1 Han Congenital Heart Disease in Adult Pop.ppt · PDF fileCardiac Surgery & Catheterization ... Atrial switch 1958 (Senning) Atrial switch 1964 (Mustard) Balloon septostomy (Rashkind)1966

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B. Kelly Han, MD, FACC, FSCCT

Congenital Heart DiseaseThe Changing Population

2015 Cardiovascular Nursing ConferenceAllina Heath October 7th , 2015

Disclosures

Research Support Siemens Medical

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Overview Historical review of CHD intervention Discuss The changing CHD population The most common diagnosis & long term outcomes Aortopathy Transposition of the Great Arteries & TOF Single Ventricle Heart disease

The most common surgical and cath based interventions in adults with CHD

Pregnancy Clinical followup ACHD program development

CHD Historical perspective Cardiac Surgery & Catheterization

• First heart catheterization 1929 (Forssmann)• First Aortopulmonary shunt 1944 (Blalock & Taussig)• First open heart surgery for ASD closure 1952• First Fontan palliation for tricuspid atresia 1968• First Arterial Switch 1975 (Jatene)• First Norwood for HLHS 1979 • First Fontan for HLHS 1983• First Pulmonary Balloon valvuloplasty 1982 (J Kan)• Transcatheter pulmonary valve – approved by FDA (HDE) 2010• Transcatheter aortic valve – approved by FDA in 2011

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CHD in Adults: The Changing Population

CHD survival has dramatically improved in the past generation 1960: 1% of d-TGA survived to adulthood 10% of TOF survived to adulthood No HLHS survived to adulthood

2013 95 % surgical survival for complex CHD 90% survival to adulthood 70% of single ventricle patients expected to survive to adulthood

Estimated that 66% of patients with CHD are now adults Most morbidity (and mortality) for CHD is now adulthood

32nd Bethesda Conference: “Care of the Adult with Congenital Heart Disease” JACC Vol 37,No5, 2001: 1161-98.Khairy et al. JACC 2010; 56:1149-1157.

CHD in Adults: The Changing Population

Khairy et al. JACC 2010; 56:1149-1157.

Mortality in CHD 1987-2005

Mortality has shifted from children to adults

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prevalence of congenital heart disease (CHD) in the European Union by age group. prevalence of congenital heart disease (CHD) in the European Union by age group.

Helmut Baumgartner Eur Heart J 2014;35:683-685

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2013. For permissions please email: [email protected]

Changing Prevalence of CHD

Changing prevalence of congenital heart disease (CHD) in the European Union by age group.

Loss of follow-up from age 6 to 22 years among the entire study cohort.

Andrew S. Mackie et al. Circulation. 2009;120:302-309Copyright © American Heart Association, Inc. All rights reserved.

CHD patients in Cardiology Follow Up

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The proportion of CHD patients in contact with the healthcare system by age

Andrew S. Mackie et al. Circulation. 2009;120:302-309

Copyright © American Heart Association, Inc. All rights reserved.

Sees Cardiologist

Sees Primary Care

By age 15 50% of patients did not receive cardiology care

FigurOutpatient cardiology follow-up stratified by severity of CHD

Andrew S. Mackie et al. Circulation. 2009;120:302-309

Copyright © American Heart Association, Inc. All rights reserved.

Simple CHD

Complex CHD

By age 1860% of patients did not receive cardiology care

20% of patients with complex disease do not receive cardiology care

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Morbidity in Adult CHD survivors

Many patients need repeat intervention in adulthood

Heart failure common Single Ventricle Systemic right ventricle

Arrhythmia and Heart Block Common Pacemaker placement Defibrillator placement

Mortality in CHD

Concor registry - Netherlands

Observational study 7000 pts

2.8% died in follow up

Median age at death 48.8 years

77% Cardiovascular origin CHF & Sudden death most common

Risk Factors: Age, severity of defect # interventions Endocarditis & pulmonary htn Arrhythmia (atrial/ventricular)

Mortality in Adult CHD. 2010 http://dx.doi.org/10.1093/eurheartj/ehq032

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RE-OP ATRIAL vt

Ebstein’s anomaly 30-50% 33-60% >2% Single ventricle >25% 40-60% >5% Tetralogy of Fallot 26-50% 15-25% 10-15% TGA, atrial switch 15-27% 26-50% 7-9% TGA, arterial switch 12-20% <2% 1-2% CC-TGA 25-35% >30% >2% Truncus arteriosus 5-89% >25% >2% AVSD 19-26% 5-10% <2% Atrial septal defect <2% 16-28% <2%

ACHD Rate of Intervention & Arrhythmia by Diagnosis

PACES/HRS expert concensus statement on the recognition and management of arrhythmia in adult congenital heart disease. Khairy et al. May 2014. DOI: 10.1016/j.hrthm.2014.05.009

Figure 2 Number and percentage of adult patients in the Netherlands with congenital heart disease who had arrhythmias in 2007

van der Bom, T. et al. (2010) The changing epidemiology of congenital heart diseaseNat. Rev. Cardiol. doi:10.1038/nrcardio.2010.166

Data derived from the CONCOR registry68

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Koyak Z et al. Circ Arrhythm Electrophysiol. 2012;5:101-110

ICD Implant in CHD: Age and Diagnosis

Lesion Specific FollowupAortic Coarctation

Definitions used Proximal < 1 cm from LSCA Distal > 1 cm from LSCA Aneurysm > 20% the size of the DAO,

or abnormal localized enlargement TAA hypoplasia TAA/DAO at diaphragm < 0.6

Long segment > 5mm narrowing Post intervention dimension:

Coarctation repair/DAO diaphragm Reobstruction (filling defect /stent %)

mild < 10%, 11-30% , > 30

Ductus

Pulmonary Arteries

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Long Term survival in Aortic Coarctation

Age at operation < 1 year old Long term survival 91%

Age at operation 5-10 years old Long term survival 87%

Age at operation > 10 years old Long term survival 60%

Long Term Follow-up of Simple Coarctation Repair Systemic Hypertension Premature coronary artery disease Aortic valve abnormalities Aortic aneurysm (often with patch repair) Re-coarctation 1/3 of patients in long-term follow-up had

significant cardiovascular abnormalities The most predictive factor of late complications

was AGE AT OPERATIONLong-term follow-up of patients after coarctation of the aorta repair. Toro-Salazar et al, AJC Vol 89(5)March 1 2002

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Aortic Coarctation –Surgical repair

Review of 819 pts (1946-2005) Mean age of repair 17 +/- 13 years 93% survival at 10 years 84% at 20 years 74% at 30 years

Need for reintervention Initial repair < 1 year old (31% need reintervention) Technique other than end to end

Additional Cardiac Intervention Needed: Aortic valve replacement or repair CABG Aortic aneurysm repair Mitral valve repair or replacement

Brown et al. J Am Coll Cardiol. 2013;62(11):1020-1025.

Date of download: 2/3/2015

Copyright © The American College of Cardiology. All rights reserved.

From: Coarctation of the Aorta: Lifelong Surveillance Is Mandatory Following Surgical Repair

J Am Coll Cardiol. 2013;62(11):1020-1025. doi:10.1016/j.jacc.2013.06.016

Type of Repair, by Decade

The type of aortic coarctation repair was stratified by decade of repair. Mean ± SD age, by decade, at time of operation is also included.

Figure Legend:

Between 1946 and 2005, 819 patientsEnd to end anastamosis better

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25 year old – undiagnosed COA

Aneurysm Formation 45 year old

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Coarctation of the Aorta: Lifelong Surveillance Is Mandatory Following Surgical Repair


Overall Survival

Survival of the entire cohort compared with an age- and sex-matched Minnesota population (p < 0.001).

Figure Legend:



From: Coarctation of the Aorta: Lifelong Surveillance Is Mandatory Following Surgical Repair


Survival, stratified by age ≤20 or >20 years at time of initial operation (p < 0.001).Figure Legend:

Younger age at repair associated with better survival

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17 year old, asymptomatic AAO dilation

CHD in Adults: TOF and d-TGA

Changing Mortality for d-TGA and TOF 1960: 1% of d-TGA survived to adulthood 10% of TOF survived to adulthood

2013 98% surgical survival for TOF at most institutions > 95% surgical survival for d-TGA Many adults are s/p atrial switch s/p Arterial switch are now reaching adulthood

Most morbidity (and mortality) for TOF and d-TGA is now adulthood

32nd Bethesda Conference: “Care of the Adult with Congenital Heart Disease” JACC Vol 37,No5, 2001: 1161-98.Khairy et al. JACC 2010; 56:1149‐1157.

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History of Intervention: d-TGA

1940 1950 1960 1970 1980 1990 2000 2010

Atrial septectomy (Blalock Hanlon)1950Atrial switch 1958 (Senning)

Atrial switch 1964 (Mustard)Balloon septostomy (Rashkind)1966

Rastelli procedure 1969Arterial switch operation(Jatene) 1975

Nikaidoh procedure 1984

Surgical Approach to d-TGA

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D-TGA: Residual Hemodynamic Abnormalities

Atrial switch Systemic or pulmonary venous baffle narrowing or occlusion

RV failure (systemic ventricle)

Tricuspid regurgitation (quantify by stroke volume differences)

Arterial switch Neo‐pulmonary root or branch pulmonary artery stenosis, pulmonary insufficiency

Neo‐aortic root dilation, stenosis or insufficiency

Compromise of re‐implanted coronary arteries

Rastelli Obstruction of the RV‐PA conduit or branch pulmonary arteries, pulmonary insufficiency

Obstruction of LV‐Aortic pathway in the area of VSD patch

Nikaidoh Native RVOT or RVOT conduit stenosis or insufficiency

Right coronary artery compromise due to translocation of the aorta leftward

Procedure Residual Hemodynamic Abnormalities

s/p Atrial switch

Atrial Switch survival 65% at 25 years (80% simple, 45% complex)

Sinus node dysfunction and IART- 20 yrs post-op Mustard 60% Senning 20%

Superior baffle obstruction > 40% of adults with mustard (30% considered significant)

Subpulmonary obstruction Baffle leaks – 25% increase risk of thromboembolism

Long term complications: Systemic RV failure Tricuspid regurgitation

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Systemic venous baffle (CT)

Pulmonary venous baffle

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D-TGA after arterial switch operation (ASO)

Neonatal Arterial Switch for D-TGA


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S/P Arterial Switch Operation

Quality of life and health status 11-15 years later same as normal children, better than atrial baffle

Earliest survivors now young adults Coronary event free survival 93% at one year 88% at 15 years

Hemodynamic sequelae: Coronary insufficiency Ventricular dysfunction Stenosis of great arterial anastomotic sites AI or PI Neo-aortic root dilation

D-TGA followup recommendations

Complete echo at least every two years. After prior ASO repair for d-TGA, all adults

should have at least 1evaluation of coronary artery patency some recommend imaging at 1, 5 and 15 years post

op

Adult survivors with d-TGA after ASO should have noninvasive ischemia testing every 3 to 5 years

“periodic” MRI or CT to evaluate anatomy and hemodynamics in more detail

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Coronary anatomy and Relationship to RVOT and Sternum

Aortic Root dilation after ASO

10 years post op – freedom from aortic root dilation 50% (z-score > 3)

Freedom from at least moderate AI 93%

Freedom from ao-root surgery 95% Predictors of Aortic root score > 3 PA band Later age when ASO performed

Predictors of Neo- AI PA band Discrepant great artery size Presence of VSD Age > 1 at time of ASO

Schwartz M et al. Circulation 2004 Sep 14;110(11 Suppl 1):II128‐32

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2% surgical mortality in current era

Tetralogy of Fallot:Residual Hemodynamic Lesions

TOF with Pulmonary stenosis pulmonary stenosis, pulmonary insufficiency, RV

dilation and dysfunction, aortic root dilation

TOF with Pulmonary artery atresia RV‐PA conduit, branch pulmonary artery or distal

pulmonary artery stenosis, conduit insufficiency,

RV dilation and dysfunction, aortic root dilation

TOF with Absent pulmonary valve RV‐PA conduit stenosis or insufficiency,

airway abnormalities,

pulmonary artery dilation

PVR most common re-interventionPediatric patients – primarily have homograft placedAdults – primarily have stented bioprosthetic valve placed

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McCrindle et al, Abstract 0929, Canadian cardiovascular congress 2007

Tetralogy survival by era of surgery



From: Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year Follow-Up of 490 Survivors of the First Year After Surgical Repair

J Am Coll Cardiol. 1997;30(5):1374-1383. doi:10.1016/S0735-1097(97)00318-5

Survival after correction of TOF. The first, steep phase of the survival curve lasts ∼1 year. After that time, the curve becomes linear. Each step in the curve denotes an event, and at each event the standard error is indicated by vertical bars. OP = operation.

Figure Legend:

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From: Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year Follow-Up of 490 Survivors of the First Year After Surgical Repair

J Am Coll Cardiol. 1997;30(5):1374-1383. doi:10.1016/S0735-1097(97)00318-5

Long-term survival after correction of TOF. All patients who died within the first year after correction were excluded for calculation of long-term survival. The curve shows two different phases that are distinct. The early, low risk phase lasts 25 years; thereafter, the risk increases significantly. Mortality risk (r) per year, as a linearized number, is calculated for each phase. Note the break in the y axis. OP = operation; p.o. = postoperatively.

Figure Legend:

Risk+ preop polycythemia & RVOT patch

TOF key postop issues

Pulmonary Insufficiency RV dilation and dysfunction Tricuspid regurgitation RVOTO, branch PA stenosis, hypoplasia Sustained VT Sudden cardiac death (2% per decade) AV block, flutter, afib Aortic root dilation and insufficiency Syndromal associations

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Predictors of outcome in TOF

Ventricular function based on MRI Lower LV or RV EF Older age at repair

Recovery of RV function for severe PI 71 patients (21 +/-11 yrs) Normalization when RVEDV < 150, pt age < 17.5 Cut off for non-recovery 168 ml/m2, ESV 80 ml/m2

Valve replacement in TOF Stabilization of QRS duration (decrease shows better

outcome or event free survival) Decreased V-tach and atrial arrhythmia compared to

controlsPowell et al. J Am Coll Cardiol. 2004;43(6):1068-1074Taylor et al. Circulation. 2008; 118: S182-S190Therrien. Circulation. 2001; 103: 2489-2494Lee et al. J Am Coll Cardiol. 2012 Sep 11;60(11):1005-14

Arrhythmia in Adult Tetralogy of Fallot

556 Adults Cross sectional

retrospective study 43.3% had a sustained

arrhythmia or arrhythmia intervention.

ICD placement for primary and secondary prevention

Arrhythmia Burden in Adults With Surgically Repaired Tetralogy of Fallot: A Multi-Institutional Study. Khairy et al, (Circulation. 2010;122:868-875.)

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Primary prevention ICD in CHD

Khairy P et el.Circulation 2008;117:363-370

VARIABLE POINTS

Prior shunt 2

Inducible sustained VT 2

QRS > 180 msec 1

Ventriculotomy incision 2

Non-sustained VT 2

LVEDP > 12 mmHg 2

TOTAL POINTS 12

Low Risk < 1%

Intermediate Risk 1-11.5%

High Risk> 11.5%

Conservative TherapyEP STUDY

SCD prevention

PVR after TOF (mostly symptomatic pts)

Not shown to change VT or mortality Herrild et al. circ 2009. 111:445-51

No agreed upon criteria for valve replacement Meta-analysis of 48 studies (3118 pts) Decreased pulm regurg Improved RV indexed volumes but not EF (unless

“corrected” ef Improved LV fxn by larger LVEDV Dec qrs duration Improved symptoms Largest rvs responded best but not as much decrease in

symptoms Largest reduction in PI had best RV resonse

Pulmonary valve replacement after operative repair of TOF.Cavalcanti et al. JACC vol 62, no 23, 2013.

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Pulmonary Valve ReplacementMost common ACHD procedure Surgical Catheter (Melody) since 2010 In previously placed conduits In native RVOT under investigation

Underlying diagnosis PS, s/p valvuloplasty or valvectomy Tetralogy of Fallot (with PA, Absent PV) Truncus Arteriosus D-TGA L-TGA

Indications for PV replacement

Class 1: Symptomatic patient Severe PI and decreased exercise tolerance

Class 2b: Asymptomatic Pt severe PI + Moderate to severe RV dysfunction Moderate to severe RV enlargement Symptomatic or sustained arrhythmia Moderate to severe TR

Peak gradient > 50, RV/LV pressure ratio > .7 Obstruction with progressive or severe enlargement

of RV

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Pulmonary Valve Replacement After Operative Repair of Tetralogy of Fallot:

Meta-Analysis and Meta-Regression of 3,118 Patients From 48 Studies

RVEDV indexed

RVESVindexed

PI %

RV/LV ratio

QRSduration

NYHA class

Coronary Artery Disease in Adult TOF

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Surgical Pulmonary Valve replacement

945 RVOT operations (Toronto) Median followup 8.3 yrs (birth – 31 yrs) Median age 6 yrs

25% underwent at least 2 Freedom from RVOT valve replacement

5 yrs 82% 10 years 58% 15 years 41%

Risk factors 13-65 yo Younger age at initial valve placement Smaller valve size Need for endovascular stents Increased # of previous valve

replacements

Indications Stenosis PI if increased RVEDV

Caldarone et al. JTCVS 2000.Volume 120 (6) 1022–1031.

Transcatheter Pulmonary Valve placement Conduit 136 pts 2007-2009 Placed in existing Conduit Attempted in 124

6 coronary compression

1 death, 1 conduit rupture Median peak 37, to 12 mmHg Of those with mod-severe PI (81%),

none had more than mild in short term follow-up

1 year freedom from intervention or valve dysfunction 93%

Currently pre-stent to decrease stent fracture rate

Over 4000 patients worldwide

McElhinney et al. Circulation. 2010; 122: 507-516

Conduits > 16 mm

Ballooon sizing 14-20 mm

Moderate to severe PR

Conduit stenosis

mean >35mmHg

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Imaging the Coronary relationship to RVOT prior to Melody Valve

CTA prior to repeat intervention RVOT? Sternal reentry, sternal compression

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Melody Valve Endocarditis

Single ventricle

1940 1950 1960 1970 1980 1990 2000 2010

Shunts 1940s

Fontan for TA 1971Fontan for HLHS 1983

Lateral tunnel Fontan 1980sExtracardiac Fontan 1990s

Glenn shunt 1950s

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Think of Fontan as physiology

Passive systemic venous flow to the lungs Unobstructed systemic arterial flow from single V Stage 1: BT shunt or Sano

Norwood or DKS Stage 2: Glenn (TCPC)

Stage 3: Fontan

Classic Fontan Bjork KawashimaAtrio-pulmonary

Lateral tunnel

Extracardiac

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Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients with Fontan Surgery. Khairy et al, Circulation 2008;117:85-92

Late Fontan Survival

261 patients Born 1985 or earlier Median age at Fontan

7.9 Freedom from death

or transplant 5 yrs: 93.7% 10 yrs: 89.9% 15 yrs: 87.3% 20 yrs: 82.6%



From: Predictors of Survival After Single-Ventricle Palliation: The Impact of Right Ventricular Dominance


Kaplan-Meier Survival Curves

Patients were separated into 2 groups according to era of birth. Era 1 consisted of patients born from 1990 to 1999; era 2 consisted of patients born from 2000 to 2008. HLHS = patients with hypoplastic left heart syndrome; LV = patients with a dominant left ventricle; RV no HLHS = patients with a dominant right ventricle but not a diagnosis of HLHS.

Figure Legend:

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Common Residual Hemodynamic Lesions after the Fontan procedure SVC to PA, IVC to PA or branch pulmonary artery narrowing Atrial dilation (most commonly in older atriopulmonary Fontan) Systemic venous occlusion, collaterals or aortic bronchial collaterals Clot within the Fontan system, pulmonary embolism Ventricular to aortic obstruction if aorta from an outlet chamber Ventricular dysfunction Valvular regurgitation (regurgitation can’t be determined from CT) Recurrent arch obstruction Fenestration or leak in lateral tunnel Fontan Hepatic disease Plastic bronchitis

Type of Fontan Connection

Lateral Tunnel Extracardiac Atrio-Pulmonary

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SVC

IVC

Right atrialClot

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Fenestration Closure of Fontan

Pregnancy in CHD

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Pregnancy in ACHD

Seminars in Perinatology 2014 DOI:http://dx.doi.org/10.1053/j.semperi.2014.04.015

Seminars in Perinatology 2014 38, 260-272DOI: (10.1053/j.semperi.2014.04.015) Copyright © 2014 Elsevier Inc. Terms and Conditions

World Health Organization Categories

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Seminars in Perinatology 2014 DOI:http://dx.doi.org/10.1053/j.semperi.2014.04.015

Maternal Risk Factors in ACHD

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Risk of Fetal Heart disease by CHD Lesion

Summary

Patients with CHD are living to adulthood Most morbidity and mortality occurs in

adulthood Many patients are not being followed by

cardiologists We need to improve ACHD care Transition clinics –educate patients and parents Collaboration with adult cardiology

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MACC Midwest Adult Congenital Clinic Collaboration between Children’s and

Abbott Northwestern Hospital (Minneapolis Heart Institute)

Adult and pediatric cardiology care Inpatient and Outpatient care Drs Chris Carter and David Burton Drs John Lesser and Chuck Gornick Dr. Mike Samara Dr. Paul Sorajja Danielle Johnson, PA

MACC number: 612-863-5001 Dr. Han: beeper 612-538-7577 CHC 612-813-8800 Cell 612-310-7982

Documents

1 Han Congenital Heart Disease in Adult Pop.ppt · PDF fileCardiac Surgery & Catheterization ... Atrial switch 1958 (Senning) Atrial switch 1964 (Mustard) Balloon septostomy (Rashkind)1966