The modified natural history of congenital heart disease Matthias Greutmann, MD Adult Congenital Heart Disease Program University Hospital Zurich, Switzerland matthias.greutmann@usz.ch
Are we ready for the future? Matthias Greutmann, MD Adult Congenital Heart Disease Program University Hospital Zurich, Switzerland matthias.greutmann@usz.ch
Objectives Natural history of complex CHD Effect of surgical modificantion Current situation Outlook and needs for the future
Survival Chance of survival to adulthood with complex CHD 100 80 % 60 40 20 0 1940 1950 1960 1970 1980 1990 2000 2010 Modified from Dr. H. Kaemmerer, Munich
Norwood
Transposition of the great arteries
Survival (%) Natural history of TGA 100 80 60 PFO only ASD VSD + PS 40 20 0 0 1 2 3 4 5 6 7 8 Age (years) Liebman, Circ 1969
Survival (%) Modified history of TGA 100 80 60 40 20 Atrial switch Arterial switch - early Arterial switch contemporary 0 0 2.5 5 7.5 10 12.5 15 17.5 20 Age (years) Liebman, Circ 1969 Williams, EJCTS 2003 Wong, HLC 2008
Improved Survival % 100 80 60 40 20 20 25 40 55 BT-Shunt Fallot-repair 70 Atrial switch 80 85 90 Norwood Arterial switch Fontan-operation 0 1940 1950 1960 1970 1980 1990 2000 2010 Modified from Dr. H. Kaemmerer, Munich
Number of patients Number of patients with CHD 25000 20000 15000 10000 5000 0 1985 1990 1995 2000 children adults Marelli, Circ 2007
Number of patients Shift to Adults: complex CHD 2500 2000 1500 1000 500 0 1985 1990 1995 2000 children adults Marelli, Circ 2007
Prevalence All congenital heart defects: 4.1 / 1000 adults Complex defects: 0.38 / 1000 adults Marelli, Circ 2007
Number of adults with CHD Switzerland 7.8 Mio Europe 730 Mio 32 000 3 000 000 3000 280000
Mortality Shift Age at death (years) 90-94 84-89 80-84 75-79 70-74 65-69 60-64 55-59 50-54 1987-88 2004-05 45-49 40-44 35-39 30-34 25-29 20-24 15-19 10-14 5-9 1-4 <1 30% 20% 10% 0% 0% 10% 20% 30% Proportion of all deaths CHD deaths General population Khairy, JACC 2010
Mortality Shift Age at death (years) 90-94 84-89 80-84 75-79 70-74 65-69 60-64 55-59 50-54 1987-88 2004-05 45-49 40-44 35-39 30-34 25-29 20-24 15-19 10-14 5-9 1-4 <1 30% 20% 10% 0% 0% 10% 20% 30% Proportion of all deaths CHD deaths General population Khairy, JACC 2010
Who are these patients?
Case 1: Female, 23 years old Left atrial isomerism single right ventricle, common atrium, severe pulmonary stenosis Palliated with left modified BTshunt
Palliated cyanotic CHD RV Hypoplastic LV Single atrium
Case 2: Male, 45 years old Tricuspid atresia Classic Glenn shunt Fontan operation Now: Failing Fontan
Fontan procedure tricuspid atresia single LV giant RA LA
Case 3: male, 23 year old Transposition of the great arteries RV LV PV-baffle Atrial switch
end-stage heart failure
What is the outcome of adults with complex CHD?
506 deaths among 12 499 patients 18% 13% 3% 34% 32% SCD Cardiac - not sudden Perioperative Noncardiac Unknow n AHA abstract, 2010
deaths Deaths per decade 400 1980-89 1990-99 2000-09 300 200 187 230 100 68 0
deaths Total patients Deaths vs. total patients 400 1980-89 1990-99 2000-09 8000 300 200 100 0 6000 4000 2000 0
Disease Complexity No of deaths 250 200 150 100 50 0 1980-89 1990-99 2000-09 1 2 3 4 5 simple moderate severe
Mode of death Simple Moderate Severe % 100 Non- Cardiac 80 60 40 Cardiac 20 0 1 2 3 4 5 p < 0.001
Age of death Changes in age of death 80 70 60 50 40 30 20 10 0 p = 0.7 p = 0.01 p = 0.002 Simple Moderate Severe 1980-89 1990-99 2000-09
Most common entities Fontan palliation (9%) Tetralogy of Fallot (11%) Subaortic right ventricles (13%) Cyanotic defects / Eisenmenger (29%) 13% of all patients but 62% of all deaths
Number of deaths Most common entities 60 1980-89 1990-99 2000-09 50 40 30 20 10 0 Cyanotic Fontan TOF TGA Cyanotic Fontan TOF TGA Cyanotic Fontan TOF TGA
Number of pts Active & alive Patients Tetralogy of Fallot TGA Cyanotics Fontan 1984 1989 1994 1999 2004 2009
Alive vs. deceased patients 2009 100% 80% 60% 40% alive death 20% 0% 1 2 3 4 Tetralogy Fontan TGA Cyanotics
Conclusions I There is an emerging population of young adult survivors with complex CHD These patients are at high risk of premature death at young age
Outlook and needs Transplantation Communication
Is transplantation the solution?
Number of adults with CHD Switzerland 7.8 Mio Europe 730 Mio 32 000 3 000 000 3000 280000
506 deaths among 12 499 patients 18% 13% 3% 34% 32% SCD Cardiac - not sudden Perioperative Noncardiac Unknow n AHA abstract, 2010
deaths Total patients No. of Transplantations 400 1980-89 1990-99 2000-09 8000 300 200 6000 4000 100 0 2 26 22 2000 0
No. of Transplantations Year of Transplant Listings Transplants Davies, Circ 2011
Reasons not to transplant CHD
% survival Outcome after Transplantation 100 75 Non-CHD CHD Fontan 50 25 0 2 4 6 8 10 Years Davies, Circ 2011 Lamour, JACC 2009
Communication Do we communicate enough?
Percent What do patients think 55 50 40 30 Students CHD mod. CHD - complex 20 10 0 < 46 46-55 56-65 66-75 76-85 86-95 >95 Self-estimated life expectancy Reid, JACC 2006
Communication 200 Adults with CHD 35 ± 15 years, 80% mod. or great disease complexity 48 Health care provider Tobler et al, IJC 2010
Patients Have you ever discussed EOL planning with a member of your medical team? % 100 80 60 40 20 0 99% No 1% Yes
Patients Do you think a member of your medical team should bring up EOL discussions? 80 77% % 60 40 20 23% 0 No Yes
Provider 100 80 How often do you typically discuss EOL issues with outpatients? 89% % 60 40 20 13% 49% 0 Simple M oderate Great p < 0.001 compared to patient group
Patients Do you think a member of your medical team should bring up EOL discussions? 100 80 77% 79% 77% % 60 40 20 0 Simple M oderate Great
Timing When do you think is the best time to bring up EOL discussions with patients? 70 60 50 Patients Provider % 40 30 20 10 0 w hile healthy first complication admitted / dying w hile healthy Before getting sick, while healthy first complication first life-threatening illness or complication admitted / dying first admitted with life-threatening complication or dying p < 0.001
Are we ready for the future? Novel therapeutic approaches Better prognostication Better communication
Thank you! Matthias Greutmann, MD Adult Congenital Heart Disease Program University Hospital Zurich, Switzerland matthias.greutmann@usz.ch