Congenital Heart Disease(CHD) Caused by improper development of the structure of the heart and great vessels Happened during

ing the 1st-8th weeks of fetal development Idiopathic(No known cause)

Risk Factors Maternal Infection(Rubella) Drugs and alcohol intake Family History of CHD Genetic/Chromosomal Disorders

Major Classifications 1. Acyanotic Heart Defect(L-R Shunting) 2. Cyanotic Heart Defect(R-L Shunting) I. ACYANOTIC HEART DEFECT(L-R)

1. DISORDERS WITH INCREASED PULMONARY BLOOD FLOW -abnormal connection between the great arteries that allows blood to flow from the high pressure left side or the heart to the low pressure right side of the heart. a. b. c. d. VSD-Ventricular Septal Defect ASD- Atrial Septal Defect PDA- Patent Ductus Arteriosus AVC- Atrioventricular Canal Defect

2. DISORDERS WITH THE OBSTRUCTION OF BLOOD FLOW -blood leaving the heart meets an area of anatomic narrowing(stenosis), causing obstruction to blood flow.

a. CoA-Coarctation of the Aorta b. AoS- Aortic Stenosis c. PS- Pulmonic Stenosis

ACYANOTIC HEART DEFECT DISORDERS WITH INCREASED PULMONARY BLOOD FLOW

VENTRICULAR SEPTAL DEFECT

-abnormal opening in the wall between the two ventricles Assessment Easy fatigue Loud harsh murmur at the Left Sternal Border at the 3rd or 4th ICS. Thrill(vibration) may be palpate ECG reveals right ventricular hypertrophy Cardiac catheterization reveals increased O2 saturation at Right side of the heart

Therapeutic Management 85% of VSD are small and close spontaneously Moderate in size VSD may be closed by cardiac catheterization Larger size VSD over 3mm require open heart surgery Exceptionally large requires Silastic or Dacron Patch Nursing Management Cardiac catheterization site at Right Femoral Vein NPO 6 hours before the procedure Protect site of catheterization. Avoid flexion of joints proximal to site Asses for complications:infection, thrombus formation-check for pedal pulse

ATRIAL SEPTAL DEFECT

Abnormal communication between left and right atrium due to failure of the foramen ovale to close More common in girls

Assessment
S2 harsh murmur sound at the 2nd or 3rd ICS ECG reveals right ventricular hypertrophy Easy fatigue Cardiac catheterization reveals increased O2 saturation in the right atrium

Therapeutic Management
Interventional Cardiac Catheterization Open heart surgery between 1 and 3 years of age(w/o repair puts child at risk for infectious endocarditis and emboli during pregnancy) Silastic or Dacron Patch for large defect

PATENT DUCTUS ARTERIOUSUS

-failure of the artery(ductus arteriosus)connecting the aorta and the pulmonary artery to close within 1-3 months

Assessment
Machinery Murmur at upper left sternal border Wide Pulse Pressure Bounding femoral pulses ECG reveals ventricular hypertrophy

Therapeutic Management
Indomethacin or Ibuprofen(NSAID, Prostaglandin Inhibitors-facilitates closing) Ligatation of PDA by 3-4 y/o via thoracotomy procedure If Indomethacin is given assess for side effects: o Decrease glomerular filtration o Impaired platelet aggregation o Decrease GI and cerebral blood flow Ibuprofen- drug of choice due to fewer side effects and used as prophylasxis in preterm infants

ATRIOVENTRICULAR CANAL DEFECT

also called ENDOCARDIAL CUSHION results from the incomplete fusion of endocardial cushion which is the septum of the heart at the junction of the atria and ventricles Assessment is the same with Ventricular Septal Defect most common cardia defect in DOWN SYNDROME Infant usually has mild to moderate CHF Surgical Treatment: either pulmonary artery banding or repair via cardiopulmonary bypass

ACYANOTIC HEART DEFECT DISORDERS WITH OBSTRUCTION OF BLOOD FLOW

COARCTATION OF THE AORTA

-stricture/stenosis at the segment just after the aortic arch Signs and Symptoms Mild:(-) femoral pulses Severe: headache, vertigo, leg cramps, fatigue, epistaxis BP 20mmHg higher in arms Systolic Murmur at the base of the heart Hypertension

AORTIC STENOSIS
-narrowing of the aortic valve causing: Resistance to flow from the left ventricle Decreased cardiac output Left ventricular hypertrophy Pulmonary Vascular Congestion

Signs and Symptoms

Faint Pulse Hypotension Tachycardia Dizziness with long standing Chest pain similar to angina(when active) Inability to suck

Therapeutic Management Betablocker or Calcium Channel Blocker to reduce hypertrophy Surgery: o Aortic Valvotomy o Balloon Angioplasty

II.

CYANOTIC HEART DEFECT 1. DISORDERS WITH PULMONARY BLOOD FLOW

-involve some type of the obstruction to pulmonary blood flow that causes pressure increase in the right side of the heart -this result in deoxygenated blood invading the systemic circulation Tricuspid Atresia Tetralogy of Fallot

TRICUSPID ATRESIA
-Failure of the tricuspid valve to develop -there is no communication between the right atrium and the right ventricles -blood flows through an ASD to the left side of the heart bypassing the lungs -blood reaches the lungs via PDA Signs and Symptoms Cyanosis Tachycardia Dyspnea Signs of chronic hypoxemia with clubbing of fingers Therapeutic Management Prostaglandin E1(ensure Ductus remain open) Systemic to Pulmonary Artery Shunt Atrial Septomy Pulmonary Artery Banding Bidirectional Glenn Stunt(cavopulmonary anastomosis) Modified Fontan procedure

TETRALOGY OF FALLOT
-four abnormalities that results in the insufficiently oxygenated blood pumped to the body -VSD, Overriding Aorta,PS, Right Ventricular Hypertrophy -develop syncope(fainting) and hypoxic episodes caused by decrease blood and oxygen to the brain usually during crying or after feeding

*Note If baby begins to have hypoxic episode administer oxygen and place in a knee chest or squatting position to trap blood in the lower extremities and keep the heart from being overwhelmed Signs and Symptoms Cyanosis Clubbing Thrill/Murmurs at 2nd-4th ICS to clavicular area Chest deformity Severe Dyspnea Retarded Growth Therapeutic Management O2 administration after 1 month old to wait for the complete closure of the ductus arteriosus No valsalva maneuver, fiber diet laxative Morphine Sulfate to reduce the ventilatory drive and decrease systemic venous return Propanolol to aid in pumonary artery dilation Palliative repair-BLT(Blalock Taussig Pocedure) Brock Procedure-complete procedure