Supervisor

Eka Gunawijaya, MD, Paed

Made Adi Purnami, MD

INTRODUCTION
Anatomy of Cor Triatriatum
Upper
Lower

Rare congenital cardiac anomaly in which a

fibromuscular membrane divides the atrium
in two
Most the left atrium is divided into an
upper chamber that receives the pulmonary
veins and a lower chamber that is related to
the left atrial appendage and the mitral valve
orifice.

INTRODUCTION
Epidemiology of Cor Triatriatum

Cor triatriatum.....

Cor triatriatum.....

Approximately 75%
Approximately
75%
of patients die
in
of infancy
patients(generally
die in
infancy
(generally
from pulmonary
from
pulmonary if the
hypertension)
hypertension)
if the
defect is unrepaired
defect is unrepaired

Most patients
Most
patients
presents
during
presents
infancyduring
evidence
infancy
evidence
of low
cardiac
of output,
low cardiac
imcluding
output,
pallor,imcluding
diminished
pallor,
diminished
peripheral
pulses,
peripheral
pulses,
and tachypnea
and tachypnea

Cor triatriatum is
Cor
triatriatuma is
essentially
form
essentially
a form
of left atrial
inflow
of obstruction
left atrial inflow
and
obstruction
and signs
presents with
presents
with signs
an symptom
of
anpulmonary
symptom of
venous
pulmonary
venous
obstruction
obstruction

Cor triatriatum.....

Cor triatriatum.....

The purpose of this paper

to keep us more alert to this disease, to

know the clinical symptom, diagnosis,
treatment, and prognosis about

Cor Triatriatum

The Case
JN, a one month old boy was referred to Sanglah
Hospital on November 23th 2010 due to shortness of
breath.
shortness of breath since 2 week prior to admission and
worsening since I week prior to admission

Blue in his lips when he was crying since 2 week

prior admission
Feeding difficulties since the shortness of breath
occur, and as a result he had weight lost in two weeks
There was no cough, fever and common cold within two weeks

The Case

Hewas
was
He
hospitalizedinin
hospitalized
Tabananhospital
hospital
Tabanan
oneweek
weekwith
with
forforone
diagnosis
diagnosis
aspiration
aspiration
pneumonia.
pneumonia.
treatedwith
with
treated
cefotaxime.
cefotaxime.

Bornfull
fullterm,
term,
Born
sectionsecarean
secarean
section
andnot
notcry
cryafter
after
and
delivered.Birth
Birth
delivered.
weight3200
3200
weight
grams.No
Novisible
visible
grams.
abnormalities
abnormalities
werefound.
found.
were

Hehad
hadbreast
breast
He
feedingafter
after
feeding
birth.
birth.
Theimmunization
immunization
The
historywas
was
history
complete
complete
accordingtotohis
his
according
age.
age.

PHYSICAL
EXAMINATION

Pulse rate

190 per minute regular,

RR

: 78x/minute

Axillary temp : 36.2C.

Weight

3.7 kg

SpO2

64% ( 90% with oxygen 10 lpm with hood)

Nutritional status : mild malnourished

PHYSICAL
EXAMINATION

There were no pale conjungtivas nor icteric sclera. Her lip

looked cyanosis
The ENT no abnormalities on ear. There was nasal flare seen.

No palpable lymph nodes nor nuchal rigidity

Shape of the thorax wall was normal. Chest was symetrical.
There were subcostal, intercostals and suprasternal chest
indrawing. The breath sounds were broncovesicular, with
rales in lung base without wheezing.

CHEST
EXAMINATION

Example text

no

Go ahead and
replace it with
Inspection
:
your own text.
precordial
bulging,
This is an
example text.

Example
text
Palpation

:
Ictus cordis
was
Go ahead
andpalpable but
replace it withon the 3thICS
not accentuated
your own text.
left MCL,
RV heave (+), thrill
This is an
(-), LVtext.
lifting (+)
example
Example text

Go ahead and
replace it with
Auscultation : your own text.
This is an with
pulmonary second sounds
example text.
th- th

accentuation of the
gallop and
diastolic murmur on the 2 3 intercostals space in left
parasternal line grade III/6

PHYSICAL
EXAMINATION

Working Diagnosis
Syok et causa suspected cardiogenic dd/ septic
CHF with functionally Ross IV +
Non Cyanotic Congenital Hearth disease
suspected Ventricle Septal Defect (VSD)
dd/Patent Ductus Arteriosus (PDA)
Impending respiratory failure.

chest X-ray
Cardiomegaly with CTR 72%,

Right ventricle
and prominent
pulmonal
segment

Enlarged
from right
atrium

increased of pulmonary vascularization

ECG and
Echocardiography
RAD, LVH, RVH, RAH and
LAH, no prolongation of P-R
interval

Severe MS, Mild AR, slight

decrease LV systolic function
(EF 60%)

The Laboratories
Complete Blood
Count

Electrolyte

throat swab coccus gram positive.

Culture of the throat swab r No growth

Others

Based on clinical manifestations, laboratories

finding, radiology finding

Rheumatic heart diasease with chronic valve

lesion, fungtional heart failure NYHA class III
Malnourished

During 10 days hospital care.

Malnourished Undernourished
Underwent mitral valve replacement
using mechanical mitral valve
Warfarin for lifelong
Counseling.

The prevalence of RHD

patient was first

diagnosed with RHD at
11 years old

2002-2003 WHO criteria for the diagnosis of RHD

Diagnostic categories
Primary episode of RF

In

Criteria
Two major or one major and two minor
manifestations plus evidence of preceding group A
this
case
streptococcal infection

Recurrent attack of RF in a patient Two major or one major and two minor
without established rheumatic heart manifestations plus evidence of preceding group A
patient presenting for the first streptococcal
time with severe
mitral stenosis with
disease
infection

mild aorta regurgitation.

Recurrent attack of RF in a patient with Two minor manifestation plus evidence of preceding
established rheumatic heart disease
group A streptococcal infection
Rheumatic chorea
Insidicus onset rheumatic carditis
Chronic valve lesions of RHD (patients
presenting for the first time with pure
mitral stenosis or mixed mitral valve
disease and/or aortic valve disease)

Other major manifestations or evidence of group A

streptococcal infection not required
Do not required any other criteria to be diagnosed as
having rheumatic heart disease

RHEUMATIC HEART DISEASE

Valve Involvement
In this case
we found severe mitral stenosis, mild aorta insufficiency,
and mild tricuspid regurgitation

mitral valve

Combination
mitral and
aortic valves

Aortic
valvar

Combined
mitral, aortic, and
tricuspid

Mitral Stenosis
Mild :
1.5-2.5 cm2

Severe :
< 1.0 cm22

Normal
:
4-6 cm2

Moderate:
1.0-1.5 cm2

Cas
Opening
e mitral valve 0.5 cm2 (severe stenosis) with annulus
valve was 38 mm and slight decrease left ventrical systolic
function with ejection fraction 60%.

Physical Examination
Aorta
Regurgitation
Diastolic murmur

There is accentuation of P2
when pulmonary
Case
hypertension is present.

Single first sound,

presystolic murmur at
apex area (ICS IV
MCL sinistra), grade
4/6, with
rumbling
mitral
soundstenosis
during diastolic
accentuated
sound,
phase,first
andheart
radiating
an opening
a midalong snap,
axillaand
line.
diastolic rumble, presystolic
murmur

Electrocardiography
Mitral stenosis
LAE. Broad, notched P waves
are seen in the limb leads, or
biphasic P wave in leads V1 dan
V2.
RAE, RAD,and RVH the
pulmonary hypertension

Case
RAD (900-1200), notched P waves are seen in the limb leads, biphasic
P wave in leads V2, right and left ventrikel hypertrophy with no
prolongation of PR interval.

The New York Heart Association (NYHA) Functional Classification

Class

Patient Symptoms

Class I (Mild)

No limitation of physical activity. Ordinary physical activity does

not cause undue fatigue, palpitation, or dyspnea (shortness of
In this case
breath).

Class II (Mild)

Slight limitation of physical activity. Comfortable at rest, but

ordinary physical activity results in fatigue, palpitation, or
dyspnea.

Class III
(Moderate)

Marked limitation of physical activity. Comfortable at rest, but

less than ordinary activity causes fatigue, palpitation, or
dyspnea.

Class IV
(Severe)

Functional
NYHA
III
Unable to carryCHF
out any physical
activityclass
without discomfort.

Symptoms of cardiac insufficiency at rest. If any physical activity

is undertaken, discomfort is increased.

....Discussion
Treatment

1
2
3

Bed rest

Heart failure therapy

Adequate prevention

Kas
us received anti heart failure therapy, adequate prevention of
the patient

reccurence, BPG 1.2 million IU, and was planned to undergo a mitral valve
replacement procedure.

Indication Invasive therapy

Score
Leaflet mobility

Leaflet thickening

Leaflet calcification

1
2

Highly mobile valve with only leaflet tips restricted

Leflet mid and base portions have normal mobility

3
4

Valve continues to move forward in diastole, mainly from the base

No or minimal forward movement of the leaflets in diastole

1
2

Leaflets near normal in thickness (4-5 mm)

Mid-leaflets normal, considerable thickening of margins (5-8 mm)

Thickening extending through the entire leaflet (5-8 mm)

Considerable thickening of all leaflet tissue (>8-10 mm)

1
2
3

A single area of increased echo brightness

Scattered areas of brightness confined to leaflet margins

4
Subvalvular thickening

Case

Brightness extending into the mid-portion of the leaflets

Extensive brightness throughout much of the leaflet tissue

1
2

Minimal thickening just below the mitral leaflets

3
4

Thickening extending to the distal third of the chords

Thickening of chordal structures extending uo to one third of the chordal length

Extensive thickening and shortening of all chordal structures extending down to the papillary
muscles

The Wilkin score is 14, so we proceed with valve replacement procedure.

Valve Selection

In this case,
the patient wish to minimize the risk of the re-operation and will take or has
existing indication or anticoagulation. There for, we choose to use mechanical
valve

Outcome
Mechanical
valve
replacement

Valve related morbidity and

complications of lifelong
anticoagulant are foremost

Little is known about the long-term outcome of mechanical valve

replacement

Search
Result:
Journal
The complications of anticoagulation
with mechanical valves do influence the
outcome. But, proper anticoagulation and follow-up, the complication can be
Prosthetic Valve Replacement in Adolescents with Rheumatic
diminished.
Heart Disease

Anticoagulation
Warfarin:
adequate anticoagulant but high risk
to embryopathy

Female

Pregnancy

Heparin:
less effective anticoagulant more maternal
complications, more protective of the fetus
In this case, female adolescent warfarin 2 mg/kg/day continuously for lifelong
So, we recommend for her parents with careful counseling prior to or shortly after the
diagnosis of pregnancy and discussion of the risks associated with available anticoagulant
options.

The choice of anticoagulant regimens

for mechanical heart valve
thromboprophylaxis when pregnancy is
very difficult.
We recommend for careful counseling prior to
or shortly after the diagnosis of pregnancy and
discussion of the risks associated with
available anticoagulant options.

PICO
Problem : How is the prognosis of child with cor triatriatum
whom underwent repair?
P Population/problem : Children whom underwent repair of
cor triatriatum
I Intervention :
C Comparison : O Outcome
: Outcome
CLINICAL QUESTION
:
How is the outcome of children with cor triatriatum whom
underwent repair?
Journal
Outcome After Reapair of Cor Triatriatum
SEARCH STRATEGY
Selcen
Yaroglu,
Sitaram Emani,
McElhinney
Keywords:
children,
cor triatriatum,
surgeryDof
andBoutcome
Am J Cardiol 20011;xx:xxx

JOURNAL SUMMARY

CRITICAL APPRAISAL ON PROGNOSIS

I. IS THIS EVIDENCE ABOUT PROGNOSIS VALID?
1.

Was a defined, representative sample of patients Yes

assembled at a common point in the course of their
disease?

2.

Was follow-up of study patients sufficiently long and Yes, Follow-up was
complete?
complete
Since before to after repair

3.

Were objective outcome criteria applied in a blind

fashion?
If subgroups with different prognosis are identified:
Was there adjustment for important prognostic
factors?
Was there validation in an independent group of
test-set patients?

4.

Valid

Unclear

Yes.
Age, gender, and anomalies
associated cor triatriatum
was adjusted.
Unclear

CRITICAL APPRAISAL ON PROGNOSIS

II. IS THIS VALID EVIDENCE ABOUT PROGNOSIS IMPORTANT?
1. How likely are the outcomes In patient with preoperative and
over time?
early postoperative
echocardiography studies with
Doppler evaluation, there was
significant reduction in the mean
gradient across the membrane,
from 11.5 mmHg to 0 mmmHg
2. How precise are the prognostic p <0.001
estimates?

Important

CRITICAL APPRAISAL ON PROGNOSIS

III. CAN WE APPLY THIS VALID, IMPORTANT EVIDENCE
ABOUT PROGNOSIS TO OUR PATIENT?
1. Is our patient so different from those in the No
study that its results cannot apply?
2. Will this evidence make a clinically Yes,
important impact on our conclusions about In this large surgical series
what to offer or tell our patient?
of patient who underwent
repair of cor triatriatum,
there were no case of
significant residual or
recurrent cor triatriatum

Applicable

Indication Invasive therapy

Mitral stenosis

Types of Prosthetic Valves

1. Bioprosthetic valve
2. Mechanical prosthetic valve.
Advantages

Disadvantage

Mechanical

Good durability

Bulky and space

occupying.
Life long anticoagulation
needed
Complications:
thromboemboli

Bioprosthethic

anticoagulants not Not durable: just 10

so much
years or less
necessary
thromboembolism
less.

Mechanical Valve

Porcine Valve

Carapetis. Lancet
2005;366:155

Rheumatic feverpathogenesis
Rheumatic fever is a
classic example of
molecular mimicry

Modified Centor Score & Management Approach

( McIsaac - JAMA 2007)
Criteria

Points

*Temperature >38 C
*Absence of Cough
*Swollen Tender Cervical Node
*Tonsillar Swelling / Exudate
*Age: 3 - 14 years

1
1
1
1
1

15 44 years
45 years or older
Total Score :

0
-1
(

Management Approach:
SCORE:

0-1
2-3
>4

No Further Testing or ABX Therapy.

Culture All
Treat Empirically .