY via the median sternotomy- vertical incision along the sternum * NONSURGICAL DEVICE PLACEMENT -performed in cardiac catheterization laboratory -device is placed via a catheter in a large femoral vessel, threaded up into the heart and across the ASD.

ATRIAL SEPTAL DEFECT: TREATMENT

murmurs (describe location, timing, frequency and intensity.)

AUSCULTATION

Phosphodiesterase

Amrinone Milrinone

POST OP COMPLICATIONS: VENTRICULAR SEPTAL DEFECT

Atrial/ ventricular arrhythmias Complete heart block residual shunt or leaking at the site of VSD patch

infant or child is generally asymptomatic soft murmur (widely split S2)

CLINICAL MANIFESTATIONS: ASD

- arrhythmias - heart block ST edema around or surgial interruption of the conduction system.

COMPLICATION: ASD

DIAGNOSIS: ATRIOVENTRICULAR SEPTAL DEFECT

CXR Echocardiogram (ECG)

1. the amount of blood flow to the pulmonary system is decreased resulting in shunting of UNOXYGENATED blood from the RIGHT side to the LEFT side of the heart. 2. mixing of oxygenated and unoxygenated blood in the systemic circulation. 3. infants are HYPOXIC and CYANOTIC 4. POLYCYTHEMIA 5. Increased risk of cerebral strokes, bleeding disorders

DEFECTS WITH DECREASED PULMONARY BLOOD FLOW

* murmur during routine exam * CXR - increased hart size * ECHOCARDIOGRAM - shows location and size of the defect

DIAGNOSIS: ATRIAL SEPTAL DEFECT

CXR - cardiac enlargement, pulmonary congestion Echocardiogram - determine congenital defects or cardiomyopathy

DIAGNOSIS: HF

CHILDREN OR ADOLESCENTS - CM

DIMINISHED PULSES DYSPNEA PALLOR ORTHOPNEA EDEMA HEPATOMEGALY

• Systolic heart failure: HF with reduced EF • Diastolic heart failure: HF with preserved ventricular function

Divided into two subtypes: Left Heart Failure

SYMPATHOMIMETICS

Drugs used therapeutically that mimic the catecholamines epinephrine, norepinephrine, and dopamine. Also called adrenergic agonists.

GOALS OF THEARAPY: - Reducing volume overload - improving contractility - reducing afterload - decreasing cardiac output

Goals of theraphy

Family history incidence in high for 1st degree relative.

HISTORY

CARDIAC OUTPUT

Heart Rate (HR) x Stroke Volume (SV) = CO

dysmorphic cyanosis (best inspected under bright natural light). clubbing of fingers

INSPECTION

DIAGNOSTIC TESTS

LABORATORY TESTS PULSE OXIMETRY Electrocardiogram (ECG TEST) Holter Monitor Event monitoring

PULMONARY STENOSIS

NARROWING OF THE PULMONARY VALVE AND OBSTRUCTION TO BLOOD FLOW FROM THE RIGHT VENTRICLW TO THE LUNGS. 8-10% of all CHD

- Allow frequent short periods of time for feeding - Pay attention to infant cues: back arching, averting of eyes: child wants to take a break from feeding

NURSING INTERVENTIONS: HF

normal CO in an infant is - 200ml/kg/min Adolescents - 100 ml/kg/min

Normal CO

1. DIGOXIN TOXICITY: (2) upset GI and Halo sign 2. monitor HR and rhythm 3. hold for HR below than normal for age 4. monitor serum K

Nx Consideration: HF

normal infants may be associated with congestive heart failure.

PALPATION FOR HEPATOMEGALY

- elective closure before the age of 5; use of coils to occlude PDA in the cardiac catheterization laboratory. - for too large PDA; surgical ligation or tying off is indicated.

PATENT DUCTUS ARTERIOSUS TREATMENT - OLDER CHILDREN

INDOMETHACIN IV/oral Ibuprofen-effective as indomethacin with fewer side effects

PATENT DUCTUS ARTERIOSUS TREATMENT - PREMATURE closure of the PDA is attempted medically with infusion of

Mild to Moderate; infant is asymptomatic Severe; - Dyspnea upon exertion - fatigue - Cyanosis

PULMONARY STENOSIS - CLINICAL MANIFESTATIONS

TRUNCUS ARTERIOSUS

RESULTS FROM A FAILURE OF THE EMBRYOLOGIC TRUNK TO DIVIDE INTO THE PULMONARY ARTERIES AND THE AORTA. 1.4% among all CHD; truncus arteriosus can be associated with DiGeorge SYndrome

• Peripheral edema • Ascites • Enlarged liver & spleen • JVD • Weight gain • Increased peripheral venous pressure

RIGHT HF: Symptoms

TREATMENT - TRUNCUS ARTERIOSUS

Surgical Repair within 1-2 weeks of life; 1. Pulmonary Arteries are removed from the common trunk and the defect left in the trunk wall is sutured closed. 2. the VSD is closed and a valved HOMOGRAFT CONDUIT is placed connecting RV to the PAs.

Pulmonary congestion: CC PT • Cough • Crackles • Pink-tinged sputum • Tachypnea • Tachycardia Fatigue Cyanosis Dyspnea

Symptoms of Left HF

CLINICAL MANIFESTATION: ASD

TACHYCARDIA TACHYPNEA GALLOP RHYTHM RETACTIONS DIMINISHED PULSES

NEWBORNS OR INFANT - CM

TACHYCARDIA TACHYPNEA GALLOP RHYTHM RETACTIONS DIMINISHED PULSES

CONGESTIVE HEART FAILURE

THE INABILITY OF THE CARDIOVASCULAR SYSTEM TO PROVIDE ADEQUATE CARDIAC OUTPUT TO MEET METABOLIC DEMANDS OF THE BODY.

- VOLUME OVERLOAD - RESULTANT POLMONARY AND - SYSTEMIC VENOUS CONGESTION.

THESE DISEASES RESULT IN IPAIRED MYOCARDIAL CONTRACTILITY WHICH CAUSES

Valvuplasty Valvotomy

TREATMENT - PULMONARY STENOSIS

1.DIURETIC THERAPY - primary intervention for fluid volume overload. Furosemide (Lasix) - primary diuretic for children. 2. INOTROPES - medications that improve cardiac contractility CARDIAC GLYCOSIDES (DIGOXIN)

TREATMENT: HF

CARDIAC OUTPUT

The volume of blood ejected from the left side of the heart in one minute.

4. ACE inhibitors 5. ARBs 6. Nitrates 7. Nutrition Breast milk fortifier

Treatment for CHF

Left Heart Failure

Usually caused by hypertension, coronary artery disease, & valvular disease

PULMONARY STENOSIS

a significant increase in the RVP ST obstruction of blood flow form the RV to the PA which may lead to RV failure decreased amount of blood flow to the lungs.

PRELOAD

amount of blood in the ventricle at the end of diastole and just prior to systole.

Valvotomy

an incision into a cardiac valve to correct a defect

congenital heart defects

are the most common causes of CHF in infants.

Blalock- Taussing shunt

assure pulmonary blood flow until surgical repair is performed.

Blalock- Taussing shunt

assure pulmonary blood flow until surgical repair is performed. - Complete repair is usually performed between 3 to 12 months of age goals of repair: Widen the RIGHT VENTRICULAR outflow tract and to close the VSD.

ATRIOVENTRICULAR SEPTAL DEFECT

blood flow patterns are dependent on the resistance to the pulmonary and systemic circulations

TRUNCUS ARTERIOSUS

both oxygenated and unoxygenated blood mix together and are ejected from the ventricles out the common trunk. blood flow is then delivered to both the pulmonary and systemic circulation.

CONGENITAL HEART DEFECTS - Correction

complete repair is possible in the neonatal period to 2 to 4 years of age

RHEUMATIC FEVER

conditions that affect the heart, joints, brain, and skin.

CLINICAL MANIFESTATIONS - TRUNCUS ARTERIOSUS

cyanosis, CHF, heart murmur

CARDIOMYOPATHY

disease of the heart muscle resulting in poor pump function.

pulsus paradoxus

excessive variation in systolic pressure with respiration.

SURGICAL CORRECTION is the primary treatment

for infants with CHF ST congenital defects

TRUNCUS ARTERIOSUS

in the 1st few hours, pulmonary vascular resistance is elevated resulting in little increase in pulmonary blood flow and unoxygenated blood is pumped through the systemic circulation making the infant mildly CYANOTIC. when PVR begins to fall, pulmonary flow increases making the shunt L to R.

Phenylephrine IV

inc vascular resistance

ENDOCARDITIS

infection of the inner lining of the heart.

MYOCARDITIS

inflammation of the heart muscle

VENTRICULAR SEPTAL DEFECT

large hole between two ventricles lets venous blood pass from the right to the left ventricle and out to the aorta without oxygenation - most common congenital heart defect; 50% among infants and children.

PATENT DUCTUS ARTERIOSUS

left to right shunts ; blood from the high pressure aorta flows directly into the low pressure pulmonary artery and pulmonary circulation.

PATENT DUCTUS ARTERIOSUS - DIAGNOSIS

murmur best heard below the clavicle CXR ECHOCARDIOGRAM

ATRIAL SEPTAL DEFECT

normal increased pressure on the left side of the heart, blood flows from the L to R across the ASD which leads to increased volume on the right side of the heart.

Blalock- Taussing shunt

performed at 4-6 weeks as a palliative measure - asystemic venous-to-pulmonary connection is established from the brachiocephalic artery or right subclavian artery to the right pulmonary artery through a conduit or graft

"tet spells"

place the infant in a knee chest position - will decrease systemic venous return of unoxygenated blood as well as increase systemic vascular resistance

CARDIOPULMONARY BYPASS

procedure that temporarily circulates and oxygenates a patient's blood during the portion of heart surgery where the heart is stopped

TACHYARRHYTHMIAS

pulse rates that are abnormally fast

BRADYARRHYTHMIAS

pulse rates that are abnormally slow

CARDIOPULMONARY BYPASS

removal of unoxygenated blood via a venous cannula and the delivery of oxygenated blood back via the aortic cannula

AFTERLOAD

resistance that the ventricle must overcome when ejecting blood.

PATHOPHYSIOLOGY

small PDA; asymptomatic large PDA; signs of CHF TACHYCARDIA TACHYPNEA GALLOP RHYTHM RETACTIONS DIMINISHED PULSES

ATRIOVENTRICULAR SEPTAL DEFECT - Treatment

surgical repair ; close the atrial and ventricular septal defects and then construct new mitral and tricuspid valves from the common AV valves. the infant is on CBP

CLINICAL MANIFESTATIONS: VSD

tachypneic diaphoretic fatigues easily underweight for age

PATENT DUCTUS ARTERIOSUS

term newborn; closes within 12 hours and completely closed by 2-3 weeks. results from failure of normal transition from fetal to postnatal circulation INCIDENCE 5-10% of CHD ; on premature 45%; premature infants

ATRIOVENTRICULAR SEPTAL DEFECT

the atria and ventricles are incompletely separated. Common defect with trisomy 21 * CAUSES THE TRICUSPID AND MITRAL VALVES TO DEVELOP INAPPROPRIATELY. 4-10% of CHD 40% of children with Trisomy 21

CONTRACTILITY

the intrinsic ability of the myocardial fiber to shorten and produce a forceful contraction.

INCIDENCE - TETRALOGY OF FALLOT

the most common cyanotic heart defect; 9-14% of all CHD

Volume Resuscitaton

to dec blood viscosity

Morphine Sulfate

to relive sx of agitation and break hyperpnea

Sympathomimetics and Phosphodiesterase

used for px with acute heart failure who do not respond to other medications.

Valvuplasty

using a balloon tipped catheter to dilate cardiac valve. if vavulplasty is not performed, Valvotomy is the tx of choice

Right heart failure

• Can be caused by left ventricular failure, right MI, or pulmonary hypertension • Right ventricle cannot empty completely • May be secondary to pulmonary problems (COPD)