Congenital Heart Defects Presentation

8/20/2019 Congenital Heart Defects Presentation

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Congenital Heart DefectsMODULE F

Chapter ElevenCardiac System

Pages 348 - 359

Objectives

• Differentiate between the different

congenital heart diseases and state

whether they are cyanotic and acyanotic.

• Describe the defect present with each

congenital heart disease.

• Describe the surgical repair (if any) for

each congenital heart disease.

Cardiac Defects

• Patent Ductus Arteriosus

• Atrial Septal Defect

• Ventricular Septal Defect

• Tetralogy of Fallot

• Transposition of the Great Arteries

• Coarctation of the Aorta

• Anomalous Venous Return

• Truncus Arteriosus

• Hypoplastic Left-Heart Syndrome

Web Sites

• http://www.childrensheartinstitute.org/edu

cate/defects/defects.htm

Normal Cardiac Blood Flow

Ductus Arteriosus

• Fetal Circulation Component

• Connects Pulmonary Artery to Aorta

• Shunts blood away from lungs

• Maintained patent by presence of

prostaglandins

• Closure secondary to:

• Increase in PaO2_

• Decrease in level of prostaglandins

Patent Ductus Arteriosus

• 5-10% of all births (1 of 2000 live births)

• 80% of premature babies

• 2-3 times more common in females than

males.

• 5th or 6th most common congenital cardiac

defect.

• Often associated with other defects.

• May be desirable with some defects.

• Morbidity/Mortality related to degree of

blood flow through PDA.

Pathophysiology - PDA

• With a drop in pulmonary arterial pressure

(reduction in hypoxic pulmonary vascularconstriction), blood will flow through PDA.

• LEFT TO RIGHT SHUNT

Increased pulmonary blood flow may lead topulmonary edema.

• Reduced blood flow to all postductal organs

• NEC

• If pulmonary artery pressure rises above Aorticpressure, blood will move in the other direction.

• RIGHT TO LEFT SHUNT

Diagnosis - PDA

• Loud grade I to grade III systolic murmur

at left sternal border.

• Washing machine

• Echocardiography

Treatment - PDA

• Restrict fluids.

• Diuretics

• Prostaglandin Inhibitors - Indomethacin

Surgical closure (ligation).

Atrial Septal Defect

• 6-10% of all births (1 of 1500 live births)

• 2 times more common in females than males.

• Types:

• Ostium Secundum (at or about the Foramen Ovale)

•Sinus Venous (at about the point where SVC and IVCattach to Right Atrium)

• In 1950 most children with ASD did not reach the

first grade. Today, first year surgery facilitates

normal growth and development.

ASD P h h i d

ASD: Pathophysiology andDiagnosis

• Pathophysiology

• Left to Right Shunt

• Inefficient recirculation of good blood through

pulmonary arteries.

May not manifest symptoms and may befound later in life.

• If defect is significant, may cause problems

later in life due to inefficiencies.

• Diagnosis

• Murmur

Treatment - ASD

• Surgical closure.

• Non-Surgical closure via cardiac

catheterization.

Ventricular Septal Defect

• 1% of all births (2 to 4 of 1000 live births)

• Vast majority the hole is small.

• In 1950, fatal. Today almost all VSD can be

closed successfully, even in small babies.

Lillehei was the first person in history tocorrect both ASD and VSD on 8/31/54.

VSD: Pathophysiology & Diagnosis

• Pathophysiology

• May be isolated or associated with other

congenital cardiac defects.

• With normal PVR:

• LEFT TO RIGHT SHUNT

• With elevated PVR (RDS):

• RIGHT TO LEFT SHUNT

Diagnosis• Echocardiography

Treatment - VSD

• Nothing if VSD is small.

• With CHF or Failure to Thrive: Surgical

closure.

http://64.143.41.32/news.html

Tetralogy of Fallot• 1% of neonates.

• Most common of the cyanotic cardiac diseases.• Mortality increases with age (1 year-old has a

25% mortality, 40 year-old has 95%).

• In 1950, fatal. Today, less than 5% mortality withchildren operated on in infancy, leading normallives.Four Defects• Pulmonary Artery Stenosis (determinant factor related

to severity)• VSD (usually large)

• Overriding Aorta

• RV hypertrophy

Tetralogy of allot agnos s an

Tetralogy of allot: agnos s an Treatment

• Tet Spells

•CXR: Boot-shaped Heart

• Diagnosed with echocardiography.

• Surgical correction.• Reparative or Palliative (Blalock-Taussig)

Blalock-Taussig

• Something

the LordMade.

• Vivien

Thomas

Com lete T ns osition of the

Complete Transposition of theGreat Arteries

• Second most common form (5-7%) of

congenital cardiac anomalies.

• Aorta arises from RV and Pulmonary

Arteries from LV.

• Without an abnormality, life would not

be possible.

• ASD

• VSD (30-40%)

• PDA

Transposition Diagnosis and

Transposition – Diagnosis and Treatment

• Diagnosis

• Chest X-Ray: “Egg on aString”

• Cardiac Catheterization (?)

•Treatment• Balloon septostomy during

cardiac cath.• Rashkind’s Procedure

• Reestablish Foramen Ovale

• Prostaglandin E1 to keepPDA open.

• Surgical Correction• Jantene Operation

Coarctation of the Aorta

• 7% of congenital cardiac defects.

• Constriction of the aorta.

• Results in severely reduced blood flow.

• Increased work on the heart leading to

CHF and cardiovascular collapse.

• Location of narrowing determines the

clinical signs.

• Usually associated with PDA, VSD and a

defective aortic valve.

Location of Coarctation

• Pre-Ductal

• Less common but more serious

• Associated with VSD, PDA, Transposition

• Post-Ductal

• More common

• Often associated with collateral circulation

beyond coarctation, which minimizes effect.

• Diagnosed by a difference in blood pressurebetween lower extremities and upper ones.

• Pressure in upper extremities > lower

oarctat on agnos s an

oarctat on – agnos s an Treatment

• Diagnosis

• Chest X-Ray

• Cardiac catheterization

• Treatment

• Support with inotropic agents (Dopamine).

• Prostaglandins to maintain PDA.

• Surgical repair

Anomalous Venous Return

• Return of pulmonary venous blood to the

right atrium instead of the left.• ASD is present to sustain life.

• Can also be partial.

• Cyanosis usually present.

• Diagnosed with echocardiography.

• Surgical correction with reimplantation of

pulmonary veins.

Truncus Arteriosus

• Defect in which one large vessel arises fromright and left heart over a large VSD.

• Cyanosis is often present.

CHF common.• Diagnosed with echocardiography and

cardiac catheterization.

• Surgery:

• Separate pulmonary arteries from truncus.

• Closure of VSD

• Create valved connection between RV and

Pulmonary Artery

Repair of Truncus

Arteriosus

Hypoplastic Left-Heart Syndrome• Several anomalies:

• Coarctation of the aorta• Hypoplastic left ventricle

• Aortic and mitral valve stenosis or atresia.

Cyanotic defect.• Right heart pumps blood to body through

Closure of PDA results in hypotension,shock, and death.• Maintain hypoxemia with normalized CO2

levels.•

“40-40 Club”

1 Patentforamenovale

2 Coarctationof the aorta

3 Patent ductus

arteriosus4 Narrowed

5 Hypoplasticleft ventricle

6 Aortic atresia

Surgical Treatment of Hypoplastic Left

Surgical Treatment of Hypoplastic LeftHeart Syndrome

• Three separate

surgeries.• Norwood procedure

• First few days after birth.

• Glenn Shunt (CavoPulmonary Connection)• 3-9 months of age

• Fontan Procedure•

2 years of age• Less wait because of

damage from pulmonaryhypertension.

St ge I No wood P ocedu e

Stage I - Norwood Procedure

Stage II Glenn Shunt

Stage II - Glenn Shunt

Stage III Fontan Procedure

Stage III – Fontan Procedure

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