ACYANOTIC CONGENITAL HEART DISEASES

By Meilleurs Amis
Remember: INCREASED VOLUME LOAD- ASD, VSD, PDA, AVSD Lonelybitter
ATRIAL SEPTAL ATRIOVENTRICULAR VENTRICULAR SEPTAL PATENT DUCTUS
DEFECT SECUNDUM SEPTAL DEFECT DEFECT ARTERIOSUS
Descriptio Present at the site of Also called Endocardial MOST COMMON cardiac Abnormal persistence
n fossa ovalis cushion defect malformation 25% of of a normal fetal
Consists of contiguous atrial CHD vascular channel
and ventricular septal most common are the between the
defects with markedly MEMBRANOUS TYPE(in pulmonary artery
abnormal AV valves the posteroinferior and aorta
Component of heart defects position, anterior to the
in asplenia and septal leaflet of the TV)
polysplenia syndrome
Pathophys Despite the large In Ostium primum SMALL lesion <5mm The higher aortic
iology pulmonary blood defects L-R shunt across VSD is pressure pressure postnatally,
flow(PBF), pulmonary the atrial defect and MV restrictive RVP is blood shunts left to
arterial pressure (PAP) ( occ. TV) insufficiency The normal right through the
is usually normal shunt is usually moderate LARGE DEFECT >10mm ductus, from the
because of the to large, PAP is normal or RV and LV pressures aorta to the
absence of a high only mildly increased are equalized pulmonary artery
pressure In Complete AVSD- the L-R Qp:Qs ratio-described the
communication occurs at both the atrial magnitude of the
between the and ventricular levels intracardiac shunts
pulmonary and Additional shunting from
systemic circulations the LV to RA because of
absence of the AV septum
Shunting Size of defect Level of PVR (Pulmonary Size of the defect Resistance offered
depends Relative compliance vascular resistance) Level of pulmonary by the ductus
on: of the RV and LV vascular resistance Level of PVR
Relative PVR and
SVR
History ASYPTOMATIC Failure to thrive Small VSDs- Small PDA-
Large defect rarely Repeated respiratory tract asymptomatic asymptomatic
produce clinical HF in infections Moderate to large Large PDA- result in
childhood Exercise intolerance VSDs- delayed growth heart failure,
May have subtle failure Easy fatigability and development, retardation of
 to thrive and Signs of CHF dyspnea, feeding physical growth and
exercise intolerance difficulties, profuse frequent lower
perspiration, decreased respiratory tract
exercise tolerance, infections and
repeated pulmonary atelectasis
infections, CHF are
common during infancy
Physical RV systolic lift at the Hyperdynamic precordium Small shunts- loud, Small shunt- (+)
Examinati LPSB S1 normal or accentuated harsh or blowing HSM classical
on S2 widely split and S2 wide, fixed splitting LPSB continuous
fixed in all phases of Pulmonary systolic Large shunts- less harsh machinery
respiration ejection murmur but more blowing murmur LUSB and
SYSTOLIC EJECTION preceded by a click holosystolic murmur normal peripheral
MURMUR (SEM) AT Low pitched mid diastolic (HSM) at the left pulses
THE LEFT rumble at the LLSB parasternal border Large shunts-
MIDSTERNAL Harsh apical holosytolic (LPSB) tachycardia, apical
BORDER murmur of mitral Mid-diastolic ,low pitched diastolic rumble,
(LMSB)/LEFT UPPER regurgitation rumble at the apex due bounding peripheral
STERNAL BORDER to incrased blodd flow arterial pulses and a
(LUSB) caused by across the MV and wide pulse pressure
increased flow indicates Qp:Qs >/= 2:1 PVOD- accentuated
across the right P2, right-to-left
ventricular outflow shunt results in
tract(RVOT) into differential cyanosis
the pulmonary (cyanosis of lower
artery half of the body
only)
ATRIAL SEPTAL ATRIOVENTRICULAR VENTRICULAR SEPTAL PATENT DUCTUS
DEFECT SECUNDUM SEPTAL DEFECT DEFECT ARTERIOSUS
ECG RAD (Right axis Superior/Extreme QRS Small VSDs- normal Small PDA normal
deviation) axis Moderate VSDs- LAE, Moderate PDA LAE,
RVE LVH LV
rsR pattern on R Large VSDs- LAE, Large PDA LAE, left
precordial leads combined ventricular ventricular or
hypertrophy biventricular
hypertrophy
Chest X- Xray- RIGHT side will Cardiomegaly Small defect- Normal Small PDA normal
ray be enlarged! (RAE Increased pulmonary size heart, normal PVM size heart and
 and RVE) and vascular markings Large defect- normal PVM
pulmonary Prominent MPA Cardiomegaly of varying Large PDA
vascualrity is degrees: LAE, LVE, BVE; cardiomegaly ,
INCREASED Increased PVM, prominent
prominent MPA pulmonary artery
with increased
pulmonary vascular
markings
Treatment Surgical or MEDICAL: MEDICAL: Requires surgical or
transcatheter device Management of CHF Treatment of CHF: catheter closure
closure for all SBE prophylaxis diuretics, digitalis Small PDA
symptomatic patients High calorie formulas -rationale for closure
and also for SURGICAL: Correct anemia is prevention of
Asymptomatic Early surgical repair(3-8 SBE prophylaxis: bacterial
patients with a Qp:Qs months): early maintenance of good endarteritis.
ratio of at least 2:1or PVOD (pulmonary vascular dental hygiene and -closed with
those with RVE occlusive disease antibiotic intravascular coils
Timing of elective Non-surgical Large PDA
pulmonary HPN)
closure:umbrella - closure is
surgery: after the 1st
device accomplished to
year and before
treat heart failure.
school entry
-closed with an
PERCUTANEOUS INDICATIOSN FOR
umbrella-like
CATHETER DEVICE SURGERY:
device
CLOSURE procedure Any age with large defects
of choice in whom clinical sxs and
Surgical treatment -
failure to thrive cannot
less than 1%
be controlled medically
Closure of the ductus
Between 6 and 12 months
is indicated in
old with large defects
asymptomatic
associated with
patients -1 yr. of
pulmonary hypertension
age
even if symptoms are
controlled medically
Older than 24 months with SURGICAL CLOSURE:
Indications:
a Qp:QS ratio greater
than 2:1 PDA regardless of size
Those WITH is an indication for
SUPRACRISTAL VSD of surgery
 any size because of the Timing: anytime
HIGH RISK FOR AV between 6 months
REGURGITATION and 2 years when
diagnosis is made
PROCEDURE: ligation
and division
through left
posterolateral
thoracotomy
without CP bypass
Natural NO CHANCE OF Membranous and Spontaneous
History SPONTANEOUS muscular small VSDs: closure does not
CLOSURE spontaneous closure in usually occur in
Heart failure occurs 1 2 30-50% of during the 1st full term infants
months of life 2 years of life, may (structural
Recurrent pneumonia decrease in size with age abnormality)
Without intervention: Inlet and CHF or recurrent
most die by 2-3 years of infundibulardefects:d pneumonia, PVOD
age o not become smaller or Infective endocarditis
PVOD close spontaneously Aneurysm of PDA: rare

ATRIAL SEPTAL ATRIOVENTRICULAR VENTRICULAR SEPTAL PATENT DUCTUS

DEFECT SECUNDUM SEPTAL DEFECT DEFECT ARTERIOSUS
Prognosis Small to moderate Unrepaired complete AV Results of primary surgical Spontaneous closure
sized ASDs may septal defect depends on repair are excellent and after infancy is
close spontaneously the magnitude of the L-R complications are rare extremely rare
Well tolerated during shunt, the degree of
childhood and Sxs do elevation of the PVR and Surgical risks higher in Small PDA - live a
not usually appear the severity of the AV muscular septum normal span with
until the 3rd decade insuffieciency Death from particularly apical few or no cardiac
or later cardiac failure during defects and multiple symptoms but late
Infective infancy before the advent VSDs manifestations may
Endocarditis is of early surgery occur
extremely rare and PVOD in patients without Long term prognosis after
SBE prophylaxis is surgery surgery is excellent Large and
not recommended Late post-operative moderate sized
complications PDA- cardiac
Atrial arrhythmias and heart failure most often
 block Progressive occurs in early
narrowing of the LVOT infancy but may
requiring surgical revision occur later in life.
Worsening of AV valve
regurgitation requiring
Most Most common form Common in TRISOMY 21 MOST COMMON cardiac PDA is associated with
common/ of atrial septal (DOWN SYNDROME) malformation 25% of maternal rubella
Must defect CHD infection during
knows Of all types of atrial PATHOLOGY most common are the early pregnancy.
septal defect, ONLY Components of Components MEMBRANOUS
of TYPE(in
SECUNDUM TYPE COMPLETE ECD PARTIAL ECDthe posteroinferior FUNCTIONAL
CLOSE Ostium primum Ostium primum position, anterior to the CLOSURE OF
SPONTANEOUSLY ASD ASD septal leaflet of the TV) DUCTUS
Associated with Inlet VSD ARTERIOSUS:
autosomal dominant Cleft in AMVL Cleft in AMVL CLASSIFICATION OF constriction of medial
HOLT-ORAM septal leaflet of VSD and smooth muscle
SYNDROME TV PERIMEM Mostofcommon defect
the ductus 10 (80%)
BRANOUS -15 hours after
PATHOLOGY OF ASD OUTLET 5-7%birth
Type of Cardiac Catheterization- May cause AR
ASD Gooseneck deformity INLET 5-8%
ANATOMIC
Ostium 50-70% (most Mitral valve or aortic valve posterior and inferior to perimembranous defect
CLOSURE:
secundu common) present deformity and distortion of MUSCULAR 5-20%
Permanent changes in
m in 10% of APVR the LVOT Highest chance of closure
the endothelium and
Ostium 15% isolated ASDs Central: mid-muscular
sub-intimal layers of
primum 30% as part of ECD Apical: multiple apparent channels on RV side,
the ductus 2 to 3
(endocardial maybe single defect on LV side
weeks of age
cushion defect) Marginal: along RV septal junction
Sinus 10% of all ASDs Swiss cheese septum:large numbers of muscular
Re-opening of
venosus located at entry of defects
Constricted Ductus:
defect SVC to RA
Before true anatomic
Coronar rare
closure occurs,
y sinus communication
functionally closed
defect between CS and LA
ductus may be dilated
by:
reduced arterial
PO2
increased PGE
 in asphyxia and
various pulmonary
diseases ductal
closure is delayed
in high altitude

ACYANOTIC CONGENITAL HEART DISEASES

Remember: INCREASED PRESSURE LOAD- PS, AS, COA (OBSTRUCTIVE LESIONS)
PULMONARY STENOSIS
Descripti 8-12% of all CHD
on Common in NOONANS SYNDROME
TYPES: valvar, subvalvar, or supravalvar
Valve cusps are deformed and result to
incomplete opening during systole
Patho Results in increased right ventricular
physiolo systolic pressure and wall stress
gy leads to hypertrophy of the right
ventricle
Severity depends on the size of the
restricted valve opening
Severe cases RVSP > SASP
Mils cases RVSP is only mildly or
moderately elevated
History Depends on the degree of obstruction
and the +/- of IAC
Mild to mod. PS- usually asymptomatic
and well developed
Severe-signs of CHF
Physical Adynamic precordium
Exam Split S2 SEM LUSB radiating to the back
Associated with MATERNAL RUBELLA
INFECTION like PDA
By Meilleurs Amis
AORTIC STENOSIS
3-6% of all CHD;
more common among males narrowed aorta or
aortic valve
valvular, subvalvular, supravalvular
Hemodynamics:
increase resistance to LV outflow increase LV
work LV hypertrophy LV failure
Depend on the severity of the obstruction
Critical AS CHF first weeks of life
Mild/moderate asymptomatic; occasionally
exercise intolerance
Severe exertional chest pain, easy fatigability,
syncope
Dependent on the degree of obstruction to LVOT
Mild AS pulse, heart size, and apical impulse are
all normal
Mild to moderate AS asstd. With an early systolic
ejection click at the apex and LPSB; normal
splitting of S2
Severe AS cardiomegaly, diminished pulses, LV
apical thrust
Lonelybitter
COARCTATION OF THE AORTA
Constriction of the aorta of varying degrees occur at any
point from the transverse arch to the iliac bifurcation
98% occur just below the origin of the left subclavian art. At
the origin of the ductusarteriosus (JUXTADUCTAL COA)
Results in decreased blood flow anterograde through the Ao
valve and due to abnormal extension of contractile ductal
tissue into the aortic wall
INFANTILE TYPE COA asstd. w/ arch hypoplasia
ADULT TYPE isolated juxtaductal COA
M>F
Feature in TURNER SYNDROME and is asstd with bicuspid
aortic valve in 70%
Mild to Modearate Asymptomatic
Severesigns of congestive heart failure and pulm. artierial
hypertension
Classical sign disparity in pulsation and BP in the
arms and legs
Femoral, popliteal, post. Tibial and dorsalispedis pulses are
weak in contrast to bounding pulses of the arms and
carotid vessels
BP in the legs is lower than the arms (NSBP in legs is 10-20
mmHg higher than the arms)
90% systolic hypertension in the UE> 95th % for age
Short SEM LUSB transmitted to the left infrascapular area
 ECG Mild normal
Moderate to severe-RAD,RAE, RVH
Chest X- Normal PVM
ray Prominent main PA
Cardiomegaly (if CHF develops)
Medical MILD PS no intervention
Therapy MODERATE OR SEVERE PS require
relief of obstruction
BALLOON VALVULOPLASTY
treatment of choice
Those with severely thickened PV
surgery
Critical PS urgent PPBV or surgical
valvotomoy
Mild to Moderate- Normal
Severe LVH w/strain pattern
Normal heart size Prominent Asc. Aorta Normal
Aortic knob
BALLOON VALVULOPLASTY procedure of choice
SURGERY for rapidly progressive subAo AS with
gradient of 40-50 mmHg or (+) AR
Discrete subaortic AS resection of the discrete
subaortic AS resection of the discrete memb. w/o
damage to the Ao valve
AORTIC VALVE REPLACEMENT NECESSARY
Choice of procedure depends on the age of the
patient
usually normal in young children
Older patients LVH
Neonates and young infants RVH, BVH
Depend on age and effects of hypertension and collateral
circulation
Cardiomegaly, pulmonary congestion, rib notching
Neonates w/ severe COA PGE1 infusion to open the ductus
and re-establish adequate LE flow; early surgery
Older infants w/ CHF but good perfusion anti-congestive
measures to improve clinical status before surgical
intervention
Older children w/ significant COA treated soon after the
diagnosis
BALLOON ANGIOPLASTY procedure of choice for re-
coarctation