Download ASD, Ostium secundum

Congenital Heart Diseases:
Atrial Septal Defect
Patient History
This is a case of G.L., a 21 year old
female, complaining of easy fatigability
and occasional chest pain who was
referred for cardiovascular evaluation prior
to employment. She was diagnosed to
have “heart disease” in childhood. Past
medical history revealed frequent upper
respiratory tract infections.
Physical Examination
• General: Hypoesthenic,
narrow A-P diameter
• Vital Signs: BP: 100/80 mmHg
PR: 75 bpm
RR: 20 cpm
BMI: 15 kg/m2
Physical Examination
• Thorax and Lungs:
symmetrical chest expansion,
resonant on all lung all fields,
(-) crackles
Physical Examination
• Cardiovascular Examination:
JVP and CAP – normal
at base: S1 is normal followed by a
grade 3/6 crescendo
decrescendo
murmur
S2 wide with fixed splitting
at apex: multiple clicks
Expiration
Inspiration
JVP
CAP
3/6
3/6
A2 P2
C C C
A2 P2
C C C
12- Lead ECG
•
•
•
•
Normal sinus rhythm
Right ventricular hypertrophy
Incomplete right bundle branch block
Diffuse ST-T changes
Chest Radiograph
• Cardiomegaly with multi-chamber
enlargement
• Pulmonary congestion
2D- Echocardiography
• ASD, ostium secundum type
• Markedly dilated right bentricle with adequate
wall motion and contractility with evidence of RV
pressure and volume overload
• Dilated right atrium without thrombus
• Dilated main pulmonary artery
• TR, severe
• PR
• Moderate pulmonary hypertension
• Reverse E/A across mitral valve
Salient Features
•
•
•
•
•
21 y/o, female
Easy fatigability and occasional chest pain
Childhood “heart disease”
Frequent URTI
Hypoesthenic with BMI of 15 kg/m2
PE
CXR
ECG
2D Echo
ASD, ostium
secundum
type
Left lower
Cardiomegal Right
Dilated right
parasternal y with multi- ventricular
ventricle with
lift
chamber
hypertrophy RV pressure
enlargement
and volume
overload
Severe TR
Systolic
Dilated main
grade 3/6
pulmonary
crescendoartery
decrescendo
Pulmonary
murmur at
PE
CXR
Fixed and
widely split
S2
Pulmonary
congestion
Multiple
clicks at
apex
Cardiomegal
y with multichamber
enlargement
ECG
2D Echo
Moderate
pulmonary
hypertension
Reverse E/A
across mitral
valve
Right atrium
and ventricle
dilated
Inspiration
Expiration
3/6
3/6
JVP 3 cm at 300
CAP
S1
C1 S2
S1
C1 S2
Diagnosis
• Cardiac Diagnosis:
Etiologic: Congenital Heart Disease
Anatomic:ASD, TR, PR
Dilated RA & RV,
Dilated Main Pulmonary Artery
Physiologic: not in failure
Functional: Class II-B
• Pulmonary Hypertension
DIFFERENTIAL
DIAGNOSIS
Hx
Patient
ASD
VSD
PDA
RHD
“heart
disease” in
childhood
Prolonged
symptomfree period
most children
with small
defects remain
asymptoma-tic
premature
birth,
perinatal
distress, or
perinatal
hypoxia
may be
present
(+) upper
respiratory tract
infection
Mitral stenosis
frequent
URTI
easy
fatigability
occasional
chest pain
Palpitations
Fatigue
Dyspnea on
exertion
Orthopnea
Frequent
respiratory
infections
Symptoms
of right
ventricular
failure
In patients
with
Eisenmenger
syndrome,
symptoms in
adult life
consist of
exertional
dyspnea,
syncope and
hemoptysis
R-L shunt
leads to
cyanosis,
clubbing, and
erythorocytosis
-dyspnea and
cough on
exertion
-orthopnea and
PND
-hemoptysis
Mitral
regurgitation
-dyspnea and
cough on
exertion
-orthopnea and
PND
-ankle edema
P.E.
Patient
ASD
Hyposthenic,
narrow AP chest
diameter
Prominent RV
impulse
Normal JVP and
CAP
Left lower
sternal lift
Normal S1 ff. by
gr. 3/6
crescendodecrescendo
murmur
S2 wide with
fixed splitting
Multiple clicks at
apex
VSD
loud, harsh,
or blowing
holosystolic
S1 normal or
murmur is
split, with
heard best
accentuation of
over the
TV closure sound lower LSB in
the 3rd or
Wide & fixed
4th ICS
splitting of the S2
Systolic ejection
murmur (heard in
pulmonic area)
Diastolic rumble
across the
tricuspid valve
Neck vein
distention
Ascites
Edema
PDA
RHD
precordial
activity is
increased
Mitral stenosis
diastolic thrill at
the apex
S1 and P2 are
accentuated
S2 is split or
fixed
OS of the mitral
valve on
expiration
(+) carvallo's
sign
apical
impulse is
laterally
displaced
S1 normal,
displaced
S2 typically
cardiac apex obscured by
with a similar murmur
holosystolic
murmur
Continuous
machineryapical
like murmur
diastolic
rumble and
Bounding
third heart
peripheral
sound (S3)
pulses
Mitral
regurgitation
(+) systolic thrill
at the apex
holosystolic
murmur
-(+) S4
ECG
Patient
ASD
VSD
PDA
RHD
Normal sinus
rhythm
Ostium
secundum
defects –
incomplete right
bundle block &
right axis
deviation
May be
normal
LVH with a
larger PDA
LVH
RVH
Incomplete
RBBB
Diffuse ST-T
changes
Ostium primum
defects – left
anterior
hemiblock &
left axis
deviation
With larger
defects
there are
various
degrees of
right axis
deviation
associated
with right
ventricular
Enlargement
CXR
Patient
ASD
VSD
PDA
RHD
Cardiomegaly
with multichamber
enlargement and
pulmonary
congestion
Shunt vascularity
(inc. pulm.
vascular
markings)
Right
Ventricular
enlargement
LAE
Increased
pulmonary
vascular
markings
PAE
Mitral stenosis
-(+) kerley B
lines
-concentric
hypertrophy of
left atrium
-cephalization
-prominent main
pulmonary artery
and branches
-constriction of
arteries in the
middle and
peripheral lung
zones
Right ventricular
enlargement
Enlargement of
the pulmonary
artery segment in
P-A view
LVE
PVE
Mitral
regurgitation
-eccentric
hypertrophy of
the left atrium
-LA and LV
enlargement
-equalization,
cephalization
-mitral annulus
calcification
Echo
Patient
ASD
VSD
PDA
RHD
ASD, ostium
secundum type
Enlargement of RV
color
flow can
show the
shunting of
blood from
the left
ventricle to
the right
LAE
Mitral stenosis
-mitral orifice
<4cm
-concentric
hypertrophy of
left atrium
-annular
calcifications
Markedly dilated
right ventricle with
adequate wall
motion and
contractility with
evidence of RV
pressure and volume
overload
Dilated RA w/o
thrombus
Dilated MPA
Severe TR
PR
Mod. Pul. HPN
Reverse E/A across
mitral valve
Negative-contrast
image at the site of
defect (saline
injection)
Doppler – abnormal
pressure of left-toright blood flow
across the septum
VSD's can
result in a
shunt from
below the
tricuspid
valve to
below the
pulmonary
valve.
Continuous
flow from the
aorta into the
main
pulmonary
artery
Mitral
regurgitation
-eccentric
hypertrophy of
the left atrium
-LA
enlargement
-hyperdynamic
LV
-annular
calcifications/
LV dyskinesis
-ruptured
chordae
tendineae
Radiologic Findings
• L to R Shunt
ASD, Ostium
secundum
Cardiomegaly
Accentuated
pulmonary
vascular
markings
ASD
Accentuated
pulmonary
vascular
markings
MPA convex
L to R Shunting
VSD
Accentuated
pulmonary
vascular
markings
Convex MPA
R anterior
oblique
Displacement
of the
esophagus
RA, RV and LV
enlargement
Pulmonary Arterial Hypertension
•
Causes
– Primary/ Idiopathic
•
Genetic
– Secondary
•
•
1.
2.
3.
Cardiac
Pulmonary
hypoxic vasoconstriction
decreased area of the pulmonary vascular bed
volume/pressure overload
Secondary Pulmonary Arterial
Hypertension
• Hypoxic Vasoconstriction
– COPD and obstructive sleep apnea
– Due to down regulation of endothelial nitric
oxide synthetase
• Decreased Area of Pulmonary Bed
– Occurs when loss of vessels exceed 60% of
the total pulmonary vasculature
– Occurs in patients with collagen vascular
disease like CREST and scleroderma. And
those with chronic emboli
• Volume/ Pressure Overload
– Seen in patients with left to right intracardiac
shunts
– May passively occur in patients with left atrial
hypertension and left ventricular dysfunction,
mitral valve disease and hose with aortic
stenosis
Pulmonary Arterial Hypertension
• Common Features
– Medial hypertrophy
– Eccentric and concentric intimal fibrosis
– Recanalized thrombi
– Plexiform lesions
There is medial hypertrophy and marked intimal hyperplasia of this muscular pulmonary artery. These
changes are the morphologic response to increased pulmonary vascular pressures, such as may be
seen in patients with chronic obstructive lung disease, pulmonary thromboembolism, or left-sided heart
disease (e.g., mitral stenosis). Occasionally, elevated pulmonary pressures are seen without
identifiable cause (idiopathic or primary pulmonary hypertension).
A. PA chest film demonstrates mild cardiomegaly with normal pulmonary
arterial markings. There is pulmonary venous congestion, fluid within the
horizontal fissure and prominent Kerley B lines (indicative of lymphatic
engorgement).
Lateral chest film show marked venous congestion with fluid visible in
both the horizontal and oblique fissures.
Pulmonary Arterial Hypertension
• Chest radiograph
– The classic finding on a chest radiograph from
a patient with PAH is enlargement of central
pulmonary arteries, attenuation of peripheral
vessels, and oligemic lung fields.
– Findings of right ventricular and right atrial
dilatation are possible.
A posterior-anterior (PA) chest radiograph demonstrates enlargement of the main pulmonary artery
and right pulmonary artery .The peripheral pulmonary arteries are reduced in caliber. The lung
volumes are normal without evidence of underlying pulmonary parenchymal disease.
A lateral radiograph also demonstrates enlargement of both the right and left pulmonary arteries.
Cardiomegaly, with predominant right ventricular enlargement is present.
Pulmonary Venous Hypertension
• Occurs secondary to increased resistance
to pulmonary venous drainage
• Associated with diastolic dysfunction of the
left ventricle and valvular dysfunctions
(aortic and mitral valve stenosis and
regurgitation)
Pulmonary Venous Hypertension
• Features
– Capillary congestion
– Focal alveolar edema
– Dilatation of interstitial lymphatics
The alveolar septa appear thickened, and the alveolar spaces contain numerous
pigment-laden macrophages. A few alveolar spaces contain pink edema fluid. Note
the thick-walled pulmonary vessel.
Acute Pulmonary Congestion and Edema
Management
• Medical
• Watch out for dev’t of complications
» Arrhythmias, CHF, endocarditis
• Operative repair: definitive
– Patch of pericardium or of prosthetic material
– Percutaneous transcatheter device closure
– Indications:
• symptomatic
• significant L to R shunting
– Qp:Qs ≥ 1.5:1
– RA or RV enlargement – radiographic, cardiac catheterization
• think otherwise if:
– PVR > 12 U/mL / significant pulmonary hypertension
– Eisenmenger – R to L shunting with cyanosis
Management
• Results
• Surgical repair w/ mortality rates near zero
• Complications: atrial arrhythmias, left atrial
hypertension
• Transcather closure
– Air embolism (1-3%), thromboembolism from device (12%), systemic, pulmonary venous obstruction (1%),
perforation of atrium/aorta (1-2%), atrial arrhythmias (13%)
Schwartz’s Principles of Surgery, 8th Ed.