Critical Case in Congenital Heart Disease, What's The Sign and Initial Treatment For Them (Prof. Dr. Dr. Mulyadi M.djer, SpA (K) )

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CURICULUM VITAE

Name : Prof. Mulyadi M. Djer, MD, SpA(K), PhD


Place / Date of Birth : Padang, 29 October 1964
Adress : Jl. Taman Sari VIII/23, Jatinegara Baru, Buaran,
Jakarta Timur 13940. Phone 021 48636322
Current Position : Lecturer and Medical Staff, Department of Child
Health FKUI-RSCM Jakarta
Organization : Head of Cardiology Coordinating Working Group
(UKK Kardiologi) Indonesian Pediatric society (IDAI)
Educational Qualifications:
Year: 1989Degree: Medical Doctor (MD) Institution: FKUI
1997 Pediatric Specialist (SpA) FKUI
2003 Pediatric Cardiologist FKUI
2005 Consultant Pediatric Cardiologist [(SpA(K)] IDAI
2008 Doctor of Phylosophy (PhD) FKUI
2016 Professor FKUI
Awards, Fellowship, Grants:
2001-2002 Fellowship training in Pediatric Cardiology at Institut Jantung Negara
(National Heart Institute), Kuala Lumpur, Malaysia
2004 Live course in Pediatric Cardiac Intervention, Beijing, China
2004 & 2006 Live course in Pediatric Catheter Intervention , Kuala Lumpur, Malaysia
2004 Short course in Pediatric Cardiac Intensive Care, Miami, USA
2005 & 2007 International Workshop on Interventional Pediatric Cardiology, Millan, Italy
2005 Live course in Pediatric Interventional Cardiology and Emerging New
Technique in Cardiac Surgery, Buenos Aires, Argentina
2006 Live course in Pediatric Interventional Cardiology and Adult Congenital
Heart Disease, Las Vegas, USA
2009 Live course in Pediatric and Adult Interventional Cardiac Symposium,
Cairns, Australia
CRITICAL CASE IN CONGENITAL HEART
DISEASE
Whats the Sign and Initial Treatment for
Them?

Prof. Mulyadi M. Djer, MD, SpA(K), PhD

Department of Child Health


Medical School University of Indonesia
Structures of the heart

120/80

25/15

5
120/8
25/5
performance

Preload
Afterload

Contractili

ty

Congenital Heart Disease


Incidence of Congenital
Heart Disease

The incidence: 8-10 in 1000 live birth11


Indonesia:

Total population : 254,900,0002


2

Birth rate: 2.3 %


Incidence CHD per year: 50,000


cases

1. Wren. Pediatric cardiovascular medicine. 2nd ed.


Oxford:
Blackwell Publ.; 2012. p. 268-75.
2. Badan Pusat Statistik, Susenas 2014 dan 2015
Classification of CHD

Acyanosis
Acyanosis
Normal
Normal pulmonary
pulmonary blood
blood flow
flow
Pulmonary
Pulmonary Stenosis
Stenosis (PS)
(PS)
Aortic
Aortic Stenosis
Stenosis (AS)
(AS)
Coarctatio
Coarctatio Aorta
Aorta (CoA)
(CoA)
Increased
Increased pulmonary
pulmonary blood
blood flow
flow
Patent
Patent Ductus
Ductus Arteriosus
Arteriosus (PDA)
(PDA)
Atrial
Atrial Septal
Septal Defect
Defect (ASD)
(ASD)
Ventricular
Ventricular Septal
Septal Defect
Defect (VSD)
(VSD)

Cyanosis
Cyanosis
Normal
Normal pulmonary
pulmonary blood
blood flow
flow
TGA
TGA without
without PS
PS
Increased
Increased pulmonary
pulmonary blood
blood flow
flow
TGA
TGA with
with VSD
VSD
Truncus
Truncus arteriosus
arteriosus
Total
Total anomaly
anomaly pulmonary
pulmonary veinvein drainage
drainage
Decreased
Decreased pulmonary
pulmonary blood
blood flow
flow
ToF
ToF
Pulmonary
Pulmonary atresia
atresia
Ticuspid
Ticuspid atresia
atresia
CCHD Early Symptom (-)

Diagnosis
Symptom (+)
Spontaneous

Growth & dev monitor


Early Closure
palliative/definitif Medical
(-) Re
treatment
se treatment Gr sp
n ow on
spo th s e
Re & (+
De )
v
N
>

Interventional Surgery

>
treatment

Palliative Definitive
1-2 year
Hybrid
intervention
>
Critical Case in CHD

Critically CHD

Heart failure

Cyanotic spell

Supraventricular tachycardia

Tamponade
Critically Congenital Heart
Disease

Complex CHD in which circulation to


lungs /systemic depend on PDA
Duct dependent pulmonary

circulation
Pulmonary Atresia

Duct dependent systemic circulation


Hypoplastic left heart syndrom


Duct dependent mixing circulation


Transposition of great artery



Critically CHD

Duct Dependent Pulmonary Duct Dependent Systemic Duct Dependent Mixing


Circulation Circulation Circulation
Persistent pulmonary
hypertension of newbo
newb
Hyperoxia test
Oxygen Ventilation PaCO2 PaO2 Values
Concentration Status Goal
(%) PPHN Lung RL
Disease Cardiac

21 %-room air Spontaneous 40 40 40 40

100 %-hyperoxia Spontaneous 40 40 >100 40


or MV
100 %-pre and Spontaneous 40 >10-15 <5 <5
postductal shunt or MV

100 %- MV 20-25 >100 >150 40


hyperoxia, (Mechanical
hyperventilation ventilation)

Thompson TR, The Cyanotic Newborn


Infant
http://www.med.umn.edu/img/assets/922
3
Persistent pulmonary
hypertension of newborn
Preductal Pulse Oximetry

Aorta
Pulmonary Artery Ductus Arterious

Atrium
Left
Ventricle Ventricle
Postductal
Pulse Oximetry
Oxygenated
Mixed
Deoxygenated
Medical Treatment
1.
1. Initial treatment:
Prostaglandin E1
Critical CHD

To open PDA

Fast response

Doses 10 nanogram/kg/minute

Side effect:

Apneu

Hypotension

Balloon Atrial Septostomi

Transposition Great Artery


Balloon Atrial Septostomy
Balloon Atrial Septostomy
Balloon Atrial Septostomy
PDA stenting

Hypoplastic Left Heart Syndrome


PDA stenting
Blallock Tausig Shunt
(BT shunt)
Blallock Taussig Shunt
(BT shunt)
Heart Failure
Heart Failure

Heart Failure is clinical


syndrome the heart is
unable:

To pump enough blood to
the body to meet its needs

To dispose of systemic or
pulmonary venous return
adequately
Combination of both

26
Congenital heart disease

No Age of Causes
Onset
1 At birth HLHS
Volume overload lesion:
Severe TR or PR
Large systemic arteriovenous fistula
2 First wk TGA
PDA in small premature infants
HLHS
TAVPR
Others:
Systemic arteriovenous fistule
27
Congenital heart disease

No Age of Causes
Onset
3 1-4 wk CoA
Critical AS
Large L to R shunt lesions (VSD, PDA) in
preterm infants
All other lesions previously listed
4 4-6 wk Some L to R shunt lesions such as AVSD
5 6 wk-4 mo Large VSD
Large PDA
Others such as ALCAPA
28
Pathophysiology and
Pathogenesis

Cardiac output: the


amount of blood that
the ventricle pump per
minute

CO= HR X SV

CO:
Preload

Afterload

Contractility


Determinant of ventricle
function

Contractility

Preload Afterload
Stroke volume

Synergistic LV Contraction
Wall integrity Heart rate
Valvular competence

Cardiac output
LV Dysfunction

Volume Pressure Loss of Decrease


Overload Overload Myocardium Contractility

LV Dysfunction
End Systolic Volume
Cardiac
output
End Diastolic Volume

Hipoperfusion Pulmonary congestion


Heart Failure
The Donkey Analogy
Ventricular dysfunction limits a patients ability to perform
the routine activities of daily living
Heart failure ...Medical treatment
preload

Diuretic;


Frusemide : 1-2mg/kg/day
2X
Sprironolakton:

0-10 kg: 6.25mg/kg 12H; 11-20 kg: 12.5


12H mg/kg 2X; 21-40 kg: 25 mg/kg 12H;
>40 kg: 25 mg/kg 12H

afterload
Vasodilator


Captopril: 0.3-6 mg/kg/day
...Medical treatment

Contractility

Dopamine : 5-10g/kg/minute
Dobutamine: 5-10 g/kg/minute

Digoxin (g/kg/day)

Digitalization
Maintenance
Premature 20 5
< 30 day 30 8
< 2 year 40-50 10-12
> 2 year 30-50 8-10
Effects of anticongestive medications on the Frank-Starling relationship
for ventricular function

Cohn JN. N Engl J Med 1997;297:27-31 35


Inotropic
Digitalis compounds
Like the carrot placed in front of the donkey
Diuretic
Diuretics, ACE Inhibitors
Reduce the number of sacks on the wagon
Beta Blocker
-Blocker
Limit the donkeys speed, thus saving energy
Afterload-Reducing Agent
Arteriolar vasodilator (hydralazin), Venodilators
(nitroglycerin),
mixed vasodilator (Captopril)
Reduced afterload
CYANOTIC SPELLS

paroxysmal hyperpnea

hypoxic spells

anoxic spells

blue spells
Pathogenesis
...Medical treatment
Cyanotic spell
a. Kneechest position
a.

b. Acid-base correction
b.

c. Sedation: Morphin sulphat 0.2


c.
mg/kg IM/SC
d. Propranolol: 0.01-0.25 mg/kg
d.
(average 0.05 mg/kg) IV slowly
Knee Chest Position
Supraventricular
Tachycardia
Mechanism of SVT

Mechanism of
SVT
Automaticity

Reentry
Mechanism of SVT

Automaticity
Automaticity
Acceleration

Acceleration of
of phase
phase 4
4
Source:

Source:
Atrium

Atrium
AV junction

AV junction
His

His bundle
bundle
Ventricle

Ventricle
Pulmonary

Pulmonary vein
vein
SVC

SVC

Cause

Metabolic
Metabolic disorder
disorder

Hypoxia
Hypoxia

Hypokalemia
Hypokalemia

Hypo
Hypo magnesemia
magnesemia

acidosis
acidosis
Mechanism of SVT

Reentry
Condition

Two

pathway
make
closed
circuit
Block in 1

pathway
Antegrad

in normal
conductio
nretrogr
ad in
block
SVT
SVT
Clinical Manifestation

SVT in infants
Irritability

Feeding problem

Tachypneu

Pale

Vomit

Heart rate: 200-300 times/minutes

Heart failure

Circulatory arrest
Clinical Manifestation
SVT in Children
Sign and symptom less severe

then infant
Rare to have heart

failure/circulatory arrest
Symptom: palpitation/chest

discomfort
Heart rate < SVT in infants

Clinical Manifestation
SVT chronic
SVT long lasting: week-

months
HR < SVT infant or

children
Symptom influenced by
Management

Acute treatment
Vagal maneuvers

Adenosine

Electrical cardioversion


Long-term treatment
Consideration:

Age

Symptoms

Mechanism of SVT

Treatment option

No treatment

Chronic or periodic medication


Catheter ablation

Anti-tachycardia pace maker


Surgery

Short-Term management
of SVT

Delacretaz. NEJM 2006;354:1039-51


Radiofrequency Catheter
Ablation

Used a definitive
therapy since 1989

Using intracardiac
catheters,
radiofrequency energy is
used to desiccate a
small, well-
circumscribed area of
cardiac tissue thought to
be essential to the
arrhythmia circuit, such
as the accessory
connection
Radiofrequency Catheter Ablation
Radiofrequency Catheter Ablation
Tamponade
Pericardial Effusion

Pericardial effusion (PE) is defined as


the presence of an abnormal amount
and /or character of fluid in the
pericardial space.

The most common etiology of PE are


viral infection, pyogenic bacterial,
tuberculosis, collagen vascular
disease, malignancy and idiopathic.
Pediatr cardiol 2000;21:363-7
radiology findings

Result : Cardiomegaly, minimal pleural


effusion, lung infiltrat and lymph node
ECG

Sinus rhythm, HR 120 x/min, normal


axis, normal QRS complex, flat T wave

V5

Before After
Echocardiography

PE

RV
LV

LV PE
PE

Result : Massive pericardial effusion and


cardiac tamponade

Suggestion : Pericardiocentesis immediately



Subxiphoid approach procedure
pericardiocentesis
Subxiphoid approach procedure
pericardiocentesis
Summary

CHD is most common congenital


abnormality in children

Critical case of CHD: critical CHD,


heart failure, cyanotic spell, SVT
and tamponade

Early detection and prompt


treatment is important
Acknowledgement

Dr.
Dr. Mazeni
Mazeni Alwi,
Alwi, MRCP
MRCP (Kuala
(Kuala Lumpur)
Lumpur)
Dr.
Dr. Hasri
Hasri Samion,
Samion, MMed
MMed Paed
Paed (Kuala
(Kuala Lumpur)
Lumpur)
Dr.
Dr. Mulyadi
Mulyadi M.
M. Djer,
Djer, SpAK
SpAK (Jakarta)
(Jakarta)
Dr.
Dr. Sukman
Sukman T. T. Putra,
Putra, SpAK,
SpAK, FACC,
FACC, FESC
FESC (Jakarta)
(Jakarta)
Prof.
Prof. Bambang
Bambang Madiyono,
Madiyono, SpJP,
SpJP, SpAK
SpAK (Jakarta)
(Jakarta)
Prof.
Prof. DR.
DR. Sudigdo
Sudigdo Sastroasmoro,
Sastroasmoro, SpAKSpAK (Jakarta)
(Jakarta)
Dr.
Dr. Ismet
Ismet NN Oesman,
Oesman, SpAK
SpAK (Jakarta)
(Jakarta)
Dr.
Dr. Najib
Najib Advani,
Advani, SpAK,
SpAK, MMed
MMed Paed
Paed (Jakarta)
(Jakarta)
Dr.
Dr. Syarif
Syarif Rohimi,
Rohimi, SpA
SpA (Jakarta)
(Jakarta)
Dr.
Dr. Sasmito
Sasmito Nugroho,
Nugroho, SpA
SpA (Yogyakarta)
(Yogyakarta)
Dr.
Dr. Noormanto,
Noormanto, SpAK
SpAK (Yogyakarta)
(Yogyakarta)
Dr.
Dr. Mahrus
Mahrus A.A. Rahman,
Rahman, SpAK
SpAK (Surabaya)
(Surabaya)
Dr.
Dr. Ria
Ria Nova,
Nova, SpAK
SpAK (Palembang)
(Palembang)
All
All Fellow
Fellow of
of School
School ofof Pediatric
Pediatric Cardiology
Cardiology
Ziyat M Hijazi (Chicago): KL Live 2011 in Kuala
Lumpur Malaysia
Thankyou

Kurtz Amplatz: PICS 2005 in Buenos Aires,


Argentina

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