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

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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

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CHDs that do not Require Operation

• Functionally normal bicuspid aortic valve
• Mild pulmonary valve stenosis
• Small interatrial connection
• Small VSD!!!
• Uncomplcicated L-transposition

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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

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Conditions with Specific Interest

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

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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

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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

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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

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Detechable Coil
Amplatzer Plug
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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

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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

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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

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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

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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

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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

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MRI

• Right ventricle size
• Right ventricle ejection fraction

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MRI II

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

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Trace PR
Severe PR
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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

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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
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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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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

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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

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Stent implantation in LPA stenosis in Fontan
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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

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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

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Heart Failure in ACHD

• Chronic Treatment
• ACE inhibitors
• Diuretics
• ?-Blockers
• Aldosterone antagonism
• Digitalis
• Acute Treatment
• Dopamine, dobutamine
• Milrinone
• Biventricular pacing

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Sudden Cardiac Death
Adults with CHD
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Sudden Death

• Surgically repaired Tetralogy of Fallot
• Atrial switch operation D-Transposition
• Aortic stenosis
• Coarctation of aorta

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