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Management%20of%20Adult%20Congenital%20Heart%20Disease

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Management of Adult Congenital Heart Disease Alpay eliker MD. Hacettepe University Department of Pediatric Cardiology – PowerPoint PPT presentation

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Title: Management%20of%20Adult%20Congenital%20Heart%20Disease


1
Management of Adult Congenital Heart Disease
  • Alpay Çeliker MD.
  • Hacettepe University
  • Department of Pediatric Cardiology

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Major Issues in ACHD
  • Primary Operation or intervention
  • Reoperation or reintervention
  • Heart Failure
  • Arrhythmia
  • Sudden Death

5
CHDs that do not Require Operation
  • Functionally normal bicuspid aortic valve
  • Mild pulmonary valve stenosis
  • Small interatrial connection
  • Small VSD!!!
  • Uncomplcicated L-transposition

6
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

7
Conditions with Specific Interest
  • Aortic coarctation
  • Left-to-right shunts
  • Repaired tetralogy of Fallot
  • Atrial switch procedures
  • Fontan circulation

8
Coarctation of Aorta
  • Major Concerns
  • Residual hypertension, aneursym formation,
    recoarctation
  • SurvivalHypertension
  • Hypertension
  • Operation between 20-40 yrs may result 80
    residual hypertension.
  • Operation age
  • 20-40 yrs 25 yr survival 75
  • gt40 yrs 15 yr survival 50

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Left-to-Right Shunt Lesions
  • Major problem is pulmonary vascular disease
  • Unrestricted VSDs 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

11
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

12
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 PDAs can be closed surgically

13
Detechable Coil
Amplatzer Plug
14
Cardiac SurgeryFrequent Complications in some
CHDs
  • 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

15
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

16
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

17
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

18
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

19
ECG and Holter
  • Positive late potentials and wide QRS (gt180 msec)
    is well-known risc factors associated with
    ventricular tacyhcardia
  • Ventricular ectopic beats and nonsustained
    monomorphic VT are other factors related with SMVT

20
MRI
  • Right ventricle size
  • Right ventricle ejection fraction

21
MRI II
  • Degree of Pulmonary regurgitation
  • Determining fibrotic and aneursymatic areas
  • Time consuming

22
Trace PR
Severe PR
23
Cardiac EPS in Fallot Patients
  • Common AV conduction disturbance
  • Common atrial flutter
  • Infrequent inducible SMVT
  • Ablation in tolerated VTs
  • ICD in fast VT or cardiac arrest

24
Hacettepe Experience EPS in Fallot Patients

Result Patient No
NORMAL 12 40
SSS 1 3.3
AVCD 3 10
SSSAVCD 3 10
NS AFL 2 6.7
SSSAFL 1 3.3
S AFL 2 6.7
Fibro-flutter 1 3.3
SSSNSVT 2 6.7
NSVT 3 10
TOTAL 30 100
30 patients after 11 years tetralogy repair
25
Reoperation in Tetralogy
  • Residual VSD with a QP/QSgt1.5
  • Residual PS with RV/LVgt2/3
  • 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

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27
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

28
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

29
Stent implantation in LPA stenosis in Fontan
30
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

31
Coronary sinus angio
Coronary sinus lead in place
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  • 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

34
Heart Failure in ACHD
  • Chronic Treatment
  • ACE inhibitors
  • Diuretics
  • ?-Blockers
  • Aldosterone antagonism
  • Digitalis
  • Acute Treatment
  • Dopamine, dobutamine
  • Milrinone
  • Biventricular pacing

35
Sudden Cardiac Death
Adults with CHD
36
Sudden Death
  • Surgically repaired Tetralogy of Fallot
  • Atrial switch operation D-Transposition
  • Aortic stenosis
  • Coarctation of aorta

37
Tesekkürler
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