Congenital Heart Disease 1.Docx

heart disease

conginital

cyanotic

decrease pulmonary blood flow

increase pulmonary blood flow

aquierd

reumatic heart disease

non-cyanotic

mixed

L-RT shunt

obstructive stenosis

TOF

TGA

TAPVR

VSD

Pulmonary stenosi

tricusped

truncus arteriosus

HLHS

ASD

aortic stenosis

PDA

coarctation of aorta

atresia

endocardial cushion defect

kawasaki disease

infections

A) Cyanotic congenital heart disease

( the common complication of them that they are increase the risk of cerebral

thromboembolism and cerebral abscess)

Decrease pulmonary blood flow Tetralogy of fallot

Tricuspid Atresia

Definition

characteristic Clinical picture Investigation Treatment

*Due to abnormal septation of truncus arteriosus into the aorta and pulmonary artery . *there is 4 abnormalities : VSD , pulmonary stenosis , overriding aorta and hypertrophy of right ventricle .

*most common cyanotic congenital heart disease . * according the severity of pulmonary stenosis it could appear either in infancy or at first or second year of life .

Absence of tricuspid valve lead to hypoplastic of right ventricle . PDA and VSD is important for pulmonary blood flow and survival . VSD can occur as a part of the disease

-Hypoxic (tet) spells . –rapid and deep breathing - cyanosis ( degree of it depends on pulmonary stenosis) -right ventricle impulse -single S2

*ECG > RT.axis deviation and RT.ventricular hypertrophy *CXR> Boot shaped heart sign *Echo .

Treatment of hypoxic spells : -O2 -knee chest position -morphine-sulphate -a-adrenergic agonist (phenylephrine ) *palliative shunt surgery *complete surgical repair .

*Severe cyanosis *single S2 *VSD murmur if VSD present .

*ECG > L.ventricle hyprertrophy , sup QRS axis . Rt. Atrial hypertrophy. *CXR> normal or mild enlargement of the heart. *Echo

Presence of VSD is important for the baby , if it is small or absent give prostaglandin E1 , to keep ductus arteriosus open . stages of surgery : 1-blalock taussing procedure 2-bidirectional glenn 3-fontan procedure

Cyanotic congenital heart disease increase pulmonary blood flow Transposition of great artery

Truncus arteriosus

Definition Transposition of great artery secondary to abnormality of septation of the truncus arteriosus

Result from failure of septation of the truncus arteriosus during first 3-4 week of gastation . single arterial trunk arises from the heart with a large VSD immediately below the trunk valve .

characteristic *most common cyanotic losion that present in the newborn period . *if there is no mixing death occur early in life . *usually VSD, ASD ,PDA occur as part of the disease . If not diagnosed at birth , the infant may develop signs of heart failure .

Clinical Investigation picture - cyanosis ( degree of it depends on the amount of mixing. -queit tachypnea -single S2 -VSD murmur may present -may present with HF . - cyanosis ( degree of it depends on the amount of pulmonary blood flow ) -tachypnea -cough -peripheral pulses are usually pounding . -single S2 -may be cystolic ejection click -systolic murmur -signs of HF .

Treatment

*ECG > RT.axis deviation and RT.ventricular hypertrophy *CXR> egg on a string sign *Echo

- prostaglandin E1 to maintain ductul patency . -pallon atrial septostomy . -atrial switch usually done at the first 2 weeks of life

*ECG > combined ventricular hypertrophy and cardiomegaly . *CXR> displased pulmonary artery . *Echo

-anticongistive drugs -surgical repair : VSD closure , placement of conduit between the RT.ventricle and pulmonary artery .

Cyanotic congenital heart disease Mixed

Definition Total anomalies pulmonary venous return

Disruption of the development of normal pulmonary venous drainage during 3ed week of gastation , results one of 4 abnormalities . all of pulmonary viens fail to connect to the ledt atrium and return abnormally via RT side of the heart . they may have supracardiac , infracardiac , cardiac , mixed drainage .

characteristic

Clinical picture

Investigation

Treatment

The most important determinant of presentation is the presence or absence of obstruction to the pulmonary venous drainage . infant without obstruction have minimal cyanosis and nay be asymptomatic . -hyperactive RT , ventricular impulse . -widely split S2 . -systolic ejection murmur at the left sternal border . - mid-diastolic murmurs at the lower left sternal border -poor of growth infants with obstruction present with cyanosis , marked tachypnea , and dyspnea , and sighn of RT. Side heart failure including hepatomegaly .

Infant wighout obstruction : *ECG > cinsistant with RT ventricular volume overload *CXR> cardiomrgaly *Echo Infants with obstruction : *ECG> RT.axis deviation and RT.ventricular hypertrophy *CXR: normal or mild enleargment of the heart , and varieng degree of pulmonary edema that appear as pneumonia . ( snow man sign on CXR )

Surgery repair : common pulmonary vie nary opend into the left atrium .

Hypoplastic left heart syndrome

Failure of development of mitral or aortic valve or the aortic arch .

Most common cause of death from cardiac defects in the first month of life .

-sign of HF -pulses are weak or absent -S2 single and load -no murmur -cyanosis may be minimal but low cardiac output gives a grayish color to the cool , mottled skin .

*ECG > RT. Ventricular hypertrophy , decreased left ventricular forces . *CXR> cardiomegaly with pulmonary vonous congestion or pulmonary edema . *Echo

- prostaglandin E1 . -correction acidosis -surgical repair : norwood procedure , bidirectional glenn and fontan procedure )

B) ACyanotic congenital heart disease Left to right shunt

Ventricul ar septal defect

Definition

characteristic

Clinical picture

Investigati on

Treatment

Ventricular septum contain 4 component : -muscular septum, -endocardial cushion septum, -supracristal septum, -membranous septum . VSD occur when any of these component fails to develop normally.

*most common congenital heart disease . *peri-membranous VSD are the most common of all VSD. *large VSD are not symptomatic at birth , at first 6 to 8 weeks of life it decrease , the amount of shunt increase , and symptoms may develop .

Small VSD : little shunt so it may asymptomatic but have a loud murmur . Had 2 important sign : (important in OSCE) 1- thrill at left lower sternal border . 2- loud pan systolic murmur ar left lower sternal border . moderate to large VSD : results in pulmonary over circulation and HF . Presentation is usually early and can present at 1 week of age with sign and symptoms of Heart failure : Breathlessness during feeding Poor feeding Sweating during feeding ,Failure to thrive ,Tachypnoea ,Tachycardia Hepatomegaly -Recurrent chest infection one of the presentation *large shunt increase flow

Small VSD normal in ECG and CXR . large VSD: *ECG > left atrial and ventricular hypertrophy . *CXR> cardiomegaly , enlargement of L.Ventricle , increase in pulmonary blood flow and increase in pulmonary artery silhouette.

Small : no need for treatment , up to 90% it will close spontaneously . initial treatment of moderate to large VSD include ; diuretics , digoxin and afterload reduction . most of it close surgically All children with VSD must be given antibiotic prophylaxis to prevent bacterial endocarditis

Atrial septal defect

Failure of septum growth , or excessive reabsorption of tissue lead to ASD .

Patent ductus arteriosus

Failure of normal closure of ductus arteriosus . which is important to keep open in fetal

There is 3 types : *septum secundom defect with the hole in the region of the foramen ovale , is the most common type . *septum premium ( partial arterioventricular defect ) common in down syndrome . * the least common ASD is the sinus venous defect .

across mitral valve so middiastolic murmur at the apex may be heard ( Soft or no systolic murmur ). * Parasternal Thrill *there may found splitting of S2 and intensity of P2. -even with large ASD infants are rarly symptomatic . -prominent RT.ventricular impulse ( at the left lower sternal border) 2 most important sign : (important in OSCE) 1-systolic ejection murmur . Soft murmur at upper left sternal edge . 2-fixed split S2 . -large shunt may result in a middiastolic murmur at left lower sternal border .

Small PDA asymptomatic moderate to large PDA : shunts can produce the symptoms of heart failure . exam. Finding : 2 most important sign : (important in OSCE)

*ECG > RT.axis deviation and RT.ventricular hypertrophy . *CXR> cardiomegaly and prominent pulmonary artery .

Medical management rarely recommended . if a shunt is still present at around 3 years , closure usually recommended. many secondum ASD closed by surgical device , while premium and sinus venous defect require surgical closure .

Small VSD normal in ECG and CXR . large VSD: *ECG > vary from normal to evidence of left

In Preterm Baby: I/V indomethacin or Oral Brufen if no contraindication to these medications If Failed Medical :Treatment

life , it allows blood to flow from pulmonary artery to the aorta .

Endocard ial cushion defect

Also called arterioventricular canal defect , may br complete or partial. Failure of the septum to fuse with the endocardial results in abnormal arterioventricualr valves as well . the complete defect defect results in

* complete endocardial cushion defect are most commonly in a child with down syndrome .

1-widened pulse pressure (Bounding pulse , or collapsing pulse ) 2-continuous , machine like murmur ( can be heard over mid-clavicular area . -large shunts can cause middiastolic murmur over mitral area ( at the apex ) and hyper dynamic pericardium . -thrill may be palpable –splitting S2 and intensity of P2. -FTT -symptom of HF usually develop. -symptoms may be earliar and more severe with significant arterio-ventricular valve insufficiency. -presence of murmur varies depending on the amount of shunting . -if there is large VSD , single S2 may be found . - poor growth of child.

ventricular hypertrophy *CXR>full pulmonary artery silhouette , and increase pulmonary vasculature .

Small PDA can be closed in Cardiac Cath Lab with COIL at 1 year If Large and S/S of Failure to thrive or Pulmonary HTN Surgical Ligation at 3-5 month

*ECG > L. axis deviation and combined ventricular hypertrophy. *CXR> cardiomegaly with increased vascularity . *Echo ( diagnostic test )

Initial management includes diuretics , digoxin , afterload reduction for treatment for treatment of HF . surgical repair is required .

premium ASD , posterior or inlet VSD , amd cleft in the anterior leaflet of the mitral and septal leaflet of the tricuspid valve .there may be arterioventricular valve insufficiency

ACyanotic congenital heart disease Obstructi ve stenosis

Definition Pulmona ry stenosis

Failure of development in early gestation of the three leaflet of the valve , insufficient reapportion of infundibular or insufficient canalization of the peripheral pulmonary arteries. Can be valvular , supvavular and supravalvular .

characte ristic

Clinical picture

Investigation

Treatment

Mild pulmonary stenosis : asymptomatic . moderate to severe stenosis : exertional dyspnea , easy fatigability . newborn with severe stenosis may be cyanotic because right to left shunt at the atrial level . - systolic ejection murmur at the second left intercostal space with radiates to the back . -thrill may be present . -S2 may be widely split . - in severe stenosis there may be RT.ventricular impulse . -valvular stenosis may result click that varies with respiration .

Mild stenosis: normal in ECG and CXR . moderate to severe : *ECG > Rt. axis deviation and Rt. ventricular hypertrophy . *CXR> heart size may be normal , dilatation of the main pulmonary artery . *Echo

Usually Nothing do with mild stenosis . Balloon vavoplasty . surgical repair require if balloon unsuccessful .

Aortic stenosis

Failure of development of the three leaflet of the valve , insufficient reapportion of tissue around the valve . Can be valvular , supvavular and supravalvular .

* secundom defect with the hole in the region of the foramen ovale . is the most common ASD . * the least common ASD is the sinus venous defect .

Mild to moderate stenosis : asymptomatic . severe stenosis : exertional dyspnea , exertional chest pain and syncope. newborn with critical stenosis may present with symptoms of HF . - systolic ejection murmur at the right second intercostal space with radiates to the neck . -systolic ejection click often is heard . -thrill may be present at the . -the aortic component of S2 may be decreased in intensity

Mild stenosis: normal in ECG and CXR . moderate to severe : *ECG > L. ventricular hypertrophy . *CXR> L. ventricular hypertrophy , dilatation of ascending aorta or aortic knop . *Echo

The degree of aortic stenosis frequently progresses with growth and age. Aortic insufficiency usually develop . serial follow up with Echo is indicated . Balloon vavoplasty . surgical repair require if balloon unsuccessful .

Coarcta tion of the aorta

During development of aortic arch area near the insertion of the ductus arteriosus fails to develop correctly , resulting in narrowing of the aortic lumen .

*Usually juxta-ductal in position

Symptoms develop when aortic ampula of the ductus closes . In infantile coarctation symptoms : poor feeding , respiratory distress , shock may develop before 2 weeks of life . classicaly the femoral pulses are weeeker and delayed compared with the right radial pulse ( radiofemoral delay ) - there may be no murmur but S3 is often present . older children may be asymptomatic of have leg discomfort during exercise , headache , or epistaxis . Arterial hypertension in the arms with low blood pressure in the lower extremities is classic and weeker pulse in femoral artery ( radio-femoral delay ) the murmur is typically best heard in the left inter-scapular area of the back . if there is abnormal aortic valve , there is systolic ejection murmur and systolic ejection click .

Done by Arwa Al-ahmadi , reviewed by shada albogami and Arwa Alahmadi (1436 H )

In infantile coarctation marked cardiomegaly , Rt ventriculat hypertrophy and pulmonary edema in older children : l.ventricular hypertrophy and midly enlarged heart . hypertrophy Rib-notching may also be seen in older children ( > 8 years ) *echo

*IV infusion of prostaglandin E1 . , inotropic agent , diuretics and other supportive care . balloon angioplasty surgical repair most commonly perform