Rhythm ProblemsRhythm ProblemsAtrioventricular Septal Atrioventricular Septal

DefectDefectAlpay Çeliker MD.Alpay Çeliker MD.

Hacettepe UniversityHacettepe University

Department of Pediatric Department of Pediatric Cardiology Cardiology

Conduction System in Conduction System in AVSDAVSD

Normal HeartNormal Heart AV node is located in AV node is located in

the triangle of Kochthe triangle of Koch

AV Septal DefectAV Septal Defect AV node is located AV node is located

posteriorlyposteriorly

ECG in AVSDECG in AVSD Prolonged PR intervalProlonged PR interval Left axis deviation and Left axis deviation and

counterclockwise frontal plane loopcounterclockwise frontal plane loop

1. Elongation of the anterior division of LBB

2. Anomalous development of anterior division of LBB

3. Interruption of the anterior division by anomalous insertion of chorda tendinea

ECG in AVSD IIECG in AVSD II Incomplete RBBB pattern in 84 %Incomplete RBBB pattern in 84 % Evidence of atrial enlargement 54 %Evidence of atrial enlargement 54 % Q wave in VQ wave in V66 84 % 84 % Additional factors that influences Additional factors that influences

ECGECG Size of ASD or VSDSize of ASD or VSD Amount of mitral and tricuspid Amount of mitral and tricuspid

regurgitation regurgitation Pulmoner vascular resistancePulmoner vascular resistance Associated defectsAssociated defects

Mechanisms of Mechanisms of ArrhythmiasArrhythmias

Abnormalities inherent to Abnormalities inherent to malformationmalformation

Hemodynamic and hypoxic stress Hemodynamic and hypoxic stress upon heartupon heart

Sequela of reparative surgerySequela of reparative surgery Residual hemodynamic problemsResidual hemodynamic problems

Rhythm Problems in Rhythm Problems in AVSDAVSD

Preoperative Rhythm ProblemsPreoperative Rhythm Problems

Perioperative Rhythm ProblemsPerioperative Rhythm Problems

Postoperative Rhythm ProblemsPostoperative Rhythm Problems

Preoperative Preoperative ArrhythmiasArrhythmias

Acquired atrial tachyarrhythmiasAcquired atrial tachyarrhythmias Late operationLate operation Atrial fibrillation may be seen 20 % and Atrial fibrillation may be seen 20 % and

causes clinical deteriorationcauses clinical deterioration AV blockAV block

Perioperative Perioperative ArrhythmiasArrhythmias

Junctional Ectopic TachycardiaJunctional Ectopic Tachycardia

AV BlockAV Block

AVSD & AVSD & Perioperative Perioperative ArrhythmiasArrhythmias

With With arrhythmiarrhythmiaa

No No arrhythmarrhythmiaia

AVSD AVSD PatientsPatients

2121 2424

Mean ageMean age 0.9 0.9 ±± 2.1 2.1 1.4 1.4 ±± 1.9 1.9

IncompletIncomplete resulte result

9/119/11 2/112/11

Pfammater et al. J Thorac Cardiovasc Surg 2002; 123: 258-262

AVSD with ArrhythmiaAVSD with ArrhythmiaN=21N=21

AJRN= 8

SSSN=7

CAVBN=1

A FlutterN=1

JETN=1

Ectopic BeatsN=1

Higher ACC, ECC time Higher ACC, ECC time and TpI levels and TpI levels

Junctional Ectopic Junctional Ectopic TachycardiaTachycardia

ventricular rateventricular rate Loss of AV synchronyLoss of AV synchrony

Cardiac OutputCardiac Output Adrenergic ToneAdrenergic Tone Heart RateHeart Rate

JET: ECG DiagnosisJET: ECG Diagnosis QRS configuration is similar to sinus or QRS configuration is similar to sinus or

atrial paced beatsatrial paced beats Rapid ventricular rate Rapid ventricular rate > or => or =to atrial rateto atrial rate Dissociated atrial activityDissociated atrial activity or retrograde or retrograde

1:1 conduction or Wenckebach1:1 conduction or Wenckebach Failure to respond adenosine, Failure to respond adenosine,

overdrive pacing or cardioversionoverdrive pacing or cardioversion Warm-up phenomenon Warm-up phenomenon

Perioperative Perioperative JETJET

Increased duration of Increased duration of postoperative postoperative ventilation and CICU ventilation and CICU staystay

incidence with incidence with ventricular muscle ventricular muscle band resection, higher band resection, higher cardiopulmonary cardiopulmonary bypass temperature, bypass temperature, transatrial RVOTO transatrial RVOTO relief relief

Postop JETN=37/343

10 %

FallotN= 25/114

21.9 %

AVSDN=6/5810.3%

VSDN=6/161

3.7 %

De-Leval group. J Thorac Cardiovasc Surg 2002; 123: 624-630.

RVOT resectionRVOT resectionMore importantMore importantThan VSD closureThan VSD closure

Treatment in Postop JETTreatment in Postop JET

General MeasuresGeneral Measures Optimize sedation/hemodynamicsOptimize sedation/hemodynamics Correct feverCorrect fever CatecholaminesCatecholamines

AV SynchronyAV Synchrony Class I and II AADClass I and II AAD Hypothermia + ProcainamideHypothermia + Procainamide IV AmiodaroneIV Amiodarone

Treatment Modalities in JETTreatment Modalities in JET

0

10

20

30

40

50

Cat Fever Sync Dig IB, I I ,

IV

Proc Hypo Comb

Ineff ective

Poss. Eff ective

Eff ective

Walsh ED, et al. J Am Coll Cardiol, 1997; 29: 1046-1053

Walsh ED, et al. J Am Coll Cardiol, 1997; 29: 1046-1053

IV AMIODARONEN=11

SECONDARY THERAPYN=5

HYPOTHERMIAN=3

HYPO&PROCN=1

CAT REDUCTIONN=1

INITIAL THERAPYN=6

SUCCESS 10/11 SUCCESS 10/11

Laird et al. Pediatr Cardiol 2003; 24: 133-137.

JETJET

Optimize hemodynamic variables, respiration, electrolytes, sedation, fever control

Discontinue Catecholamines

Atrial PacingAtrial Pacing*

JT rate > 200 bpm orPersistent rate 170-200 bpm

AADHypothermia

Atrial pace slightly faster than JET from epicardial wires or Esophagus*not an isolated therapy if JET not an isolated therapy if JET

rate >200 bpmrate >200 bpm

AMIODARONEAMIODARONEPROCAINAMIDEPROCAINAMIDE

Core temperature 33-350 C using posterior cooling blanketunder sedation, mechanic ventilation and paralysis

AV BlokAV Blok

Postoperative AV block has been Postoperative AV block has been reported to occur in 0-3.5 %.reported to occur in 0-3.5 %.

50 % of postoperative AV block 50 % of postoperative AV block resolves within the 8 days.resolves within the 8 days.

Permanent pacemaker implantation Permanent pacemaker implantation after 15 days is prudent.after 15 days is prudent.

Postop CAVBPostop CAVBTemporary PacingMonitor 7-10 days

NSR or 1o AVB

Type 1, 2o AVB

NSR, 1NSR, 1oo AVB, AVB, RBBB, LADRBBB, LAD

Type II, 2o AVB

30 AVB

EPSEPS

Permanent PacemakerPermanent Pacemaker

InfraHisianInfraHisianBlockBlock

Cardiac Pacing in AVSDCardiac Pacing in AVSD

SSS & Good AV Conduction: AAIRSSS & Good AV Conduction: AAIR SSS & AV Conduction Disturbance: DDDSSS & AV Conduction Disturbance: DDD AV Block: DDDAV Block: DDD Small Child ( <15 kg): Epicardial implantSmall Child ( <15 kg): Epicardial implant SSS or AV Block with Atrial Tachycardia: SSS or AV Block with Atrial Tachycardia:

Antitachycardia PMAntitachycardia PM Late Recovery of AV Conduction: 10 %Late Recovery of AV Conduction: 10 %

Perioperative and Perioperative and Longterm ArrhythmiasLongterm Arrhythmias

Arrhythmia Arrhythmia TypeType

Perioperative Perioperative

N-%N-%Long-termLong-term

N-%N-%TotalTotal

N-%N-%

SVTSVT 18 (5)18 (5) 12 (4)12 (4) 24 (7)24 (7)

At FibrillationAt Fibrillation 7 (2)7 (2) 21 (6)21 (6) 25 (8)25 (8)

At FlutterAt Flutter 7 (2)7 (2) 6 (2)6 (2) 13 (4)13 (4)

AV BlockAV Block 5 (2)5 (2) 4 (1)4 (1) 9 (3)9 (3)

Premature Premature SVB & VBSVB & VB

22 33 3 (1)3 (1)

El-Najdawi et al. J Thorac Cardiovasc Surg 2000; 19: 980-90.

Atrial ArrhythmiasAtrial Arrhythmias Atrial FibrillationAtrial Fibrillation Isthmus Dependent Atrial Flutter (IDAF)Isthmus Dependent Atrial Flutter (IDAF) Intraatrial Reentrant Tachycardia (IART)Intraatrial Reentrant Tachycardia (IART)

Risk of Atrial Reentry Risk of Atrial Reentry TachycardiaTachycardia

High Risk (> 10 %)High Risk (> 10 %) Fontan palliationFontan palliation Mustard-SenningMustard-Senning Total correction for Fallot or DORVTotal correction for Fallot or DORV Sinus venosus or late repair of ASD IISinus venosus or late repair of ASD II

Moderate Risk (1-10 %)Moderate Risk (1-10 %) TAPVRTAPVR Ebstein’s anomalyEbstein’s anomaly Complete AVSDComplete AVSD Mitral valve replacementMitral valve replacement

Low Risk (<1 %)Low Risk (<1 %) Early repair ASD IIEarly repair ASD II VSD repairVSD repair

IART or IDAF

Therapy Of Atrial Therapy Of Atrial ArrhythmiasArrhythmias

DC Cardioversion DC Cardioversion AAD: Class Ic, IIIAAD: Class Ic, III AAD & PMAAD & PM Transcatheter RF AblationTranscatheter RF Ablation Arrhythmia SurgeryArrhythmia Surgery

Correction of residual defects Correction of residual defects Surgical ablation Surgical ablation Maze procedureMaze procedure

Transcatheter AblationTranscatheter Ablation Atrial Fibrillation: His Atrial Fibrillation: His

Ablation Ablation IDAF and IART: IDAF and IART:

Creation of Block Line Creation of Block Line Use of saline irrigated Use of saline irrigated

catheterscatheters Use of 3D Anatomic Use of 3D Anatomic

MappingMapping

Efficacy

ApplicationApplicationProblemsProblems

Treatment FailuresAdverse Effects

CostAAD

RFA ATP

ArrhythmiaArrhythmiaSurgerySurgery

Treatment Methods in Atrial Tachyarrhythmias

Sudden Death and AVSDSudden Death and AVSD

Cardiac DefectCardiac Defect Incidence 1000 Incidence 1000 pt/yearpt/year

Aortic StenosisAortic Stenosis 5.45.4

D-TGAD-TGA 4,94,9

Fallot TetralogyFallot Tetralogy 1,51,5

Aortic CoarctationAortic Coarctation 1,31,3

AVSDAVSD 0,90,9