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HONG KONG CORE CARDIOLOGY CERTIFICATE COURSE
8 DECEMBER 2019
MODULE 5
TOF, TGA AND OTHER COMPLEX CONGENITAL HEART
DISEASES
Pak-Cheong CHOW Department of Paediatric Cardiology Queen Mary Hospital Hong Kong SAR
Congenital Heart Diseases
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Anatomy & Morphology Situs
Atrial morphology Septal defect
AV valve Ventricular morphology
Ventricular outflow Great vessels
Haemodynamics Acyanotic vs cyanotic Pressure vs Volume
Pulmonary plethora vs oligaemia SBF vs PBF
Presentation & Complications
Function
NYHA class
Surgery / Intervention
Medications / Devices
Genetics
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Conceptual issue
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Cure: “bring back to health; get rid of disease”
Repair: “to bring back something worn or damaged to a good condition”
Residua: “that which remains; that which is left over”
Sequel: that which follows or arises out of an earlier happening”
CHD could not be cured Chronic (life-long) illness with paediatric-onset
Timeline Approach
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TETRALOGY OF FALLOT 5
Apitz, Anderson & Redington, 2009
Normal RVOT TOF
TOF Morphology
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Etienne-Louis Arthur Fallot Contribution à l'anatomie pathologique de la maladie bleue (cyanose cardiaque). Marseille médical 1888; 2: 77-93
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Bertranou, Blackstone, Hazelrig & Kirlin, Am Heart J, 1978
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35% died before 1 yo
50% before 3yo
70% before 10yo
95% before 40y
Most common cyanotic congenital heart disease ~ 1 in 3,500 live births 7-10% of all congenital heart diseases
Tetralogy of Fallot (TOF)
TOF Repair
Karl 2012
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Trans-annular patch
Trans-atrial
Trans-pulmonary Trans-ventricular Reparative surgery:
- Closure of VSD (1)
- Relief of right ventricular outflow obstruction (2)
Cyanosis abolished
Survival rates by 32 - 36 years: 85-86%
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Late outcome
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serious adverse events: death, heart failure admission, ventricular arrhythmia and cardiac transplant
all adverse events: serious adverse event, endocarditis, atrial arrhythmia, defibrillator and pacemaker implantation
Repaired Tetralogy of Fallot – long-term problems
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Repaired TOF – Pulmonary regurgitation
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P1/2t <100ms PR index <0.77 D/S VTI (PW)
Repaired TOF – Pulmonary regurgitation
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Shinebourne et al 2006
PRF ≤25%: mild
25-40%: moderate ≥40%: severe
CMRI: i-RVEDV i-RVESV
RVEF cRVEF
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Singh, H. S. et al. (2013) , Nat. Rev. Cardiol. doi:10.1038/nrcardio.2013.127
Pulmonary Valve Replacement
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Tweddell et al, Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann, 2012;15:27-33
Operative risk Cardiopulmonary bypass RVOT reconstruction Concomitant surgery
Non-operation Vascular access RVOT morphology-dependent Coronary artery compression
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Mean difference Standard error p-value
Indexed RVEDV (ml/m2) - 62.73 2.59 <0.001
Indexed RVESV (ml/m2) - 38.09 2.42 <0.001
Non-corrected RVEF (%) + 1.00 0.86 0.24
Corrected RVEF (%) + 21.26 2.91 <0.01
Indexed LVEDV (ml/m2) + 6.70 0.68 <0.001
Indexed LVESV (ml/m2) + 1.44 0.99 0.147
LVEF + 1.82 0.66 0.006
QRS duration (ms) - 2.86 1.39 0.039
NYHA class - 0.86 0.10 <0.001
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Impact of PVR – NYHA , QOL , CPX
Discigil et al, JTCVS 2001 NYHA
QOL
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Impact of PVR – Survival, VT, SCD
Survival, VT, SCD – no significant difference between PVR and control
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Risk of PVR – Valve dysfunction, RV size and function,
Redo / Reintervention
Survival Free from second Redo-PVR
Freedom from reintervention or SVD
5 yr 94.8±3.1% 94.5±3.1% 89.2±4.7%
10 yr 83.7±8.0% 58.8±11.9% 32.0±13.3%
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PVR – benefit vs risk
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PVR – Indications
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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease
PRF ≥ 25%:
RVEDVI ≥ 160ml/m2
RVESVI ≥ 80ml/m2
RVEF <45% / LVEF <55%
RVSP ≥ 2/3 systemic (RVOTO)
Progressive objective CPET
Symptom due to PR
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Repaired TOF – Sudden Death & VT
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TOF & Aortic Disease
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Aortic regurgitation in adults after surgical repair of tetralogy of Fallot.
Trojnarska O, Siwińska A, Mularek-Kubzdela T, et al. Kardiol Pol. 2003
Aortic root disease in tetralogy of Fallot. Tan JL, Gatzoulis MA, Ho SY. Curr Opin Cardiol. 2006.
Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy.
Niwa K. Int J Cardiol. 2005
Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY. Circulation. 2005
Arterial haemodynamics in patients after repair of tetralogy of Fallot: influence on left ventricular after load and aortic dilatation.
Senzaki et al. Heart. 2008.
TOF & Aortic Dissection
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Aortic dissection late after repair of tetralogy of Fallot. Kim, et al. Int J Cardiol 2005
Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging.
Rathi VK, et al. Int J Cardiol 2005.
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IV-1 Pulmonary Valve Replacement [PVR] for moderate (PRF >25%) or severe (PRF >40%) pulmonary regurgitation • Symptom [include dyspnea, chest pain, and/or exercise intolerance referable to PR or
otherwise unexplained] • Asymptomatic patients with any 2 of the following:
• Mild or moderate RV or LV systolic dysfunction [RVEF <45%, LVEF <55%] • Severe RV dilation (RVEDVI ≥160 mL/m2, or RVESVI ≥80 mL/m2, or RVEDV ≥2x
LVEDV) • Progressive reduction in objective exercise tolerance
• Symptomatic or sustained atrial or ventricular tachyarrhythmia related to RV dilation • Significant [moderate to severe] tricuspid regurgitation [concomitant tricuspid valve
surgery needed] • Residual lesions requiring surgical interventions IV-2 Repair of residual VSD: • when Qp/Qs > 1.5:1 • when significant LV volume overload IV-3 RVOT reconstruction [including PVR] for residual pulmonary stenosis: • RVSP ≥ two-third of the systemic pressure • Peak-to-peak instantaneous echocardiography gradient > 50mmHg • Progressive and/or severe dilation of RV • Significant RV dysfunction • Not amenable by catheter-based intervention IV-4 Aortic root surgery: • When aortic root dimension > 55mm [asymptomatic] • When aortic dissection is present IV-5 Aortic valve surgery for significant aortic valve regurgitation • when associated with symptom or progressive LV dilation / dysfunction
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TRANSPOSITION OF GREAT ARTERIES 27
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TGA – associated lesions: VSD, PS
Cohen et al, JASE 2016
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Associated lesions: Ventricular septal defect – 40-45% Pulmonary stenosis [LVOT obstruction] – 25% Coarctation of aorta – 5% Pulmonary hypertension
Anderson & Weinberg, 2005
Transposition of Great Arteries
Without interventions poor survival
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Transposition of Great Arteries – Surgery
Anatomical repair Arterial switch Rastelli operation
Physiological repair Atrial switch
[Senning , Mustard]
Nagre, J Cardiovasc Med Cardiol 2016
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Survival: Atrial switch vs ASO
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TGA – Atrial Switch Opertion (Senning / Mustard)
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Late systemic RV dysfunction: 11-37% (Dos et al 2005, Khairy et al 2005, Moons et al 2004, Sarkar et al 1999, Wilson et al 1998)
TGA Atrial Switch: Systemic RV
(Roos-Hesselink et al 2004)
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Progressive decline of ventricular function and clinical condition
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Mechanisms:
Anatomic RV: unfavourable geometry
Pre-operative cyanosis & peri-operative damage
Perfusion mismatch
Tricuspid regurgitation (cart or horse?)
Neurohumoral activation
Diminished cardiac reserve (baffles)
Arrhythmias
Dys-synchrony
TGA Atrial Switch: Systemic RV
Kantor & Redington, 2010
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ECG: JR, IART
TGA Atrial Switch: Atrial arrhythmia
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TGA s/p Atrial Switch: Arrhythmia
TGA s/p Atrial Switch:
Arrhythmia & Sudden cardiac death
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TGA s/p Atrial Switch: Baffle complications
Dilated PA
Pulmonary venous baffle
Occluder at baffle leak
IVC channeling to ‘LA’
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TGA s/p Atrial Switch: QMH FU Recommendations
43 Modality Frequency recommended
Parameters / Variables
Clinical Every visit Exercise tolerance [NYHA class] Orthopnoea / paroxysmal nocturnal dyspnea Palpitations, syncope Pan-systolic murmur of tricuspid regurgitation Signs of SVC / IVC obstruction
ECG At least yearly Rhythm, rate, QRS duration, CXR Baseline, and then
clinically indicated
Cardiothoracic ratio
Echocardiography Every 1 – 3 years [more frequent if systemic RV dysfunction and / or Tricuspid regurgitation present]
Systemic RV size and function Tricuspid regurgitation Baffle leak / stenosis Subpulmonary LV size and function Pulmonary (LVOT) stenosis Feature of pulmonary hypertension: LV size, mitral regurgitation gradient, pulmonary regurgitation gradient
Cardiac MRI ^ Baseline, and then every 2-3 years
Systemic RV and subpulmonary LV volumes and function Baffle leak / stenosis LGE for myocardial fibrosis
Cardiopulmonary exercise test
Baseline, and when subjective exercise intolerance
Exercise duration Peak VO2 (absolute value and percentage of predicted) Heart rate response – chronotropic competence Exercise-induced arrhythmia
Modality Indication Parameters / variables
Holter Baseline, and then every 2-3 years, and when clinically indicated: palpitations, syncope, ECG abnormalities
Rhythm Heart rate Atrial / ventricular arrhythmia
Diagnostic cardiac catheterization
Symptomatic heart failure, Imaging evidence of significant systemic RV dysfunction; New-onset of arrhythmia; Attempted catheter-based interventions e.g. balloon dilation of baffle stenosis, occlusion of baffle leak
Systemic RV EDP, systolic function Tricuspid regurgitation Baffle stenosis or leak (shunting) Subpulmonary LV Systolic pressure & EDP, systolic function LVOT (pulmonary) obstruction
Electrophysiological study
History of unexplained syncope History of cardiac arrest / sustained atrial or ventricular tachyarrhythmia Ablation / ICD implantation to be considered at the same setting
Sinus node function Conduction study VT study
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Arterial switch operation (ASO) - Sequelae
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TGA s/p ASO: Pulmonary Stenosis
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Reported incidence of 1–42% Freedom from PS at 23 years: - Of ≥36mmHg: 34.0±18.0% - Of ≥20mmHg: 17.7±9.6%
Ao
PA
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TGA s/p ASO: Neo-aortic valve and Root
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Ao
TGA s/p ASO: Coronary artery
Year Post-ASO Freedom from coronary events
1 92.7%
5 92.5%
10 91%
15 88.2%
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TGA s/p ASO: QMH Recommendation
58 Modality Frequency recommended Parameters / Variables
Clinical Every visit Exercise tolerance [NYHA class] Palpitations, syncope Chest pain (ischaemic)
Coronary risk factors Baseline, then every 2-3 years
Blood pressure Body weight and height Fasting lipid profile + glucose Smoking status
ECG At least yearly Rhythm, rate, ST changes
CXR Baseline, then when clinically indicated
Cardiothoracic ratio Differential flow pattern
Echocardiography * Every 1 – 3 years [more frequent if aortic root dilation present]
LV size and function, regional wall motion abnormalities Aortic root size, aortic regurgitation Pulmonary artery stenosis Residua of other cardiac anomalies: ASD, VSD, CoA
Cardiac MRI ^ Baseline, then every 3 years
RV and LV volumes and function Pulmonary artery stenosis Aortic root dimension Myocardial perfusion study and LGE for myocardial fibrosis
Cardiopulmonary exercise test
Baseline, then every 3 years or when subjective impaired exercise tolerance
Exercise duration Peak VO2 (absolute value and percentage of predicted) Exercise-induced arrhythmia Exercise-induced myocardial ischemia
Holter Baseline or when arrhythmia suspected
Rhythm Heart rate Atrial / ventricular arrhythmia
Modality Indications
CT coronary angiogram When myocardial ischemia is suspected or evident from other investigations
Diagnostic cardiac catheterization
When coronary artery angiography is needed When non-invasive investigations are inadequate When further surgery / intervention is being considered
Electrophysiological study♯
History of unexplained syncope History of cardiac arrest / sustained atrial or ventricular tachyarrhythmia [Ablation / ICD implantation to be considered if no treatable haemodynamic lesion]
Complex CHD 60
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Classification of congenital heart diseases
ACHD of Great Complexity
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Complexity of Congenital heart disease: -Simple -Moderate -Great
Fontan Operation or Fontan/Kreutzer Operation
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A palliative surgical procedure used in children with complex congenital heart diseases Diverting the systemic venous blood from the right atrium to the pulmonary arteries without passing through the morphologic pulmonic ventricle
Initially described in 1971 by Dr Fontan and Dr Kreutzer separately as a surgical treatment for tricuspid atresia
APC LT ECFC
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Fontan Operation – Survival
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Single Ventricle Surgery Timeline
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Pulmonary artery band
Single Ventricle
Systemic-to-Pulmonary Shunt
Fontan Operation
Cavopulmonary connection
Increased PBF
Decreased PBF
Neonatal - early infancy
Infancy Childhood
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Single Ventricle – Ventricular Dysfunction
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Before CPC / Fontan:
• Volume overload
• Hypoxia
• Ventricular dilation and overgrowth
After CPC / Fontan:
• the ventricle is unloaded to 50–70% of normal for BSA, but to 50% if significant overgrowth had occurred perform at its closing volume
• Pulmonary vascular resistance limits ventricular filling
• Progressive diastolic dysfunction + Reduced contractile function, impaired ventricular-arterial coupling
CPC
Fontan Circulation
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Cardiac output: - Ventricular function - PVR
Age ↑: - ↓Ventricular function - ↓PVR Cardiac output ↓ Fontan Failure
Fontan Circulation
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“Fontan failure”: • Variably defined • Constellation of
complications due to inefficient circulation with or without myocardial failure.
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Fontan Operation – Arrhythmia
IART in Fontan
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Fontan Associated Liver Disease (FALD)
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Abnormalities of liver biomarkers: 75% Coagulation abnormalities: 25 -64% Histological cirrhosis: 58% Radiological abnormalities: 91% Hepatic abnormality is common and almost universal
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Fontan Associated Liver Disease (FALD)
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Fontan Associated Liver Disease (FALD)
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Fontan FU – QMH
75 Modality Frequency recommended Parameters / Variables
Clinical Every visit Exercise tolerance [NYHA class] Palpitations, syncope Ascites SpO2
ECG At least yearly Rhythm, rate, PR interval Any atrial arrhythmia (especially IART)
CXR Every 1 – 3 years Cardiothoracic ratio Pleural effusion
Echo^ Every 1 – 3 years -Ventricular size and function -Atrioventricular valve regurgitation -Ventricular outflow obstruction -Aortic valve regurgitation -Intra-cardiac thrombus -Residual atrial shunting / fenestration [agitated saline injection may be needed] -Any obstruction of Fontan circuit [especially IVC-Conduit anastomosis]
Cardiac MRI Baseline (≥ 10 years post-Fontan) and then Every 3 years
- SVC, IVC, Fontan pathway - for any stenosis - RA dimension [for atriopulmonary connection only] -Pulmonary artery anatomy, size and presence of stenosis -Ventricular size and function [+/- LGE presence and extent] -Presence of thrombus [intra-cardiac, Fontan circuit] -Pulmonary venous connections stenosis
Modality Frequency recommended Parameters / Variables
Cardiopulmonary exercise test
Baseline (≥ 10 years post-Fontan); and then every 2-3 years or when subjective impaired exercise tolerance
Exercise duration Peak VO2 Oxygen saturation VE/VCO2 slope Exercise-induced arrhythmia
Holter Baseline (≥ 10 years post-Fontan) ); and then every 2-3 years
Rhythm Heart rate Atrial / ventricular arrhythmia
Blood Every visit PT, INR (if patient is on warfarin)
Yearly Liver enzymes♯: ALT, AST, ALP, GGT Serum albumin Bilirubin: total and direct Renal biochemistry: Na, K, Ur, Cr, eGFR CBC Ca, PO4, PTH, Vitamin D assay@ TSH / fT4 (patients on amiodarone)
Baseline (≥ 10 years post Fontan) and at least yearly
Alpha-fetoprotein♯
USG Abdomen♯ Baseline (≥ 10 years post Fontan) and then every 1-2 years
Liver size and echogenicity; Any focal hepatic lesion liver Hepatic elastography
CT Liver (contrast, triphasic); or MRI Liver
If there is hepatic lesions or elevated AFP or abnormal liver enzymes
Hepatic size, cirrhotic change, characteristics of hepatic nodule
Cardiac Catheterization
Baseline (≥ 10 years post-Fontan); and then when clinically indicated
Pulmonary vasodilators in Fontan
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Wang et al.
Mean difference Standard error p-value
mPAP (mmHg) -2.25 -3, -1.5 <0.001
Peak VO2 (ml/min/kg) 1.42 +0.21, 2.63 0.02
6MWD (m) 134 86.1, 181.9 <0.001
NYHA -0.39 -0.72, -0.05 <0.001
Mortality 0.35 0.09, 1.34 0.126
2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease
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Fontan Failure – Surgical Management
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Fontan conversion: conversion of atriopulmonary connection to extra-cardiac total cavopulmonary connection
atriopulmonary connection
extra-cardiac total cavopulmonary
connection
Fontan Failure – Surgical Management
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Interventions / Surgery for Fontan Failure: • Fenestration • Takedown of Fontan circuit • Ventricular assist devices • Heart Transplantation
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Fontan Failure – Negative Pressure Ventilation
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Iron lungs for Polio patients
Cyanotic Heart Disease
MULTI-SYSTEM INVOVLEMENT !
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Cyanotic Heart Disease: Hb & iron status
Secondary erythrocytosis [ polycythemia]
Chronic hypoxaemia erythropoeitin erythrocytosis
Compensated :
an equilibrium with stable Hb & haematocrit in an iron-replete state
absent or mild hyperviscosity symptom
Decompensated :
failed to establish an equilibrium with rising haematocrit
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JACC 1996
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*microcytosis : MCV <82
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Phlebotomy in Cyanotic Heart Disease
Vongpatanasin et al 1998 Warnes et al AHA 2008
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Management issue in cyanotic patients
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Guidelines for the Management of GUCH / ACHD
CANADIAN CARDIOVASCULAR SOCIETY 2009 CONSENSUS CONFERENCE ON THE GUIDELINES FOR THE MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE Ariane Marelli (Section Editor), Luc Beauchesne (Section Editor), Annie Dore (Section Editor), Marla Kiess (Section Editor), Omid Salehian (Section Editor), Timothy Bradley , Jack Colman, Michael Connelly , Louise Harris , Paul Khairy , Seema Mital , Koichiro Niwa , Erwin Oechslin, Nancy Poirier , Markus Schwerzmann , Dylan Taylor , Isabelle Vonder Muhll , Helmut Baumgartner, Lee Benson , David Celermajer, Matthias Greutmann , Eric Horlick, Mike Landzberg, Folkert Meijboom , Barbara Mulder, Carole Warnes , Gary Webb
https://www.onlinecjc.ca/article/S0828-282X(10)70352-4/pdf
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https://watermark.silverchair.com/ehq249.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAAAmswggJnBgkqhkiG9w0BBwagggJYMIICVAIBADCCAk0GCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQMOGicq4YzMpuwRsRiAgEQgIICHhZUSCPHaSAoJwRIPYwp1rzKsVDU-A0gCXpZS-fEV1RSDT-M3NXvZBF68HNKw83KZILD4VkfiHA64oHbTO4uS-VoZcA-kqQoWilJnag2Z6GNRoafPebR2f01iZuRzZeJSfrIDkyzrwCnXPc4B9ANZTN_4gLftIDDzr48epyuBA7xVF_ZBRRe3Fv0XdpOGhUw0MuxSzZQuz2VFb_aT65fVkADgdx3WUfwK_v275UF_6yfsGr9R-nhxHcFxdSWHB7Xuw69FLn5Bbwb6wvPsCncXl9OE2qntiyAXUXreOYDb1PyUqMmYfRqfGfp9LPu1qwjWFldk5LjipGQZOkNszKy3433TV3To3Z8FBDBj_rUzxtyh_qQsygsMaCSXht9uME_MTPm17SVIf76K4aLr9OwkTQD3RTniw2TmwK9Dm732fV-I7WMV3Xfkee0V7REAUFYcpFwOmhnjYY8Ks0jCg54agLs7wDxWCGNSidJEWeRWs-LZ1B7RcOPLA2JIF6ukhOVI5XoCR8gcoOs1a1dPjAn0ysiLPFQKi5Uwr5nTZnlOjIB_62GMnOZwGfFqfPY0zO8m7TTKTBLgEMv9ylV4h3ajyJxnQ1tE3hveI6TlyveSn16a9VVkNNcy0hctKESZF5LNZI_M4UW4hbOvM1riswnjX6OpLGx2BZRMupfZcRfqwZmvam83r8mnz3-9z9dDnZH9cuwRzp3ghXdzlkM150Z
https://www.ahajournals.org/doi/pdf/10.1161/CIR.0000000000000603
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