Management of Adult Congenital Heart

Disease

Alpay Çeliker MD.Hacettepe University

Department of Pediatric Cardiology

Congenital Heart Defects in Newborn8%

Cardiac Operation60 %

Possibility to reach adulthood85%

Major Issues in ACHD

Primary Operation or intervention

Reoperation or reintervention Heart Failure Arrhythmia Sudden Death

CHD`s that do not Require Operation

Functionally normal bicuspid aortic valve

Mild pulmonary valve stenosis Small interatrial connection Small VSD!!! Uncomplcicated L-

transposition

Types of Surgery for Congenital Heart Disease

Curative: No postoperative residua, sequelae, or complications

Reparative: Anatomic repair or reconstruction with obligatory postoperative residua or sequelae

Palliative: Basic morphologic anomaly is neither repaired or reconstructed

Reoperative: Late reoperation after reparative or palliative surgery

Organ transplantation

Conditions with Specific Interest

Aortic coarctation Left-to-right shunts Repaired tetralogy of Fallot Atrial switch procedures Fontan circulation

Coarctation of Aorta Major Concerns:

– Residual hypertension, aneursym formation, recoarctation

Survival&Hypertension– Hypertension

•Operation between 20-40 yrs may result 80% residual hypertension.

– Operation age •20-40 yrs 25 yr survival 75%•>40 yrs 15 yr survival 50%

Isolated Aortic Coarctation

Surgery Balloon Dilation

Stent Implantation

Dangerous!!!

Balloon Expandable StentsCovered Stents

Neointima FormationI. Method in Recoarc

Left-to-Right Shunt Lesions

Major problem is pulmonary vascular disease

Unrestricted VSD`s rarely reach adult age without PAH

PDA and ASD can be successfully managed by transcatheter methods

Small VSD should be followed clinically, unless AVP and Aortic regurgitation

May result with Eisenmenger syndrome

ASD Closure ASD II can be

closed by interventional methods.

Two major problem may contribute– Pulmonary

vascular disease– Decreased left

ventricle compliance

– Balloon occlusion test should be performed

PDA Closure Small PDA Endarteritis Moderate size PDA Left ventricle

and atrial dilation Large PDA Pulmonary vascular

disease Transcatheter closure avoids from

general anasthesia, thoracotomy Large PDA’s can be closed

surgically

Detechable Coil

Amplatzer Plug

Cardiac Surgery&Frequent Complications in some CHD’s Total correction for tetralogy of Fallot

– Atrial and ventricular arrhythmias– Pulmonary regurgitation

Atrial switch procedures for D-TGA– Atrial arrhythmias, Sick sinus syndrome– Right ventricle failure– Baffle obstruction

Fontan circulation– Atrial arrhythmias, sick sinus syndrome– Protein losing enteroptahy– Conduit obstruction

Late Complications after Tetralogy Repair

Endocarditis Aortic Regurgitation LV Dysfunction Residual RVOT

Obstruction Residual Pulmonary

regurgitation RV Dysfunction Exercise Intolerance Heart Block Atrial Fl and Fib Sustained Ventricular

Tachycardia Sudden Cardiac Death

Total Correction and Arrhythmias

Ventricular arrhythmias– Late operation\Long follow-up

duration– Residual VSD– Severe Pulmonary regurgitation

Atrial arrhythmias Sinus node and AV conduction

disorders

Risk Assessment ECHO

– Residual VSD, PS– Degree of Pulmonary& Tricuspid

Regurgitation– Right ventricle status

ECG– Prolonged QRS duration – Abnormal late potentials

Holter– Ventricular ectopy, NSMVT or SMVT

Exercise– Increased ectopy, VT

Invasive EPS MRI

ECHO It is helpful in

determining left ventricle function, residual VSD and residual PS

There is no concensus determining Pulmonary regurgitation with ECHO

Right ventricle ejection fraction can not be measured

ECG and Holter Positive late potentials and wide QRS

(>180 msec) is well-known risc factors associated with ventricular tacyhcardia

Ventricular ectopic beats and nonsustained monomorphic VT are other factors related with SMVT

MRI Right ventricle size

Right ventricle ejection fraction

MRI II

Degree of Pulmonary regurgitation

Determining fibrotic and aneursymatic areas

Time consuming

Severe PR

Trace PR

Cardiac EPS in Fallot Patients Common AV conduction

disturbance

Common atrial flutter

Infrequent inducible SMVT

Ablation in tolerated VT’s

ICD in fast VT or cardiac arrest

Hacettepe Experience: EPS in Fallot Patients

Result Patient No %

NORMAL 12 40

SSS 1 3.3

AVCD 3 10

SSS+AVCD 3 10

NS AFL 2 6.7

SSS+AFL 1 3.3

S AFL 2 6.7

Fibro-flutter 1 3.3

SSS+NSVT 2 6.7

NSVT 3 10

TOTAL 30 100

*

*: 30 patients after 11 years tetralogy repair

Reoperation in Tetralogy Residual VSD with a QP/QS>1.5 Residual PS with RV/LV>2/3 RVOT aneursyms Branch PS & Pulmonary regurgitation Severe pulmonary regurgitation with;

– Right ventricle enlargement– New onset tricuspid regurgitation– Ventricular tachycardia– Deteriorating exercise intolerance

Significant aortic regurgitation

Mustard & Senning Procedures

Right ventricle dysfunction– ACE inhibitors,

digitalis, diuretics Atrial flutter

– AA treatment, catheter ablation, antitachycardia pacemaker

Sick sinus syndrome– Brady pacing

Baffle obstruction– Surgery or

intervention

Fontan Circulation Arrhythmia: 41 % sustained IART

and many of them SSS findings Protein Losing Enteropathy (PLE) Ventricular Dysfunction Thromboembolism Conduit obstruction Pulmonary artery stenosis Pulmonary arterivenous fistulae Plastic bronchitis

Stent implantation in LPA stenosis in Fontan

Fontan & Arrhythmia

SSS or AV Block– Epicardial pacing– Pacing from

coronary sinus IART or atrial

flutter– DC cardioversion– AA drug therapy– Catheter ablation

with 3D mapping– Arrhythmia surgery

Coronary sinus angio

Coronary sinus lead in place

PLE– Diuretics– Supplemental albumin infusion– High protein and medium-chain

triglyceride intake– Oral steroids, heparin– Atrial fenestration

Thromboembolism: – Anticoagulation and antiplatelet

therapy Heart Failure

– Conversion to Cavopulmonary anastomosis

Heart Failure in ACHD Chronic Treatment

– ACE inhibitors– Diuretics -Blockers– Aldosterone antagonism– Digitalis

Acute Treatment– Dopamine, dobutamine– Milrinone

Biventricular pacing

Adults with CHD

Sudden Cardiac Death

Sudden Death

Surgically repaired Tetralogy of Fallot

Atrial switch operation D-Transposition

Aortic stenosis Coarctation of aorta