Dr. Mazeni Alwi

Institut Jantung Negara (National Heart Institute)

Kuala Lumpur

Atrial Septal Defects with Severe Pulmonary Hypertension: Shall I close

it? How to close it?

4th Asia Pacific Congenital & Structural Heart Intervention Symposium 2013

Hong Kong

Pulmonary arterial hypertension (PAH): Definition

• mean pulmonary artery pressure of > 25 mmHg at rest • normal pulmonary artery wedge pressure <15mm Hg

4th world symposium of PAH 2008, Dana point

Diagnosis and assessment of PAH Badesch et al , J. Am. Coll. Cardiol. 2009;54;S55-S66

Pulmonary vascular resistance > 3 woods unit

Definition of severe PHT ?

Not clinically Eisenmenger’s Still left right shunt of colour Doppler

Limitations of measuring PVR and Qp:Qs based on Fick’s formula by oxymetry

Mean PA pressure Mean Ao pressure

> ? 0.75 > ? 0.9

MacDonald ST et al. Heart, March 2011 Vol 97 No 5

• ? Why patients with same degree of PAH demonstrate individual differences in symptoms, natural Hx and responses to treatment

• PDA and VSD with severe PHT behave differently from

ASD? • Confounding issue – ? Is primary/iPHT the main issue

and ASD is co-incidental

Problems with Congenital Heart Disease Left Right Shunt

10 year old male Moderate – large ASD “Eisenmenger’s” features at 6 years of age ? 1° PHT

45 years, female Very large ASD PA pressure 80% systemic ~ still left right shunt

• Determine those with PAH-CHD whose lesion is still operable/have reversible pulmonary vascular disease

• How to accurately determination of degree of pulmonary vasoreactiviy/reversibility?

Management Dilemma

• Choose not to close : Patient may progress to Eisenmenger syndrome • Choose to close : Risk of progressive pulmonary vascular disease right heart failure, premature death

How to decide? Evaluation of operability

• Systematic approach

• Combination of clinical & investigative information

• Clinical, ECG, CXR

• Degree of left - right shunt echocardiogram

haemodynamic calculation

• Response to pharmacological vasodilation

(inhaled NO, 100% oxygen or inhaled illoprost)

• Lung biopsy (microscopic finding)

No/mild PAH Moderate-severe PAH

Very severe PAH

Qp: Qs > 2.0 1.5-2.0 < 1.5

Pulmonary vascular resistance ( wood unit)

< 3 >4- 10 > 10

Vasodilator test positive variable negative

Difficult /controversial What value of PVR?

Risk of post operation pulmonary hypertensive crisis Long term risk of progressive pulmonary vascular disease

ASD and PAH : Decision to close

PAH-CHD : IJN experience

• Since 2005 sildenafil used to prime pulmonary bed

• Children & Adults

• CHD (simple ASD, VSD & PDA)

• Pulmonary arterial hypertension PVR 4-?15 woods unit, ( the cut off PVR limit

reduced with time and experience) Mean pulmonary artery pressure to aortic

pressure ratio PA: Ao > 0.5 (ASD) or > 0.8 for VSD, PDA

Qp:Qs ratio 1.5-2

Methods Medical history, P/E

CXR ,ECG, 2 DE

Baseline 6 min walk test

Cardiac catheterization

Baseline ocular test

Sildenafil 50mg tds or 1-2 mg/kg/day

Clinical review

6 min walk test, 2DE

3 months

6 months

Repeat Cardiac catheterization, acute vasoreactivity test dobutamine stress

Trial occlusion/ fenestrated closure of defect/surgical closure

PVR > 4- ?10 w/u PA : Ao >0.5 0r > 0.8

Pre 2005, no sildenafil

Contraception

NT pro BNP

Vasodilator and Stress challenge

• Nebulised Illoprost 20mcg • Omron nebuliser • 15 mins post nebulising : restudy • 100% 0xygen or iNO ( ventilated patients) • Dobutamine stress to achieve 80% targeted heart rate

Positive vasodilator challenge During acute vasodilator testing (with NO, PgI2 or adenosine)

Decrease in mPAP >10 mmHg To reach a mPAP < 40 mmHg With a normal or a high cardiac output

Transcatheter trial occlusion

• Trial of transcatheter occlusion with device • Monitor hemodynamics for 24-48 hours, device still attached

to cable • If stable, release device • If unstable, remove device

(Mainly applicable to PDA, very few ASD cases due to large defect size)

Post procedure

• Surgical patients Precautions during induction, and post op care. (inotropes, ventilation, PAH drugs (iNO ) • Sildenafil continued at least up to 1 year post closure until

repeat cardiac catheterization • Monitor for progression of pulmonary vascular obstructive

disease Clinical, 6MWT, Echo, NT pro BNP • Repeat cardiac catheterization at 1 year, 3-5 years

25 patient enrolled

Baseline cath

trial closure n=23

Suprasystemic PAP

Not Close n=2

SILDENAFIL (7)

PDA close n=18 FAILED

6M post sildenafil n=8, non sildenafil n=15

SURGERY

NONE (11)

SILDENAFIL (10) NO sildenafil (15)

n=2

n=1

Follow up: Alive n=6 Died n=1

Follow up: Alive n= 7 Died n=3 Defaulted n=3

PDA size (mm): 9.93 ± 2.95 Device Use : ASO (n=10) ADO (n=8)

PDA with PHT

0.00

20.00

40.00

60.00

80.00

100.00

PRE SILDENAFIL 6 MONTHS SILDENAFIL

1 YEAR POST 3 YEARS POST 5 YEARS POST

Mean PA

NO

YES

0.00

0.10

0.20

0.30

0.40

0.50

0.60

0.70

0.80

0.90

1.00

PRE SILDENAFIL 6 MONTHS SILDENAFIL

1 YEAR POST 3 YEARS POST 5 YEARS POST

PA:Ao

NO

YES

PDA-PAH :Post occlusion Sildenafil pre occlusion vs no sildenafil

ASD with PAH : IJN experience

• Prospective • Adults > 18yrs • Moderate size secundum ASD (>2cm) • Pulmonary arterial hypertension (PVR 4-10 woods unit, mean pulmonary artery pressure

to aortic pressure ratio > 0.5 (PA: Ao), Qp:Qs ratio <2) • Not clinically Eisenmenger’s

Objective • Review the effect of 6 months of sildenafil 50mg tds on

the hemodynamic parameters • Outcome of closure/fenestration of ASD

ASD and PAH: IJN Experience

Patient on Sildenafil No closure ASD occlusion Fenestrated closure

TOTAL no of patient 14 3 12

AoP (mmhg) 93+21.3 76.7+17.62 91,8+10.5

PAP(mmhg) 54.3+9.21 48.7+16.3 48.9+4.9

PA:Ao 0.66+0.15 0.72+0.26 0.59+0.11

Qp:Qs 1.92+0.43 2.22+1.02 2.35+0.91

Ao Sat (%) 94+6.5 98.3+1.2 96+1.56

PVR (woods unit) 8.24+3.91 6.02+2.1 7.29+2.28

RESULTS

No procedure (n=14) Progression of PHT

Cath 2 – 6 months post Sildenafil n=29

ASD with PAH (n=32) Cath 1 - pre Sildenafil

Suitable for Fenestrated ASD closure (n= 12)

ASD occlusion (n=3) by device

Defaulted n= 3

PVR <8 woods unit Consider closure

HAEMODYNAMICS 3.02.2012 14.09.2012

Room air Post Illoprost Room air Post Illoprost

Qp:Qs 2.2:1 2.4:1 2.46:1 2.39:1

PVR( woods unit) 10.8 16.3 8.29 8.56

3.02.2012 14.09.2012 (post Sildenafil)

Surgical closure with fenestration

ASD and PAH: IJN Experience

OUTCOME

No procedure

(n=15)

Fenestrated ASD closure (n= 12)

ASD occlusion (n=3)

1 died (after 1 year

post closure)

2 died after 3 years sildenafil

11 fenestrated closure

(1 abandoned)

n=1

ASD and PAH: IJN Experience

OUTCOME ON FOLLOW UP

Patients post fenestration had lower PVR and mean PA pressure compared to those closed

Duration follow up YEAR SD

NONE 2.37 1.81

FENESTRATED 2.45 1.22

CLOSE 3.22 1.13

0

10

20

30

40

50

60

70

PRE 6 MONTHS 1 YEAR POST 3 YEARS POST

ME

AN

PA

PA PRESSURE

NAB

PSP

AM

NS

NR

BAJ

FMS

NA

NJ

MM

WNW

FENESTRATED - CATH

Fenesterated ASD Closure

0

0.1

0.2

0.3

0.4

0.5

0.6

0.7

0.8

0.9

PRE 6 MONTHS 1 YEAR POST 3 YEARS POST

PA

:AO

PA:AO RATIO

NAB

PSP

AM

NS

NR

BAJ

FMS

NA

NJ

MM

PA:Ao

RATIO PRE 6M 1Y POST

NS 0.82 0.67 0.77

NAB 0.53 0.53 0.56

Summary

Secundum ASD with severe PHT

• Very difficult management decision No agreed definition (? pre-Eisenmenger’s, PA/Ao pressure > 0.6) Limitations of current methods of evaluation • Role of vasodilator therapy to prime pulmonary vascular bed

before intervention

Intervention

• Selected cases after detailed evaluation – clinical, echo, haemodynamics pre and post vasodilator

Rigorous counselling and consent process • Surgical closure with fenestrated patch for suitable cases • Limited role of device closure (usually very large defects) • ? Role of individualized device size with fenestration