Prenatal Interventions

for

Congenital Heart Anomalies

Carol B. Benson, MD No Disclosures

Fetal Cardiac Interventions

Indications:

Aortic stenosis developing HLHS(hypoplastic left heart syndrome)

Hypoplastic left heart syndromewith restricted atrial septum

Pulmonic stenosis developinghypoplastic right ventricle

Aortic Stenosis Developing intoHypoplastic Left Heart Syndrome

Intervention – Aortic valve dilationGoal:

Prevent

Reverse

Minimize degree of

Hypoplastic

leftheart

syndrome

Critical aortic stenosis or aortic atresia↓

Obstruction of blood flowthrough left ventricle

↓Damage to left ventricle myocardium

↓Poor left ventricle contractility

& growth↓

Hypoplastic left heart syndrome

Aortic Stenosis Developing intoHypoplastic Left Heart Syndrome

Critical aortic stenosis or aortic atresiaProgression of ultrasound findings

Dilated left ventricle↓

Echogenic endocardium↓

Poor left ventricle contractility↓

Small noncontractile left ventricle

Aortic Stenosis Developing intoHypoplastic Left Heart Syndrome

Reade ASdeveloping HLHS

Aortic Stenosis progressingto HLHS

Oneweeklater

20weeks

Kelly AS developing HLHS

Aortic stenosis developed into HLHS31 weeks

Lembke hypopl lv 3 & 2

Hypoplastic left ventricle

Procedure: Aortic valve dilatationPreop assessment

Full fetal surveyKaryotypeLeft ventricular sizeLeft ventricular contractilityLeft ventricular fibroelastosisFlow across aortic valveWidth of aortic valve

Aortic Stenosis Developing intoHypoplastic Left Heart Syndrome

Reining AS preop

Before procedure

EndocardialFibroelastosis

Aortic stenosisEccentric jet of flow

Pecor preop

Beforeprocedure

Palomo preop 1and clip 3

Before procedure

Procedure: Aortic valve dilatationPosition fetusParalyze & anesthetize fetusSet needle approachInsert needle into left ventricle

pointing towards aortic valveExchange trocar with wireGuide wire across aortic valvePass balloon catheter across valveInflate balloon

Aortic Stenosis Developing intoHypoplastic Left Heart Syndrome

Diagram of needle in heart

Aortic Valve Dilation

Fetal position must be optimalSwaby 5 & Rand 3

Fetal Position

23 weeks 22 weeks

Paralyze & Anesthetizethe Fetus

Inject thigh or buttocksMiller paralyze 1-3

Needle Insertion

Use continuous ultrasound guidance

Select needle entry site

Select angle

Modify insertion as needle is advanced

Atherton4 & Crawford3 needle in LV

23 weeks

Jungert 4 & Crawford3 needle in LV

23 weeks 23 weeks

Wire Insertion

Remove trocar

Insert wire

Pass wire across aortic valve

Dietzel wire 9& 10 wire 8

21 weeks

Balloon Catheter

Pass catheter over wire across valve

Inflate balloon

Atherton wire & balloon 4&7

23 weeks

Deslauriers catheter & balloon 1, 3, 4

25 weeks

Reining2,3,4

21 weeksAfter Removing the Needle

Assess flow across the valve

Atherton post op flowImmediately after procedure

Beane post op flow 9, 11, 12

Immediatelyafter procedure

Deffea post op flow

After procedure

Deslauriers pre & post

25 weeks

Preop Postop

LV

Mulvihill preop 2 & post op 2

Preop Postop

Flaherty preop & post op

Preop Postop

LV

LV

Challenges

Fetal positioning (must be ideal)

Difficulty entering thorax

Left ventricular collapse

“White out” of left ventricle

Wire does not cross aortic valve

stiff, atretic valve

needle not pointing at the valve

Hayes wire to mitral valve 3

Complications

Fetal bradycardia

requiring resuscitation

Pericardial effusion

Pleural effusion

Fetal demise

Balloon rupture

Anmar postprocedure brady & effusion

Postprocedure bradycardia& pericardial effusion

Hamblin arm injection& RV injection

Bradycardia requiring resuscitation

Deffea RV injection

Pleural effusion postop day 1

Hypoplastic Left Heart with Restricted Atrial Septum

Obstructed pulmonary venous returnHigh Perinatal MortalityGoal of procedure:

Create channels throughthe atrial septum

To improve pulmonary blood flowTo allow pulmonary venous return

to right heart across septum

Left to right flow across atrial septumNecessary for survival after birthOxygenated pulmonary blood

must reach systemic circulationRight ventricle pumps blood

for both pulmonary andsystemic circulations

After birth, intervention to enlargeatrial septal defect

Hypoplastic Left Heart with Restricted Atrial Septum

In utero develop high pulmonaryarterial and venous pressure

Causes pulmonary cystic changesfrom dilated lymphatics

Causes pulmonary hypertensionLimited flow through pulmonary

circulation in uteroPersistent limited flow after birth

Hypoplastic Left Heart with Restricted Atrial Septum

Intervention –Create hole in atrial septum

Goal:Open pathway for blood

returning from lungs to reachright ventricle

Decompress pulmonary veins toprevent pulmonary hypertensionand cystic changes in lung

Hypoplastic Left Heart with Restricted Atrial Septum

Procedure: Atrial septostomyPosition, paralyze & anesthetize fetusSet needle approachInsert needle into right atrium

towards or across atrial septumExchange trocar with wirePosition wire across septumAdvance balloon catheter across septumInflate balloon

Hypoplastic Left Heart with Restricted Atrial Septum

Crawford position preop 1 & 2

HLHS & restricted atrial septum – 31 weeks

Pomrink position preop 1

HLHS& restrictedatrialseptum34 weeks Vanderwerken HLHS

restricted septum

HLHS & restricted atrial septum30 weeks

LA

Dilated pulmonary veins

Dobbs HLHS restrictedAS & pulm dysplasia

HLHS with restrictedseptumDilated

Pulmonary lymphaticsNeedle Insertion

Use continuous ultrasound guidance

Select needle entry site

Select angle

Modify insertion as needle is advanced

Pomrink into RA Philips 1 & 2

Lewis into RA 1&2LA

Wire & Balloon Insertion

Remove trocar

Insert wire

Pass wire into left atrium or

pulmonary vein

Insert balloon

Inflate balloon

Lewis wire advanced 3&4

LA

Lewis balloon 5&6

Lewis pull out 6Moreaux wire &

balloon 1,4,6

22 weeks HLHS withrestricted atrial septum

Vandenberg balloon& post 6&8

29 weeks HLHS with restricted atrial septum

Flow from LA to RA

Bailey HLHS restricted septum

HLHS Preop24 weeks

PostopSeptotomy

Dodds HLHS septostomy 3,4,5

Septotomy

Phillips flow across septum

Immediatelyafter

procedure

Stent Placement

Pass stent catheter over wire

to straddle septum

Insert balloon to expand stent

Remove cannula, wire, & catheter

Crawford 3 4 5stent placement

Stent placedacross septum

31 weeks

Crawford 9 & 12stent placement

Stent placedacross septum

31 weeks

Vanderwerkenstent placement

Stent catheter across septum

Vanderwerkenstent placement

Stent across septum

Dixonstent placement 1,2

Evolving HLHS with restricted septumStent placement at 30 weeks

LA

Dixonstent placement 4&6

Evolving HLHS with restricted septumStent placement at 30 weeks

LA

Watson stentpostop

HLHS with restricted septumPostop Stent

LA

Flow from LA to RA

Pulmonic Stenosis/AtresiaDeveloping

into Hypoplastic Right Heart

Pulmonic atresia (or critical stenosis)diagnosed prenatally

~ two-thirds will havesingle ventricle after birthhypoplastic right ventricle

Pulmonic Stenosis/AtresiaDeveloping

into Hypoplastic Right Heart

Intervention – Pulmonic valve dilationGoal:

Prevent

Reverse

Minimize degree of

Hypoplastic

rightheart

Healey PS developinghypopl RV

Hypoplastic RV Pulmonic atresia

Goebel PS surgery

Pulmonic atresia intact

ventricular septum27 weeks

Goebel PS surgery

Pulmonic atresia intact ventricular septum Total Experience – 193 cases(202 procedures)

Aortic valve dilation (138 in 136 patients)115 Successful technically23 Unsuccessful technically

Atrial septostomies (35)32 Successful technically

(+ 5 stent placement)3 Unsuccessful technically

Pulmonic valve dilation procedures (14)9 Successful technically1 Partially successful technically4 Unsuccessful technically

Mitral valve dilation procedures (2)2 Successful technically

Rescue procedures (13 in 9 patients)2000–2016

77 technically successful procedures35 – two ventricles (not HLHS)35 – hypoplastic left heart syndrome

7 – demise or TAB23 technically unsuccessful procedures

3 – two ventricles (not HLHS)*11 – hypoplastic left heart syndrome3 – demise or termination

First 100 Aortic ValveDilation Procedures

*Late procedures, 28w, 28w, & 32w

First 100 Aortic ValveDilation Procedures

Success rate improved with experience

88%68% 78% 78%Successful

First 100 Aortic ValveDilation Procedures

Outcome improved with experience& better selection criteria

88%68% 78% 78%

0

5

10

15

20

25

1 2 3 4

Number of Patients

Patient Order in Quartiles

Demise

1V

2V

p‐value=0.4

52%44%28%28%

2ventricles

Thank you