Classifications

Cyanotic defects. Acyanotic defects.

Acyanotic Defects
Involves heart or circulatory anomalies that involve either a stricture to the flow of blood or a shunt that moves blood from the arterial to the venous system (Left-to-right shunt)

Cyanotic
Occurs when blood is shunted from the venous to the arterial system as a result of abnormal communication between the two systems (right-to-left shunts)

Another classification system has been established that addresses the hemodynamic and blood floe patterns of the disorder rather than their effect, and these are the following:

Increased pulmonary blood flow. Decreased pulmonary blood flow. Obstruction of blood flow leaving the heart. Mixed blood flow in which oxygenated and deoxygenated blood mix within the heart or great arteries.

Patent Ductus Arteriosus (PDA)

when ductus arteriosus fails to close, blood will shunt from the aorta (oxygenated) to the pulmonary artery (deoxygenated) because of the increased pressure in the aorta. The shunted blood returns to the left atrium of the heart, passes to the left ventricle, out to the aorta and shunts back to the pulmonary artery.

Assessment:
Wide pulse pressure Classic machinery murmur, which is continuous from systole into diastole. Dyspnea on exertion and easy fatigability. Under weight thin child. Bounding pluses due to increased systolic pressure.

Therapeutic Management:
IV indomethacin or ibufropen prostaglandin inhibitors as prescribed
This encourage closure of ductus Nrsg Mgmt:
If indomethacin is given, assess for possible side effects, including reduced glomerular filtration, impaired platelet aggretation and diminished GI and cerebral blood flow

 It can also be closed by insertion of Dacron-coated stainless steel coils by interventional cardiac catheterization when the child is 6 months to 1 tear of age. Large defects can be closed surgically by ductal ligation.

Atrial Septal Defect (ASD)

Abnormal opening between the atrium, allowing blood directed from the higher pressure left atrium to flow to the lower pressure right atrium. The resulting left to right shunting of blood places. A blood places a burden on the right side of the heart resulting in an increased pulmonary blood flow.

2 types of ASD
Ostium Primum (ASD1)
The opening is at the lower end of the septum

Ostium Secundum (ASD2)

The opening is near the center of the septum

Assessment:
Harsh systolic murmur Echocardiography with color flow Doppler may reveal enlarged right side of the heart and increase pulmonary circulation Cardiac catheterization may reveal the separation in the atrial septum and the increaed 02 saturation in RA.

Management:
Surgery between 1 and 3 years of age Large defects require open surgery and cardiopulmonary bypass Very large defects, a Silastic or Dacron patch may be sutured into place to occlude the space.
Post operatively, carefully observe for arrhythmias

Ventricular Septal Defect (VSD)

Ventricular septal defect is an abnormal opening between the right and left ventricles. Due to higher pressure in the left ventricle, blood shunts from left to the right across the septum.

Assessment:
When shunting begins at 4 to 8 weeks of age, the child demostrates
Easy fatigue Loud harsh pansystolic murmur Thrill may be palpable

Therapeutic Management:
Cardiac catheterization moderate in size Open heart surgery surgery larger (3mm) Silastic and Dacron patch
Assess for arrhythmias postop Prophylactic antibiotics are given for 6 mos to prevent bacterial endocarditis

Atrioventricular canal defect

AKA endocardial cushion defect Blood flow between all four chambers of the heart.

Assessment
Right ventricular hyperthrophy Increased pulmonary blood flow Fixed s2 splitting

Management
Surgery
Observe for jaundice Prophylactic anticoagulation and antibiotic therapy may be given

Pulmonary Stenosis (PS)

Narrowing of the pulmonary valve or the pulmonary artery just distal to the valve. Inability of the right ventricle to evacuate blood by way of the pulmonary artery because of the obstruction leads to right ventricular hypertrophy.

Assessment:
Mild PS: asymptomatic, discovered on routine examination. Severe PS: may present by right heart failure in infancy: cyanosis and cardiac failure may occur, poor exercise tolerance with exertional dyspnea due to insufficient blood flow to the lungs to meet the need for increased cardiac output during exertion. ECG shows gross right ventricular hypertrophy. Radiography shows enlargement of the heart. The murmur and changes in the second heart sound can be analyzed with echocardiography.

Management:
Balloon Angioplasty
A catheter with an uninflated balloon at its tip is inserted and passed through the heart into the stenosed valve. As the balloon is inflated, it breaks valve adhesions and relieves stenosis.

Aortic Stenosis (AS)

Narrowing or stricture of the aortic valve prevents blood from passing freely from the LV of the heart into the aorta.

Assessment:
Most of the children are asymptomatic PE murmur, a rough systolic sound heard loudest in the 2nd right interspace Thrill at suprasternal notch Decreased CO
Faint pulses Hypotension Tachycardia Inability to suck for long periods When the child is active chest pain

Management
Stabilization with beta-blocker or Ca channel blocker to reduce hypertrophy Balloon valvuloplasty

Coarctation of the Aorta

Narrowing of the lumen of the aorta due to a constricting band. Because it is difficult for blood flow to pass through the narrowed lumen of the aorta, BP increases proximal to the coarctation and decreases distal to it.

Assessment:
Slight coarctation absence of palpable femoral pulses Obstruction proximal to the left subclavian artery absent brachial pulses When children grow older leg pain on exertion Soft or moderately loud systolic murmur

Management
Interventional Angiography (Balloon Catheter) Surgery - Narrowed portion of the aorta is removed and new ends of the aorta are anastomosed
Pre op Digoxin and diuretics are given Post op assess for abdominal discomfort Elevated BP antihypertensive agents

Transposition of Great Arteries

Aorta arises from the right ventricle instead of the left, and the pulmonary artery arises from the left ventricle instead of right.

Assessment
Cyanotic from birth No murmur Echocardiography enlarged heart

Management
Prostaglandin may be administered to keep ductus arteriosus patent Surgical correction done at 1 week to 3 months of age

Total Anomalous Pulmonary Venous Return

The pulmonary veins return to the right atrium or superior vena cava instead of to the left atrium as they normally would

Assessment
Mildly cyanotic Tire easily

Mangement
Surgery involves reimplanting the pulmonary veins into the left atrium

Truncus Arteriosus
One major artery or trunk arises from the left and right ventricles in place of separate aorta and pulmonary artery vessels.

Hypoplastic Left Heart Syndrome

The left ventricle is non-functional There may be accompanying mitral or aortic valve atresia. The nonfuctioning LV lacks adequate strength to pump blood into the sytemic circulation. This causes the RV to hypertrophy as it tries to maintain the entire action of the heart

Assessment
Mild to moderate cyanosis

Management
Prostaglandin Therapy to increase blood supply to the aorta Heart transplant

Tetralogy of Fallot
The classical tetralogy consists of: Pulmonary stenosis. Ventricular septal defect. Overriding of the aorta: aorta receives blood from both ventricles and this is the cause of cyanosis. Right ventricular hypertrophy.

 Because of the pulmonary stenosis, pressure builds up in the right side of the heart. Blood then shunts from this area of increased pressure into the left ventricle and overriding aorta. The extra effort involved to force blood through the stenosed pulmonary artery causes the fourth deformity, hypertrophy of the RV.

Assessment
Polycythemia Dyspnea Growth restriction Clubbing of fingers Squatting or knee chest position when resting Syncope

 Lab findings Increased hemoglobin, Hct and total RBC as well as reduced O2 sat Echocardiography and ECG Enlarged chamber of the right side of the heart

Management
In hypoxic episode Administer O2 Place baby in knee- chest position Administer morphine sulfate If not Propanolol may be given orally Blalock-Taussig procedure
Can create a shunt between the aorta and pulmonary artery This allow blood to leave the aorta and enter the pulmonary artery, oxygenate in the lungs, and return to the left side of the heart, the aorta and the body.

Tricuspid Atresia
Extremely serious disorder because the tricuspid valve is completely closed, allowing no blood to flow from the RA to RV. Instead, blood crosses through the patent foramen ovale into the LA, bypassing the lungs and the step of oxygenation

Assessment
When the FO and DA closes extreme cyanosis, Tachycardia and dyspnea

Management
PGE1 IV infusion to ensure that the ductus remains open Fontan procedure construction of vena cava-to-pulmonary shunt

Congestive Heart Failure (CHF)

CHF is the inability of the heart to pump an adequate amount of blood to the systemic circulation to meet the metabolic demands of the body. In children CHF most frequently occurs secondary to structural abnormalities that result in increased blood volume and pressure. CHF is a symptom caused by an underlying cardiac defect, not a disease it self, since it is usually the result of an excessive workload imposed on a normal myocardium

 Heart failure is often separated into two categories; right sided and left sided failure. In right sided failure, the right ventricle is unable to pump blood effectively into the pulmonary artery resulting in increased pressure in the right atrium and systemic venous circulation. Systemic venous hypertension causes Hepatosplenomegaly and occasionally edema. In left sided failure, the left ventricle is unable to pump blood into the systemic circulation resulting in increased pressure in the left atrium and pulmonary veins. The lungs become congested with blood, causing elevated pulmonary pressure and pulmonary edema.

Clinical Manifestation:
Tachypnea Rapid breathing Hepatomegaly Irritable Restless Abdominal pain caused by liver distention Dyspnea, orthopnea Rales Bloody sputum when coughing Difficulty feeding

Therapeutic Management
The goals of treatment are to: Improve cardiac function. Remove accumulated fluid and sodium. Decrease cardiac demands. Improve tissue oxygenation and decreased oxygen consumption.

Acquired Cardiovascular Disorders

Acute Rheumatic Fever (ARF)

It is an autoimmune disease that occurs as a reaction to group A beta-hemolytic streptococcal infection Inflammation from the immune response leads to fibrin deposits on the endocardium and valves (mitral) as well as in the major body joints.

Assessment:
Guidelines for the diagnosis of initial attack of Rheumatic fever
Major Manifestations Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules Minor Manifestations Clinical findings Arthralgia Fever Laboratory findings Elevated acutephase Reactants Erythrocyte Sedimenation rate C- reactive protein

Management
Full course of rheumatic fever is 6 to 8 weeks. Bedrest on acute phase Monitor VS especially pulse rate (sign of improvement) Obtain apical pulse for a full minute to measure effect of activity of the pulse rate

 Penicillin Therapy
Eliminate group A beta-hemolytic streptococci

Oral Ibufropen
Reduce inflammation and joint pain

Corticosteroids
Reduce inflammation

Phenobarbital and diazepam

Reduce purposeless movements of chorea

Persistent Pulmonary Hypertension

Results when the pulmonary vascular resistance present at birth because of unopened alveoli fails to fall normal. PPH occurs because hypoxia and acidosis from respiratory difficulty cause vasoconstriction of the pulmonary artery.

Kawasaki Disease
Mucocutaneous lymph node syndrome A febrile, multisystem disorder that occurs almost exclusively in children before the age of puberty.

Assessment
High fever 39 40 C Lethargic and irritable Reddened swollen hands and feet Conjunctivitis strawberry tongue and red, cracked lips Rashes Cervical lymph nodes are enlarged Abdominal pain Anorexia Diarrhea Desquamation of palms and soles

Management
Acetylsalicylic acid (aspirin) or Ibuprofen
Decreases inflammation and blocks platelet aggregation

Abciximab
Platelet receptor inhibitor

IV immune globulin
Reduce immune response

Endocarditis
Inflammation and infection of the endocardium or valves of the heart Caused by streptococci of the viridans type

Assessment
Pale Anorexia Weight loss Arthralgia Malaise Chills Sweating at night Petechiae of the conjunctiva or oral mucosa Hemorrhage of fingernails and toenails LUQ abdominal painL Lab proteinuria and hematuria

Management
Antibiotic
Prevent infectious endocarditis

Penicillin IV through central venous access

Allows quick dilution and distribution

Cardiomyopathy
Refers to structural or functional abnormality of the ventricular myocardium that occurs following an infection such as adenovirus, cytomegalovirus, or HIV/AIDS

VALENCIA, EVANGELINE A. BSN 4A