Download Evaluation And Treatment Of Common But Non

Evaluation And Treatment Of
Common But Non-Complex
Congenital Heart
Defects/Problems
John E. Pliska, M.D.
Chief of Pediatric Cardiology
McLane Children’s Hospital Scott & White
Texas A&M College of Medicine
Temple, Texas
Discussion Topics
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Atrial Septal Defects
Ventricular Septal Defects
Patent Ductus Arteriosus
Premature Atrial Contractions
(Complexes)
Premature Ventricular Contractions
Normal Cardiac Physiology
Cardiac shunts:
Left-to-right Examples
Patent
Ductus
Arteriosus
Atrial Septal
Defect
Ventricular
Septal
Defect
ASD case
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3 week old male infant with heart
murmur
Echo-Moderate size secundum atrial
septal defect with right atrial
enlargement, normal biventricular fxn
ECG with sinus rhythm and RVH
Feeding well, no resp distress, +wt gain
(BW 3kg, Wt: 3.35kg)
ASD continued
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Discharged home with plans for
outpatient follow-up with pediatric
cardiology
Atrial Septal Defects
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Types
Clinical Presentation
Treatment
Follow-up and Consideration for Closure
(cath vs surgical)
Anatomic types of ASDs
ASDs Basic Echo Diagram
Sinus Venosus ASD
Secundum ASD
Primum ASD
ASD Basic Types
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Secundum atrial septal defect
Primum atrial septal defect
Sinus venosus defect
BUT THAT’S NOT THE
WHOLE PICTURE……..
ASD Types
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Patent foramen ovale
Ostium secundum atrial septal defect
Superior vena caval atrial septal defect
Inferior vena caval atrial septal defect
ASD in the septum intermedium
Coronary sinus atrial septal defect
Ostium primum atrial septal defect
The Atrial Septum
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http://www.med.uc.edu/embryology/ch
apter7/animations/contents.htm
Patent Foramen Ovale
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Between the superior rim of the septum
secundum on the right atrial side and septum
primum on the left atrial side
Normally closes due to overlapping of the
septum secundum and the septum primum
Abnormally high atrial pressures can cause
the two septa to pull apart
Patency 25% at autopsy
Ostium secundum ASD
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The floor of the fossa ovalis is thin ostium
primum, bounded by the limbic septum
Defects in the septum ovale or septum
primum are called “ostium secundum ASD”
and make up 69% of ASDs
Defects may be multiple (fenestrated)
Results from developmental failure of the
septum primum or secondary fenestrations in
the septum primum
Ostium secundum ASD - Echo
Superior vena caval ASD /
“Sinus venosus ASD”
Coronary sinus ASD
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Rare
Appears as large ostium of the coronary sinus
A defect in the “roof” of the coronary sinus
Invariably a persistent left SVC to LA is
present
Noted due to the relatively frequent isolated
finding of persistent left superior vena cava
draining through an enlarged coronary sinus
which does not have an associated ASD or
intracardiac shunt, and is benign clinically
Persistent Left SVC draining
via enlarged coronary sinus
Ostium Primum ASD
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15% of ASDs (30% if includes AV
Septal Defects)
Is a defect in the atrioventricular
septum
Therefore most often has an
endocardial cushion type inlet VSD as
part of the defect
Cleft mitral valve present
Ostium Primum ASD
Partial atrioventricular septal defect
“Atrial Septal Aneurysm”
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Echocardiographic definition is somewhat
arbitrary
Hanley, et al: Dilatation protruding at least 1.5
cm beyond the plane of the atrial septum or if
septal phasic excursion during
cardiorespiratory cycle exceeded 1.5 cm and
if the base of the protrusion was at least 1.5
cm in diameter
Stroke associated with R-L shunting and
thrombus formation in some types of ASA
ASD Hemodynamics
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Primarily a left-to-right shunt lesion
 Therefore enlarged RA and RV
May shunt right-to-left with elevated right
atrial and/or elevated right ventricular
pressure and elevated pulmonary vascular
resistance, especially if tricuspid regurgitation
present
Right-to-left shunt risk increased during
pregnancy
Atrial Septal Defects – When
To Be Concerned
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If associated with other congenital
cardiac anomalies
Generally tolerated well during infancy
and childhood with closure indicated at
2-4 years of life
If patient at risk for stroke: eg, pulmonary
hypertension, thrombi/hypercoagulable state
present, intractable atrial dysrhythmia
Atrial Septal Defect Closure
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Cath lab
interventional
procedure – device
closure
Surgical repair –
primary closure vs
patch closure
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Timing – 2+ to Five
years
Heart size and
hemodynamics
normalize shortly
after closure
Increased risk of
atrial arrhythmia if
late closure
ASD Closure
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Device closure in cath lab, or surgical
CHF and pulmonary hypertension (35% by age 40
yrs) may develop if not closed
Atrial arrhythmias may occur in adults (30% by age
50 yrs)
Cerebrovascular accident (CVA) results from
paradoxical embolization through ASD
Indications for closure: L-R shunt > 1.5:1, ASD >
5mm with RV enlargement
ASD Repair – Cath Lab
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Appropriate size and location of the
Secundum ASD may be closed in cath lab
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Smaller children (< 3 yrs) may not be
candidates because of the size of the
vascular catheters
ASD Repair - Surgical
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Usually 2 to 5 yrs of
age – suture or
pericardial patch
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Performed to avoid
RV dilatation and
scarring, PA HTN?
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Can be done at any
size
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? Need for long term
follow-up
ASD Surgical Closure (patch)
VSD Case
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Near full term infant male delivered by NSVD.
Birth weight 2.9 kilograms. Normal APGAR
scores. DOL 1 a grade 2-3/6 harsh systolic
murmur heard on exam. Stable VS without
cyanosis or feeding difficulty. Murmur still
present 8 hours later and clinically stable.
Echocardiogram: small, mid-muscular
ventricular septal defect. Baby discharged
home with plans for outpatient f/u.
Anterior muscular VSDs
Ventricular Septal Defects
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Types
Clinical presentation scenarios
Treatment
Intervention (surgical vs cath)/timing
Ventricular Septal Defects
Types
Looking from
right ventricle.
Size and
Location of VSD
affect amount of
shunt.
Right Atrium
Pulmonary Valve
Ventricular septal defect
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Produce left-to-right shunt which increases as
the infants pulmonary artery pressure and
pulmonary vascular resistance decrease with
normal maturing of the lungs after birth
Early surgical intervention (1 month) if
significant VSD or significant heart failure
Some moderate to large “muscular”
ventricular septal defects are more
successfully “closed” in the cath lab
VSD Closure
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Surgery
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Cath Lab
Case: Patent Ductus
Arteriosus
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Init eval: 6 day old baby boy delivered at 35 weeks EGA with birth
weight of 2.4 kg. Mild tachypnea and continuous murmur.
Cardiomegaly. Echo: moderate PDA with L-R shunt, LA and LV
enlargement.
Rx with indomethacin:
4 days later: Continuous murmur still present, heart size improved.
Feeds OK. + wt gain. Echo: Small to mod PDA L-R shunt. Normal LA &
LV size with nl biV function.
Laxis given every other day and baby improved and discharged home.
F/U 3 months later: Continuous murmur present, feeding well, pos
weight gain. Echo: Small to mod PDA L-R, no cardiac enlargement.
F/U 9 months of age: Cont murmur, nl growth. Echo: sm to mod PDA
L-R, mild left atrial enlargement, Borderline enlarged LV.
Referral for PDA closure: performed successfully in cath lab 1 year of
age
PDA Chest X-Ray
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Pre-closure
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Post-closure
Patent Ductus Arteriosus
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Isolated vs Associated with other CHD
Ductal Dependent CHD
Clinical Presentation Scenarios
Treatment
Closure of PDA (surgical vs cath)/timing
Ductal Dependent Congenital
Heart Disease; Examples
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Transposition of the Great Arteries
Severe Pulmonary Stenosis or
Pulmonary Atresia (with VSD/without
VSD)
Tricuspid Atresia with restrictive VSD
Critical Aortic Stenosis
“Interrupted Aortic Arch-Coarctation Ao
Patent Ductus Arteriosus
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Ductus arteriosus present at birth,
constricts and “closes” by 3 days after
birth
Oxygen constricts the ductus arteriosus;
PGE1 relaxes the ductus arteriosus
Persistent PDA shunts right-to-left with
pulmonary hypertension and elevated
pulmonary vascular resistance – “Wait for
shunt to change to L-R before considering
closure”
Patent Ductus Arteriosus
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Shunt becomes left to right as lungs
“mature” (decreased PA pressure and
decreased pulmonary vascular
resistance)
With increasing left to right shunt,
cardiomegaly, poor lung function and
congestive heart failure ensue
Patent Ductus Arteriosus
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Left to right
ductal shunt (1)
Increased
pulmonary blood
flow
If significant –
enlarged left
atrium and LV
PDA
Patent ductus arteriosus:
Early Intervention
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Treatment: Oxygen, Indomethacin,
Ibuprofen, Diuretic, Surgical Closure
If fails medical treatment for ductal closure
and has clinical signs and symptoms of CHF
(tachypnea, inability to wean from vent,
cardiomegaly, poor weight gain), or other
heart defects contributing to left to right
shunt, surgery for ductal ligation should be
considered early
PDA
Options for closure in infant
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Medical: O2, Indomethacin, Ibuprofen
Surgical: Ligation (metal clip)
Interventional: If baby several months
old may be considered for catheter
interventional procedure in which a
“feathered” coil or ductal closure device
is placed to occlude the ductus
arteriosus
PDA closure in the
catheterization laboratory
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“Feathered” coil may
be deployed to close
small patent ductus
arteriosus
Amplatzer ductal
occluder used for
larger PDAs
PDA closed with “coil” in the
catheterization laboratory
PACs - Case
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Fetal bradycardia
Delivered; frequent PACs (no SVT)
Resolve with time (days)
Premature Atrial Contractions
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PACs
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Premature Atrial Contractions
Premature Atrial Complexes
How common
Clinical presentation scenarios (Irreg heart
beats-pauses with bradycardia)
Treatment
Concerns (red flag abnormalities on ECG)
Follow-up
Premature Atrial Contractions
(PACs)
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PACs or atrial premature beats (APCs)
Early electrical activation of left or right atrium
May be conducted or non-conducted to ventricles
PACs with “aberrancy” may look like PVCs
Early beats with pause or non-conducted both lead
to irregular heart rate
Common in otherwise structurally and functionally
normal hearts; rarely occur with underlying
cardiomyopathy or atrial tumor
PACs may be caused mechanically by central
venous lines in the atria
Premature Atrial Contractions
- PACs
PACs
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Occur in 14% of infants (all with fewer
than 12 PACs/hour) (up to 21% in
older children)
Infants generally remain asymptomatic
PACs may be noted during fetal
monitoring (may have fetal ventricular
bradycardia if atrial bigeminy present)
PACs benign in most all cases: rare that
PACs are trigger for SVT or Atrial Flutter
PACs – When To Be
Concerned
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If runs of tachycardia (SVT) present
If evidence of cardiac dysfunction
If persisting after interventions to move
central line or correct metabolic
abnormality
Premature Atrial Contractions
What to Do?
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Electrocardiogram ordered if irregular
heart beats noted
If no evidence of runs of tachycardia on
monitoring no treatment needed
Echocardiogram if concern for congenital
heart disease
No additional follow-up needed; most
resolve or diminish significantly in the first
weeks of life
PVCs - Case
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Irregular heart beat in newborn,
Frequent PVCs
Check lytes, Mg++, Ca++
Runs of tachy, bradycardia?
Premature Ventricular
Contractions - PVCs
Incessant V-tachycardia in an
infant
Premature Ventricular
Contractions
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PVCs
How common
Concerns
Treatment
Follow-up
Premature Ventricular
Contractions
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Less common than PACs
Almost always benign
Usually resolve
Usually never need treatment
Frequent PVCs may warrant echo
PVCs – When To Be
Concerned
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If runs of tachycardia present (> 3
beats)
Evidence of ventricular dysfunction
(echo)
If prolonged QTc present (ECG)
Usually improve/resolve if metabolic
abnormalities corrected (Ca++, Mg++)
Bradycardia / Heart Block
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Bradycardia in infants usually related to increased
vagal tone or “blocked” PACs
Premature atrial contractions (PACs) generally benign
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Not benign if associated with SVT
Complete heart block often associated with maternal
lupus erythmatosis or Sjogren’s disease
Adequate heart rate > 60 BPM
Lower heart rates may cause cardiomegaly, cardiac
dysfunction, or congestive heart failure
Early pacemaker placement may be needed