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Management

of Grown-up Congenital Heart


Disease (GUCH)

Yulius Patimang

Division of Pediatric Cardiology and Congenital Heart Disease


Department Cardiology and Vascular Medicine
Faculty of Medicine Universitas Hasanuddin/
Cardiac Center Wahidin Sudirohusodo Hospital
GUCH has been new expanding population
not only in Indonesia but also in the world
GUCH patients outnumbered the pediatric
CHD patients
Improved diagnoses
Improved intervention and surgical technique
Improved post operative care
Advance medical management
Diagnosed 1st time in adulthood

Mortality & Morbidity

Growing Population of GUCH


GUCH Profile in NCCHK
Age Distribution of GUCH Patients
40

30

20

10

0
Age 17-20 Age 21-30 Age 31-40 Age 41-50 Age 51-60 Age 61-65
Severity of GUCH
Patients
65% of GUCH are
moderate to severe
cases

35% 30%

35%

1. Zaidi AN, Daniels CJ. Moss and Adams heart disease in


infants, childrenm and adolescents including the fetus and
complex CHD young adult. Volume 2.8th edition. Philadelpia.Lippincott
Williams&Wilkins. 2013. Pages: 1463-1465.
moderate-severe CHD 2. Moodie. Douglas. Texas Heart Institute Journal. 2011; 38(6):
705-706.
simple CHD 3. Amaral, Fernando, et al. Arq Bras Cardiol.2010
Jun;94(6):707-13.
BCPS Prosedure
Fontan Prosedure
Norwood prosedure
vascular lesions arrhythmia
residual
shunts
sudden death

LATE COMPLICATIONS
right heart failure
left heart failure
valvular Pulmonary Hypertension
diseases Infection heart failure
Zaidi AN, Daniels CJ. The Adolescent and adult with congenital heart disease. In: Allen HD et.al (editor). Moss and Adams
heart disease in infants, childrenm and adolescents including the fetus and young adult. Volume 2.8 th edition.
Therapeutic considerations

Medical management is largely supportive (e.g. for


heart failure, arrhythmias, pulmonary and systemic
arterial hypertension, prevention of thrombo-
embolic events, or endocarditis) and significant
structural abnormalities usually require
interventional treatment
CHD general problems
Heart Failure

Heart failure is a frequent problem in the GUCH population

The few available data on heart failure treatment in GUCH


patients are not conclusive and are derived from small patient
numbers

Cardiac resynchronization therapy (CRT) has gained


increasing interest for use in GUCH patients with congestive
heart failure
CHD general problems

Arrhythmias and sudden cardiac death


ICD implantation is indicated in survivors of
cardiac arrest after exclusion of reversible
causes
Sustained VT should undergo invasive
haemodynamic and EP evaluation.
Invasive haemodynamic and EP evaluation is
reasonable in patients with unexplained
syncope and impaired ventricular function
CHD general problems

Surgery during adulthood may be required in


various situations:
Patients with prior repair and residual or new
haemodynamic complications.
Patients with prior palliation.
CHD general problems

Infective endocarditis
High risk particularly in:
Cyanotic CHD without surgical repair, residual
defects, palliative shunts
Prostetic valves
Residual defects (generally) after surgical or
cath. closure
Patients with previous IE
general problems

Pregnancy
Generally well tolerated
Extremely High risk (high mortality 30-50%):
Severe pulmonary art. hypertension (Eisenmenger sy)
High risk
Cyanosis (esp. SpO2 <85%)
Stenotic left valve disease (AS, MS)
Poor EF LV (<40%)
Aortic roof dilation (Marfan sy)
Arrhythmias
Specific problems-Atrial septal
defect
Atrial septal Defect

RA LA

RA LA

RV LV
RV LV
Atrial septal defect -
treatment
ASD secundum (if possible catheterisation
device closure is prefer)
Atrial septal defect

During balloon
sizing
Atrial septal defect

ASD after occluded


using ASO
Atrial septal defect -
treatment
Device closure has become first choice for secundum
defect closure when feasible from morphology

Stretched diameter <38 mm


Sufficient rim of 5 mm except towards the
aorta
Atrial septal defect -
treatment
ASD sinus venosus superior connected with
partial anomalous pulmonary venous
connection PAPVC (mostly right upper
pulmonary vein to RA) L-R shunt
Only surgical treatment
Big ASD generally operated
Atrial septal defect -
treatment
Additional considerations:
Exercise/sports: No restrictions in
asymptomatic patients, limitation to low-
intensity recreational sports in PAH patients
Pregnancy: The risk from pregnancy in
patients without pulmonary hypertension is
low
IE prophylaxis: Recommended for 6 months
after device closure
Ventricular septal defect
Ventricular septal defect

RA
LA
RA LA

RV LV RV LV
Ventricular septal defect

Management:

Definitive : VSD closure


Surgery

Transcatheter closure
Ventricular septal defect

VSD before occlusion


Ventricular septal defect

VSD after occluded


using Device
Ventricular septal defect -
treatment
Additional considerations
Exercise/sports: No restrictions are required
in patients after VSD closure, or with small
VSD without pulmonary
hypertension,significant arrhythmias, or LV
dysfunction
Pregnancy: Pregnancy is contraindicated in
Eisenmenger syndrome
IE prophylaxis
Patent Ductus Arteriosus
Patent Ductus Arteriosus

RA LA
RA LA

RV LV
RV LV
Patent Ductus Arteriosus-
treatment
Device closure is the method of choice

Surgery is reserved for the rare patient with a duct too large
for device closure or with unsuitable anatomy such as
aneurysm formation
Patent Ductus Arteriosus
Patent Ductus Arteriosus

PDA after occluded


using Device
Patent Ductus Arteriosus-
treatment
Additional considerations
Exercise/sports: No restrictions in
asymptomatic patients before or after
intervention without pulmonary hypertension
Pregnancy: No significantly increased risk for
pregnancy in patients without pulmonary
hypertension
IE prophylaxis
Tetralogy of Fallot

4 anatomic
malformations:
Right Ventricular
Hypertrophy
RVOTO
Overriding of the
aorta
Ventricular Septal
Defect
Tetralogy of Fallot

Surgical treatment VSD closure + relief of


RVOTO (with resection of the infundibulum and
pulmonary valvotomy)
Common complication in adulthood (after
repair) :
Pulmonary regurgitation, Residual RVOTO, Aortic
root dilation with valve regurgitation, RV dilation
and dysfunction, Residual VSD
All patients should be followed-up in specialized
GUCH centers
Total Correction Prosedure of TOF
Tetralogy of Fallot -
treatment
Advantages of early total correction
Prevents cerebral hypoxia,cerebral embolism,
abscess and hematological changes
Decrease RVP
Providing adequate Pulmonary Blood Flow
Thank you
Grown-up Congenital
Heart Disease (GUCH)

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POPULA PROBLEMS
TION