SUMANDEEP NURSING COLLEGE

Piparia, vadodara

Seminar
On
Congenital heart disease
[Nursing management]

Date- /05/2011.

Submitted To Submitted By
Mrs. Mahalakshmi Priyakant Bhatt
Asst. Prof. M. Sc. Nursing 2 nd year Child
Health Nursing Child Health Nursing

Master plan
Topic-congenital heart disease
 Presented by- Priyakant bhatt
Guided by- mrs. Mahalakshmi(asst. prof.)

Introduction
ACYANOTIC CHD (definition, etiology, clinical manifestation, Pathophysiology &
management)
 Ventricular septal defect [VSD]
 Atrial septal defect [ASD]
 Patent ductus arteriosus [PDA]
 Atrio-ventricular canal [AVC]

CYANOTIC HEART DISEASE (definition, etiology, clinical manifestation,

Pathophysiology & management)
 Tetralogy of fallot [TOF]
 Tricuspid atresia [TA]
 Transposition of great arteries [TGA]
 Truncus arteriosus.
 Hypoplastic left heart syndrome.
 Total anomalous pulmonary venous return.
 Eisenmenger syndrome or complex.

OBSTRUCTIVE LESION (definition, etiology, clinical manifestation, Pathophysiology &

management)
 Coarctation of aorta.
 Aortic valve stenosis.
 Pulmonary valve stenosis
 Congenital mitral stenosis.

Research articles

Bibliography

Name Of The Student:- Mr. Priyakant Bhatt

Name Of The Guide:- MRS. Mahalakshmi

Topic:- congenital heart disease

Unit:- 1ST Unit

Date:- 31/5/2011

Time: -

Venue: - M.Sc. Nursing 2nd year classroom

Class Taught: - second year M.Sc. Nursing

Method Of Teaching:- Lecture Cum Discussion

Audio Visual Aids:- Power Point Presentation, Transparencies, Black Board

GENERAL OBJECTIVE:- at the end of the seminar group will be able to know about the definition,
etiological factors, clinical manifestation and management of the congenital heart disease, and apply this
knowledge into the clinical setting.

SPECIFIC OBJECTIVES: - AT THE END OF THE SEMINAR GROUP WILL BE ABLE TO –

1) DEFINE congenital heart disease
2) EXPLAIN various etiological factors of the congenital heart disease
3) Discuss the various acyanotic heart disease in detail
4) Discuss the various cyanotic heart disease in detail
5) Discuss the common obstructive lesion in congenital heart disease.
6) Describe the nursing management for congenital heart disease

Congenital heart diseases

 Congenital heart disease is the structural malformations of the heart or great vessels, present at birth.
It involves the chambers, valves, and great vessels arising from the heart. It is the most common congenital
malformations. Congenital heart disease occurs in 0.5–0.8% of live births. The incidence is higher in
stillborns (3–4%), abortuses (10–25%), and premature infants (about 2%)
 COMMON CONGENITAL HEART MALFORMATIONS
CHD can be grouped into three categories
ACYANOTIC CHD- there is a increase pulmonary blood flow due to right to left shunt. It includes
 Ventricular septal defect [VSD]
 Atrial septal defect [ASD]
 Patent ductus arteriosus [PDA]
 Atrio-ventricular canal [AVC]

CYANOTIC HEART DISEASE

 Tetralogy of fallot [TOF]
 Tricuspid atresia [TA]
 Transposition of great arteries [TGA]
 Truncus arteriosus.
 Hypoplastic left heart syndrome.
 Total anomalous pulmonary venous return.
 Eisenmenger syndrome or complex.

OBSTRUCTIVE LESION
 Coarction of aorta.
 Aortic valve stenosis.
 Pulmonary valve stenosis
 Congenital mitral stenosis.

ETIOLOGY AND INCIDENCE

The cause of most congenital heart defects is unknown, but rapid progress is being made in
identifying the genetic basis of many congenital heart lesions. Most cases of congenital heart disease were
thought to be multifactorial and result from a combination of genetic predisposition and environmental
stimulus.
 CHD affects 8 to 12 of every 1,000 neonates.
 Exact cause of CHD is unknown in 90% of cases.
 The heart begins as a single cell and develops into a four-chambered pumping system during the
third to eight weeks of gestation.
 Associated factors for CHD include:
o Fetal or maternal infection during the first trimester (rubella).
o Chromosomal abnormalities (trisomy 21, 18, 13).
o Maternal insulin-dependent diabetes.
 o Teratogenic effects of drugs and alcohol.
 Syndromes that include CHD:
o Marfan's syndrome: mitral valve prolapse (MVP), dilated aortic root.
o Turner's syndrome: aortic valve stenosis (AVS), coarctation of the aorta (CoA).
o Noonan's syndrome: dysplastic pulmonary valve.
o William's syndrome: supravalvular pulmonary stenosis (PS).
o DiGeorge syndrome: interrupted aortic arch (IAA), truncus arteriosus, and transposition of
great arteries (TGA), tetralogy of Fallot (TOF).
o Down syndrome (trisomy 21): atrioventricular (AV) canal defect, ventricular septal defect
(VSD). About 50% of children with Down syndrome have a CHD.

ATRIAL SEPTAL DEFECT

ASD is an abnormal communication between the left and right atrium. ASDs account for 9% of CHDs.
There are three types:

 Ostium secundum ASD: the most common type of ASD; abnormal opening in the middle of the
atrial septum.
 Ostium primum ASD: abnormal opening at the bottom of the atrial septum; increased association
with cleft mitral valve and atrioventricular defects.
 Sinus venosus ASD: abnormal opening at the top of the atrial septum; increased association with
partial anomalous pulmonary venous return.

Pathophysiology and Etiology

 Blood flows from the higher-pressure left atrium across the ASD into the lower-pressure right atrium
(left-to-right shunt).
 Increased blood return to the right heart leads to right ventricular volume overload and right
ventricular dilation.
 Increased pulmonary blood flow leads to elevated pulmonary artery pressures.

Clinical Manifestations

 Usually asymptomatic.
 Clinical symptoms vary depending on type of associated defects:
o CHF (usually not until the third or fourth decade of life).
o Frequent upper respiratory infections (URIs).
o Poor weight gain.
o Decreased exercise tolerance.

Diagnostic Evaluation

 Auscultation: soft systolic ejection murmur heard best at the left upper sternal border; widely split,
fixed second heart sound.
 Chest X-ray: varies; normal to right atrial and ventricular dilation, increased pulmonary markings.
 ECG: varies; right axis deviation and mild RVH or right bundle-branch block.
 Two-dimensional echocardiogram with Doppler study and color flow mapping to identify the site of
the ASD and associated lesions and document left-to-right flow across the atrial septum.
  Cardiac catheterization usually not needed for initial diagnosis; performed if defect can be closed
using an atrial occlusion device (device can be used only in ostium secundum defects).

Management

 Medical management:
o Monitor and reassess (spontaneous closure rate is small but may occur up to age 2).
o Treatment with Anticongestive therapy (digoxin and Lasix) may be necessary if signs of CHF
are present (usually not until third to fourth decade of life if ASD unrepaired).
o Infective endocarditis prophylaxis for 6 months after surgery or atrial occlusion devise is
used.
 Cardiac catheterization for placement of an atrial occlusion device for ostium secundum defects.
 Surgical intervention:
o Primary repair: suture closure of the ASD.
o Patch repair of the ASD.

Complications

 CHF (rare).
 Infective endocarditis.
 Embolic stroke.
 Pulmonary hypertension.
 Atrial arrhythmias.

VENTRICULAR SEPTAL DEFECT

A ventricular septal defect is an abnormal opening in the septum between and right and left ventricles. It is
the most common acyanotic congenital heart disease with left to right shunt. It is found approximately 25%
of all CHD.
Pathophysiology and Etiology
 Blood flows from the high-pressure left ventricle across the VSD into the low-pressure right
ventricle and into the PA, resulting in pulmonary overcirculation.
 A left-to-right shunt because of a VSD results in increased right ventricular pressure and increased
PA pressure.
 The increased pulmonary venous return to the left side of the heart results in left atrial dilation.
 Long-standing pulmonary overcirculation causes a change in the pulmonary arterial bed, leading to
increased pulmonary vascular resistance. High pulmonary vascular resistance (PVR) can reverse the
blood flow pattern that leads to a right-to-left shunt across the VSD (Eisenmenger's syndrome),
resulting in cyanosis. Once this develops, the child is no longer a candidate for surgical repair.
VSDs vary in the size (small and restrictive to large and nonrestrictive defect), number (single versus
multiple), and type (perimembranous or muscular).
Clinical manifestation

 Small VSD usually asymptomatic; high spontaneous closure rate during the first year of life.
 Large VSDs.
o CHF: tachypnea, tachycardia, excessive sweating associated with feeding, hepatomegaly.
o Frequent URIs.
o Poor weight gain, failure to thrive.
o Feeding difficulties.
o Exertional dyspnea
 o Biventricular hypertrophy
o hepatomegaly
o Decreased exercise tolerance.

Diagnostic Evaluation

 Auscultation: harsh systolic regurgitant murmur heard best at the lower left sternal border (LLSB);
systolic thrill felt at LLSB, narrowly split S2.
 Chest X-ray: varies; normal or cardiomegaly and increased pulmonary vascular markings.
Pulmonary vascular markings are directly proportionate to the amount of left-to-right shunting.
 ECG: varies; normal to biventricular hypertrophy.
 Two-dimensional echocardiogram with Doppler study and color flow mapping to identify the size,
number, and sites of the defects, estimate pulmonary artery pressure, and identify associated lesions.
 Cardiac catheterization usually not needed for initial diagnosis; may be needed to calculate the size
of the shunt or to assess PVR. May be performed if defect can be closed using a ventricular
occlusion device (device can be used only in muscular defects).

Management
Small VSD

 Medical management:
o Usually no Anticongestive therapy is needed.
o Infective endocarditis prophylaxis for 6 months after surgical implantation of a ventricular
occlusion device.
 Cardiac catheterization for placement of a ventricular occlusion device for muscular defects (for
Qp:Qs > 2:1).
 Surgical intervention is usually not necessary.

Moderate to Large VSD

 Medical Management:
o CHF management: digoxin and diuretics (furosemide, spironolactone) and afterload
reduction.
o Avoid oxygen; oxygen is a potent pulmonary vasodilator and will increase blood flow into
the PA.
o Increase caloric intake: fortify formula or breast milk to make 24 to 30 cal/oz formula;
supplemental nasogastric feeds as needed.
o Infective endocarditis prophylaxis for 6 months after surgery/ventricular device occluder.
 Cardiac catheterization for placement of a ventricular occlusion device for muscular defects (for
Qp:Qs > 2:1).
 Refer for surgical intervention.
o Usually repaired before age 1.
o One-stage approach: preferred surgical plan; patch closure of VSD.
o Two-stage approach: first surgery is to band the PA to restrict pulmonary blood flow; second
surgery is to patch close the VSD and remove the PA band.

Long-Term Follow-Up

 Monitor ventricular function.

 Monitor for subaortic membrane and double-chamber RV.
Complications

 CHF.
 Frequent URIs.
 Failure to thrive; poor weight gain.
 Infective endocarditis.
 Eisenmenger's syndrome.
 Pulmonary hypertension.
 Aortic insufficiency.

PATENT DUCTUS ARTERIOSUS

The ductus arteriosus is a normal fetal connection between the left PA and the descending aorta.
During fetal life, blood flow is shunted away from the lungs through the ductus arteriosus and directly into
the systemic circulation. PDAs are common in premature neonates who weigh less than 1,500 g. They
account for 5% to 10% of CHDs, excluding premature neonates.
Pathophysiology and Etiology

 During fetal life, the ductus arteriosus allows blood to bypass the pulmonary circulation (fetus
receives oxygen from the placenta) and flow directly into the systemic circulation.
 After birth, the ductus arteriosus is no longer needed. Functional closure usually occurs within 48
hours after birth. Anatomic closure is completed by age 2 to 3 weeks.
 When the ductus arteriosus fails to close, blood from the aorta (high pressure) flows into the low-
pressure PA, resulting in pulmonary overcirculation.
 Increased pulmonary blood flow leads to a volume-loaded LV.

Clinical Presentation
Small to Moderate-Sized PDA
Usually asymptomatic.
Large PDA

 CHF, tachypnea, frequent respiratory tract infections.

 Poor weight gain, failure to thrive.
 Feeding difficulties.
 Decreased exercise tolerance.

Diagnostic Evaluation

 Auscultation: continuous murmur heard best at left upper sternal border. Hyperactive precordium
with large PDAs.
 Wide pulse pressure; bounding pulses.
 Chest X-ray: varies; normal or cardiomegaly with increased pulmonary vascular markings.
 ECG: varies; normal or LVH.
 Two-dimensional echocardiogram with Doppler study and color flow mapping to visualize the PDA
with left-to-right blood flow.
 Cardiac catheterization is not needed for the initial diagnosis.

Management

 In the symptomatic premature neonate: indomethacin given I.V.

 Medical management:
o Monitor growth and development.
o Reassess for spontaneous PDA closure.
o Increase caloric intake as needed for normal weight gain.
o Diuretics: furosemide (Lasix), spironolactone (Aldactone).
o Infective endocarditis prophylaxis for 6 months after surgery or coil occlusion.
  Cardiac catheterization:
o For small PDAs coil occlusion.
o For larger PDAs a closure device may be used.
 Surgical management through PDA ligation.

Complications

 CHF, pulmonary edema.

 Infective endocarditis.
 Pulmonary hypertension/pulmonary vascular occlusive disease.
 Recurrent pneumonia.

Cyanotic heart disease

TETRALOGY OF FALLOT
TOF is the most common complex congenital heart defect; it accounts for 6% to 10% of all CHDs.
The four abnormalities of TOF include the following:

 A large, nonrestrictive VSD.

 Aortic override.
 Pulmonary stenosis (right ventricular outflow tract obstruction).
 Right ventricular hypertrophy.

Pathophysiology and Etiology

 Degree of cyanosis depends on the size of the VSD and the degree of right ventricular outflow tract
obstruction (RVOTO).
 Obstruction of blood flow from the right ventricle to the PA results in deoxygenated blood being
shunted across the VSD and into the aorta (right-to-left shunt causes cyanosis).
 RVOTO can occur at any or all of the following three levels: pulmonary valve stenosis, infundibular
stenosis, or supravalvular stenosis.
 The right ventricle becomes hypertrophied as a result of the increased gradient across the RVOT.
 Minimal RVOTO results in a pink TOF variant, with the physiology behaving more like a large,
nonrestrictive VSD.

Clinical Manifestations

 Clinical manifestations are variable and depend on the size of the VSD and the degree of RVOTO.
 Cyanosis.
o Neonate may have normal oxygen saturations; as the infant grows, the RVOTO increases and
the oxygen saturation falls.
o Neonate with unacceptably low oxygen saturation needs PGE 1 infusion to maintain ductal
patency and adequate oxygen saturation.
o Cyanosis may initially be observed only with crying and with exertion.
 Polycythemia.
 Decreased exercise tolerance.
 A common clinical manifestation years ago was squatting, a posture characteristically assumed by
older children to increase systemic vascular resistance and to encourage increased pulmonary blood
flow. Squatting is rarely seen currently because TOF is now surgically repaired during the first year
of life.
 Hypercyanotic spells (formerly known as Tet spells): a life-threatening hypoxic event with a
dramatic decrease in oxygen saturations; mechanism is usually infundibular spasm, which further
obstructs pulmonary blood flow and increases right-to-left flow across the VSD.
 o Typical hypoxic spells occur in the morning soon after awakening; during or after a crying
episode; during or after a feeding; during painful procedures such as blood draws.
o Typical scenario includes tachypnea, irritability, and increasing cyanosis, followed by
flaccidity and loss of consciousness.
o Home treatment for the caregiver: soothe the infant and place him or her in a knee-chest
position; notify the health care provider immediately.
o Hospital treatment includes knee-chest position, sedation (morphine), oxygen, beta-
adrenergic blockers (propranolol, esmolol) to relax the infundibulum, and administration of
medications to increase systemic vascular resistance (phenylephrine).
o Hypercyanotic spells usually prompt the cardiologist to refer for surgical intervention.

Diagnostic Evaluation

 Auscultation: harsh systolic ejection murmur heard best at the upper left sternal border (RVOT
murmur); single second heart sound; during a hypercyanotic spell the murmur disappears.
 Chest X-ray: varies; normal or decreased pulmonary vascular markings. The heart may appear boot
shaped because of a concave main PA with an upturned apex resulting from RVH.
 ECG: varies; normal or RVH.
 Two-dimensional echocardiogram with Doppler study and color flow mapping to identify the
structural abnormalities, estimate the degree of RVOTO and assess the coronary artery pattern.
 Cardiac catheterization is usually not needed for the initial diagnosis. May be performed before
surgical intervention to identify the location and number of VSDs, the PVR, the degree of RVOTO,
and the presence of any coronary abnormalities.

Management

 Medical Management:
o Monitor oxygen saturation level.
o Monitor growth and development.
o Monitor for hypercyanotic spells (many spells go unnoticed by parents).
o Infective endocarditis prophylaxis (lifelong).
o Restrict strenuous activity and participation in competitive sports.
 Balloon pulmonary angioplasty (rarely).
 Surgical intervention: Palliative versus definitive repair.
o Many centers prefer definitive, one-stage repair.
o Potential obstacles for one-stage repair: abnormal coronary artery distribution (left anterior
descending arises from right coronary artery and crosses RVOT); multiple VSDs; hypoplastic
branch pulmonary arteries; small infant weighing less than 5.5 lb (2.5 kg).
o Palliative surgery: modified Blalock-Taussig shunt (BT shunt); tube Gore-Tex graft between
the left subclavian artery and the PA: increased pulmonary blood flow results in higher
oxygen saturations.
o Definitive surgery: patch closure of VSD, relief of right ventricular outflow tract obstruction;
with or without transannular patch across the pulmonary valve.

Long-Term Follow-Up

 Assess RV outflow tract, monitor degree of pulmonary insufficiency.

 Monitor RV function and exercise tolerance.
 Monitor for arrhythmias.

Complications

 Hypoxia.
 Hypercyanotic spells.
 Polycythemia.
  CHF: rare; associated with pink TOF.
 Right ventricular dysfunction.
 Ventricular arrhythmias.
 Infective endocarditis.

TRICUSPID ATRESIA

Tricuspid atresia involves the absence of the tricuspid valve and hypoplasia of the right ventricle. Associated
defects such as an ASD, VSD, or PDA are necessary for survival. Tricuspid atresia accounts for 1% to 3%
of CHDs.

Pathophysiology and Etiology

 With TA, systemic venous return enters the right atrium and cannot continue into the RV; blood
flows across an atrial septal opening into the left atrium.
 Pulmonary blood flow occurs through a PDA or VSD.

Clinical Manifestations

 Cyanosis.
 Tachypnea.
 Feeding difficulties.

Diagnostic Evaluation

 Auscultation: murmurs vary depending on the associated lesions; single second heart sound.
 Chest X-ray: pulmonary vascular markings related to the amount of pulmonary blood flow (usually
decreased); normal to slightly increased cardiac silhouette.
 ECG: superior axis; right and left atrial hypertrophy; LVH.
 Two-dimensional echocardiogram identifies the atretic tricuspid valve and hypoplastic RV; Doppler
study and color flow mapping documents the right-to-left atrial shunt and the size of the PDA or
VSD.
 Cardiac catheterization may be necessary to delineate anatomy.

Management

Medical Management

 Stabilize with PGE1 infusion.

 Intubate and ventilate as needed.
 Inotropic support as needed.
 Infective endocarditis prophylaxis (lifelong).

Surgical Management

 First surgery neonate:

o BT shunt indicated if pulmonary blood flow is insufficient.
o Pulmonary artery band indicated if pulmonary blood flow is excessive.
o No treatment is required if pulmonary blood flow is balanced.
 Second surgery: ages 6 to 9 months:
o Bidirectional Glenn shunt: end-to-side anastomosis of the SVC to the right PA.
 Third surgery: ages 18 months to 3 years:
o Fontan completion: IVC to PA connection (extracardiac conduit or intracardiac baffle).
Complications

 CHF.
 Persistent pleural effusion (especially after Stage II and Stage III repairs).
 Thrombus formation in the systemic venous system.
 Infective endocarditis.
 Rarely, heart block.

TRANSPOSITION OF THE GREAT ARTERIES

Transposition of the great arteries (TGA) occurs when the PA arises off the left ventricle and the aorta arises
off the right ventricle. It accounts for 5% to 10% of CHDs. Associated lesions include VSD, ASD, PDA, PS,
and CoA.

Pathophysiology and Etiology

 This defect results in two parallel circulations:

o The right atrium receives deoxygenated blood from the inferior vena cava (IVC) and superior
vena cava (SVC); blood flow continues through the tricuspid valve into the right ventricle
and is pumped back to the aorta.
o The left atrium receives richly oxygenated blood from the pulmonary veins; blood flow
continues through the mitral valve into the left ventricle and is pumped back into the PA.
 To sustain life, there must be an accompanying defect that allows mixing of deoxygenated blood and
oxygenated blood between the two circuits, such as PDA, ASD, PFO, or VSD.
 Neonates born with TGA with an intact ventricular system are usually more cyanotic and sicker than
neonates born with TGA with a VSD.

Clinical Manifestations

Symptoms evident soon after birth; clinical scenario is influenced by the extent of intercirculatory mixing.

 Cyanosis.
 Tachypnea.
 Metabolic acidosis.
 CHF.
 Feeding difficulties.

Diagnostic Evaluation

 Auscultation: varies; no murmur or a murmur related to an associated defect, single S 2.

 Chest X-ray: varies; neonate chest X-ray usually normal; cardiomegaly with a narrow mediastinum
(egg-shaped cardiac silhouette) and increased pulmonary markings; or decreased pulmonary
markings with pulmonary stenosis.
 ECG: RVH or biventricular hypertrophy.
 Two-dimensional echocardiogram with Doppler study and color flow mapping identifies the
structural abnormalities: transposed vessels, coronary artery pattern, and degree of mixing across the
atrial septum plus associated lesions.

Management

Medical Management

 Stabilize with PGE1 infusion.

  Treat pulmonary overcirculation with digoxin and diuretics as needed.
 Intubate and ventilate as needed.
 Inotropic support as needed.
 Infective endocarditis prophylaxis (lifelong).

Cardiac catheterization

 Balloon atrial septostomy (Rashkind) is indicated for severe hypoxia to create or improve atrial level
mixing.

Surgical Management

 Arterial switch operation (Jatene)—procedure of choice:

o Ideally performed during the first week of life.
o The aorta and PA are switched back to their anatomically correct ventricle above the level of
the valve.
o Coronary arteries are transferred to the new aorta.
o Associated lesions are also repaired at this time.
 Rastelli operation—performed for TGA, VSD, and PS.
o Repaired during the first year of life.
o VSD patch repaired to include LV to aortic outflow continuity with pulmonary blood flow
provided via an RV to PA homograft.
 Atrial switch operation: Mustard or Senning procedure:
o Rerouting of atrial blood flow: RA → mitral valve → LV → PA and LA → tricuspid
valve → RV → Ao
o Restores oxygenated blood into the systemic system and deoxygenated blood guided to the
pulmonary system.
o Disadvantages:
 RV is left as the systemic ventricle will develop RV dysfunction.
 Increased incidence of atrial dysrhythmias and baffle obstruction.

Complications

 Severe hypoxia.
 Multiorgan ischemia.
 Arrhythmias.
 RV dysfunction.
 Coronary artery obstruction leading to myocardial ischemia or death.

Truncus arteriosus

It accounts for 1% to 4% of all congenital heart disease.it is marked by incomplete division of the common
great vessel, the truncus arteriosus which normally divide into the pulmonary artery and pulmonary valve
and the aorta and aortic valve. This failure in division results in a single large vessel and single valve, which
gives rise to the pulmonary, systemic and coronary circulations. The ventricular septum fails to develop at
the same time, and therefore and associated VSD is present. The common truncal arteriosus vessel override
the VSD and receive the blood from both right and left ventricles. There are four classification of truncus
arteriosus related to the site of origin of the pulmonary artery from the common truncal vessel. The truncal
valve is not a normal semilunar valve and can be stenotic or regurgitant.

Pathophysiology
 Desaturated blood enters the right atrium and flows through the tricuspid valve into the right ventricle.
Saturated blood from the left atrium flows through the mitral valve and into the left ventricle. The
desaturated and saturated blood mixes in the ventricles at the level of the VSD and common ventricular
outflow tract. The common great vessel send this mixes blood to the systemic, pulmonary and coronary
circulations.

Oxygen saturation depends on the volume of pulmonary blood flow, related to the pulmonary vascular
resistance the greater this flow; the more symptoms of CHF, decreased cardiac output and potential for
coronary artery ischemia. The ventricles are under pressure and volume overload.

Clinical manifestations

Unrestricted flow to the pulmonary artery results in pulmonary congestion and severe CHF.

Harsh systolic murmur

Bounding pulse

Widened pulse pressure

Management

Medical management is aimed at the reducing the efforts of CHF and preventing Polycythemia.

CHF is treated with digoxin and diuretics

Surgical management- newborns who do not respond to early medical management may benefit from
pulmonary artery banding and total corrective surgery is also choice of surgical intervention.

The corrective repair includes closing the VSD and placement of a conduit from the right ventricle to the
pulmonary artery. A valvoplasty of the truncal valve, which is the neoaortic valve, may be performed to
improve valvular competence. Blood flow postoperatively is normal.

Hypoplastic left heart syndrome

It accounts for 1% of all congenital heart disease. It is seen more frequently in males than females.
Approximately 95% of all affected infants who are untreated will die within the first months of life.

Inadequate development of the left side of the heart results in only one effective ventricle. The syndrome
may include aortic valve atresia, hypoplasia of the left ventricle, atresia or hypoplasia of the ascending aorta
and mitral valve stenosis or atresia. Most infants have an intact ventricular septum.

Saturated pulmonary venous blood return is unable to flow from the left atrium through the rest of the left
side of the heart. It is shunted left to right through a patent foramen ovale into the right atrium, where it
mixes with desaturated blood. Mixed saturated blood travels through the right ventricle to the main
pulmonary artery. A portion of blood flows through the branch pulmonary arteries and to the lungs. A
portion flows from the pulmonary artery through the PDA to the descending aorta. From the aorta this mixed
saturated blood provides systemic and coronary blood supply. The coronary blood supply is from retrograde
flow in the ascending aorta to the coronary arteries.

Clinical manifestation

Tachypnea

Early CHF from increased pulmonary blood flow

Systemic hypoperfusion

Shock

Infants appear grayish blue in color with dyspnea and hypotension.

Management

The management recommendations include option of supportive care only, surgical staged repair or cardiac
transplantation. The management option depend on the facility, team and country.

Emergency management addresses correction of the acid base and electrolyte imbalances and establishment
of ductal patency with prostaglandin.

Eisenmenger’s complex

A congenital cyanotic heart defect consisting of ventricular septal defect, dextraposition of the aorta,
pulmonary hypertension with pulmonary artery enlargement and hypertrophy of the right ventricle.

Obstructive lesions
COARCTATION OF THE AORTA
CoA is a discrete narrowing or a long segment hypoplasia of the aortic arch, usually in the juxtaductal
position. It accounts for 8% to 10% of congenital heart defects.
Pathophysiology and Etiology

 The discrete narrowing or hypoplastic segment of the aorta increases the workload of the left
ventricle (increased LV systolic pressure).
 In a neonate with critical CoA, lower body blood flow occurs through the PDA (right-to-left
shunting).
 In the older child, collateral vessels grow and bypass the coarctation to perfuse the lower body.

Clinical Manifestations

 The neonate with critical CoA (ductal dependent lesion):

o Asymptomatic until the PDA begins to close.
o After PDA closure: severe CHF, poor lower body perfusion, tachypnea, and acidosis,
progressive circulatory shock, absent femoral and pedal pulses.
 The child or adolescent with CoA:
o Usually asymptomatic normal growth and development.
o Hypertension in the upper extremities, with absent or weak femoral pulses.
o Nosebleeds, headaches, leg cramps.

Diagnostic Evaluation

 Auscultation varies; nonspecific systolic ejection murmur.

 Chest X-ray cardiomegaly and pulmonary edema or pulmonary venous congestion.
 ECG varies; normal or right ventricular hypertrophy (RVH) in infants and LVH in older children.
 Two-dimensional echocardiogram with Doppler study and color flow mapping identifies area of
aortic arch narrowing and associated lesions (bicuspid aortic valve, VSD, PDA).
 Invasive studies (cardiac catheterization) usually not needed to make the initial diagnosis; may need
aortic angiography to identify collateral vessels before surgery.
 Cardiac magnetic resonance imaging may be done to noninvasively assess the location and degree of
narrowing and identify collateral vessels.
Management

 Medical management:
o Resuscitation and stabilization with PGE1 infusion: monitor for complications related to PGE1
therapy (fever, apnea).
o Intubation and ventilation as needed.
o Infective endocarditis prophylaxis (lifelong).
o Anticongestive therapy (digoxin and Lasix) and inotropic support as needed.
o Assess renal, hepatic, and neurologic function.
 Balloon angioplasty may be indicated for infants who are a high surgical risk.
 Surgical intervention: usually performed as soon as the diagnosis is made.
o Subclavian flap repair (Waldhausen procedure).
o End-to-end anastomosis.
o Dacron patch repair.

Coarctation in the Child or Adolescent

 Surgical intervention.
o End-to-end anastomosis.
o Dacron patch.
 Medical management for hypertension (beta-adrenergic blockers).
 Infective endocarditis prophylaxis (lifelong).

Recurrent Coarctation in the Neonate or Child

 Balloon angioplasty in the cardiac catheterization laboratory.

 Redo surgical intervention.

Complications

 Systemic hypertension.
 CHF.
 Cerebral hemorrhage.
 Infective endocarditis.
 Left ventricular failure.
 Aortic aneurysm.

AORTIC STENOSIS
Congenital AS may be caused by a bicuspid aortic valve with fused commissures that does not open
completely, by a hypoplastic aortic valve annulus or by stenosis above or below the aortic valve
(subvalvular or supravalvular stenosis). The result is turbulent blood flow across the aortic valve and into the
ascending aorta. Patients with AS must be evaluated for additional left heart lesions that include CoA, mitral
valve stenosis, hypertrophic Cardiomyopathy, and hypoplastic left heart. AS is the most common form of
left ventricular outflow tract obstruction. It accounts for 3% to 6% of congenital heart defects. AS may occur
at any age, and it occurs more commonly in boys than in girls. In most children, it is a progressive lesion
that creates LVOTO.
Pathophysiology and Etiology

 Blood flows at an increased velocity across the obstructive valve or stenotic area and into the aorta.
 During systole, left ventricular pressure rises dramatically to overcome the increased resistance at the
aortic valve.
  Myocardial ischemia may occur because of an imbalance between the increased oxygen
requirements related to the hypertrophied left ventricle (LV) and the amount of oxygen that can be
supplied.
 Left-sided heart failure results in an increased LV end diastolic pressure that is reflected back to the
left atrium and pulmonary veins.

Clinical Manifestations
Neonate

 Severe congestive heart failure (CHF).

 Metabolic acidosis.
 Tachypnea.
 Faint peripheral pulses, poor perfusion, poor capillary refill, cool skin.
 Poor feeding and feeding intolerance.

Child and Adolescent

 Chest pain on exertion, decreased exercise tolerance.

 Dyspnea, fatigue, shortness of breath.
 Syncope, light-headedness.
 Palpitations.
 Sudden death.

Diagnostic Evaluation

 Auscultation.
o Systolic ejection murmur heard best at right upper sternal border, radiates to neck.
o Ejection click.
o S2 splits normally or narrowly.
 Electrocardiogram (ECG): left ventricular hypertrophy (LVH) with a strain pattern may be seen in
severe cases.
 Chest X-ray: increased cardiac silhouette increased pulmonary vascular markings. A prominent
aortic knob may be seen occasionally from poststenotic dilatation with valvular AS.
 Echocardiogram: two-dimensional echocardiogram with Doppler study and color flow mapping to
visualize the anatomy and to estimate the gradient across the valve and through the aorta.

Management
Neonate

 Stabilize with prostaglandin E1 (PGE1) infusion to maintain cardiac output through the PDA.
 Inotropic support as needed.
 Intubation and ventilation as needed.
 Infective endocarditis prophylaxis (lifelong).
 Cardiac catheterization: aortic balloon valvuloplasty or aortic balloon angioplasty.
 Surgical valvotomy, commissurotomy, or myectomy/myotomy.

Child and Adolescent

 Medical management with close follow-up to monitor increasing gradient across the aortic valve or
through the aorta.
 Restrict strenuous exercise and anaerobic exercise (e.g., weight lifting).
 Restrict participation in competitive sports.
 Aortic balloon valvuloplasty or aortic balloon angioplasty.
 Infective endocarditis prophylaxis (lifelong).
 Surgical intervention.
 o Surgical valvotomy, commissurotomy, or myectomy/ myotomy.
o Aortic valve replacement.
 Mechanical prosthesis (St. Jude valve).
 Ross procedure (pulmonary autograft)

Complications

 CHF and pulmonary edema.

 Dizziness, light-headedness, and syncope.
 Palpitations, arrhythmias.
 Infective endocarditis.
 Sudden death.

NURSING CARE OF THE CHILD WITH CONGENITAL HEART DISEASE

Nursing Assessment

 Obtain a thorough nursing history.

 Discuss the care plan with the health care team (cardiologist, cardiac surgeon, nursing case manager,
social worker, and nutritionist). Discuss the care plan with the patient, parents, and other caregivers.
 Measure and record height and weight. Plot on a growth chart.
 Record vital signs and oxygen saturations.
o Measure vital signs at a time when the infant/child is quiet.
o Choose appropriate-size blood pressure (BP) cuff.
o Check four extremity
 Assess and record:
o Skin color: pink, cyanotic, mottled.
o Mucous membranes: moist, dry, cyanotic.
o Extremities: check peripheral pulses for quality and symmetry; dependent edema; capillary
refill; color and temperature.
 Assess for clubbing (cyanotic heart disease).
 Assess chest wall for deformities; prominent precordial activity.
 Assess respiratory pattern.
o Before disturbing the child, stand back and count the respiratory rate.
o Loosen or remove clothing to directly observe chest movement.
o Assess for signs of respiratory distress: increased respiratory rate, grunting, retractions, nasal
flaring.
o Auscultate for crackles, wheezing, congestion, and stridor.
 Assess heart sounds.
o Determine rate (bradycardia, tachycardia, or normal for age) and rhythm (regular or
irregular).
o Identify murmur (type, location, and grade).
 Assess fluid status.
o Daily weights.
o Strict intake and output (number of wet diapers; urine output).
 Assess and record the child's level of activity.
o Observe the infant while feeding. Does the infant need frequent breaks or does he or she fall
asleep during feeding? Assess for sweating, color change, or respiratory distress while
feeding.
o Observe the child at play. Is play interrupted to rest? Ask the parent if the child keeps up with
peers while at play.
o Assess and record findings relevant to the child's developmental level: age-appropriate
behavior, cognitive skills, gross and fine motor skills.

Nursing Diagnoses
  Impaired Gas Exchange related to altered pulmonary blood flow or pulmonary congestion
 Decreased Cardiac Output related to decreased myocardial function
 Activity Intolerance related to hypoxia or decreased myocardial function
 Imbalanced Nutrition: Less Than Body Requirements related to excessive energy demands required
by increased cardiac workload
 Risk for Infection related to chronic illness
 Fear and Anxiety related to life-threatening illness

Nursing Interventions

Relieving Respiratory Distress

 Position the child in a reclining, semi-upright position.

 Suction oral and nasal secretions as needed.
 Identify target oxygen saturations and administer oxygen as prescribed.
 Administer prescribed medications and document response to medications (improved, no change, or
worsening respiratory status).
o Diuretics.
o Bronchodilators.
 May need to change oral feedings to nasogastric feedings because of increased risk of aspiration with
respiratory distress.

Improving Cardiac Output

 Organize nursing care and medication schedule to provide periods of uninterrupted rest.
 Provide play or educational activities that can be done in bed with minimal exertion.
 Maintain normothermia.
 Administer medications as prescribed.
o Diuretics (furosemide, spironolactone):
 Give the medication at the same time each day. For older children, do not give a dose
right before bedtime.
 Monitor the effectiveness of the dose: measure and record urine output.
o Digoxin:
 Check heart rate for 1 minute. Withhold the dose and notify the physician for
bradycardia (heart rate less than 90 beats/minute [bpm]).
 Lead II rhythm strip may be ordered for PR interval monitoring. Prolonged PR
interval indicates first-degree heart block (dose of digoxin may be withheld).
 Give medication at the same time each day. For infants and children, digoxin is
usually divided and given twice per day.
 Monitor serum electrolytes. Increased incidence of digoxin toxicity associated with
hypokalemia.
o Afterload-reducing medications (captopril, enalapril):
 When initiating medication for the first time: check BP immediately before and 1
hour after dose.
 Monitor for signs of hypotension: syncope, light-headedness, faint pulses.
 Withhold medication and notify the physician according to ordered parameters.

Improving Oxygenation and Activity Tolerance

 Place pulse oximeter probe (continuous monitoring or measure with vital signs) on finger, earlobe, or
toe.
 Administer oxygen as needed.
 Titrate amount of oxygen to reach target oxygen saturations.
 Assess response to oxygen therapy: increase in baseline oxygen saturations, improved work of
breathing, and change in patient comfort.
  Explain to the child how oxygen will help. If possible, give the child the choice for face mask
oxygen or nasal cannula oxygen.

Providing Adequate Nutrition

 For the infant:

o Small, frequent feedings.
o Fortified formula or breast milk (up to 30 cal/oz).
o Limit oral feeding time to 15 to 20 minutes.
o Supplement oral feeds with nasogastric feedings as needed to provide weight gain (ie,
continuous nasogastric feedings at night with ad-lib by-mouth feeds during the day).
 For the child:
o Small, frequent meals.
o High-calorie, nutritional supplements.
o Determine child's likes and dislikes and plan meals accordingly.
o Allow the parents to bring the child's favorite foods to the hospital.
 Report feeding intolerance: nausea, vomiting, diarrhea.
 Document daily weight (same time of day, same scale, same clothing).
 Record accurate inputs and outputs; assess for fluid retention.
 Fluid restriction not usually needed for children; manage excess fluid with diuretics.

Preventing Infection

 Maintain routine childhood immunization schedule. With the exception of RSV (Synagis) and
influenza, immunizations should not be given for 6 weeks after cardiovascular surgery.
 Administer yearly influenza vaccine.
 Administer RSV immunization for children younger than age 2 with complex CHD and those at risk
for CHF or pulmonary hypertension.
 Prevent exposure to communicable diseases.
 Good hand washing.
 Report fevers.
 Report signs of URI: runny nose, cough, increase in nasal secretions.
 Report signs of GI illness: diarrhea, abdominal pain, irritability.

Reducing Fear and Anxiety

 Educate the patient and family.

 Provide the family with contact phone numbers: how to schedule a follow-up visit; how to reach a
cardiologist during the work week, evenings, weekends, and holidays.

Family Education and Health Maintenance

 Instruct the family in necessary measures to maintain the child's health:

o Complete immunization.
o Adequate diet and rest.
o Prevention and control of infections.
o Regular medical and dental checkups. The child should be protected against infective
endocarditis when undergoing certain dental procedures.
o Regular cardiac checkups.
 Teach the family about the defect and its treatment.
o Provide patients and families with written and verbal information regarding the CHD. Offer
appropriate Internet resources for information about CHD and medical and surgical treatment
options.
o Signs and symptoms of CHF
 o Signs of hypercyanotic spells associated with cyanotic defects and need to place child in
knee-chest position.
o Need to prevent dehydration, which increases risk of thrombotic complications.
o Emergency precautions related to hypercyanotic spells, pulmonary edema, cardiac arrest (if
appropriate).
o Special home care equipment, monitors, oxygen.
 Encourage the parents and other people (teachers, peers) to treat the child in as normal a manner as
possible.
o Avoid overprotection and overindulgence.
o Avoid rejection.
o Promote growth and development with modifications. Facilitate performance of the usual
developmental tasks within the limits of the child's physiologic state.
o Prevent adults from projecting their fears and anxieties onto the child.
o Help family deal with its anger, guilt, and concerns related to the disabled child.
 Initiate a community health nursing referral if indicated.
 Stress the need for follow-up care.
 Encourage attendance in support groups for patients and families.

Bibliography:-

 Hockenberry & Wilson David, “Wong’s essentials of pediatric nursing”, 8 th edt.2009, Mosby Elsevier, St.
Louis, Missouri. Page no.862-891.

 Marlow R, Dorothy& Redding A. Barbara, “Text book of pediatric nursing”, 6 th edt.2007, Saunders
Company, Philadelphia, Pennsylvania, Page no.

 Ghai O.P., Gupta piyush et al, “essential pediatrics”, 6th edt.2006, CBS publishers & Distributers, New-
Delhi, page no.395-422.

 Gupte suraj “the short textbook of pediatrics” 11 th edition (2004) jaypee brother medical publishers p.
ltd. Page no.359-374.

 Bhatt Swarna Rekha, “Achar’s textbook of pediatrics”, 4 th edt.2009, universities press (India) private
limited, Hyderabad, page no.-

 Datta parul, “pediatric nursing”, 2 nd edt.2009, Jaypee brothers medical publishers (p) ltd. New-Delhi,
page no.320-328

 Mrs.Tambulwadkar R.S., “Pediatric nursing”, 2nd edt.2005, Vora medical publications, Bombay, page
no.

 Internet Sources- www.google.com