MR. SURENDRA SHARMAASSIST. PROFESSORAMITY

UNIVERSITY,GURGOAN

CONGENITAL HEART DISEASES

INCIDENCE:The overall incidence of congenital heart

diseases is about 8 – 10 percent per 1000 live births

Defect Percentage VSD 25 – 30 % PDA 10 %ASD 10 %Coarctation of aorta 6 %TOF 5- 9 %

ETIOLOGY AND INCIDENCE   Hereditary factors CHD affects 8 – 12 of every 1000 neonates Associated factor for CHD includeØ     Fetal or Maternal infection during the first trimester

(Rubella) Ø     Chromosomal abnormality (Trisomy 21, 18, 13)Ø     Maternal diabetes Ø     Teratogenic effects of drugs and alcohol  Syndromes that include CHDØ     Marfan`s syndrome : Mitral value prolapse Ø     Turner’s syndrome : Aortic value stenosis, COAØ     William’s syndrome : Dysplastic pulmonary value Ø     Down syndrome : Triosomy

 Congenital Heart diseases

Cyanotic heart Acyanotic heart Obstructive

diseases ( R to L shunt ) diseases ( L to R ).Coarctation of aorta

Fallots tetrology . VSD . Vascular ring

Transposition of greater . ASD . Pulmonary stenosis

vessels . PDA . Aortic

stenosis Tricuspid atresia

VENRICULAR SEPTAL DEFECT (VSD)

Definition

VSD is an abnormal communication between the two ventricles

PathophysiologyBlood is shunted from the left to the right ventricles in most of the cases due to the relatively high pressure of the left ventricles chamber

If the defect is large, the amount of blood shunted in to the right ventricles may be quite large resulting in increased workload for both ventricles

Right ventricles increased right ventricular out put and pulmonary

enlargement develops

Blood increased return to the left atrium, thus increasing the work of the left ventricles, resulting in bi – ventricular hypertrophy

Pulmonary over circulation cause a change in the pulmonary arterial bed, leading to increased pulmonary artery vascular

resistance

High pulmonary vascular resistance can reverse the blood flow pattern that leads to right to left shunt across the VSD

(Eisenmenger`s Syndrome) resulting in cyanosis

Clinical manifestation  

Small VSD`s : usually a symptomatic; High spontaneous closure rate during the first year of life

Large VSD`s:      CHF – tachypnea, tachycardia, excessive sweating       Frequent URI       Poor weight gain, Failure to thrive       Feeding difficulties      Murmur present      Pulmonary vascular obstructive diseases

 

Investigation

X. - ray chest - ventricular hypertrophy

Small -Normal Moderate VSD - shunt vascularity (pulmonary plethora)

Ventricular hypertrophy

o       Pulmonary artery increased size. Large VSD - Biventricular hypertrophy Increased pulmonary trunk Left arterial enlargement.

– Enlarged main pulmonary artery

--- Right ventricular hypertrophy

--- Peripheral pruning with apparent decrease in shunt

--- vascularity.

NORMAL HEART VSD - HYPERTROPHY

ECG

Echo and Doppler study

Cardiac cauterization study

Angiocardiography

Management of VSD:Aims: -

1.     To achieve normal growth by controlling ccf2.     Prevention and treatment of anemia3.     Prevention and treatment of infective endocarditis

1. Medical management: -1.CHF management :digoxin and diuretics(furasemide,

spironolactone) and after reduction 2. Avoid oxygen : - oxygen is a potent pulmonary

vasodilator and will increase blood flow in to the P.A3.Increase caloric intake: fortify formula or breast milk to

make 24 to 30 caloz formula, supplemental nasogastric feeds as needed.

4.Ineffective endocarditis prophylaxis for 6 months after surgery.

2.Surgical treatment: -Corrective surgery done in first 2 years of life

prevents progression of pulmonary hypertension

Surgeries: -1.Corrective surgery –patch graft – Dacron / Natural

2.Palliative surgery – Pulmonary artery banding

Complication:  C.H.F Recurrent respiratory tract infection Ineffective endocarditis Failure to thrive: poor weight gain Pulmonary arterial hypertension and

elsenmengerisation Aortic or tricuspid regurgitation Right ventricular outflow tract obstruction.

ATRIAL SEPTAL DEFECT(ASD)

Definition: -Atrial septal defect is an abnormal communication between the two atria.

Pathophysiology:

Blood flows from the higher pressure left atriumacross the ASD in to the lower pressure right

atrium. 

Increased blood return to the right heart leads to right

ventricular volume over load.

Right ventricular dilatation

Increased pulmonary blood flow leads to elevated pulmonary artery pressure.

Clinical manifestation: -1.     Usually a symptomatic2.     CHF3.     Frequent upper respiratory tract infection4.     Poor weight gain5.     Decreased exercise tolerance.

Diagnostic evaluation: - X-Ray Right atrial and ventricular enlargement-

enlargement of pulmonary artery E.C.G Auscultation: soft systolic ejection murmur heard

best at the left upper sternal border. Cardiac caterization

Management: -1.Medical management

a).Monitor and reassessb).Treatment with anticongestive therapy (digoxin and lasix) may be necessary. if signs of CHF are presentc).Infective endocarditis prophylaxis for 6 months after surgery or atrial occlusion devise is used. 

2. Cardiac catherisation for placement of an atrial occlusion device for ostium secundam defects.

3.Surgical intervention: a)     Primary repair suture closure of the ASD.b)     Patch repair of the ASD.

Complication:CHF

Infective endocarditis

Pulmonary hypertension

Atrial arrhythmias. 

Patent ductus arteriosus (PDA)

Definition This defect, which normally occurs during fetal

life, short circuits the normal pulmonary vascular system and allows blood to mix between the pulmonary artery and the aorta. Prior to birth, there is an open passageway between the two blood vessels, which closes soon after birth. When it does not close, some blood returns to the lungs. Patent ductus arteriosus is often seen in premature infants.

Pathophysiology: During fetal life, the ductus arteriosus allows blood to by pass the pulmonary circulation and flow directly in to the

systemic circulation.

After birth, the ductus arteriosus is no longer needed. Functional closure usually occurs within 48 hrs after birth.

When the ductus arteriosus fails to close blood from the aorta ( high pressure) flows in to the lower pressure PA.  

Resulting in pulmonary over circulation 

Increased pulmonary blood flow leads to a volume- loaded LV.

Clinical manifestation:1.     Growth retardation2.     External dyspnea3.     CCF4.     Pericardial pain5.     Cough6.     Dyspnoea7.     Tachypnoea.8.     Dyspnoea9.     Tacycardia10. Hepato splenomegali11. Machinery murmur. It is harsh and may be localized to second left intercostals space or transmitted to left clavicle to lower down (ie) left sternal border. It is accomplished by a thrill.

Diagnostic evaluation: -

Chest X-Ray- cardiomegaly

ECG

ECHO

Cardiac catherization; raised pressure in right ventricles and pulmonary artery.

Management: -

1.In the symptomatic premature neonate; Indomethacin. Given IV.

2.Medical management:

a)      Monitor growth and development

b)     Reassures for spontaneous PDA closure

c)      Increase caloric intake as needed for normal weight gain

d)     Diuretics: furusemide (lasix), spironolactone (Aldactone).

e)      Ineffective endocarditis prophylaxis for 6 months after surgery.

3.Cardiac catherization:a)      For small PDAs coil occlusionb)     For large PDAs closure device may be used.

4.Surgical management through PDA ligation. 

Complication: 1.     CHF, pulmonary oedema2.     Infective endocarditis3.     Pulmonary hypertension4.     Recurrent pneumonia.

 

TETRALOGY OF FALLOT

PATHOPHYSIOLOGY

The blood normally returns from the systemic circulation to the systemic circulation to the right atrium and right

ventricles

 

 

The outflow of blood from the right ventricles is resisted by the pulmonary stenosis so that the blood flows through the

ventricular septal defect in to the aorta

There is right to left shunt. Hypertrophy of the right ventricles occurs as a result of the pressure exerted against

the pulmonary stenosis.

Because, the blood from the right ventricles is unoxygenated, cyanosis results.

Clinical manifestation: -1. Cyanotic episodes: cyanotic spells may occur while crying and after feeding. After cyanotic spells, there may be limpness, fatigue and fainting.2. Dyspnoea3. Delayed physical growth and development4. Pansystolic murmur may be heard at the middle to lower sternal borders5. Cyanosis- may be seen mucous membrane of the lips, mouth and pharynx and in fingernails and toe- nails.6. Clubbing of the fingers7. Paroxysmal dyspneic attacks (anoxia, “ blue “ spells) occur during the first 24 months of life and last for a few minutes to hours.

Diagnosis: -1.Blood studies.

2.X-Ray chest

3.ECG- right ventricular hypertrophy.

4.Echo- evidence of the aortic override, thick anterior right ventricular wall and large aorta.

Medical and Nursing management:Palliative and corrective surgery for tetrology of

fallot is being done in infants and children of all ages.

Transposition of the great arteries

This congenital heart defect, the positions of the pulmonary artery and the aorta are reversed, thus:

o The aorta originates from the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs.

o The pulmonary artery originates from the left ventricle, so that most of the blood returning from the lungs goes back to the lungs again

Pathophysiology: -

In this anomaly the aorta has its origin in the right ventricles and pulmonary artery has its origins in the left ventricles.

Hence, the aorta carries unoxygenated blood to the systemic circulation and the pulmonary circuit carries oxygenated blood

back to the lungs. 

 

The pulmonary venous return is to the left atrium and the systemic veins returns to the right atrium.

There is two separate circulatory systemic exist, one pulmonary and one systemic. An infant can survive with this

malformation initially only if an associated with defect or PDA is present 

There co-existing lesions provide a means for mixing venous and arterial blood.

Clinical manifestation: -

1.     Cyanosis from neonatal period and polycythemia

2.     Congestive cardiac failure

3.     Hypercapnoea due to low arterial oxygen

4.     Delayed growth and development

5.     Metabolic acidosis

6.     Clubbing of the finger and toes.

Diagnostic evaluation: -

1.     Physical examination – if defect is there murmur can be heard

2.     X-ray- cardiomegaly and increased pulmonary vasculature

3.     Fluoroscopy- egg shaped” cardiac contour can be identified

4.     Echo- Right ventricular hypertrophy

5.     ECG

6.     Angiocardiography

Cardiac catherization

Treatment: -

Procedure used for the treatment of transposition of the great vessels are palliative and corrective.

Coarctation of the aorta

Aortic coarctation is a narrowing of part of the aorta (the major artery leading out of the heart). It is a type of birth defect. Coarctation means narrowing. It accounts for 8 -10% of CHD and is 2 to 5 time more common in male.

Obstructive

Causes

The aorta carries blood from the heart to the vessels that supply the body with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to pass through the artery.

Aortic coarctation is more common in Turner syndrome.

Coarctation of the aorta may be seen with other congenital heart defects, such as:

Bicuspid aortic valveDefects in which only one ventricle is presentVentricular septal defect

Clinical manifestationAsymptomatical until the PDA begin to

closeAfter PDA closure:-Sever CHFTachypneaAcidosisPrograsive circulatory shockAbsent femoral and pedal pulses

Chest painCold feet or legsDizziness or faintingDecreased ability to exerciseFailure to thriveLeg cramps with exerciseNosebleedPoor growthPounding headache

Diagnostic evaluation

1.     Physical examination –The pulse in the groin (femoral) area or feet will be weaker than the pulse in the arms or neck (carotid). Sometimes, the femoral pulse may not be felt at all and murmur sound can be heard ,2.     X-ray- cardiomegaly 3.    ECG

4. Echo- Right ventricular hypertrophy 5.     Angiocardiography

6. Cardiac catherization 7. Heart CT may be needed in older children 8. MRI or MR angiography of the chest may be

needed in older children  

Management1. Medical MenagementResuscitation and stabilization with Prostaglandin E1

infusionIntubation and ventilation as neededInfective endocarditis prophylaxisAnticongestive theraphy( digixin and lasix)Assessment of renal ,hepatic,and nurologic function.2. Ballon angioplasty may be indicated for infants who are a

high surgical risk.3. Surgical intervention: usually performed as soon as the

diagnosis is madeSubclavian flap repairEnd to end anastomosisDacron patch repair 4. hypertention management is needed for the older

children

ComplicationAortic aneurysmEndocarditis (infection in the heart)Heart failureKidney problemsParalysis of the lower half of the body (a

rare complication of surgery to repair coarctation)

Severe high blood pressure

Pulmonary stenosis

Pulmonary valve stenosis is a heart valve disorder that involves the pulmonary valve.

This valve separates the right ventricle (one of the chambers in the heart) and the pulmonary artery. The pulmonary artery carries oxygen-poor blood to the lungs.

Stenosis, or narrowing, occurs when the valve cannot open wide enough. As a result, less blood flows to the lungs.

Causes

Narrowing of the pulmonary valve is usually present at birth (congenital). It is caused by a problem that occurs when the unborn baby (fetus) is developing. The cause is unknown, but genetics may play a role.

Pulmonary valve stenosis is a rare disorder.In some cases, pulmonary valve stenosis

more in families.

Clinical manifestation

These infants are usually found to have a murmur on a routine heart examination.

When the valve narrowing (stenosis) is moderate to severe, the symptoms include:

Bluish color to the skin (cyanosis) in some patientsChest painFaintingFatiguePoor weight gain or failure to thrive in infants with

severe blockageShortness of breathSudden deathSymptoms may get worse with exercise or activity.

Diagnostic evaluationPhysical examination:- The health care provider

may hear a heart murmur when listening to your heart using a stethoscope. Murmurs are blowing, whooshing, or rasping sounds heard during a heartbeat.

Tests used to diagnose pulmonary stenosis may include:

Cardiac catheterizationChest x-rayECGEchocardiogramMRI of the heart

TreatmentSometimes, treatment may not be needed if the disorder is

mild.When there are also other heart defects, medications may be

used to:Help blood flow through the heart (prostaglandins)Help the heart beat strongerPrevent clots (blood thinners)Remove excess fluid (water pills)Treat abnormal heartbeats and rhythmsPercutaneous balloon pulmonary dilation (valvuloplasty) may

be performed when no other heart defects are present.This procedure is done through an artery in the groin.The doctor sends a flexible tube (catheter) with a balloon

attached to the end up to the heart. Special x-rays are used to help guide the catheter.

The balloon stretches the opening of the valve.Some patients may need heart surgery to repair or replace the

pulmonary valve. The new valve can be made from different materials. If the valve cannot be repaired or replaced, other procedures may be needed.

Complications

Abnormal heartbeats (arrhythmias)DeathHeart failure and enlargement of the right

side of the heartLeaking of blood back into the right

ventricle (pulmonary regurgitation) after repair

NURSING CARE OF THE CHILD WITH CONGENITAL HEART DISEASES.

Nursing Assessment: - Obtain a through nursing history Discuss the care plan with the health care team

(cardiologist, cardiac surgeon, nursing care manager, social worker, nutrition list)

Measure and record height and weight plot on a growth chart

Record vital signs and oxygen saturations.   Measure vital signs at a time when the infant / child is

quit.

  Choose appropriate size blood pressure cuff

  Check four extremities BPxl.

Assess and record.

       Skin color, pink, cyanotic, mottled

       Mucous membranes; moist, dry, cyanotic

   Extremities: check peripheral pulses for quality and symmetry, dependent edema, capillary refill, color and temperature.

Assess for clubbing (cyanotic heart disease0Assess chest wall for deformities; prominent pericardial activity.

Assess respiratory patternBefore disturbing the child, stand back on

count the respiratory rate.Loosen or remove clothing to directly

observe chest movementAssess for signs of respiratory distress;

increased respiratory rate, granting, retraction, nasal flaring.

uscultate for crackles, wheezing, congestion, and strider.

Assess heart sounds.Determine rate (bradycardia, tachycardia) and

rhythm( regular or irregular)Identity murmur (type, locations, and grade)

     Assess fluids status.      Daily weights   Strict intake and output (number of wet diaper,

urine output)      Assess and record the child’s level of activity     Observe the infant while feeding, does the

infant need frequent breaks or child asleep during feeding, assess for sweating, color change, or respiratory distress while feeding.

  Observe the child at play, is play interrupted to rest

  Assess and record findings relevant to the child’s development level, age appropriate behavior, cognitive skill, gross and fine motor skills.

Summary: -

So far we have discussed about congenital heart diseases, cyanotic heart disease like fallots tetrology, transposition of great arteries and acynotic heart disease like VSD, ASD, PDA and Nursing care of the child with congenital heart disease.