‘ The Pedi-Cardiac Lecture ’ Part 2

Pediatric Cardiovascular DisordersJerry Carley MSN, MA, RN, CNE

Concept Map: Pediatric Cardiac Conditions

Distribution of Congenital Heart Defects by anatomical location

PDA

PDA

Persistent Ductus Arteriosus

PDA Incidence 10% of all reported CHDs One of the most common benign defects Ductus normally closes within hours of birth Connection between the pulmonary artery

(low pressure) and aorta (high pressure) High risk for pulmonary hypertension

Ductus Arteriosus Fetal Structure, Connecting

Function

Pulmonary Artery

Aorta

Blood from (R))Ventricle

Pulmonary Artery

Reenters Aorta

Allows Blood to Bypass Lungs

Effects / Symptoms

Tachypnea

Dyspnea

BoundingPulse

RecurrentPneumonia

Heart Failure

PulmonaryHypertension

Cardiomegaly

Murmurs

DifficultyFeeding

FTT

TiresEasily

InfectiveEndocarditis

Treatments

SpontaneousClosure

Medication Indomethacin(Indocin)

Ibuprofen(Motrin)

SurgeryHeart Catheterization

Usually by2 years

Nursing Care

Closed HeartSurgery

Diagnosis and Treatment

Diagnosis by Chest x-ray – enlarged heart and dilated

pulmonary artery Echo-cardiogram – show the opening between

pulmonary artery and aorta

Treatment

Indomethocin (Indocin) given po – constricts the muscle in the wall of the PDA and promotes closure

Cardiac Catheterization – coil is placed in the open duct and acts like a plug

Closed heart surgery – small incision made between ribs on left hand side and PDA is ligated or tied and cut

ASD

Atrial Septal Defect

ASD 10% of defects Blood in left atrium flows into right atrium Pulmonary hypertension Reduced blood volume in systemic

circulation If left untreated may lead to pulmonary

hypertension, congestive heart failure or stroke as an adult.

Pathophysiology

Lower PressureIn ® Atrium

Effects / Symptoms

Short Stature

Heat Murmur

Dyspnea

PulmonaryHypertension

Cardiomegaly

Arrhythmia

Treatments

Large ASDs:SurgicalClosure

Heart Catheterization

Patching

Nursing Care

Oxygenated blood From lungs shuntedTo ® Atrium from(L) d/t ASD

Blood recirculates back to the lungsVia pulmonary arteries

TranscatheterBalloon

R L

Suturing

ASDLeft Right

Diagnosis and Treatment

Diagnosis: heart murmur may be heard in the pulmonary valve area because the heart is forcing an unusually large amount of blood through a normal sized valve.

Echocardiogram is the primary method used to diagnose the defect – it can show the hole and its size and any enlargement of the right atrium and ventricle in response to the extra work they are doing.

Treatment

Surgical closure of the atrial septal defect After closure in childhood the heart size will

return to normal over a period of four to six months.

No restrictions to physical activity post closure

VSD

Ventricular Septal Defect

VSD 30% of congenital heart defects Opening in the ventricular septum Left-to-right shunt Right ventricular hypertrophy Deficient systemic blood flow

Pathophysiology Hole in VentricularSeptum R L Shunting

Increased PulmonaryFlow & Pressure

Effects / Symptoms

Tachypnea

Tachycardia

EnlargedPulmonary Arteries

RecurrentPneumonia

PulmonaryHypertension

Cardiomegaly

Murmurs

Treatments

Medication

Diuretics

Captopril(ACE Inhibitor)

Surgery

Heart Catheterization

Nursing Care

Open-HeartSeptalPlasty

PulmonaryEdema

DyspneaPaleness

FTT

SweatingWhileFeeding

Frequently seenWith other anomalies,e.g., TOF

CongestiveHeartFailure

Digoxin

Eventually, will becomeR LShunt if Not Treated !

VSD

Small holes generally are asymptomatic Medium to moderate holes will cause

problems when the pressure in the right side of the heart decreases and blood will start to flow to the path of least resistance (from the left ventricle through the VSD to the right ventricle and into the lungs)

This will generally lead to CHF

Diagnosis and Treatment

Diagnosis – heart murmur – clinical pearl a louder murmur may indicate a smaller hole due to the force that is needed for the blood to get through the hole.

Electrocardiogram – to see if there is a strain on the heart

Chest x-ray – size of heart Echocardiogram – shows size of the hole and

size of heart chambers

Treatment VSD

CHF: diuretics of help get rid of extra fluid in the lungs

Digoxin if additional force needed to squeeze the heart

FTT or failure to grow may need higher calorie concentration

Will need prophylactic antibiotics before dental procedures if defect is not repaired

Surgical Repair

Over a period of years the vessels in the lungs will develop thicker walls – the pressure in the lungs will increase and pulmonary vascular disease

If pressure in the lungs becomes too high the un-oxygenated blood with cross over to the left side of the heart and un-oxygenated blood with enter the circulatory system.(Becomes a Right Left Shunt)

If the large VSD is repaired these changes will not occur.

COA

Coarctation of Aorta

COA 7 % of defects Congenital narrowing of the descending aorta 80% have aortic-valve anomalies Difference in BP in arms and legs (severe

obstruction)

Diagnosis and Treatment

In 50% the narrowing is not severe enough to cause symptoms in the first days of life.

When the Ductus Arteriosis closes a higher resistance develops and heart failure can develop.

Pulses in the groin and leg will be diminished Echocardiogram will show the defect in the

aorta

Treatment

Prostaglandin may be given to keep the DA open to reduce the pressure changes

The most common repair is resection of the narrowed area with re-anastomosis of the two ends

Surgical complications – kidney damage due to clamping off of blood flow during surgery

High blood pressure post surgery – may need to be on antihypertensives

Antibiotic prophylactic need due to possible aortic valve abnormalities.

PS

Pulmonary Stenosis

PS 7% of defects Obstruction of blood flow from right ventricle Hypertrophy of right ventricle If severe cyanosis due to right-to-left shunt

Pulmonary Valvular Stenosis

In pulmonary valvar stenosis the pulmonary valve leads to narrowing and obstruction between the right ventricle and the pulmonary artery.

Thickened tissue become less pliable and increases the obstruction

Right ventricle must work harder to eject blood into the pulmonary artery.

Pathophysiology Abnormality ofPulmonary ValveLeaflets

Effects / Symptoms

Asymptomatic(usually)

® VentricularHypertrophy

DilatedPulmonaryArtery

Feeding Problems

PulmonaryHypertension

Dyspnea

Potential ®VentricularFailure

FTT

S/S®HeartFailure

Treatments

Indomethacin(Indocin)

Ibuprofen(Motrin)

Surgery

Heart Catheterization

Usually by2 years

Nursing Care

Sometimes part ofDiGeorge Syndrome

Leakage ofPulmonary ValveWhen closed

TiresEasily

TranscatheterBalloon

Stenting

Diagnosis and Treatment

Diagnosis: heart murmur is heard – clicking sound when the thickened valve snaps to an open position.

Electrocardiogram would be normal Echocardiogram most important non-invasive

test to detect and evaluate pulmonary stenosis

Cardiac Catheterization – to measure pressures and measure the stenosis

Treatment

Cardiac Catheterization to dilate the valve and open up the obstruction.

Open- heart procedure would only needed for more complex valve anomaly.

TOF

Tetralogy of Fallot (TOF)

6% of all CHD defects Most common cardiac malformation

responsible for cyanosis in a child over 1 year

1. Narrowingof the Pulmonic Valve

2. Thickening of Right VentricularWall

3. Displacement ofAorta over ventricularseptal defect

4. VentricularSeptal defect

Right Left

Pathophysiology

Pulmonic ValveNarrowing

(R) VentricleHypertrophy

Displacement ofAorta

Effects / Symptoms

Tachypnea

Dyspnea

CentralCYANOSIS

Heart Failure

Cardiomegaly

Harsh SystolicEjection Murmur

DifficultyFeeding

FTTTiresEasily

Treatments

Surgery

Nursing Care

Ventricular SeptalDefect (VSD)

R L

TetSpells

UsuallySelf-Limiting

CloseVSD

Relieve ®VentricularOutflow

FingerClubbing

TOF

Four Components VSD Pulmonary stenosis – narrowing of pulmonary

valve Overriding of the aorta – aortic valve is enlarged

and appears to arise from both the left and right ventricles instead of the left ventricle

Hypertrophy of right ventricle – thickening of the muscular walls because of the right ventricle pumping at high pressure

Clinical Manifestations

Dependent on degree of right ventricular outflow obstruction.

Right-to-left shunt Clubbing of digits “tet” spells - ‘hyper-cyanotic episodes’

treated by flexing knees forward and upward Severe irritability due to low oxygen levels

Children with T.O.F. exhibit cyanosis during episodes of crying or exertion.

Knee-chest Position

Child with a cyanotic heartdefect squats (assumes a knee-chest position) to relievecyanotic spells. (“tet spells “)

Nurse puts infant in knee-chestposition.

Diagnosis

Cyanosis (central) Oxygen will have little effect on the cyanosis Loud heart murmur Echocardiogram – demonstrates the four

defects characteristic of tetralogy

Treatment

If oxygen levels are extremely low prostaglandins may be administered IV to keep the PDA open

Complete repair is done when the infant is about 6 months of age

Correction includes Closure of the VSD with dacron patch The narrowed pulmonary valve is enlarged Coronary arteries will be repaired Hypertrophy of right heart should remodel within a few

months when pressure in right side is reduced

Long Term Outcomes

Leaky pulmonic valve that can lead to pulmonary insufficiency

Arrhythmias after surgery Heart block – occasionally a pacemaker is

necessary Periodic echocardiogram and exercise stress

test or Holter monitor evaluation

End of Part 2