Adult Congenital Heart

Disease

Esti Hindariati
Consultant Cardiologist
Over a million adult survivors
of congenital heart disease

Major advances in cardiac imaging

Surgical techniques
Catheter-based technologies
Medical therapy
Understanding of the natural history of
specific cardiac lesions
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
Left Ventricular Outflow Tract (LVOT)
Bicuspid Aortic Valve (BAV)
Right Ventricular Outflow Tract
(RVOT)
Tetralogy of Fallot
Ecstein Anomaly
Univentricular Heart
Disorders of the Blood Vessels
Coarctation of the Aorta
ATRIAL SEPTAL
DEFECT
Types of ASD based on
anatomic location
1.Type Secundum
Most common, 75%
Women > Men
Mitral Valve Prolapse
2.Type Primum
15%
Downs Syndrome, AV Canal
Defects, Cleft Mitral Valve
3.Type sinus venosus superior
10%
Anomalous Pulmonary
Venous Return
4.Type sinus venosus inferior
(rare)
5.Unroofed coronary sinus
defect (rare)
ASD
 Pathophysiology

The hole in the atrial septum

can cause mild left to right
shunting from the higher
pressure LA into the lower
pressure RA
Dilatation in the right-sided
chambers

Develop symptoms

At the end of the disease process:

Pulmonary Hypertension
Reversal of the shunt flow & cyanosis
Eisenmenger Syndrome
 Clinical Findings & Diagnosis
Prolonged Asymptomatic Course
Symptoms Clinical Findings
Reduced exercise Usually pink
tolerance Cyanosis, suggests
severe PH
Tiredness Right ventricular heave
Exertional dyspnea Ejection systolic murmur
at the left sternal
Palpitations border (relative PS)
Atypical chest pain Wide & fixed split of the
2nd heart sound; a loud
Frequent respiratoty pulmonary component
tract infection reflects severe PH
Signs of right-heart Diastolic murmur at the
lower left sternal border
failure (relative TS)
Diagnostic
Studies
ECG
Chest X-ray
Echocardiography
MRI
Catheterization
Atrial tachyarrhytmias (Atrial Flutter,
Atrial Fibrillation)
Coronary sinus rhytm
Incomplete RBBB
Right axis deviation
Right atrial enlargement
Right ventricular hypertrophy
1st degree AV Block
Enlarged cardiac silhouette (RA &
RV)
A prominent, dilated pulmonary
artery
Dilated hilar vessels
 The Gold Standard
TTE
(most ASDs)

TEE
(sinus venosus ASD & specifically measure the
size of any ASD)
Presence & type of defect
Exact defect size
Distance of defect rims (TEE)
Quality of atrial septal margins
around the defect (TEE)
Right ventricular size
Qp / Qs
Pulmonary atery pressure
Any aother associated congenital
anomalies
Entry of pulmonary veins
Cardiac MRI
Often used when the diagnosis is unclear after
echo studies
Catheterizati
on

Is not required to established the Dx

It is indicated:
When there is a need to determine
pulmonary vascular resistance &
pulmonary vascular reactivity in the
presence of PH
Selective coronary angiography (pts
 Treatment

Surgical Catheter-Based
Closure Closure
(Appropriately-sized ostium
Endocarditis prophylaxis
secundum ASD)is not
ASD Closure Indicated:
Qp : Qs shunt ratio > 1.5 : 1.0
Evidence of dilatation of the right
heart chambers (RV volume overload)
Symptoms attributed to the ASD

Contraindications to closure:
Irreversible pulmonary arterial hypertension
Poor LV function
VENTRICULAR SEPTAL
DEFECT
Types of VSD based on
anatomic location
1.Perimembranous
60-80 %
2.Inlet VSD
8%
3.Outlet VSD
5-7 %
4.Muscular
5-20 %
5.Gerbode defect
Shunt between the
LV & RA
Usually small & rare
VSD
 Pathophysiology

Blood flow preferentially from

the higher-pressure LV into
the lower pressure RV

LV volume overload

Heart Failure

At the end of the disease process:

Pulmonary Hypertension
Reversal of the shunt flow & cyanosis
Eisenmenger Syndrome
 Clinical Findings & Diagnosis
Symptoms Clinical Findings
A minor VSD is Prominent &
usually hyperactive precordium
asymptomatic Systolic thrill at the
lower sternal border
palpitations
Loud, harsh systolic
Dyspnea murmur, in the 3th -4th
Fatigue left parasternal
intercostal space
Recurrent upper
Moderate shunt P2
respiratory will increase
infection LV chamber
enlargement
Diagnostic
Studies
ECG
Chest X-ray
Echocardiography
MRI
Catheterization
The ECG recording in pts with a small VSD
maybe normal
Moderate VSD LA, LV hypertrophy (LV
volume overload)
>Moderate VSD LVH & RVH
Eisenmenger RVH
 In a small VSD = heart size & pulmonary vasculature is
normal
A moderate or large VSD = enlargement of the LA, LV &
pulmonary artery
The central & peripheral pulmonary vascular markings are
increased
Echocardiography
The size, type & location
Qp : Qs
Pulmonary artery
pressure
Size of cardiac chamber
To assess spontaneous
defect closure
Sensitive To assess prolapse of the
technique (95%)
aortic cusp into the
defect, presence &
degree of AR
Presence of a right to
 Catheterizati
on

It is indicated:
In the event of inconclusive results of
noninvasive assessment
To obtain a more specific finding
To assess the severity of RV outflow tract
obstruction
To exactly determine PAP & resistance
To test the response to vasodilating agents in
 Treatment
Small VSD do not cause symptoms & do not
require intervention

Larger VSD usually require closure if:

LV chamber dilatation
Elevated PA vascular resistance
Qp : Qs > 1.5

Surgical Catheter-Based
Closure Closure
Endocarditis prophylaxis is required
PATENT DUCTUS
ARTERIOSUS
 PDA
Embryologic
remnant
Connects the roof
of the pulmonary
artery to the
proximal
descending aorta
5-10 % of all CHD
Closes functionally
during the first 48
Considered abnormal if it remains widely patent beyond
hours
second weeks after birth
of life
PDA
 Pathophysiology

Left to right blood flow across

the PDA

LV chamber volume overload

LA & LV enlargement

Heart Failure

At the end of the disease process:

Pulmonary Hypertension
Reversal of the shunt flow & differential
cyanosis
Eisenmenger Syndrome
 Clinical Findings & Diagnosis
Most PDA lesions are well tolerated
throughout adolescence
Symptoms Clinical Findings
Small PDA = Central cyanosis is
asymptomatic not evident
Dyspnea Clubbing & cyanosis
Palpitations occurs in thelower
Atypical chest pain limb (R to L)
Significant L to R Continuous murmur
or machinery
shunt heart
failure murmur
Eisenmenger no
murmur, prominent
the 2nd heart sound
 Small PDA = normal ECG
LA overload, LV hypertrophy
Prolonged PR interval
P Pulmonal & RVH (Eisenmenger
Syndrome)
Echocardiography
The size, type & location
Qp : Qs
Pulmonary artery
pressure
Size of cardiac chamber
To assess spontaneous
defect closure
Sensitive To assess prolapse of the
technique (95%)
aortic cusp into the
defect, presence &
degree of AR
Presence of a right to
 In a small PDA = Normal CXR
Pulmonary vascular marking
The central pulmonary arteries are prominent
Calcification of the duct between (older adults)
Echocardiography

The mainstay diagnostic

method
The method of choice to
confirm & to characterize
the PDA
 Catheterizati
on

It is limited to certain patients:

Prior to elective closure of the PDA
In the presence of PH and increased PVR to
determine their reversibility
Aortic angiography describes the morphology
of the ductus
Selective coronary angiography
 Treatment

Closure of the ductus is indicated for any patient

with symptoms or in asymptomatic patients with
evidence of left heart enlargement

Surgical Catheter-Based
Ligation Closure
Endocarditis prophylaxis is no longer
recommended
 AHA Guidelines Cardiac Conditions
For Which IE Prophylaxis is
Recommended
Prosthetic cardiac valve
Previous IE
Congenital Heart Disease (CHD)
Unrepaired cyanotic CHD, including palliative shunts &
conduits
Completely repaired congenital heart defect with
prosthetic material or device, wheter placed by surgery or
by catheter intervention, during the first 6 months after
the procedures
Repaired CHD with residual defects at the site of or
adjacent to the site of a prosthetic patch or prosthetic
device (which inhibits endothelialization
Cardiac transplantation recipients who develop cardiac
valvulopathy
Thank
You