* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Download Adult Congenital Heart PatientsLookup
Management of acute coronary syndrome wikipedia , lookup
Remote ischemic conditioning wikipedia , lookup
Coronary artery disease wikipedia , lookup
Electrocardiography wikipedia , lookup
Hypertrophic cardiomyopathy wikipedia , lookup
Rheumatic fever wikipedia , lookup
Arrhythmogenic right ventricular dysplasia wikipedia , lookup
Cardiac contractility modulation wikipedia , lookup
Heart failure wikipedia , lookup
Myocardial infarction wikipedia , lookup
Lutembacher's syndrome wikipedia , lookup
Atrial septal defect wikipedia , lookup
Heart arrhythmia wikipedia , lookup
Quantium Medical Cardiac Output wikipedia , lookup
Congenital heart defect wikipedia , lookup
Dextro-Transposition of the great arteries wikipedia , lookup
Adult Congenital Heart: Current Status & Long Term Issues 25th Annual CV Symposium Marc D. Knepp, MD November 4, 2016 I HAVE NO FINANCIAL DISCLOSURES Objectives 1. Define the current status of Congenital Heart Disease in the Adult 2. Identify co-morbidities of Grown Up Congenital Heart Disease 3. Discuss the importance of long term follow up and disease specific counseling Congenital Heart Disease • Malformation of the heart present at birth • Most common birth defect – 8 per 1000 live births • Common Forms – Ventricular Septal Defect (VSD) – Atrial Septal Defect (ASD) – Tetralogy of Fallot (TOF) – Transposition of the Great Arteries (TGA) • 10% diagnosed as an Adult Treatment of CHD • Medical therapy only temporizes – 50% survival to adulthood • First surgical intervention, 1938 – PDA ligation, Gross & Hubbard, Children’s Boston • Universally fatal until the 1940s – Shunt surgery devised by Dr. Helen Taussig – Performed by Dr. Arthur Blalock • November 1944, Johns Hopkins Early Congenital Heart Surgery • 1954– Dr. C.W. Lillehei performed the first TOF repair – Cross Circulation Cross Circulation Cardio-Pulmonary Bypass • Revolutionized the Care of CHD Survival of Unrepaired Tetralogy of Fallot Betranou EG, Blackstone EH, Hazelrig JB, Kirklin JW. Life expectancy without surgery in tetralogy of Fallot. American Journal of Cardiology 1978 Survival of Repaired TOF Murphy JG, Gersh BJ, Mair DD et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993: 329:593-599 But some work yet to do….. Nollert et al. Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year FollowUp of 490 Survivors of the First Year After Surgical Repair. JACC 1997: 1374-1383 Survival doesn’t mean cured • In 2015, >90% of newborns with CHD will live until adulthood • Even with successful neonatal or childhood repairs, many will still need procedures and follow up thru life www.achaheart.org Diller et al Circulation 2015 Diller et al Circulation 2015 Cause of Death Adult Congenital in the US • Over 1.3 million Adult Congenital patients – >50% of all congenital heart patients – 35-40,000 CHD pts/yr turn 18 • 18 different distinct Congenital Heart Lesions • Adults with CHD have 3-4x higher rates of ER visits, hospitalizations, and ICU admits, compared to general population 1. www.achaheart.org 2. Facing Lifesaving Heart Surgery, Twice. Wall Street Journal, 8/20/12 Annual ACHD Hospitalizations Agarwal et al. JAHA 2016 Age of Admitted ACHD Patient Cost of Hospital ACHD Care Long Term Follow Up Issues • Heart Failure • Arrhythmia – Sudden Cardiac Death • Re-operations/interventions • Pregnancy • Guidelines, whenever possible, “evidence based” • Total of 647 references used • Expert consensus used when lacking evidence Seen Regularly in ACHD clinic • • • • • Single Ventricle Cyanotic Transposition Truncus Arteriosus Conduits Periodic ACHD follow up • • • • • • Tetralogy of Fallot Aortic Coarctation Complicated VSD Complicated ASD Anom Pulm Veins Ebstein’s anomaly Non-routine ACHD patients Tetralogy of Fallot • Repairs in 1950s • Improved survival • Longterm issuses – Heart failure – Arrhythmia – Pulmonary Valve replacement – Sudden Cardiac Death Aortic Coarctation • • • • • • Hypertension Re-coarctation Aneurysm Cerebral Aneurysm Early CAD Premature Death D-Transposition Atrial Switch Repair of DTGA • • • • Mustard/Senning 1960s Arrhythmia Systemic RV failure Arterial Switch for DTGA • • • • • • Jatene 1980s Heart Failure PA stenosis Aortic root dilation Coronary ischemia Hypoplastic Left Heart Fontan Palliation—Single V Fontan Issues….. • Surgery in 1970s – DILV – Tricuspid Atresia • Norwood procedure – HLHS, 1980-90s • 70% survival to adulthood • • • • • • • Arrhythmia Systolic failure “Fontan” failure PLE Liver Failure/HCC Renal Failure Cyanosis PREGNANCY Pregnancy in ACHD • With more survivors and less morbidity more ability to make babies • Most woman with CHD can carry a successful pregnancy • Risk of CHD Recurrence – On average 5-8% – Up to 50% • Cardiac disease is rapidly becoming the #1 cause of peri-partum mortality Normal Physiologic Changes • Reduced Systemic Vascular Resistance BP falls early in gestation, 10mmHg below baseline by 2nd trimester • 30-50% increase in intravascular volume & cardiac output • Increased lower extremity venous pressure • Fluctuation in cardiac output during labor & delivery • Increased risk of thromboembolism Hemodynamic Changes Summary Kaleschke & Baumgartner. Heart 2011. For Example…. With decreased SVR/BP… Eisenmenger Syndrome • Long standing left to right shunt (usually thru VSD) that has become right to left shunt due to development of Pulmonary Hypertension. • Contra-indication to pregnancy – 50% mortality Another example “Flood behind the Dam” Heart Failure Example “Pump Failure” ACHD in Peoria • One of the 20 busiest clinics in US – Over 1400 visits per year • Formal transition program from Pediatric to Adult Congenital Team • ~40% of patient visits at Congenital Heart Center are adults THANK YOU! QUESTIONS?