You are on page 1of 33

CURRICULUM VITAE

Nama

: dr. Lucia Kris Dinarti SpPD SpJP (K)

Tempat, tanggal lahir

: Yogyakarta, 2 Maret 1961

Current Education :

Internist

: Universitas Gadjah Mada (1999)

Cardiologist

: Universitas Indonesia (2003)

Cardiologist Consultant

: Universitas Indonesia (2006)

Current Position:

Staf Bagian Kardiologi dan Kedokteran Vaskular Universitas Gadjah Mada/RSUP


dr. Sardjito Yogyakarta

Kepala Instalasi Rawat Jantung RSUP dr. Sardjito Yogyakarta

Clinical Approach to
Grown Up Congenital
Heart Disease (GUCH)
Lucia Krisdinarti
Cardiology Department and Vascular Medicine
Medical Faculty UGM/Sarjito Hospital
YOGYAKARTA

Introduction
GUCH can be divided into 2 groups : those who
have not previously had an intervention and
those who have.

Commonly divided into non cyanotic (L R)


and cyanotic (R L) categories based on
direction of shunting

In USA, incidence of GUCH increase 5% /year.


Data of incidence in Indonesia not available

Incidence of CHD in RSUP dr. Sardjito 2014

Age

Gender
(Hidayati et.al, 2015)

Incidence of CHD in Children and Adult


in Sarjito Hospital 2014

Children

Adult
(Hidayati et.al, 2015)

Acyanotic Heart Disease


Atrial septal defects (ASD)
Ventricular septal defects (VSD)
Patent ductus arteriosus (PDA)
Obstruction to blood flow
Pulmonic stenosis (PS)
Aortic stenosis (AS)
Aortic coarctation

(ESC, 2010)

Cyanotic Heart Disease


Tetralogy of Fallot
Tricuspid atresia (TA)

Total anomalous pulmonary venous return (TAPVR)


Truncus arteriosus
Transposition of the great vessels
Hypoplastic left heart syndrome (HLH)
Pulmonary atresia (PA) / critical PS

Double outlet right ventricle (DORV)

(ESC guidelines , 2010)

Basic Patient Assessments


Thorough patient evaluation is crucial for management of GUCH!
Patient History
- assess present and past symptoms
- look for intercurrent event and changes in medication
- question lifestyle to detect progressive changes in daily acativities

Clinical Examination
Plays a major role and includes, during follow up, careful evaluation with
regard to any changes in auscultation findings, blood pressure, and
development of sign of heart failure

ECG and pulse oxymetri


Routinely carried out along alongside clinical examination

Chest X-ray
Performed on indication but helpful during follow up
( ESC guidelines , 2010)

Further Examination
Echocardiography
- First line investigation, providing morphology of cardiac anatomy,

shunting and the surrogate for hemodynamic measurement.

Cardiac MRI
- Alternative to echo when both provide similar information but echo
cannot be obtained with sufficient quality

- second method when echo result are borderline or ambigous

Computed Tomography
Cardiopulmonary Exercise Testing
Cardiac catheterization
- assesment of PAP, PVR, shunt quantification particularly in shunt lesion
- testing of vasoreactivity
- coronary angiography before surgery in men>40 y.o, post menopausal
women, and patients with risk factors for CAD
(ESC guidelines, 2010)

Most common in adult

Commonly asymptomatic
Essentials of diagnosis:
Right ventricular heave
S2 widely split and usually fixed
Grade I-III/VI systolic murmur at the pulmonary area
RBBB in ECG; RAD for secundum type and LAD for primum
type
Cardiac enlargement on CXR
(Rao, 2005)

Three major types


Ostium secundum
most common
In the middle of the septum in the region of the foramen ovale
Ostium primum
Low position
Form of AV septal defect
Sinus venosus
Least common
Positioned high in the atrial septum
Frequently associated with PAPVR

(Rao, 2005)

Atrial Septal Defect Closures


I IIa IIb III

I IIa IIb III

I IIa IIb III

Closure of an ASD either percutaneously or


surgically is indicated for right atrial and RV
enlargement with or without symptoms.
A sinus venosus, coronary sinus, or primum
ASD should be repaired surgically rather than
by percutaneous closure.
Surgeons with training and expertise in CHD
should perform operations for various ASD
closures.
(ACC/AHA guidelines , 2008)

12

Closure
contraindicated in
severe PAH

Previously surgical;
now often closed
percutaneously using
device

Clinical findings
Grade II-IV/VI, medium- to
high-pitched,
harsh
pansystolic murmur heard
best at the left sternal border
with radiation over the entire
precordium

Chest X-ray : LV enlargement


ECG : LVH, later biventricular
hypertrophy

(Brickner et al, 2000)

(Brickner et al, 2000)

Surgical Ventricular Septal Defect Closure


VSD Closure Operations
I IIa IIb III

Surgeons with training and expertise in CHD


should perform VSD closure operations.
I IIa IIb III

Closure of a VSD is indicated when there is a


Qp/Qs (pulmonary-to-systemic blood flow ratio) of
2.0 or more and clinical evidence of LV volume
overload.

I IIa IIb III

Closure of a VSD is indicated when the patient has


a history of IE.
ACC/AHA guidelines, 2008)

15

Persistence of normal fetal vessel joining


the pulmonary artery to the aorta

Closes spontaneously in normal term


infants at 3-5 days of age

Epidemiology facts
Accounts for about 10% of all cases of CHD
Higher incidence of PDA in infants born at high altitudes
(> 10,000 feet)
More common in females

Pulses are bounding and pulse pressure is widened


Characteristically has a rough machinery murmur which peaks at S2 and
becomes a decrescendo murmur and fades before the S1
(Brickner et al, 2000)

Most common cyanotic lesion (7 to 10% of all CHD)


Typical features

Cyanosis after the neonatal period


Hypoxemic spells during infancy
Right-sided aortic arch in 25% of all patients
Systolic ejection murmur at the upper LSB
(Brickner et al , 2000; Perloff et al, 2009)

Special Issues of CHD in Adult


Pregnancy
Complication of Pulmonary Hypertension/
Eisenmenger Syndrome

Mortality Rate

Pregnancy in GUCH

Maternal Cases of ASD patients in


RSUP dr. Sardjito
Total 23 pregnant ASD patients from January 2013 Dec 2014;
who came in the Delivery/Maternal Unit
PH incidence

Mode of delivery

Caesarean
Spontaneus

PH /ES

7 (25%)

non PH

5 (22%)

18 (78%)

16 (75%)

Mortality
6 (27%)
Survive

SeverePH

Died
17 (73%)

(Krisdinarti & Anggrahini, 2014)

Euro Heart Survey :


Decreased survival in PAH- related CHD

Engelfriet et al., 2007

Eisenmenger Syndrome
Eisenmenger syndrome is pulmonary hypertension with a reversed central
shunt

An uncorrected large left-to-right shunt causes irreversible rise in PVR


leading to reversal of or bidirectional shunt flow with resultant hypoxemia

Eisenmenger syndrome is not a congenital defect, but a pathophysiologic


condition

Based on RHC; total number of the patients July 2012 Dec 2014: 124
45

no PH

39

40

mild PH (25-40 mmHg)


mod-severe PH >41 mmHg

patients No.

35
30
23

25

21

20
15
8

10
5

10
6

3
0

0
<20

21-40

41-60

Age Category

>61
(Krisdinarti & Anggrahini, 2014)

Moderate-Severe PH (mPAP>41 mmHg)

mild PH (mPAP 25-40 mmHg)


3

<20

21-40

17

41-60

19

>61

31

(75%)

<20
21-40
41-60
>61

High Incidence of Severe PH in young female age groups 21-40 y.o :


Natural History for Eisenmenger?
Genetic background ?
Or ASD coincide with IPAH/HPAH ?

(Krisdinarti & Anggrahini, 2014)

Mortality in CHD with PH in


RSUP.Dr.Sarjito Yogyakarta
Causes of death
Sudden death (30%)
Congestive heart failure (25%)

Hemoptysis (15%)
Pregnancy
Perioperative mortality of non-cardiac surgery
Infectious diseases

( Krisdinarti & Anggrahini, 2014)

Mortality Cases of ASD in RSUP dr. Sardjito


Number of Mortality patients : 19 of 247 patients

PH severe/
Eissenmenger Right Heart Failure
Lung Infection
6%

PA dissection
Sudden Cardiac Death
Arrythmia VF
Sepsis

Death
7%

No PH 1%

All cause mortality

(Krisdinarti & Anggrahini, 2014)

SUMMARY
GUCH still become a big problem in Indonesia.
Case finding is important
Special issue : pregnancy, pulmonary
hypertension, high mortality rate.

Early screening is needed to decrease the


problem

Thank You

Infective Endocarditis
Cardiac conditions associated with the highest risk of adverse
outcome from endocarditis for which prophylaxis with dental
procedures is reasonable
Condition

Congenital Specific Condition*

Previous infective endocarditis

Unrepaired cyanotic CHD, including palliative


shunts and conduits

Prosthetic cardiac valve or prosthetic


material used for cardiac valve repair

Completely repaired congenital heart defect with


prosthetic material or device, whether placed
by surgery or by catheter intervention, during
the first 6 months after the procedure

*Except for the conditions listed above, antibiotic prophylaxis is


Repaired CHD with residual defects at the site or
no longer recommended for any other form of CHD.
Prophylaxis is reasonable because endothelialization of
adjacent to the site of a prosthetic patch or
prosthetic material occurs within 6 months after the procedure.
prosthetic device that inhibit endothelialization
Modified with permission to include footnotes from Wilson et al.
Prevention of infective endocarditis: guidelines from the
American Heart Association: a guideline from the American
Heart Association Rheumatic Fever, Endocarditis, and
Cardiac transplant recipients who develop
Kawasaki Disease Committee, Council on Cardiovascular
cardiac valvulopathy
Disease in the Young, and the Council on Clinical Cardiology,
Council on Cardiovascular Surgery and Anesthesia, and the
Quality of Care and Outcomes Research Interdisciplinary
Working Group. Circulation. 2007;116:1736-54. CHD indicates
congenital heart disease.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 6. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001

30

Infective Endocarditis
Congenital Cardiac Lesions and Preoperative Risk for Noncardiac Surgery
High risk
Pulmonary hypertension, primary or secondary
Cyanotic congenital heart disease New York Heart
Association class III or IV

Severe systemic ventricular dysfunction (ejection


fraction less than 35%)
Severe left-sided heart obstructive lesions
Moderate risk

Prosthetic valve or conduit


Intracardiac shunt
Moderate left-sided heart obstruction
Moderate systemic ventricular dysfunction
Warnes, et al. J Am Coll Cardiol 2008;52. Table 7. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001

31

Surgical Ventricular Septal Defect Closure


VSD Closure Operations
I IIa IIb III

Closure of a VSD is reasonable when net left-to-right


shunting is present at a Qp/Qs greater than 1.5 with
pulmonary artery pressure less than two thirds of systemic
pressure and pulse volume recording (PVR) less than two
thirds of systemic vascular resistance.

I IIa IIb III

Closure of a VSD is reasonable when net left-to-right


shunting is present at a Qp/Qs greater than 1.5 in the
presence of LV systolic or diastolic failure.
I IIa IIb III

VSD closure is not recommended in patients with severe


irreversible PAH.

32

Atrial Septal Defect Closures


I IIa IIb III

Closure of an ASD, either percutaneously or surgically,


may be considered in the presence of net left-to-right
shunting, pulmonary artery pressure less than two
thirds systemic levels, PVR less than two thirds
systemic vascular resistance, or when responsive to
either pulmonary vasodilator therapy or test occlusion
of the defect (patients should be treated in conjunction
with providers who have expertise in the management
of pulmonary hypertensive syndromes).

I IIa IIb III

Concomitant Maze procedure may be considered for


intermittent or chronic atrial tachyarrhythmias in adults
with ASDs.

33