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Congenital Heart Disease

Evaluation of congenital heart disease requires

o ECG, CXR, and hyperoxia test (measuring preductal (radial a.) arterial blood gas on room air and then
repeating on FIO2 100%. Interpretation helps narrow diagnosis
Cyanotic Heart Disease (Ductal Independent Mixing Lesions) TTTT
o Truncus Arteriosus
Single arterial vessel, therefore complete mixing of systemic and pulmonic blood
usually has ventricular septal defect, 30% have right sided aortic arch
Associated with 22q11 microdeletion DiGeorges syndrome may hypocalcemia
Clinical Manifestations
Moderate Cyanosis, CHF develops in weeks, biventricular hypertrophy on ECG
Holosystolic Murmur at L sternal border
Single Loud S2 b/c theres only 1 valve
Anticongestion = digoxin & diuretics
Surgical repair close VSD, and make conduit b/n both aorta and pulm artery
o d-Transposition of Great Arteries
Aorta arises from RV, Pulmonary A. arises from LV operates in Parallel
#1 cyanotic HD presenting in neonates, 5% of all congenital heart disease
Most have intact ventricular septum
ASD, VSD, or PDA that allows mixing is required for survival
Clinical Manifestions
Cyanosis present from birth, Single Loud S2, Right ventricular heave,
If ventricular septum defect, then harsh holosystolic murmur lower left sternal border
Right ventricular hypertrophy seen, right axis deviation on ECG, Egg-shaped heart on
PGE1 to keep PDA open
Rashkind balloon atrial septostomy atrial mixing of blood
Surgical repair switch back
o Total Anomalous Pulmonary Venous Connection without Obstruction (TAPVC)
Rare lesion, where all pulmonary venous return is directed to the Right Atrium
o May drain to: Supracardia: SVC (50%), Cardiac: Directly to RA (20%),
Infradiaphragmatic: Portal or Hepatic (20%), Mixed (10%).
Without obstruction refers to the site where the anomalous vein enters a vessel ie
there is no constriction at this site. Therefore there is increased Pulmonary blood flow,
mild Pulmonary HTN, and Moderate Cyanosis
Clinical Manifestations
Findings similar to Atrial septal defect
Right ventricular heave, wide and fixed split S2
Systolic ejection murmur at Left upper sternal border
Cardiomegaly, increased pulmonary vasculature on CXR,
ECG shows right axis deviation and Right Ventricular Hypertrophy
Rx of CHF
Surgically redirect vessels into Left Atrium Necessary in 1st month of Life
Cyanotic Congenital Heart Disease: Lesions with Ductal-Dependent Pulmonary Blood flow
o Tricuspid Atresia
No connection b/n RA and RV, leads to hypoplasia or absence of Right ventricle
30% have transposition of great arteries, 90% have VSD (this allows blood to flow from
the left ventricle to both the Pulm A. & Aorta
Most also have pulmonic stenosis
The only way for systemic blood to get to the Left side (and therefore the Pulm A. and
Aorta is through a Patent Foramen Ovale or ASD
Clinical Manifestations
Cyanosis is severe, poor feeding, tachypnea over first 2 weeks of life
VSD loud holosystolic murmur at left lower sternal border if present
PDA continuous murmur if present
ECG superior axis and Left Ventricular Hypertrophy
PGE1 to keep PDA open to maintain Pulmonary flow
balloon atrial septostomy, if atrial defect not sufficient
Blalock-Taussig Shunt Gor-Tex conduit b/n subclavian Artery and Pulmonary A.
o Pulmonic Atresia with Intact Ventricular Septum
Rare defect with pulmonary valvular and infundibular atresia with right ventricular and
tricuspid valve hypoplasia
o ie. Basically the Right Ventricle is unable to produce any pulmonary outflow
so RV is hypertensive and have Tricuspid Regurgitation.
Dependent on PDA for survival
In some cases Coronary perfusion is dependent on Right Ventricle! Therefore
decreasing RV hypertension can lead to MI and death
Clinical Manifestations
Extremely cyanotic, tachypneic, Tricuspid Regurgitation murmur on left sternal border
Continuous PDA murmur
ECG LV hypertrophy, leftward axis
PGE1 to keep PDA open to maintain Pulmonary flow
Must check coronary arteries before surgery via catheterization
If coronaries not RV dependent, then RV to pulmonary artery connection is made
o Tetralogy of Fallot
#1 cause of Cyanosis presenting during 3rd week of life
4 defects: VSD, Right ventricular outflow obstruction, Right ventricular hypertrophy,
and overriding large aorta
Clinical Manifestations
Cyanotic due to right to left shunting across VSD and decreased Pulmonary flow
Tet Spells periodic cyanosis and agitation caused be increase in RV outflow tract
resistance, increasing the R L shunt. Last minutes to hours, may resolve or progress
to death
exam: RV heave felt, Systolic ejection murmur in L upper sternum due to RV outflow
PDA cont murmur, VSD holosystolic murmur at Left lower sternal border
ECG shows RA dilation, RV hypertrophy
25% have right sided arch
Tet-spells Rx by decreasing R L shunting, by increasing systemic vascular
resistance, and dec pulm vascular resistance
o Supplimental O2, vagal maneuvers, morphine sulfate, vasoconstrictors, beta-
blockers and volume administration, also bring the childs knees to their chest
increasing systemic resistance and decreasing the shunt from Right to Left.
o pull knees of child to chest decreases preload, increases systemic resistence
o Ebsteins Anomaly
Extremely rare, where leaflets are displaced into RV cavity
Results in hypoplasia of RV, tricuspid regurg and/or stenosis
80% have Patent Foramen Ovale, with R L shunt
Massively dilated RA
Associated with Wolff-Parkinson-White (WPW) syndrome
Clinical Manifestations
Cyanosis and CHF in first few days of life
Widely fixed S2 and Tricuspid Regurg at L lower sternal border
ECG shows Right Bundle Branch Block
Delta Waves due to WPW syndrome
CXR MASSIVE cardiomegaly, with enlarged RA
PGE1 to keep PDA open to maintain Pulmonary flow
CHF therapy with Digoxin and diuretics
Surgical repair of Tricuspid has had poor results
Cyanotic Congenital Heart Disease: Lesions with Ductal Dependent Systemic Blood Flow
o Hypoplastic Left Heart Syndrome (HLHS)
#1 cause of death from congenital HD during 1st month
Syndrome with: Hypoplasia of LV, Aortic Valve stenosis or atresia, Mitral Valve
stenosis or atresia and hypoplasia of ascending aortic with coarctation of aorta.
All combine to reduce flow through the Left side
Causes L R shunt at Atrial level & R L shunt at Ductus Arteriosus
Clinical Manifestations
As ductus close CHF, moderate cyanosis, tachypnea, pulmonary rales and
Poor or absent peripheral pulses
S3 and Loud single S2
PGE1 to keep PDA open to maintain flow to the Aorta from right side
Norwood palliation: restores unobstructed systemic blood flow
o Interrupted Aortic Arch
Aortic arch is not complete and ends in: Type A: beyond L subclavian, Type B: b/n L
subclavian and L common carotids, Type C: b/n L common carotids and
brachiocephalic arteries
All flow distal to the dead-end must come from PDA (R L)
Also associated with DiGeorges syndrome due to 22q11 microdeletion
Clinical Manifestations
Pulmonary edema
Presentation similar to Critical Coarctation of Aorta (below)
PGE1 to keep PDA open to maintain systemic flow
Surgery to reanastomose aortic segments
o Total Anomalous Pulmonary Venous Connection with Obstruction
Background see above
Presence of Severe Pulmonary Edema differentiates from w/o obstruction
Clinical Manifestations
TAPVC w/ obstruction presents with extreme cyanosis, tachypnea, and dyspnea
RV heave, narrowly split S2, Ventricular gallop (S3)
ECG R axis deviation, RA dilation, RVH
CXR pulmonary edema
PGE1 should not be given as pulmonary flow is already overflowed
Management of CHF and Pulmonary edmema
Surgical redirection of aberrant vessels into left atrium
Acyanotic Congenital Heart Disease
o Atrial Septal Defects
3 types:
o Ostium secundum: midportion of atrial septum
o Ostium primum: defect in low atrial septum
o Sinus venosus: defect at junction of RA and SVC or IVC
Usually results in L R shunting, increasing Pulm blood flow
Clinical Manifestations
Usually asymptomatic
RV heave often present
Systolic ejection murmur in Pulmonic area
S2 wide and constantly split
Ostium Secundum usually spontaneously close
Ostium Primum and sinus venosus require surgical closure
o Ventricular Septal Defects
#1 congenital defect, 26% of all
5 types: conoventicular, muscular, inlet, conoseptal hypoplasia, malalignment
Clinical Manifestations
Small shunt has no symptoms
Larger, gives growth failure, CHF, chronic lower respiratory infection
With Eisenmenger physiology (R L) presents with SOB, dyspnea, chest pain, and
Smaller defect = louder murmur
If pulmonary vascular obstruction RV heave, ejection click, short systolic ejection
murmur, diastoloic murmur of pulmonary valve insufficiency, Loud S2
Most close without intervention
May require surgery if large
o Common Atrioventricular Canal
Endocardial cushion defect
Deficiency of both atrial and ventricular septa and of Mitral and tricuspid valves
Severity varies widely depeding on extend of defect.
Approx 1/3 of Downs syndrome have this;
L R shunt at atrial and ventricular level
Clinical Manifestations
Pulmonary HTN may develop
Presents with tachypnea, dyspnea, and poor feeding
Blowing holosystolic murmur at L lower sternal border due to VSD and Fixed wide S2
due to ASD
ECG right axis deviation, RA dilation, LA dilatation
Surgical repair
o Patent Ductus Arteriosus
10% of Congenital heart disease, 2:1 female, higher in premies,
connects Pulmonary a to Aorta just distal to Left Subclavian a.
Clinical Manifestations
Small no symptoms
Larger L R: CHF, slow growth, repeated Lower Resp tract infections
Reversal to R L: result of high pulm vasc resistance, causes SOB, dyspnea and
Continuous machinery murmur
Indomethacin closes PDA by decreasing PGE1 levels
Usually closes by 1 month, if not surgery is required
o Coarctation of the Aorta
Constriction of aorta, usu decending at insertion site of ductus.
If in female consider Turners syndrome
Aortic valve is Bicuspid in 80%,
Clinical Manifestations
Weak femoral pulses relative to upper
If severe may rely on PDA
Systolic ejection murmur at Apex
Visualized with Echo
Balloon angioplasty or surgical end-to-end anastomosis
o Aortic Stenosis
Rigid thinkened valvular tissue LV hypertrophy
Clinical Manifestations
Presents with cardiac collapse or harsh systolic ejection murmur at right upper sternal
border and is preceded by ejection click
If severe may depend on PDA
On CXR see poststenotic dilatation of aorta
May see inverted T waves
Open surgical valvotomy or by balloon valvuloplasty
o Pulmonic Stenosis
Pulmonary commissures are fused
Clinical Manifestations
May cause dyspnea on exertion and angina,
Ejection click varies with inspiration
Harsh ejection murmur at L upper sternal border
Balloon valvuloplasty