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Congenital Heart Disease: Case
Studies for Congenital Interventional
Procedures
Edgard A Bendaly, MD, FAAP
Congenital Interventional Cardiology
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
Glimmers of Knowledge: Ancient
Times
• 20,000 YRS AGO: Hunters
in Southern Spain
recognized importance of
heart, in thorax.
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(Reproduction of cave fresco in Benchmark
Papers in Human Physiology: Congenital
Heart Disease, edited by W. J. Rashkind)
Glimmers of Knowledge: Ancient
Civilizations
• 3000 BC, Egypt. First illustration/ description of blue
skinned individuals
• 2600 BC, China. Yellow Emperor’s Book of Medicine:
“...whenever one feels the pulse, one feels the heart. This
means the heart speaks through the vessels of the limbs”
• 460-370 BC, Greece. Hippocrates: Direct auscultation
(Hippocratic succession)
• 400-320 BC, Greece. Aristotle: noted the embryonic beating
chick heart: the heart is center and origin of life. Greeks
thought blood had inherent powers of movement.
Renaissance - 1935: The
Anatomists
Rene Laennec
• Stethoscope innovation: 1819
• Identified each cardiac cycle
had 2 heart sounds
• Emphasized inspection,
palpation, percussion,
auscultation
Willem Einthoven (1860-1927)
• String galvanometer for
recording ECG: 1903
Maude Abbott,1936: Atlas of Congenital
Heart Disease
• Comprehensive collation and
analysis of > 400 cases of CHD
• Anatomy, physical signs, and
clinical-path-physiology
correlation
• Enabled those who came later
to devise treatment
• International scope of study of
CHD
• Coined “Eisenmenger’s
complex”, used “TOF”, noted
Downs/CHD correlation
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
History
• The word catheter is derived from the Greek
word katheter, meaning to send down.
• While commonly thought to have its origin in
urology, the first catheter was associated with the
use of clysters (enemas).
• Used as both purgatives to restore the balance of
the four humors and as a means of administering
nutrients, clysters date from ancient Egyptian
times.
Old “Catheters”
• The enema bag, usually
an animal bladder, was
affixed to quill or metal
tube, and substances
were injected through
the rectum by
squeezing the tube.
Sones’ Cardiac Catheterization
Cardiac Catheterization Laboratory
New Catheters
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
PDA Closure
PDA: Prevalence and Physiology
• Represent 5-10% of congenital cardiac disease in
full term babies
• Female:male ratio is 2:1
• PDA causes excess blood flow to the lungs and
can cause symptoms like fatigue, difficult
breathing, failure to thrive or chronic respiratory
infections. Large defects can lead to heart failure
and death.
Presentation
• Small: Asymptomatic
• Large: lower respiratory tract infection, CHF,
exertional dyspnea, failure to thrive
Physical Examination
• Tachycardia and tachypnea if CHF
• Bounding peripheral pulses with wide pulse
pressure with moderate or larger defect.
• With moderate to large defect, the
precordium is hyperactive. Systolic thrill may
be present at the upper left sternal border. A
continuous (“ machinery”) murmur at the left
infraclavicular area.
EKG
• Small-moderate: normal or LVH
• Large: BVH
CXR
• Small: normal
• Moderate-large: cardiomegaly
PDA Types Based on Shape
Krichenko Type A
Krichenko Type B
Krichenko Type D
Krichenko Type C
Krichenko Type E
Indications for Closure
• Children
– Endocarditis/endarteritis risk
– Left ventricular volume overload
• Infants
– Congestive heart failure
– Failure to thrive
Historical Methods
Primary Device Options
Coils
Amplatzer Ductal
Occluder
Procedure Overview
PDA Closure
PDA Closure
PDA Closure
Results
• Acute closure rates with the ADO are 70%
• Complete occlusion in 90-95% within 24 hours
• More than 97% by 1 year
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
Atrial Septal Defect
ASD: Prevalence and Physiology
• Represent 6-10% of all cardiac anomalies
• Female:male ratio 2:1
• Occur in 1 child per 1,500 live births
• Imposes a volume
overload on the right heart
and chronic lung disease
Presentation
• Usually asymptomatic
• Asthma that is difficult to control
• Very subtle findings as mild shortness of
breath at full exertion in an athletic patient
• Failure to thrive
Physical Examination
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A relatively slender body build
Widely split and fixed S2
Grade 2-3/6 SEM
Mid diastolic rumble in large shunts
Natural History of ASD
• Symptoms early in life are rare.
• CHF rare < 40yo
• Atrial arrhythmias increase in frequency in older
patients
– 13% in > 40 yo
– 52% in > 60 yo
• PHTN in older untreated patients
– Usually > 20yo
– Predominantly in females
– Up to 1/3 of patients in 3rd decade of life
Transcatheter Treatment of ASD
• Transcatheter occlusion first described in 1976
using a double umbrella device
• Currently the Amplatzer Septal Occluder and
Helex Septal Occluder are the 2 devices with
FDA approval for ASDs in the United States.
• Elective repair recommended between 3-5
years of age
• 98.5% closure rate
ASD Device
ASD
ASD Evaluation
ASD Evaluation
ASD Closure
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
Balloon Atrial Septostomy
• Primarily used to palliate transposition of the
great arteries
• Newborns can survive several months after
this procedure
• Currently performed at the bedside under
echocardiography
Balloon Atrial Septostomy
Balloon Atrial Septostomy
Balloon Atrial Septostomy
Balloon Atrial Septostomy
Balloon Atrial Septostomy
Aortic Stenosis
Natural History of AS
Aortic Valvuloplasty
Results
• Palliation comparable to the palliation
achieved by a surgical aortic valvotomy
Pulmonary Valve Stenosis
Indications
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Gradient > 40 mmHg
Right ventricular hypertrophy
Right ventricle failure
Symptoms
Pulmonary Valvuloplasty
Pulmonary Valvuloplasty
Pulmonary Valvuloplasty
Pulmonary Valvuloplasty
Pulmonary Valvuloplasty
Results
• Valvuloplasty is the gold standard for
treatment and first line management
• In non dysplastic valve it is usually curative
• In dysplastic valve it is usually palliative until
valve is replaced
Coarctation of the Aorta
Indications for angioplasty/stent
• Gradient > 20 mmHg
• Narrowing > 50%
• Signs and symptoms
– Hypertension
– Exercise induced hypertension
– Congestive heart failure
– Severe acidosis
Stents
Coarctation Stenting
Coarctation Stenting
Coarctation Stenting
Coarctation Stenting
Results
• Depends on the underlying physiology
• Depends on the age the procedure is
performed
• Stents maintain long term vessel patency
Outline
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History of cardiology
History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary
Summary
• Cardiac cath in patients with CHD is a safe and
standard procedure when done by a
congenital interventional cardiologist
• Hospital stay is between 24-36 hours
• Interventional procedures allow either
palliation or treatment of cardiac disease
replacing or delaying open heart surgery
Summary
• PDA cause volume and pressure and overload
• Presentation and physical findings can be
subtle
• Closure in the cath lab is safe, effective and
considered the gold standard
Summary
• ASD cause volume overload on the heart
• Presentation can be subtle
• If untreated, late complications include a
shorter lifespan
• Closure in the cath lab is safe and effective
Summary
• Interventional procedures are increasing in
number and complexity
• New procedures and techniques are
constantly developed