Monaco CONGENITAL HEART DISEASE ADULTS GUCHs

Transcription

1 Monaco 2012 CONGENITAL HEART DISEASE ADULTS GUCHs

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3 DOCTORS disagree about most service provision for adults with Congen. H.D. EVEN the NAME NUMBERS NEEDS THEY agree the patients Are HERE!

4 GUCH POPULATION NAMES JEMA USMO GUCH GUCCI CACH PACH ACHD Germany Serbia Malta,Nevada USA,FRANCE Italy Canada Pacific Vancouver - USA, GUCH unit Brompton, etc [ Assoc. Childhood Heart Disorders Scotland] ACHDAC?- CYPRUS

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7 GUCH 2012 POPULATION PATIENTS PROBLEMS Novi Sad, February 2011

8 GUCH POPULATION Compared to Coronary disease and High blood pressure SMALL - WEAKNESSES and STRENGTHs

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10 GUCH Survivors Make Total GUCH Population in a Country Numbers unknown Guess statistics! (calculated from perceived survivors after surgery over years The oft quoted 1-2 MILLION USA , 000 UK 500,000 Canada CANNOT be FOUND FEWER become GUCH PATIENT population Fewer become GUCH medical population (?30%)

11 GUCH : Growth of Population Paediatric Cardiology 1990 GUCH Paediatric Cardiology 2005 GUCH Paediatric Cardiology 2012 GUCH BUT THEY ARE NOT ALL PATIENTS

12 Predicted number of Persons ALIVE is NOT Medical POPULATION

13 C.H.D.CHILD adult GUCHS Without service /organisation many LOST to follow up = INADEQUATE CARE

14 GUCH PATIENTS POPULATION? 25-40% COMPLICATED Hearts, repaired, palliated or untouched NAMED Operations Known will have COMPLICATIONS SIMPLE - occasional problems? 60-75% CURED Not AGREED around world!

15 GUCH 2012 POPULATION PATHOLOGY/PATIENTS PROBLEMS

16 TOTAL CORRECTION-C.H.D MOSTLY a SURGEON S DREAM (SAN) EXCEPT for DUCT Some VSD SOME ASD AORTO-PULM.Defect fistulae

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23 Fontan This is a THROMBOTIC Disease ALL ADULTS need Anticoagulants

24 GUCH 2012 POPULATION PATHOLOGY PROBLEMS

25 GUCH ADMISSIONS HAEMOPTYSIS ENDOCARDITIS TP. ASSESS. TOE/MRI HEART FAILURE INTERVENTION VENESECTION OTHER ARRHYTHMIA CATHETER R.B. % OF TOTAL ADMISSIONS J.S.

26 ADULT CONGENITAL HEART DISEASE Mayo Clinic Percent of Patients in Each Decade Patient (%) Acquired heart disease CAD, MI, CHF, AF 0 < >70 Age (years) CP

27 GUCH - OLDEST 2012 FONTAN EISENMENGER CTGA TRICUSPID ATRESIA COMPLEX PULM. ATRESIA DILV (Unop.) FALLOT (Unop.) MUSTARD TGA Yrs

28 GUCH HEART FAILURE PATIENTS INCREASING because LIVE LONGER! Reaching 60 s and 70 s INCREASING comorbidities

29 ELDERLY GUCH NON-CARDIAC PROBLEMS INCREASE WITH EACH DECADE

30 GUCH - NON-CARDIAC OBSTETRIC - Pregnancy Gynae. DENTAL - Wisdom Teeth NEUROLOGIST - Stroke Transient CNS GENERAL SURG. - Trauma, Gallstones SPINE SURG. - Kyphoscoliosis ENDOCRINE - Thyroid, HRT HAEMATOLOGY - RBC. Anaemia VITAL NEED for GENERAL

31 Non Cardiac Co-Morbidity Other Adult Specialist referral in 22%

32 PREGNANCY IN GUCH

33 AT RISK [COMPLEX] GUCHS with NONCARDIAC problems NEED CONSULTATION/MANAGEMENT by GUCH SPECIALIST to reduce COMPLICATIONS /DEATHS

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35 Why should PCs know about these issues? Who Should Look after GUCHS? Further Education Employment Insurance Sport Scars Contraception Anxiety

36 GUCHS have MANY NEEDS CARDIOLOGY the MOST

37 GUCH - CARDIOLOGY PROBLEMS ARRHYTHMIAS HEART FAILURE INCREASING CYANOSIS INFECTION THROMBOEMB EVENTS

38 GUCH - Type of Cardiac Surgery- Atrial Flutter 86 Pts % 20 % 17 % 15 % 14 % 12 % Number of Patients Fontan RVOT reconstruction ASD Closure Mustard Palliation Others Type of Cardiac Surgery Wei Li Int J Cardiol 2000

39 GUCH Cardiology EXPERTISE NEEDED Electrophysiologist MUST Understand GUCH anatomy Diagnostic imaging Echo Transesophageal, Intra-op,3D MRI CT INTERVENTION Angiography + Cath Don t FORGET Chest X-Ray

40 THE CARDIAC SURGEONS Made cardiologists, general physician d paediatricians work properly ulated need for paediatric cardiolo Stimulated need for PAEDIATRIC CARDIOLOGY

41 GUCH - CARDIAC SURGERY Special Problems Reoperations 70% - 1 to 10 Mortality Complication Time-Theatre,ITU Renal Failure Hemorrhage Needs Specialist Numbers not Occasional

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43 Problems with Surger Re-sternotomy with controlled re-entry into the aneurysmal RVOT

44 Mortality at Reoperatio The Advantage of tertiary centre referral Yemets et al N=85 1% Oechslin et al N=45 0% Therrien et al N=70 4% Hazekamp et al.2000 N=47 2%

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47 TECHNICAL SCOPE Interventions OPENING STRUCTURES CLOSING HOLES & CHANNELS IMPLANTING VALVES VALVE Regurgitation CONSTANT ADVANCING TECHNOLOGY and INNOVATIONS HYBRID interventions with operations VERY IMPORTANT in GUCH management As may mean no more surgery 47

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49 TOF: Implantable PV

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51 GUCH DEATHS and NEAR MISSES WCC 2008

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54 GUCH DEATHS 1990 s cf 2000 s factors / causes Reops Late Referrals Non cardiac surgery Sudden unexpected

55 GUCH DEATHS OPS / REOPS The real cause of DEATHS in relation to SURGERY INEXPERIENCE (arrogance, ignorance, non referral)

56 DIFFICULTY in TRANSFER to GUCH UNITS GUCH - Special RISKS for AT RISK MINOR OPERATIONS ENDOSCOPY THERAPY NON-CARDIAC DISEASE OTHER SPECIALISTS MONEY Follows Patient philosophy

57 GUCH s AT RISK TRANSPOSITION, Malposition DOUBLE Outlet, Inlet SINGLE, Common, One ATRESIA, Absence NAMED Operations, Diseases

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60 GUCH SUDDEN DEATHS 1:4 identifiable warning BEFORE Sarubbin et al. 1998

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65 GUCH PROBLEMS DIVERSE & UNUSUAL VARYING MEDICAL NEEDS NEW MEDICINE NEEDS SPECIALISTS all Levels ANYTHING POSSIBLE SURGERY DIFFICULT NEEDS an OPEN MIND

66 FONTAN d A G E 52 yr Operated- 1972

67 2012 GUCH major problems DIAGNOSIS Pulmonary REGURGITATION- post Fallot repair Failed RVOT valves [mostly Homografts] FONTAN problems Aortic valve,root,arch[post coarct.repair] ASDs for closure PULMONARY HYPERTENSION[Eisenmengers] TGA Mustard

68 GUCH IS NEW MEDICINE

69 GUCH patients with problems are EXPENSIVE Particularly SURGICAL!

70 ADULT CONGENITAL HEART PATIENTS Are a CHALLENGE for ALL DOCTORS

71 Congenital heart disease in adults[guchs] SCIENTIFIC DAY THANK YOU MONAC 2012

72 GUCH ISSUES/PROBLEMS 2009 NAME DEFINING POPULATION PEDIATRIC CARDIOLOGY INVESTIGATIONS BY UNTRAINED WHO DOES INTERVENTION? ADULT CARDIOLOGY CARDIAC SURGERY TRAINING SPECIALISTS FINANCE PRIVATE PRACTICE GEOGRAPHY

73 GUCH Surgery ISSUES Where? Not a childrens hospital Needs paediatric/ adult training Knowledge acquired heart disease Must have volume (> 50 minimum) Needs specialist nursing, intensivist etc Part of GUCH specialist unit Cardiac surgeon NOT chief GUCH unit GUCH Cardiologist help appointment!

74 SMALL (population) is BEAUTIFUL and GROWING!

75 Specialist GUCH UNIT CARDIAC SURGERY for GUCH 20% Admissions Presents Special Problems

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77 GUCH Cardiac Surgery Heart Hospital 236 patients Misc RV-PA conduit TCPC VSD Sub AS Ao root ASD AVR AVSD Ross

78 GUCH Surgery (n = 402) Single Ventricle 12% Septal Defect 24% Other4% RVOT / CONDUIT FAILURE 25% Aortic Valve 16% Atrio-ventricular Valve 12% Fallot / Pulmonary Atresia 7%

79 GUCH PATIENTS POPULATION? % COMPLICATED Hearts, repaired, palliated or untouched NAMED Operations Known will have COMPLICATIONS SIMPLE - occasional problems? 60-75% CURED

80 GUCH Trained Doctors ADULT PATIENTS Need ADULT Drs + Nurses, Technicians etc trained with ADULTS DRS WITH EXPERTISE

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82 GUCH - CARDIOLOGIST MUST KNOW PEDIATRIC CARDIOLOGY ADULT CARDIOLOGY Grown-Up CHD And Some General Medicine COUNTRY MUST TRAIN DR S

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85 GUCH operations Birmingham 2005/2008 Procedure Fallot PVR Secundum ASD AVSD Repair AVR SVASD ctga TVR Re-op Mustard VSD/AR PA VSD Radical repair Ebstein Cx Cyanotic Shunt PVR post-pvo RVOT Resect MVR RV-PA Conduit TVR TOF radical repair TCPC/Fontan Ross Procedure Tricuspid Other Number of Patients by Sara Thorne

86 GUCH - NATIONAL PROBLEM TRANSFER of PATIENTS DATA NEED PAEDIATRIC/ADULT CARDIOL. To WORK TOGETHER

87 VSD Pulmonary vein repair Shunt Sub-Aortic Surgery Trauma Surgery Tricuspid Valve Surgery PPM Pulmonary Trunk/Valve Surgery Intra Atrial Repair Mitral Surgery Fontan Surgery Surgery performed Aortic Surgery Aortic Surgery (Ross) Aortic Valve and Root ASD Repair AVSD Repair Coronary Artery Fallot Repair