THE ROYAL COLLEGEEXAMINATION:CARDIOLOGY STUDY CARDS 2011: ADULT CONGENITAL HEART DISEASE (ACHD)
GRADING SYSTEM FORGUIDELINESClass of RecommandationI      Benefits >>> Risks                 Should performIIA    Benefits...
GENERALRECOMMEDNATIONSIn the introduction, the guidelines describe the organization of theCanadian Health Care system and ...
GENERALRECOMMEDNATIONSGenetic diagnosis may be useful for:      •    Prognosis      •    Complementary Examinations (other...
SHUNT LESIONS• Atrial Septal Defects (ASD’s)• Ventricular Septal Defects (VSD’s)• Endocardial Cushion Defects (atrioventri...
ASD’S                                                                    Complications                                    ...
ASD’SClass IIa & IIb recommendations*•   Closure may be indicated in the absence of RV    Enlargement in the following con...
ASD’SClass III recommendations* (i.e. don’t do it)•     NO closure if irreversible PH (refer to specialized center)       ...
Mild: Low Press              sPAP/Ao Syst <0.3 or      VSD’S                                                              ...
VSD’SClass IIa & IIb recommendations*•   Closure may be indicated if:     •   History of endocarditis (esp if recurrent)  ...
VSD’SClass III recommendations* (i.e. don’t do it)•   NO closure if irreversible PH (refer to specialized center)     • PA...
5 leaflets: R-AS, R-inf, Superior & Inferior bridging leaflet, L-lateral wall leaflet    AVSD’S                           ...
AVSD’S  Class IIa & IIb recommendations*  •        There are none!Partial:     Ventricular septum intact, 2 separate AV va...
AVSD’SClass III recommendations* (i.e. don’t do it)•   NO closure if irreversible PH (refer to specialized center)     • P...
PDA   Class I recommendations*       •    No intervention for Silent PDA’s       •    Device Closure is preferred (>85% su...
PDA    Class IIa & IIb recommendations*•    Closure may is indicated for any audible PDA without     irreversible PulmHTN ...
PDAClass III recommendations* (i.e. don’t do it)•   NO closure if irreversible PH (refer to specialized center)     • PAP ...
OBSTRUCTIVE LESIONS      & Ebstein & Marfan’s•       Left Ventricular Outflow Tract Obstruction (LVOTO)*•       Coarctatio...
LVOTO    Class I recommendations*Intervention is indicated if• Supravalvular (discrete) and probably the same for diffuse ...
LVOTO Class I recommendations (cont’d)*Supravalvular and Subalvular LVOTO require an ACHD surgeonBalloon valvuloplasty and...
LVOTOClass IIa & IIb recommendations*•   RVAo may be considered if Bicuspid valve and Critical Stenosis       •   <0.6cm2 ...
LVOTOClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis unless prosthetic valve.
COARCTATION    Class I recommendations*•   Intervention should be considered for any significant coarctation, especially i...
COARCTATIONClass IIa & IIb recommendations*• There are none…
COARCTATIONClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis except in the first 6 months following repair.
RVOTO  Class I recommendations*Percutanous Intervention (Balloon>PVR) is indicated if• Valvular RVOTO with nice anatomy an...
RVOTO  Class I recommendations (cont’d)*No ACHD follow-up if trivial RVOTO (<25 Peak and Asx)ACHD follow-up if• > mild RVO...
RVOTOClass IIa & IIb recommendations*• Intervention for valvular RVOTO probably also indicated for:     •   Important arrh...
RVOTOClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis unless prosthetic valve.
DiGeorge (22q11 deletion): ToF, interrupted Ao Arch, VSD, persistent truncus                                              ...
TOFClass IIa & IIb recommendations*Complete corrective surgery should be considered for all patients, unless severe irreve...
TOFClass IIa & IIb recommendations (cont’d)*Patients with high risk (>3.5%/yr) features for SCD may benefitfrom ICD (1° pr...
TOFClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis post repair, unless prosthetic valve or leak at    s...
EBSTEIN  Class I recommendations*Intervention (ACHD surgeon, preserve native TV) is indicated for thefollowing:    • NYHA>...
EBSTEINClass IIa & IIb recommendations*There are none…
EBSTEINClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis unless cyanotic, 6 months post repair, or TV    ...
MARFAN’S  Class I recommendations*Dx of Marfans should be made according to the Ghent criteria     • Skeletal Criterion, O...
MARFAN’S  Class I recommendations (cont’d)*Follow-up in specialized multidisciplinary setting    • Annual Echo & MRI/CT Q3...
MARFAN’SClass IIa & IIb recommendations*All patients with Marfan’s should receive BB therapy, especially ifdissection has ...
MARFAN’SClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis unless 6 months post repair or prosthetic    va...
COMPLEX CONGENITALLESIONS•   Complete Transposition of the Great Arteries (TGA)•   Congenitally Corrected Transposition (c...
TGA  Class I recommendations*All patients should be followed by an ACHD specialist (especiallysurrounding pregnancy) and a...
TGAClass IIa & IIb recommendations*Re-intervention may be warranted if    • Atrial Switch and           •   Systemic AV va...
TGAClass IIa & IIb recommendations (cont’d)*Patients with high risk features for SCD may benefit from ICD (1°prevention)  ...
TGAClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis post atrial or arterial switch, unless (+) Hx    end...
CCTGA  Class I recommendations*All patients should be followed by an ACHD specialist (especiallysurrounding pregnancy) and...
CCTGAClass IIa & IIb recommendations*(Re-)intervention may be warranted if     • VSD or residual VSD     • Moderate to sev...
CCTGAClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis unless cyanotic, prosthetic valve or conduit,    r...
FONTAN  Class I recommendations*All patients should be followed by an ACHD specialist (especiallysurrounding pregnancy) an...
FONTAN  Class I recommendations*Heart transplantation should be considered for severe single-ventricledysfunction and Sx H...
FONTANClass IIa & IIb recommendations*Patients with PM/ICD should receive warfarin.All Fontan patients without a Class I i...
FONTANClass III recommendations* (i.e. don’t do it)•   NO IE prophylaxis unless cyanotic, recent redo (6months),    prosth...
SINGLE VENTRICLE  Class I recommendations*All patients should be followed by an ACHD specialist at least annuallyand all a...
SINGLE VENTRICLEClass IIa & IIb recommendations*There are none…
SINGLE VENTRICLEClass III recommendations* (i.e. don’t do it)There are none…
EISENMENGER’S    Class I recommendations*•   Cardiac catheterization (if absolutely necessary) should be performed in ACHD...
EISENMENGER’S  Class I recommendations*• Phlebotomy should only be performed in patients with proven Sx  due to erythrocyt...
EISENMENGER’SClass IIa & IIb recommendations*• Pulmonary vasodilator therapy may improve quality of life     •   Endotheli...
EISENMENGER’S Class III recommendations* (i.e. don’t do it)Unless unavoidable, the following should not be attempted      ...
CYANOTIC   Heart Disease   Class I recommendations*• NSAIDs should be avoided.• Platelets, FFP, cryoprecipitate, Vit K, an...
CYANOTIC    Heart Disease    Class I recommendations*•   All patients should have ACHD f/u including       •   Annual clin...
CYANOTICHeart DiseaseClass IIa & IIb recommendations*• Patients may undergo phlebotomy to a target Hct<65% prior to  surge...
CYANOTICHeart DiseaseClass III recommendations* (i.e. don’t do it)Women with Eisenmenger’s should avoid pregnancy         ...
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  • CJC March 2010\n
  • *especially if not referred from a specialized pediatric center\nCJC March 2010.\nwww.genetests.org\n
  • *especially if not referred from a specialized pediatric center\nCJC March 2010.\nwww.genetests.org\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • *especially if not referred from a specialized pediatric center\nCJC March 2010.\nwww.genetests.org\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • *especially if not referred from a specialized pediatric center\nCJC March 2010.\nwww.genetests.org\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training. (Class I, Level C)\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • CJC March 2010.\n*General note: All recommendations will state that imaging, but especially interventions, should be performed by experienced practitioners with specific training.\n
  • Congenital guidelines notes

    1. 1. THE ROYAL COLLEGEEXAMINATION:CARDIOLOGY STUDY CARDS 2011: ADULT CONGENITAL HEART DISEASE (ACHD)
    2. 2. GRADING SYSTEM FORGUIDELINESClass of RecommandationI Benefits >>> Risks Should performIIA Benefits >> Risks It is reasonable to performIIB Benefits > Risks May be consideredIII Risks > Benefits Either unhelpful or may be harmfulLevels of Evidence A Multiples RCT’s &/or Meta-analyses B 1 RCT or 1 large non-randomized trial C Expert opinion, small studies, retrospectives, & registries
    3. 3. GENERALRECOMMEDNATIONSIn the introduction, the guidelines describe the organization of theCanadian Health Care system and the provision of ACHD care inthis context.Please refer to the Endocarditis Section (8) of the Study Cards forIE Prophylaxis recommendations in CHD Essentially, • Unrepaired cyanotic disease (including shunts) • 1st 6 months following repair • Residual lesions suggesting unendothelialized prosthetic material
    4. 4. GENERALRECOMMEDNATIONSGenetic diagnosis may be useful for: • Prognosis • Complementary Examinations (other organ involvement?) • Reproductive counselling • Family member screeningEvaluation of ACHD should therefore include:* • Detailed family history for birth defects of all kinds (not just cardiac) • Physical examination for dysmorphic facies, eye and ear abnormalities, limb defects, other skeletal defects, other organ system involvement, neurodevelopmental delay or learning disabilities. • Family screening through ECG and/or Echo. The need for investigation should be guided by genetic susceptibility when a genetic cause is known. • Cytogenetic testing should be considered in the following situations: • Recognizable chromosomal syndrome (eg, trisomy 21) • Associated dysmorphic features • Growth retardation, developmental delay or mental retardation • Multiple congenital anomalies. • History of multiple miscarriages and/or family history of birth defects.Genetic consultation is recommended in the presence of • Associated extracardiac anomalies • Clinical suspicion of a genetic abnormality • Positive family history of birth defects.
    5. 5. SHUNT LESIONS• Atrial Septal Defects (ASD’s)• Ventricular Septal Defects (VSD’s)• Endocardial Cushion Defects (atrioventricular septal defects, AVSD’s)• Padent Ductus Arteriosus (PDA)GeneticsHolt-Oram syndrome: ASD + pre-axial limb defects (TBX5 gene)Familial ASD + progressive AVB: Nkx2.5 gene w/o AVB: GATA4 mutationsASD in context of FAS
    6. 6. ASD’S Complications Residual shunting, PAH, new-onset or Class I recommendations* recurrent arrhythmias, device embolization, impingement of valves, veins, aorta, tamponade, device thrombosis, RV failure• Closure of ASD if hemodynamically significant • Defined as an enlarged RV by MRI or Echo • Surgical or percutaneous • percutaneous preferred if technically feasible (< 38mm, Secundum type, and no associated cardiac defects requiring surgical repair)• Post Percutaneous Closure • Systematically r/o effusion at 24hrs by TTE • Chest Pain or Syncope -> Immediately R/O Erosion/Migration • 3 months & 1 Year & then “periodically” • 6 months of ASA • 6 months prophylactic antibiotics• Post Surgical Closure • Risk of tamponade/post pericardiotomy syndrome for several weeks • Peri-operative death <1%• Follow-up • Anticoagulation for AF/AFL according to guidelines • reduced occurrence if closure before 40yrs • Rate or Rhythm Control according to guidelines • ACHD Specialist follow-up if: • Repaired as an adult, elevated PAP, arythmia, or ventricular dysfunction at the time of repair, coexisting heart disease, Familial ASD with risk of AVB
    7. 7. ASD’SClass IIa & IIb recommendations*• Closure may be indicated in the absence of RV Enlargement in the following contexts: • Orthodeoxia-Platypnea • Paradoxical Emboli • Tricuspid Valve repair or replacement • PAH if Qp/Qs >1.5 or PAH is reversible• Arythmia Interventions should be considered before percutaneous intervention or at the same time as surgery• Consult EP prior to closure in the setting of sustained atrial arrhythmia
    8. 8. ASD’SClass III recommendations* (i.e. don’t do it)• NO closure if irreversible PH (refer to specialized center) • PAP >2/3 SBP (ie PAH present) • PVR >2/3 SVR *Transmission to infant of sporadic 2nd ASD: 5-10%• NO pregnancy if Eisenmenger • Otherwise well tolerated if not hemodynamically significant or repaired • Increased risk of paradoxical emboli during pregnancy and post-partum• NO IE prophylaxis if Isolated ASD or repaired ASD >6 mos without residual shunt. Recommended that closure be performed before 24yrs→mortality benefit - and probably before 40yrs→arrhythmia benefit
    9. 9. Mild: Low Press sPAP/Ao Syst <0.3 or VSD’S mPAP < 20mmHg or sPAP< 35mmHg AND Qp/Qs<1.5 Class I recommendations* Mod: High Press Syst Pressure ratio >=0.3 mPAP>= 20mmHg or• Closure of VSD if: sPAP>= 35mmHg AND Qp/Qs>1.2 • Significant VSD PVR/SVR<0.2 • Symptoms Large: High Press + PVR/SVR 0.2-0.7 • LV Volume overload • LV or RV failure/deterioration Eisenmengers: High P and Vasc Resist ratio >0.7 + Qp/Qs<1.2 • Qp/Qs > 2 • Significant RVOT Obstruction • Cath or mean Echo Gradient > 50mmHg • SubArterial (Type1) or PeriMb (type2) with >mild Ao Insuff • Severe PulmHTN • = PAPs/SBP >2/3 or PVR/SVR >2/3 • Qp/Qs 1.5 (i.e. still net significant L-to-R shunt) or evidence of reversibility on Cath• Surgical closure by ACHD surgeon • Device closure is IIb • Excellent survival if normal LV&RV function • PAP evolution variable. Therefore follow-up… • Ventricular arrhytmias & SCD may occur, especially if late repair• ACHD specialist follow-up if • Residual defect (re-intervention rare) • Elevated PVR at the time of repair • Concomitant Ao valve surgery • Residual RV or LV dysfunction • Significant atrial or ventricular arythmias • Associated cardiac lesions
    10. 10. VSD’SClass IIa & IIb recommendations*• Closure may be indicated if: • History of endocarditis (esp if recurrent) • Transvenous pacing required • Prevention of paradoxical emboli• Device Closure (as opposed to surgical) may be preformed if: • Muscular or PeriMb VSD far from valves without associated lesions. • Risk of cAVB (1-6% early or late) higher with PeriMb device closure than surgery (1-4%) • Success rates are 90-95% • Complications 10% • Hypotension, device embolization, heart block, new AI or TR
    11. 11. VSD’SClass III recommendations* (i.e. don’t do it)• NO closure if irreversible PH (refer to specialized center) • PAP >2/3 SBP or PVR >2/3 SVR & Irreversible on Cath• NO pregnancy if Eisenmenger • Otherwise well tolerated if small or moderate or repaired. • Assuming no LV dysfunction or significant cyanosis or other lesions.• NO IE prophylaxis if Isolated VSD without cyanosis or repaired VSD >6 mos without residual shunt.
    12. 12. 5 leaflets: R-AS, R-inf, Superior & Inferior bridging leaflet, L-lateral wall leaflet AVSD’S 30-60% of AVSD have DOWN’s Syndrome 70% of Down’s Syndrome have AVSD (also seen in Williams Syndrome Smith-Lemli-Opitz, Smith-Magenis) Class I recommendations*• Intervention if: • Unoperated and • Paradoxical Emboli • LV Dysfucntion • RV Volume Overload • Clinical Heart Failure • Reversible Pulm HTN • Operated and • Persistent or new HD significant defects • Left AV valve regurgitation or stenosis (from previous repair) + symptoms • Deteriorating ventricular function • Significant Subaortic Obstruction • Cath or mean Echo Gradient > 50mmHg at rest or with stress (isuprel)• Avoid TVP if residual ASD or VSD.• Surgical closure by ACHD surgeon • Device closure is not possible (in Primum ASD+Partial AVSD) • If AV valve replacement, operative risk same as MVR, but higher risk of cAVB (both early and late). • Re-Intervention 5-10%• Pregnancy • Well tolerated if repaired or NYHA Class I-II • Increased risk of paradoxical emboli if unrepaired. (prophylactic closure should be considered) • ACHD transmission 3-5% generally • Trisomy 21 transmission is 50% (reproductive counselling recommended)• ACHD specialist follow-up for all patients • High risk of progressive AVB, subaortic stenosis (5%), “mitral” regurgitation and stenosis
    13. 13. AVSD’S Class IIa & IIb recommendations* • There are none!Partial: Ventricular septum intact, 2 separate AV valve annuli, Primum ASD, Cleft Left AV valve (90% of these occur in non-Downs)Intermediate: Restrictive VSD, Primum ASD, Cleft MV. Fused anterior & post bridging leaflets → 2 distinct AV valves (spectrum b/w partial & complete)Complete: Non-restrictive inlet VSD. Primum ASD (rarely atrial septum intact). Common AV orifice (Poor prognostic features: AF/AFL, syncope, HF, hemoptysis,... eisenmengers)
    14. 14. AVSD’SClass III recommendations* (i.e. don’t do it)• NO closure if irreversible PH (refer to specialized center) • PAP >2/3 SBP or PVR >2/3 SVR & Irreversible on Cath• NO pregnancy if Eisenmenger • Otherwise well tolerated • Consider closure prior to pregnancy (see class I)• NO IE prophylaxis if Isolated AVSD without cyanosis or repaired VSD >6 mos without residual shunt or prosthetic heart valve.
    15. 15. PDA Class I recommendations* • No intervention for Silent PDA’s • Device Closure is preferred (>85% success at 1 year) • Should be planned at the same time as diagnostic cath • The presence of Ductal Ca2+ increases surgical risk • Surgical Closure is reserved for cases where device closure not feasible(>95% success) • Too large or distorted (aneurysm, post-endarteritis) • Risk of recurrent laryngeal or phrenic nerve or thoracic duct damage.Genetic associations:Char Syndrome: AD; TFAP2B- abN facies, a/hypoplasia 5th fingers middle phalanges ↑ - must check Oximetry in hands + feet
    16. 16. PDA Class IIa & IIb recommendations*• Closure may is indicated for any audible PDA without irreversible PulmHTN (ie: not Silent PDA or severe PDA)• Clinically Silent PDA post endarteritis• If significant Pulm HTN (sPAP>2/3 sBP or PVR >2/3 SVR) + Qp/Qs >1.5 or evidence of PA reactivity on Pulm vasodilat challenge (O2, NO, Prostgldin)• ACHD specialist follow-up for all • Recanalization rare, but possible • Natural history of device closure not completely known• Endocarditis prophylaxis according to guidelines
    17. 17. PDAClass III recommendations* (i.e. don’t do it)• NO closure if irreversible PH (refer to specialized center) • PAP >2/3 SBP or PVR >2/3 SVR & Irreversible on Cath• NO pregnancy if Eisenmenger • Otherwise well tolerated if small or moderate or repaired. • Assuming no LV dysfunction or significant cyanosis or other lesions.• NO IE prophylaxis if Isolated PDA or repaired >6 mos without residual shunt.
    18. 18. OBSTRUCTIVE LESIONS & Ebstein & Marfan’s• Left Ventricular Outflow Tract Obstruction (LVOTO)*• Coarctation• Right Ventricular Outflow Tract Obstruction(RVOTO)• Ebstein Anomaly• Marfan’s Syndrome *Supravalvar: often diffuse, begins at superior margin of sVals, distal to coronaries (Williams Syndrome: 7q11.23 (on FISH) microdeletion (elastin gene): neurodev delay, ‘social’ personality, ELFIN facies, cardiac malfrmtn, HYPERCALCEMIA, skeletal, renal abN - ass. periph pulm or systemic arterial stenoses-coronary/renal) Valvar: usually BAV + associated Coarct, PDA, Asc Aortopathy (BAV: 1-2% of pop, AD trait with variable penetrence, 4:1 Male: Noonan, Turner, Williams Syndromes) (Aortic atresia: Jacobsen (11q deletion), Turner, Trisomy 13, 18, Wolf-Hirschhorn (4p deletion) Syndrome) Subvalvar: 2:1 Male Often with AI - AV damaged by subvalvr jet Can be- discrete fibromusc ridge (partially or compl encircling LVOT Long fibromusc narrowing, or tunnel-like narrowing of entire LVOTO, rare: obstruction by MV insertion or accessory MV leaflet SHONE Syndrome: LV Inflow and Outflow Obstruction (eg: supravalvar mitral ring, parachute MV + subvalvar LVOTO, BAV, Ao coarct)
    19. 19. LVOTO Class I recommendations*Intervention is indicated if• Supravalvular (discrete) and probably the same for diffuse lesions, as well. • Sx or Mean gradient > 50 (cath or echo) orPeak gradient > 70 (echo) • Low operative mortality and recurrence low • Risk of aneurysms and endocarditis at the site of patches• Valvular and • Sx (dyspnea, angina, (pre-)syncope) and significant LVOTO • Mean Gradient >40 (N LVEF), AVA <1.0 or <0.6/m2 • Sx and severe regurgitation • Severe regurgitation and LVESD>55, LVEDD>75, or LVEF<50% • AoRoot replacement if dissection, prox Ao >50, progression >5mm/yr (continued surveillance either way)• Subalvular and • Sx and mean echo gradient > 50 or peak echo gradient > 70 • Gradient may be underestimated if associated VSD • Sx and progressive Ao regurgitation • Not indicated for prevention of regurgitation in adults!
    20. 20. LVOTO Class I recommendations (cont’d)*Supravalvular and Subalvular LVOTO require an ACHD surgeonBalloon valvuloplasty and Ross procedure may be considered forvalvular in the young adult with out Ca2+ • Experienced centers only • Valvotomy/plasty will usually require re-intervention eventually • Pulmonary autographs may degenerate over time and there is a risk of neo-aortic aneurysm formation • Pulmonary homographs also require follow-upReintervention is indicated if • Recurrent LVOTO (same criteria), severe AR, or restenosis with > mild AR (esp if Sx or progressive LVEF deterioration) • Re-intervention after subvalvular LVOTO repair is particularly common
    21. 21. LVOTOClass IIa & IIb recommendations*• RVAo may be considered if Bicuspid valve and Critical Stenosis • <0.6cm2 non-indexed • Mean Gradient >60mmHg• RVAo may be considered if Biscuspid valve and • Competitive athlete • Pregnancy desired• Longterm follow-up following the Ross procedure• RVAo may be considered if Biscuspid valve and • Competitive athlete • Pregnancy desired• All patients should have regular follow-up• ACHD specialist follow-up for the following: • Williams & Shone Syndromes • Complex lesions (with or without repair) • Look for: recurrence, aneurysms, endocarditis, heart block, VT/VF, & SCD
    22. 22. LVOTOClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis unless prosthetic valve.
    23. 23. COARCTATION Class I recommendations*• Intervention should be considered for any significant coarctation, especially in the young with hypertension. • 65% will have regression of hypertension • Less straightforward are the elderly, asbence of HTN, mild coarctation, significant comorbidity • Either surgery (mortality <1%) or percutaneous according to experience and patient preference • Percutaneous preferred for recoarctation (lower mortality and lower risk of aneurysm) • Unsuitable anatomy for percutaneous: Long, tortuous, arch hypoplasia• Women with significant coarctation or dilatation should undergo repair prior to pregnancy• All aptients require follow-up with an ACHD specialist with periodic MRI/CT • r/o aneursym formation, late dissection • Screen for residual/recurrent HTN (recoarct?), LV dysfn, CAD • F/U bicuspid Ao valve and screen for ascending aortopathy • Investigate headaches agressively (Berry aneurysms) • Sx (dyspnea, angina, (pre-)syncope) and significant LVOTO
    24. 24. COARCTATIONClass IIa & IIb recommendations*• There are none…
    25. 25. COARCTATIONClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis except in the first 6 months following repair.
    26. 26. RVOTO Class I recommendations*Percutanous Intervention (Balloon>PVR) is indicated if• Valvular RVOTO with nice anatomy and • Sx (dyspnea, angina, (pre-)syncope) and significant RVOTO • Peak Gradient > 50 (mean > 30) • Asx and significant RVOTO • Peak Gradient > 60 (mean > 40)Surgical Intervention is indicated if• Valvular RVOTO with dysplasia or subvalvular or supravalvular, or f pulmonary hypoplasia or PRRe-intervention is indicated if• Recurrent RVOTO (same criteria)• Severe pulmonic regurgitation assoc’d with reduced functional capacity or deteriorating RV function or substantial TR or sustained atrial or ventricular arrhythmias
    27. 27. RVOTO Class I recommendations (cont’d)*No ACHD follow-up if trivial RVOTO (<25 Peak and Asx)ACHD follow-up if• > mild RVOTO• > moderate PR • Screen for progressive/recurrent stenosis, RV dysfn/dilatation, TR, arrhythmias, or evidence of shunting (esp. R-to-L)
    28. 28. RVOTOClass IIa & IIb recommendations*• Intervention for valvular RVOTO probably also indicated for: • Important arrhythmias (A-flutter) • Associated ASD or VSD (esp. if R-toL shunting) • Recurrent endocarditis• Double-chambered RV with pullback gradient >50mmHg
    29. 29. RVOTOClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis unless prosthetic valve.
    30. 30. DiGeorge (22q11 deletion): ToF, interrupted Ao Arch, VSD, persistent truncus Palatal abN, hypertelorism, cleft palate TOF Learning disability, hearing loss, HypoCa from hypoparathyroidism Class I recommendations*All patients should be followed by an ACHD specialist and all advancedinvesitgations should be performed by staff with expertise in ACHDSurgical Intervention is indicated if• Valvular RVOTO with dysplasia or subvalvular or supravalvular, or f pulmonary hypoplasia or PRRe-intervention is indicated if• Recurrent RVOTO (same criteria)• Severe pulmonic regurgitation assoc’d with reduced functional capacity or deteriorating RV function or substantial TR or sustained atrial or ventricular arrhythmiasSustained VT and/or SCD without reversible cause should receive an ICD (2°prevention)All patients should have ACHD follow-up, preconception counselling andfollow-up during pregnancy.Endocarditis prophylaxis is recommended for unrepaired TOF, 6 monthsfollowing surgery, if there is a prosthetic valve or VSD patch leak (residualshunt)
    31. 31. TOFClass IIa & IIb recommendations*Complete corrective surgery should be considered for all patients, unless severe irreversiblePulm HTN or inadequate pulmonary arteries, esp. if: • Worsening Sx • Cyanosis with erythrocytosis • Reduction of absence of shunt murmur (suspected stenosis/occlusion) • Aneurysm formation at the site of a shunt • LV dilation due to AR or Shunt (volume overload)Re-Intervention may be indicated for: • Free PR with • progressive or moderate-to-severe RV dilatation (RVEDV > 170cc/m2) • Modereate to severe RV dysfn • Atrial or ventricular arythmias • Decreasing exercise tolerance • Residual VSD with a shunt > 1.5:1 • Residual RVOTO with RVSP/SBP >2/3 • Significant AR with LV systolic dysfunction or symptoms • AoRoot > 55mm • RVOT aneurysm, false aneurysm, or infection • Sustained atrial arrhythmias or monomorphic VT (eliminate hemodynamic cause and consult EP) • The combination of mild-moderate residual VSD, RVOTO, or PR leading to RV dilatation or dysfn or worsening symptoms
    32. 32. TOFClass IIa & IIb recommendations (cont’d)*Patients with high risk (>3.5%/yr) features for SCD may benefitfrom ICD (1° prevention) • Previous palliative shunt • QRS > 180ms • Non-sustained VT • Inducible VT • Check if Hx of palpitations/syncope, older age at repair, transannular patch, QRS>180, non-sustained VT • LV dysfunction
    33. 33. TOFClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis post repair, unless prosthetic valve or leak at site of VSD patch.
    34. 34. EBSTEIN Class I recommendations*Intervention (ACHD surgeon, preserve native TV) is indicated for thefollowing: • NYHA>II • Cardiothoracic ratio greater than 65% • Resting O2-Sat <90% • Severe TR with Sx • TIA or stroke (if associated ASD/PFO) • NOTE: if percutaneous closure, test occlusion is mandatory!All patients should have ACHD follow-up • Cyanosis? Cardiomegaly? RV dysfn? TR? TS? Arrhythmias? AVB?
    35. 35. EBSTEINClass IIa & IIb recommendations*There are none…
    36. 36. EBSTEINClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis unless cyanotic, 6 months post repair, or TV prosthesis.
    37. 37. MARFAN’S Class I recommendations*Dx of Marfans should be made according to the Ghent criteria • Skeletal Criterion, Ocular Involvement and positive FamHx or genetic testing • Skeletal Criterion, Ocular Criterion, Cardiac InvolvementAll patients with significantly dilated aortic root or malignant family history shouldavoid isometric exercise, competitive or contact sports, and scuba diving (abruptpressure changes predispose to PTX)Surgical Intervention for • AoRoot/AscAo >50mm • AoRoot/AscAo >45mm and • Rapid progression (>5mm/yr) • Progressive AR and valve-sparing surgery possible • Family history of dissection @ <50mm • Severe MR that requires surgery • AoRoot/AscAo >44mm and pregnancy desired • Other parts of the Aorta >50-60mm or progressive dilatation • Severe MR with Sx or LV dilatation/dysfn (as per valve guidelines)Longterm surveillance of the entire aorta is indicated even after repair • AoRoot/AscAo >50mm
    38. 38. MARFAN’S Class I recommendations (cont’d)*Follow-up in specialized multidisciplinary setting • Annual Echo & MRI/CT Q3yrs • Annual MRI/CT if any dilated segment is approachingsirgical indication • MRI/CT within 1 year of repair • Annual MRI/CT for minimum 3 years post-dissection, esp. if unrepaired
    39. 39. MARFAN’SClass IIa & IIb recommendations*All patients with Marfan’s should receive BB therapy, especially ifdissection has occurred or root is dilated • Strict blood pressure control paramount • Losartan may slow the progress of root dilatation (peds series)All patients with Marfan’s should receive BB therapy, especially ifdissection has occurred or root is dilated • Strict blood pressure control paramount • Losartan may slow the progress of root dilatation (peds series)
    40. 40. MARFAN’SClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis unless 6 months post repair or prosthetic valve.
    41. 41. COMPLEX CONGENITALLESIONS• Complete Transposition of the Great Arteries (TGA)• Congenitally Corrected Transposition (ccTGA)• Fontan Circulation• Single Ventricle Physiology• Eisenmenger Syndrome• Cyanotic Heart Disease
    42. 42. TGA Class I recommendations*All patients should be followed by an ACHD specialist (especiallysurrounding pregnancy) and all advanced investigations should beperformed by staff with expertise in ACHD.Before pacemaker or ICD lead implantation, patency and anatomy ofsuperior venous conduits should be assessed.Aggressive management of atrial arrhythmias with catheter ablation. • Catheter ablation of ventricular arrhythmias may also be warranted • r/o reversible hemodynamic cause of arrhythmiaSustained VT or SCD without a reversible cause -> ICDEndocarditis prophylaxis is indicated post Rastelli (valved conduit)
    43. 43. TGAClass IIa & IIb recommendations*Re-intervention may be warranted if • Atrial Switch and • Systemic AV valve (tricuspid) regurgitation without significant RV dysfn • SVC or IVC obstruction (IVC obstruction worse!) • Pulmonary Venous pathway obstruction • Baffle leak resulting in significant L-to-R shunt (>1.5), Sx, Phtn, or progressive ventricular dilatation/dsfn • Baffle leak resulting in R-to-L shunt & Sx • ArterialSwitch and • Significant RVOTO • Coronary obstruction • Severe neo-AR • Severe neo-AoRoot dilatation • Rastelli and • Significant RV-PA conduit obstruction • Severe RV-PA regurgitation with Sx, progressive RV dilatation, or arrhythmias • Severe subaortic obstruction across the tunnel (mean gradient>50) • Significant PA branch stenosis
    44. 44. TGAClass IIa & IIb recommendations (cont’d)*Patients with high risk features for SCD may benefit from ICD (1°prevention) • Sx of arrhythmias • Documented AF/AFL • Systemic ventricular dysfunction • Older Age
    45. 45. TGAClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis post atrial or arterial switch, unless (+) Hx endocarditis, residual VSD after patch closure, or 6 months following repair with prosthetic patch or device implantation.
    46. 46. CCTGA Class I recommendations*All patients should be followed by an ACHD specialist (especiallysurrounding pregnancy) and all advanced investigations should beperformed by staff with expertise in ACHD.Pacemakers are indicated for 3°AVB or advanced 2°AVB or documentedasystole >3sec. • Monitor ventricular function post implantation! • Deterioration of systemic RV function reported
    47. 47. CCTGAClass IIa & IIb recommendations*(Re-)intervention may be warranted if • VSD or residual VSD • Moderate to severe SAVV regurgitation • Hemodynamically significant RVOTO • Significant stenosis across LV/RV-PA conduit or LV-Ao tunnel • Deteriorating systemic RV functionClosure of intracardiac shunts should be considered prior to PMimplantation.If closure of shunts not possible, epicardial leads should beconsidered.
    48. 48. CCTGAClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis unless cyanotic, prosthetic valve or conduit, residual VSD or Hx of endocarditis
    49. 49. FONTAN Class I recommendations*All patients should be followed by an ACHD specialist (especiallysurrounding pregnancy) and all advanced investigations should beperformed by staff with expertise in ACHD.Patients with a history atrial thrombus, VTE, interatrial communication(ASD or fenestration), or atrial arrhythmias should receive warfarinRe-intervention isindicated for • Obstruction of Fontan circuit • Obstruction of PV return • > moderate SAVV regurgitation • Development of venous collaterals or pulmonary AVM’s resulting in cyanosis • Residual ASD or fenestration with R-to-L shunt • Subaortic obstruction (>30 peak-peak) • PLE with high systemic venous pressures or Fontan abnormality • Recurrent/poorly tolerated atrial arrhythmias refractory to medical Tx
    50. 50. FONTAN Class I recommendations*Heart transplantation should be considered for severe single-ventricledysfunction and Sx HF despite optimal TxHeart transplantation should be considered for refractory PLE. • CHD patients have poorer survival following transplantationWhen arrhythmias are present, r/o hemodynamic cause & refer to EPwith expertise in CHD (very complicated and low long term arrhythmia-freesurvival) • CHD patients have poorer survival following transplantationAll women with Fontan should have ACHD consultation prior topregnancy.
    51. 51. FONTANClass IIa & IIb recommendations*Patients with PM/ICD should receive warfarin.All Fontan patients without a Class I indication for warfarin shouldbe considered for warfarin therapy.It may be reasonable to treat ventricular dysfunction with diuretics,ACE-inhibitors, and BB. (extension of HF data)Serious refractory arrhythmia may warrant conversion to TCPCwith concomitant MAZE(Re-)intervention may be warranted if • VSD or residual VSD • Moderate to severe SAVV regurgitation • Hemodynamically significant RVOTO • Significant stenosis across LV/RV-PA conduit or LV-Ao tunnel • Deteriorating systemic RV function
    52. 52. FONTANClass III recommendations* (i.e. don’t do it)• NO IE prophylaxis unless cyanotic, recent redo (6months), prosthetic valve or extracardiac conduit material, residual patch leaks or Hx of endocarditis
    53. 53. SINGLE VENTRICLE Class I recommendations*All patients should be followed by an ACHD specialist at least annuallyand all advanced investigations should be performed by staff withexpertise in ACHD.All women contemplating pregnancy should have a comprehensiveevaluation prior.Endocarditic prophylaxis is indicated for (all) single-ventricle patients • High rate of cyanosis • Guidelines do not suggest room for individualization according to resting or exercise O2-Sat
    54. 54. SINGLE VENTRICLEClass IIa & IIb recommendations*There are none…
    55. 55. SINGLE VENTRICLEClass III recommendations* (i.e. don’t do it)There are none…
    56. 56. EISENMENGER’S Class I recommendations*• Cardiac catheterization (if absolutely necessary) should be performed in ACHD centers.• All patients should be followed by an ACHD +/- PAH specialist. • Annual comprehenisve clinical assessment, including labs • Imaging every 2-3 years• All women should receive preconception/contraception counselling by an obstetrician/gynecologist with experience in high-risk pregnancy. • Tubal ligation, intratubal stents, or progestin-only preparations• If patient is already pregnant and wished to pursue, f/u should be with specialized multidisciplinary team.• All patients should be counselled not to smoke, use drugs, and to avoid dehydration or heat exposure and excessive physical activity.• Additionnally, the ACHD specialist should be advised if non-cardiac surgery is proposed or if there has been a serious illness. • Any pulmonary infection should be treated immediately• Exception oral hygiene and regular dental check-up Q6mos• Endocarditis prophylaxis for all (cyanotic)
    57. 57. EISENMENGER’S Class I recommendations*• Phlebotomy should only be performed in patients with proven Sx due to erythrocytosis. • Prevention of iron deficiency is important• Sinus rhythm should be restored/maintained on an individualized basis• Transvenous pacing should be avoided (risk of paradoxical embolism and closure CIx)• ICD implantation is high-risk. It may be considered for 2° prevention and epicardial leads should be favoured.• Patients with AF/AFL should receive warfarin
    58. 58. EISENMENGER’SClass IIa & IIb recommendations*• Pulmonary vasodilator therapy may improve quality of life • Endothelin antagonists are beneficial and well tolerated • Prostacyclins probably work, but carry risk and burden of IV therapy • PDE5-inhibitors have been little studied in Eisenmenger’s • Patients should likely be refered to a specialized center such therapy
    59. 59. EISENMENGER’S Class III recommendations* (i.e. don’t do it)Unless unavoidable, the following should not be attempted • Pregnancy (extremely high risk) • Maternal and fetal mortality each approach 50% with each pregnancy • Maternal mortality also increased post-partum (up to 3-4 weeks) • Non-cardiac surgery, General Anesthesia • If necessary, should have a cardiac anesthetist on hand • Early ambulation and ICU post-op • Hemoptysis should be aggressively investigated & ACO stopped • Vasoldilators • Estrogen-containing OCP’s • NSAIDs • Agents that impact renal function & platelet function • Cardiac catheterization • IV acces! (filter, filter, filter) • Acute High altitude (>2500m) • Strenuous Exercise • Heat exposure & dehydration
    60. 60. CYANOTIC Heart Disease Class I recommendations*• NSAIDs should be avoided.• Platelets, FFP, cryoprecipitate, Vit K, and ddAVP can be administered to treat severe bleeding• Iron deficiency (anemia) should be treated• Hydration should be prescribed prior to procedures involving contrast media• Symptomatic gout or hyperuricemia should be treated with colchicine or probenacid and prophylaxis with allopurinol is indicated.• Patients with worsening symptoms, cyanosis, or functiona capacity should be considered for intervention • Complete repair should be considered in those eligible • If you have irreversible PAH, you are ineligible • Refer for palliative procedures/therapies or transplant
    61. 61. CYANOTIC Heart Disease Class I recommendations*• All patients should have ACHD f/u including • Annual clinical visits, incl. labs • Imaging Q2-3yrs• Endocarditis prophylaxis for all
    62. 62. CYANOTICHeart DiseaseClass IIa & IIb recommendations*• Patients may undergo phlebotomy to a target Hct<65% prior to surgery. • FFP’s can be substituted for volume replacement if specific factor deficiencies are documented.
    63. 63. CYANOTICHeart DiseaseClass III recommendations* (i.e. don’t do it)Women with Eisenmenger’s should avoid pregnancy • Non-Eisenmenger’s Pregnancy still high risk • O2-Sat >85% better

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