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

http://www.childrensheartinstitute.org/educate/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



A V R



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

PDA.

•

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

aorta

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

congenital heart defects presentation

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