Adult Congenital Heart Disease T S U N ` A M I!

Transcription

1 Adult Congenital Heart Disease T S U N ` A M I! Erwin Oechslin, MD, FRCPC, FESC Director, Congenital Cardiac Centre for Adults University Health Network Peter Munk Cardiac Centre / Toronto General Hospital

2 Outline History The adult population Do we cure CHD patients? Summary

3 st Operation in CHD X R.E. Gross Boston, USA

4 1944 -Operation Aortic Coarctation C. Crafoord Stockholm, SE

5 Tetralogy of Fallot Helen B. Taussig ( ) Baltimore, USA

6 1944 Helen B. Taussig ( ) Baltimore, USA A. Blalock ( ) Baltimore, USA

7 Tetralogy of Fallot Blalock-Taussig Shunt Dr. A. Blalock and his team

8 Alfred Blalock Vivien Thomas

9 1 st Heart Lung Machine in 1953 Open Heart Surgery Intracardiac Repair John H. Gibbon Jr

10 C.W. Lillehei, Minneapolis, USA: First Tetralogy of Fallot Repair August 31, 1954 Cross- Circulation Courtesy of Dr. W. Williams, Toronto

11 C.W. Lillehei, Minneapolis, USA: First Tetralogy of Fallot Repair 1954 VSD patch closure RVOT reconstruction Transannular patch (fig.) Valvotomy, infundibulectomy, RV patch enlargement RV-PA conduit

12 Åke Senning Zürich, CH William T. Mustard Toronto, Ca

13 Åke Senning Zürich, CH William T. Mustard Toronto, Ca

14 Åke Senning Zürich, CH William T. Mustard Toronto, Ca

15 ARTERIAL Switch Procedure Brickner ME, et al. N Engl J Med 2000;342:

16 F. Fontan

17 Potts Anastomosis Tricuspid Atresia with ASD and VSD Fontan Procedure Not suitable for biventricular repair Separation of the systemic and pulmonary circulation

18 1971: Fontan - Circulation

19 Gross Crafoord Taussig Blalock Lillehei Senning Mustard Fontan Yacoub Jatene

20 Diagnostic Imaging Right Left

21 Toronto CHD History Hospital for Sick Children Keith, Rowe, Freedom, Redington Mustard, Trusler, Williams, Van Arsdell ACHD clinic opened 1959 at Toronto General Hospital

22 Advances Diagnosis Therapy Survival

23 Survival in Tetralogy of Fallot by decade of birth at HSC, Toronto n = 1,693 ( ) Hickey EJ, Manlhiot C, McCrindle BW 2007 Heart and Stroke Foundation of Ontario Functional outcomes in Tetralogy of Fallot

24 100 Survival of Complete TGA Arterial Switch: 2000 s Survival (% Year) Atrial Switch: 1980 s Natural History: 1950 s Years Courtesy by Dr. W. Williams

25 Survival Rate in CHD and Milestones in Cardiac Surgery % BT-Shunt Fallot-OP Atrial Switch-OP Fontan-OP Arterial Switch-OP Courtesy of Dr. H. Kaemmerer, Munich

26 Outline History The adult population Do we cure CHD patients? Summary

27 Prevalence of Severe and Other CHD Marelli AJ, et al. Circulation 2007; 115:

28 Changing Age Distribution of Severe CHD Marelli AJ, et al. Circulation 2007; 115:

29 Extrapolation: Quebec Data Annual Increase ~ 1,000 Adults per Year Prevalence of Adults with CHD (4.09 per 1000) ~ 96,000 Prevalence of severe CHD in Adults (0.38 per 1000) ~ 9,000

30 CHD Patients in Ontario Medium / High Risk for Complications: 50% 31,000 38,000 Children/ Adolescents Adults (>18 yrs)

31 Canadian Disease Statistics Cystic Fibrosis Cerebral Palsy HIV / AIDS Multiple Sclerosis Parkinson Congenital Heart Disease 3,098 50,000 58,000 75, , , Sources: Cerebral Palsy Canada, Cystic Fibrosis Association, Public Health Agency of Canada; Canadian Congenital Heart Alliance

32 Workload in Outpatient Clinic by Year Numbers Visits Patients SARS closed the clinic in 2003 for several months

33 Age Distribution n = Age (yrs) Euro Heart Survey on Adult Congenital Heart Disease

34 Heterogenous Population Diagnostic groups Shunt lesions Transposition complexes, Ebstein RVOTO / LVOTO, etc. Pulmonary hypertension Surgical procedures None Palliative shunts Repair

35 Outline History The adult population Do we cure CHD patients? Summary

36 The surgeon fixed my heart: I am cured

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38 Hardware

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40 Surgery is corrective, if...?...ventricular function is normal...life expetancy is normal!...there is no need for therapeutic??? measures during follow-up!?

41 Corrective Surgery... Atrial septal defect Ventricular septal defect Patent ductus arteriosus... if treated during childhood!!

42 Long-Term Survival after ASD Closure by Age Murphy JG, et al. N Engl J Med 1990;323:

43 Emergencies 11% 6% Cardiovascular Infective Others 83% Kaemmerer H, Oechslin E,... Hess J. J Thorac Cardiovasc Surg 2003; 126:

44 Cardiovascular Emergencies 2% 7% 6% 11% 2% 19% 53% Arrhythmias Heart failure Syncope Cerebral ischemia Other cardiovascular Infections Others Kaemmerer H, Oechslin E,... Hess J. J Thorac Cardiovasc Surg 2003; 126:

45 Psychological Patient Injury

46 Long Term Challenges Arrhythmias Supraventricular / ventricular Reoperations Failure of conduits / artificial valves Residual shunt / regurgitation / obstruction Heart failure Medical therapy / heart / (lung) transplantation Biomedical and psychosocial concerns / barriers

47 Outline History The adult population Do we cure CHD patients? Summary

48 Summary The demographics of CHD population have changed: Increasing number of adults with CHD Distribution of CHD: Prevalence of pts with severe CHD is increasing more rapidly in adults than in children Equalizing numbers of adults and children with severe CHD

49 Summary Mortality and morbidity are shifting away from the young and towards the adult Workload is increasing Impact on Health Care System!

50 Congenital Heart Disease Heterogeneous population the patient profile is changing Most pts are not fixed Life-long risk for complications Expert care with knowledge and expertise in: Anatomy and phsyiology Long-term outcome Multi-disciplinary team approach in designated centres

51 Designated Centre for Pt Care Cardiology Congenital / Thoracic Surgery Intensive Care Rheumatology Psychology Anesthesia Respirology Pulmonary HT Genetics Transplantation Heart Failure Imaging Immunology

52 Patients with named conditions or operations are complex Specialized centre

53 CHD is a Continuum from Fetal Life until Adulthood

54 ACHD TSUNAMI: Catch The WAVE! Arrhythmias Heart Failure Pulmonary Hypertension Re-Intervention Psychosocial Issues Reproduction Premature Death

55 ACHD Team 2008