Adult Congenital Heart Disease: The New Reality Kathryn Rouine-Rapp, MD Professor of Anesthesia Disclosures I have nothing to disclose 1
Outline Historic perspective Our reality Common lesions Guidelines Pathways to expertise Lorraine Sweeney 1938 PDA First person to survive surgery to correct CHD BCH Dr. Robert Gros 7 yo 2
Eileen Saxon 1944 TOF First person to undergo BT(T) shunt Johns Hopkins Drs Blalock, Taussig, & Mr Thomas Age 15 months switching arteries sidetracks blood and oxygen to otherwise starved lungs 3
Our Reality 1-3 million adults USA and CA with CHD 1.8 million Europe Survival to adulthood increased from 30% in 1940s to nearly 90% today More adults vs children with CHD Median age 40 yrs FEBRUARY 23, 2014 4
Our Reality >10000 adults with CHD Increasing fraction of all non-cardiac surgery Majority underwent surgery nonteaching hospitals Increased morbidity and mortality Maxwell et al. Anesthesiology. 2013 Oct;119(4):762-9 Lesion classification Complexity classification Simple Moderate Severe 20-25% overall severe complexity 40% simple or resolved post intervention 5
9/21/2015 Patient status Unoperated Palliated Surgical or device correction Excellent uncomplicated result Residual defect Sequelae 6
Lesions Common lesions VSD, ASD, PDA Pulmonary valve stenosis Aortic valve stenosis Coarctation of the aorta Atrioventricular-septal defects TOF TGA ASD One of most common defects Four types Secundum (70%), central IAS, associated MR Primum (15-25%), near AV valves, associated cleft MV Sinus venosus (10%), associated w anomalous PV Unroofed coronary sinus (rare) 7
http://www.doctortipster.com/wp-content/uploads/2011/07/interatrial-septal-defect2.gif https://apps.childrenshospital.org/clinical/mml/viewblob.cfm?media_id=306 8
Spectrum of severity 24 yo male asx athlete, murmur detected, secundum ASD 83 yo male, in OR for CABG, new incidental finding on TEE immediately prior to CPB, SV ASD & anomalous RUPV 54 yo female, presented with DOE and new onset atrial fibrillation with RVR, TTE w RVD, L to R ASD flow VSD Four types Perimembranous (70%) Muscular (20%) Doubly-committed (subarterial) (5%) Inlet (5%) 9
http://206.47.151.137/bcdecker/figures/acs/part11_ch01_fig29.gif Spectrum of severity Large defects = heart failure/sx Qp/Qs pulmonary to systemic flow ratio defect size SVR and PVR PHTN Infective endocarditis Device closure or surgery 10
Tetralogy of Fallot Most common cyanotic defect Four lesions RVOTO (severity determines cyanosis) RVH VSD (PM) Overriding aorta http://www.heartbirthdefect.com/images/birth-defects/621x440xtetralogy-of-fallot.jpg.pagespeed.ic.4xfv8mg3um.jpg 11
9/21/2015 Spectrum of severity 61yo male for atrial flutter ablation Shunt placement Shunt revision and PM Surgical repair (10yo) Proximal LPA hypoplasia Aneurysmal RVOT patch, PI RV EF 30% Decreasing exercise tolerance Not a candidate for percutaneous intervention 12
Guidelines 2008 ACC/AHA consensus statement Adults with CHD : surgical (diagnostic, interventional) procedures that require general anesthesia or conscious sedation in adults with moderate or complex CHD should be performed in a regional adult CHD center with an anesthesiologist familiar with adult CHD patients Guidelines 2008 ACC/AHA consensus statement Adult patients with complex or high-risk CHD should be transferred to an adult CHD center for urgent or acute problems and should have a cardiologist consultation prior to procedures 13
Simple lesions Unoperated Operated Isolated mild aortic or mitral valve lesion Isolated ASD Small isolated VSD Isolated mild PV stenosis PDA Secundum ASD Sinus venosus ASD wo residua VSD wo residua Cannesson et al Anesth 2009 Training no established curriculum for education DiNardo, Baum, Andropoulous: pathways for pediatric cardiac anesthesia fellowships depend on training prior to fellowship Anesth Analg. 2010 Apr 1;110(4):1121-5 Inconsistent experience during adult cardiac anesthesia fellowships across USA (personal survey) 14
Closed claim analysis Factors: adverse events n = 21 11 (52%) cardiac procedures 10 (48%) noncardiac procedures cardiac procedures surgical technique (73%) intraoperative anesthetic care (55%) noncardiac cases postoperative monitoring/care (50%) CHD (50%) preoperative assessment or optimization (40%) Maxwell BG et al. Congenit Heart Dis. 2015 Jan-Feb;10(1):21-9 Questions to consider Status of patient Unoperated, palliated, repaired Lesion and classification Simple, moderate, severe complexity Functional status NYHA Standard of care & experts in your group When to say no 15
Summary Increasing population of adults with CHD who need our care Lesion classification Specific lesions Guidelines Training variability Reality of local care vs triage THANK YOU 16
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