adult congenital heart diseases surgical...

Adult CHD Muscat 2012

Adult Congenital Heart Diseases

Surgical Considerations

Samuel Weinstein MD MBA Director, Pediatric Cardiothoracic Surgery

Director, Adult Congenital Cardiac Surgery Children’s Hospital at Montefiore

Montefiore Medical Center

New York, NY


•*Estimated Numbers

•Warnes CA et al. J Am Coll Cardiol 2001; 37: 1170-1175

•Williams RG et al. J Am Coll Cardiol 2006; 47: 701-707

Incidence: 4 to 50 per 1,000 live births

Overall CHD mortality 3-4%

5% per year


Challenges for a growing population

• Who is going to take care of these patients

• Where is the best setting for this care

• Patients have a poor understanding of their heart

disease and are lost to follow-up

• Transition to adult medicine can be challenging

• Complex CHD is not “cured”

• Really a chronic effects of disease


• Chronic volume overload

•Left to right shunt

•Right to left shunt

• Pressure overload

•valvular disease

•subvalvular disease (LVOTO)

• systemic hypertension

• Cyanosis

• Myocardial damage due to CPB (mulitple)

• Atrial and ventricular arrhythmias

• Pulmonary Hypertension

• Coronary artery disease

• Obesity, IDDM, Pulmonary, etc.

Chronic Sequelae of ACHD/CHF


? Interventionalist

? Intensivist

?Anesthesiologist

? ICU/floor nursing

? Perfusion


Post-Operative CHF in adolescents and adults

Norozi et al, Am J Cardiology 2006;15: 1238


Operative Strategy

• Percutaneous vs hybrid vs. open repair

• Open Heart Surgery

– Ventricular Venting/Collateral Flow

– Cardioplegia

– Temperature

– Cannulation


SVC

TV

ASD 2D and 3D echocardiography


Ostium

Secundum

ASD

Septum

Primum

Coronary

Sinus

Pericardial

Patch

Closure

ASD Closure

CS


ASD device closure


ASD Device in Left Atrium


RMPV

ASD

Right

Atrium

PFO

SVC

RUPV

RMPV

Sinus Venosus ASD


Reoperations

Incidence in Acquired Adult Cardiac Surgery 10-20%

Incidence in Adult Congenital Cardiac Surgery >75%


Reoperations


Peripheral

Cannulation

Axillary Artery Fem-Fem w/distal limb

Femoral Artery

Superior Vena

Cava via IJ for

IVC occlusion


Scar on the inside, too!


Tetralogy of Fallot


Dilated RVOT

Wide Open PI in

Repaired TOF

Dilated Right Ventricle

Severe Volume

Overload

Adherence to back of

Sternum


Pulmonary Valve Replacement

Main PA

Main PA

Contegra

Right Ventricle

Graft in Situ

Beating Heart PVR


Percutaneous PVR


Coarctation of the Aorta


Repaired Coarctation Survival

Cohen et al. Circulation 1989; 80: 840-5

72% survival at 30 years

Essential Hypertension

Present as adults

Re-coarctation

Primary Presentation


Descending Aorta

Coarct

Isthmus

Left Subclavian Artery

Adult Coarct Repair Collaterals

Mobilization

Cardiopulmonary Bypass

Collateral



Percutaneous Stent Placement

Coarct Segment


Fontan Revision and Cryoablation

Classic Fontan Sequelae

– Right Atrial Dilation

• Stasis of Flow

• Decreased Cardiac Output

– Atrial Arrhythmias

• A Flutter

• A Fibrillation

– Pulmonary Vein Stenosis

– AVM’s


Fontan Revision and Cryoablation


Fontan Revision and

Cryoablation


Conclusions

• Complicated subset of patients

• Require multidisciplinary input

• No one correct way

• Consider all surgical and interventional options

• Not just bigger patients

– Co Morbidities

– Non childhood illnesses (CAD, Pulmonary, Renal, etc)

• With careful planning and in the appropriate

setting ACHD procedures can significantly

improve quality and quantity of life


Thank You

• Comments?

• Questions?


Complete Transposition of the Great

Arteries (D-TGA)

• Separate

PARRALLEL

circuits that require a

communication for

survival

• PDA

• ASD

• VSD

Brickner et al. NEJM 2000; 342: 334-342


Arterial Switch Repair Atrial Switch Repair


Late Complications of Atrial Switch Roos-Hesselink et al. Eur Heart J 2004; 25: 1264-70

Morphologic RV (systemic ventricle) failure

Systemic AV valve regurgitation (tricuspid valve)

Baffle Obstruction or Leak

At 15 years, only 50% have avoided sig morbidity


RV LV

Ao PA

TGA post Mustard Baffle

RV

LV


TGA s/p Mustard, poor RV fx, pre-

transplant


Venous

Confluence Left Atrium

Adult CHD Muscat 2012 Boneva, R. S. et al. Circulation 2001;103:2376-2381

Age-adjusted death rate due to CHDs by year of death, United States, 1979-1997

Mortality Declined by 40% from 1979 through 1997

Survival of children born with CHD is now

approximately 85-90%


Low complexity ACHD

Atrial Septal Defect (ASD)

Ventricular Septal Defect (VSD)

Patent Ductus Arteriosus (PDA)

Mild Pulmonary Stenosis

Oechslin et al. Am J Card 2000; 86: 1111-1116

Moderate Complexity ACHD

Coarctation of the aorta

Ebstein’s anomaly

AV canal

Tetralogy of Fallot

High Complexity ACHD

Transposition of the great arteries

Single ventricle

Fontan procedure

Eisenmenger syndrome


Left PAPVR

Main PA

Aorta


Post-Operative CHF in adolescents and adults

Norozi et al, Am J Cardiology 2006;15: 1238

At 40 Y of age

> 2/3 Fallot with CHF

At 40 Y

25% ASD

VSD, PDA


1 Million

Incidence of Adult Diseases in General Population



Percutaneous Stent Placement

Coarct Segment


Atrial Septal Defect

Asymptomatic

• Murmur

• Abnormal EKG

• ECHO

Symptomatic

• Exercise intolerance

• Paradoxical embolus

• Atrial arrhythmias

Lai et al. Echocardiography in Pediatric and Congenital Heart Disease 2009


Case

• 26 year old male with a history of Tetralogy of

Fallot repair at age 5. Now presents with PI, RHF.

• Most common form of cyanotic

congenital heart disease

• 4 features

• Right ventricular outflow tract

obstruction

• Overriding of the aorta

• VSD

• Right ventricular hypertrophy

• Features of Repair

Relief of right ventricular

outflow tract obstruction

• VSD Closure

• Takedown of any shunts

Success 85% at 30 year F/u


PAPVR Repair

Left Lung

Ascending Vertical Vein


Age of the Patient Population

50%


Common Procedures in Adult CHD

• Fallot: PV replacement

• Valved conduit failure

• Aortic valve, root or LVOTO

• Primary Disease (ASD, PAPVR)

• Residual lesions (VSD, obstruction, Pulmonary Vein Stenosis)

• Fontan Revision

and Cryoablation

• Transplantation


Types of Congenital Heart Disease in

Montefiore ACHD Program

ASD, VSD, PDA

Coarctation

TOF, LVOTO

Ebstein’s, AVC, Conduits

Single Ventricle,

TGA,Transplantation

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