ACHD for the Generalist

Transcription

1 Congenital Heart Disease Congenital heart disease for the generalist Andrew Aitken Chairs: John Lainchbury & TV Liew ACHD for the Generalist Andrew Aitken Wellington Hospital Outline ACHD : Why is it important to a Generalist Why do we need to know about ACHD Distinctive features of ACHD patients Special considerations Case examples How do we organize the care of our patients Increasing prevalence NZ patients DHB 150,000 Approx. 400 patients Geographical distribution Acute presentations common Chronicity of care Adult Congenital patients Followed at Montreal Heart Centre Canadian Journal of Cardiology 30 (2014) S410-S419 1

2 NZ Congenital Cardiac Surgery Predicted Future Survival for Child and Adult patients Survival Adult Survival child Adult survival Child survival pre Special Considerations Age of the patients Transition from paediatric services Complexity of anatomy Lack of reserve Poor attendance Stretching our comfort zone Pregnancy / contraception /employability / insurance issues Psychological support Case Presentation 1 Case 1 : Investigations 34F presents with SOB Prior Tetralogy of Fallot Repaired age 4 years Intermittent palpitations Examination: RV + ESM + EDM at ULSE Mild peripheral oedema Incidence 1 / 3,500 live births per 150,000 population 2

3 Case 1 : Managed with diuretics Symptoms settled No arrhythmias on holter MRI scan: RV dilated at 170 ml/m2 with RVEF 40% Free PR Mild impairment of LV function Proceeded on to Pulmonary valve Replacement Tetralogy : Treatment of Heart failure Little evidence BB / ACEI of help CRT in RBBB shows short term benefits only Pulmonary Valve Replacement has a role Improve symptoms May reduce risk of malignant arrhythmias Debate as to best timing for PVR Percutaneous Melody valves changing landscape Tetralogy : Learning Points PVR increasingly common in early adult life Improved RV size and function? Improved outcomes Experience of adult issues driving advances in surgical techniques in infants Risk stratification for sudden death improving Role of ICDs /ablation still uncertain Case Presentation : A.S. Born with AVSD Repaired age 3-4 months Left with A-V valve regurgitation Presented age 21 with severe MR / SOB and paroxysmal AF. Developed thyrotoxicosis (amio related) Proceeded to ON-X MVR and surgical MAZE 2009 Anticoagulated with warfarin. BB as back in AF / on ACEI 3

4 A.S. Pregnancy A.S. ECHO Planned pregnancy 2013 (by the patient!) Stopped ACE Inhibitor and warfarin Commenced on LMWH by GP S/B obstetrician in Wairarapa Referred to local physician Referred to WLG cardiology + high risk clinic First seen at 20 weeks gestation A.S. Anticoagulant Monitoring during pregnancy A.S. Anticoagulant Monitoring during pregnancy Haphazard! Was initially on once daily enoxaparin 0.9 Changed to bd dose on obst. review Anti- Xa levels : 0.6 Measured 5 times during pregnancy Erratic timing 0.3? True trough or peak levels Haphazard! Was initially on once daily enoxaparin 0.9 Changed to bd dose on obst. review Anti- Xa levels : 0.6 Measured 5 times during pregnancy Erratic timing 0.3? True trough or peak levels Therapeutic Levels 0 Aug-13 Sep-13 Oct-13 Nov-13 Dec-13 Jan-14 Feb-14 0 Aug-13 Sep-13 Oct-13 Nov-13 Dec-13 Jan-14 Feb-14 4

5 A.S. Plans for Delivery Planned induction of labour at 38 weeks Stop clexane on induction Heparin infusion until labour established Facilitate second stage Antibiotics peripartum Restart heparin 4-6 hours postpartum Initially lower dose for 4-6 hours Continue in hospital until INR therapeutic A.S. Plans for Delivery Planned induction of labour at 38 weeks Facilitate second stage Antibiotics peripartum Stop clexane on induction Heparin infusion until labour established Restart heparin 4-6 hours postpartum Initially lower dose for 4-6 hours Continue in hospital until INR therapeutic A.S. Learning Points (There s nothing physiological about pregnancy!) Importance of preconception planning Need for close monitoring during pregnancy Importance of multidisciplinary team / communication protocols Patient support on local level 5

6 ACHD Care in New Zealand Local ACHD Centre Single specialist surgical centre in Auckland CHD Specialist centre S.I.? CHD Specialist centre lower N.I. Local ACHD expertise in all cardiology centres One cardiologist with an interest in ACHD Support via Outreach from Specialist Surgical / Cardiology centres Specialist MDT sessions Role of videoconferencing 0.25 FTE ACHD Specialist nurse Take Home Messages Need for ongoing follow-up throughout adult life Continuity of care (locally) and successful transition Arrhythmias may be tolerated poorly Marker of significant structural decline / need for further intervention Treat vigorously (don t forget our E/P colleagues) Challenge to discuss unusual concepts Pregnancy / employment / insurance / contraception Most rewarding aspect of my work 6

7 Roles of Local ACHD Centre Delivery of shared care under established protocols Ensure the delivery of long term follow-up appropriate to need Understand when to refer the patient Basic cardiac diagnostic services Joint working with palliative care Management of low risk pregnancies Fontan: Ongoing Issues Role of Fontan conversion Role of selective pulmonary vasodilators How to monitor for end-organ damage (Management of pregnancy) Expanding indications - eg HLHS What does the future hold? Palliative procedure but when does decline occur? Cardiac transplant -? When rather than if 7

8 Case Presentation: G.W. Fontan: Surgical Developments 40M Born with tricuspid atresia. Had Fontan operation 1979 Atrio-Pulmonary connection Seen 2002 at age 25 Physically fit Rarely took aspirin Concerns re outlook A-P Fontan (Classic) Lateral Tunnel Extracardiac Conduit G.W. G.W. - progress Presented to ED with RUQ pain April 2010 Abnormal LFTs but normal hepatic U/S Noted to be in AF. ECHO severe atrial dilatation + poor flow Also increase in A-V valve regurgitation Decrease in ventricular function CTPA no pulm. embolus but RA clot Anticoagulated clexane warfarin Beta blocker for rate control Managed pain Discharged and had MRI scan at 4 weeks Large RA but no thrombus Sluggish flow but no other structural issues Referred for DC cardioversion Successful

9 G.W. - Currently Re-appears 5 years later In chronic AF Very anxious about his situation? Time to upgrade the Fontan Keen to explore other options Has DNA d last 6 months Arrhythmias in Tetralogy Atrial re-entrant 30% Ventricular 10% Sudden Cardiac Death 0.2%/year Felt largely due to VT Occas. abrupt A-V block Mechanism previously surgical scar Also ongoing strain, esp RA / RV enlargement How to Predict Risk of SCD QRS duration > 180 msec Age > 20 years Multiple cardiac operations (shunts) LV systolic or diastolic dysfunction Others RV dilatation or dysfunction Inducible arrhythmias on E/P testing Severity of PR ICDs Appropriate discharge rates 7.5 to 10%/year* Inappropriate discharges 6-10% / year Complication rates up to 30% Compared to DCM population (2 year f/up):** Decreased appropriate discharges (5 vs 23%) Increased inappropriate discharge (20 vs 4%) Younger population ( mean age 25 vs 54) *Khairy et al Circulation 2008 **Witte et Al Europace

10 Initial Operative Strategy Minimise ventriculotomy incision Transatrial and transpulmonary approach Decrease degree of PR Preservation of pulm valve function Decrease RV dilatation Increased RV pressures No evidence yet of improved outcomes! Pulm Valve Replacement PVR decreases RV size and preserves RV function Improves symptoms / reduces heart failure? evidence that decreases risk of VT/SCD Harrild et al Circulation 2009: 98 late PVR matched to controls No benefit in terms of VT / death over 10 years Tetralogy : Timing of PVR With symptoms Exercise deterioration on CPET / arrhythmias With volume change / dysfunction RV on MRI RVEDV > 150 ml/m2 RVESV > 80 ml/m2 Move towards ECHO volumetric assessment (as with AR and MR for left heart) Limited evidence that improves longer term outcomes Cardiopulmonary Exercise Testing Objective assessment of exercise tolerance VO2 max RER (VCO2/VO2) measure of degree of effort VE/VCO2 slope (minute ventilation related to CO2 production) Measure of ventilator efficiency Anaerobic threshold measure of submaximal exercise intensity HR and BP response O2 saturations ( ECG changes ; PFTs) 10

11 Transcatheter P.V. replacement Pioneered by Boenhoffer 2000 Bovine jugular vein on a stent Deployed via femoral vein (can use RIJ) 20-24mm balloon. Percutaneous Pulmonary Valve Implantation Bonhoeffer et al, London. Management of Heart failure in Tetralogy 20% TOF patients have LV dysfunction Likely increase in CHF in this population over time BNP limited utility ECHO limitations with RV assessment MRI important 11

12 Management of CHF Prognostic Effects of Pulmonary Valve Replacement in Repaired Tetralogy of Fallot: Results From a Large Multicenter Study No evidence benefit of BB / ACEI CRT Short term data only suggesting improved RV function with RBBB PVR Reduces RV size and improves RV function Outcome data lacking Timing key Circulation. 2016;134:A19888 A.S. Outcome Successful delivery with no complications in pregnancy and healthy baby BUT care not optimal No record of prenatal counselling re options Poor monitoring of LMWH Initial od regime Infrequent antixa levels Not therapeutic Anticoagulation strategy peripartum not clearly defined D/C early Fontan : Late Conversion Option in failing fontan Conversion of RA-PA to extra cardiac fontan Bypass RA and optimise flow to pulm. aa Perform cryo-ablation to treat arrhythmias Mavroudis et al : Chicago 118 pts over 13 years 1 early (0.8%) and 9 late (7.6%) deaths 6 (5%) required heart transplant within 3 years Starship experience: 9 procedures 1 death 12