1、Adult Congenital Heart Disease,Carlos Macias, MD Arizona Pediatric Cardiology Consultants,Congenital Heart Disease,An abnormality of the heart that is present at birth. It may be hereditary or due to some influence occurring during pregnancy.,Congenital Heart Disease,95% has no identifiable cause. R
2、anges in severity from fairly simple to and curable to very complex and potentially lethal.,Congenital Heart Disease,The most common birth defect. Occurs in 1/100 births,History of Congenital Heart Surgery,1940s-1950s Maude Abbott and Helen Taussig defined the anatomy and physiology of Congenital He
3、art Disease. Early 1950s, first closed heart surgery using bathtub hypothermia. 1953, First attempts at heart “bypass” surgery. 1955, first bypass surgery at Mayo Clinic.,Congenital Heart Defects Prevalence,32,000 new cases of CHD / year in the U.S. 1.5 million, world wide. 20,000 open-heart operati
4、ons / year in U.S. 85% reaching adulthood Approx 1,000,000 adults with CHD in U.S. More adults than children with CHD,Changes in Technology,Preoperative diagnosis Intraoperative care (cardiopulmonary bypass). Surgical technique Postoperative care,ACHD,For all but the most simple defects, surgical an
5、d catheter procedures are palliative rather that curative.,Acyanotic CHD (left to right shunt),Atrial septal defect (ASD) Ventricular septal defect (VSD) Endocardial cushion defect Patent ductus arteriosus (PDA),Cyanotic CHD (right to left shunt),Obstruction to pulmonary blood flow Tricuspid, pulmon
6、ary atresia Intracardiac mixing of oxygenated and unoxygenated blood Tetralogy of Fallot Transposition of the great Vessels Single ventricle,Obstructive Valvar Lesions,Pulmonary valve stenosis Aortic valve stenosis. Coarctation of the thoracic aorta,Fetal Circulation,Internal Normal Heart (Frontal V
7、iew),Atrial Septal Defect,Atrial Septal Defects (View from right side),Atrial Septal Defect Patch Repair,Amplatzer Occlusion of Atrial Septal Defect,Clockwise from above: Transcatheter delivery of Amplatzer device, which is positioned across the atrial septal defect Left: Amplatzer device in place,A
8、SD, Survival Patterns,Normal, if closed before 20 years of age. Good, but shorter than normal 25-41 yrs. Increased morbidity and mortality after 41 yrs.,ASD and PHT,Occurs in two chronologically disparate age groups. Females, late teens and 20s. Primary PHT with coincidental ASD Dont close the ASD !
9、 Patients 40 years of age Mild to mod. PHT secondary to longstanding left to right shunt. Likely to benefit from ASD closure,Ventricular Septal Defect,VSD before surgical repair,VSD after surgical repair,Ventricular Septal Defects (View from right side),Occlusion of Intracardiac and Vascular Shunts
10、Ventricular Septal Defect Occlusion,Above: Echocardiogram of muscular VSDUpper right: Fluoro image of CardioSEAL device occlusion of a VSD. Transesophageal echo probe (TEE) and pigtail catheter in place.Lower right: Amplatzer muscular ventricular septal occluder Illustration courtesy AGA Medical Gro
11、up,Eisenmenger Complex Ventricular Septal Defect (VSD) in conjunction with Pulmonary Vascular Obstructive Disease (PVOD) Arrow right to left shunt,Atrioventricular Canal Defect- Complete,Atrioventricular Canal Defect - Complete,Above: view of heart from above showing joined mitral and tricuspid valv
12、es Right: ASD atrial septal defect VSD ventricular septal defect,Atrioventricular Canal Defect- Complete,Atrioventricular Canal Defect - Partial,Repair of Atrioventricular Canal Defect - Complete,Patent Ductus Arteriosus (PDA),Patent Ductus Arteriosus Division and Ligation,Occlusion of Intracardiac
13、and Vascular Shunts Coil embolization of PDA,Left, top: Catheter crosses the PDA from the aortic side and delivers a coil.Left, bottom: Withdrawal of catheter, leaving coil in PDA,Aortic Stenosis A - Supravalvar B Valvar C - Subvalvar,1) Valvular Stenosis with a bicuspid aortic valve,RV,LA,RA,Severe
14、 Aortic Stenosis in an infant,Aortic Stenosis - Balloon Valvuloplasty,Repair of Aortic Stenosis,Ross Procedure Konno Procedure,Marfans Syndrome 1 enlarged ascending aorta 2 stretched aortic valve 3 mitral valve prolapse SV Sinuses of Valsalva,Pulmonary Stenosis A supravalvular B valvular C - subvalv
15、ular,Valvuloplasty Pulmonary Balloon Valvuloplasty,Left: Arrow indicates stenotic pulmonary valve,Coarctation of the Aorta SA subclavian artery COA coarctation AV - aortic valve,1. Coarctation of the aorta, distal to the leftsubclavian artery2. Severe aortic stenosis,Coarctation of the Aorta with se
16、vere aortic stenosis in an infant,Coarctation Repair Excision with End to End Anastamosis,Coarctation - Subclavian Flap Repair,Angioplasty Aortic Coarctation Angioplasty,Illustrations showing (left) uninflated and (right) inflated angioplasty balloon positioned within coarctation of the descending a
17、orta,Intravascular Stents Coarctation of the Aorta,Left: uninflated angioplasty balloon and stent within coarctation Middle: expansion of balloon and stent Right: deflation of balloon leaving stent wide open,Transposition of the Great Arteries L Type MV mitral valve TV tricuspid valve,Ebsteins Anoma
18、ly ASD atrial septal defect 1 atrialization of the right ventricle,Transposition of the Great Arteries D Type,Balloon Atrial Septostomy - Dynamic,Rashkind-Miller catheter Illustration courtesy Edwards Lifesciences Corp.,Transposition Repair,Aorta and pulmonary arteries are switched Opening in the at
19、rial septum is closed. Patent ductus arteriosus is divided and closed off. Coronary arteries are re-implanted,Jatene Arterial Switch Operation,Mustard Repair,D-TGA, Arterial Switch,Initial steep learning curve Operative mortality approx. 2% Flat mortality curve at 15 years,Arterial Switch, Problems,
20、Neopulmonary valve and artery Neoaortic Valve Coronary Arteries Coronary artery obstruction in 10-18% Absence of symptoms is not reliable,Arterial Switch, Late Complications,25 year Follow-up 4% operative mortality Pulmonary stenosis most frequent cause of reintervention Coronary artery problems are
21、 rare but consequences are severe. Pts usually asymptomatic. Angiography necessary.,Hutter et al. JTC. 2002,D-TGA Outcomes Mustard Outcomes (113 Survivors),80% survival at 28 years 76% in NYHA class I 97% freedom from RV failure Late unexpected sudden death 2-8%Wilson et al. JACC, 1998,Arrhythmias a
22、nd Sudden Death in Mustards and Sennings for TGA,Progressive loss of sinus rhythm (SSS) Atrial flutter more common in patients with RV dysfunction or baffle leak SD related to severe TR, RV dysfunction, uncontrolled SVT. SD, 81% occurred during exercise. SD, Role for EPS uncertain,D-TGA Outcomes Mus
23、tard Outcomes (113 Survivors),20% have to modify lifestyles 5% cant work 75% normal life 20% had educational difficulties,D-TGA Treatment options for long term failures,Orthotopic cardiac transplant Convert to Arterial switch poor results in older patients,Tetralogy of Fallot,1 ventricular septal de
24、fect (VSD), 2 pulmonary stenosis 2a infundibular stenosis, 3 enlarged aorta overriding VSD 4 right ventricular hypertrophy,Tetralogy of Fallot,Palliation with aortopulmonary shunt Primary repair earlier in infancy Prosthetic patch closure of VSD resection of sub-pulmonary stenosis transannular patch
25、 required for small pulmonary annulus,Repair of Tetralogy of Fallot,Subclavian Artery to Pulmonary Artery Anastomosis (Blalock-Taussig Shunt),Ascending Aorta to Right Pulmonart Artery Anastomosis (Waterston-Cooley Shunt),Modified Blalock-Taussig Shunt,Central Shunt,Repair of Tetralogy of Fallot,Tetr
26、alogy of Fallot Repair,TOF Long term Results,20-30 year follow-up, 90% survival Majority of pts. In NYHA, I and II Women, 93% live births (7% had major cardiac defects),Right Ventricle,Nonobstructed transannular patch often results in significant PI PI directly related to RV dilation. Decreased exer
27、cise tolerance Arrhythmias ? Sudden death,TOF, Pulmonary Valve,Complete relief of valvar/annular obstruction may result in significant PI requiring valve insertion,TOF, Pulmonary Valve Insertion,TOF Arrhythmias,Heart block Most patients have RBBB post-op RBBB + left anterior hemiblock may have sligh
28、tly higher risk of heart block but not sudden death. SVT, 2-26% Ventricular tachycardia. Major cause of late sudden death post-op.,Tetralogy of Fallot,SUDDEN DEATH: The largest single cause of late mortality associated with repaired TOF,TOF Repair in Adults,Higher risk surgery than in children Highe
29、r incidence of pre and post-op arrhythmias Improves quality of life Repair in adults is warranted !,Single Ventricle Defects,1. Atretic (missing) Tricuspid Valve. 2. Hypoplastic Right Ventricle. 3. Ventricular septal defect. 4. Atrial septal defect. 5. Pulmonary Stenosis.,Restricted Blood Flow to Lu
30、ng Tricuspid Atresia,1. Mitral valve atresia. 2. Severe aortic valve stenosis. 3. Hypoplastic left ventricle. 4. Hypoplastic ascending aorta. 5. Patent ductus arteriosus (PDA).,Restricted Blood Flow to Body Hypoplastic Left Heart Syndrome,1. Rudimentary right ventricle 2. Ventricular septal defect (
31、VSD) 3. Transposition of the great arteries 4.Double inlet left ventricle 5.Atrial septal defect (ASD) 6. Ventricular inversion 7. Left aortic arch,Little or no Restriction in Blood Flow Double Inlet Left Ventricle,Fontan Procedure Lateral Tunnel,Fontan Procedure Extracardiac,Fontan Procedure Compli
32、cations,Pleural Effusions, Chylothorax Thrombus formation Protein losing enteropathy Arrrhythmias Progressive ventricular dysfunction 15 year survival in”best Fontan candidates” is 73%.,Deaths in Adults with Congenital Heart Defects,1/4 deaths had identifiable, avoidable physician error,Inadequate A
33、ssessment,Electrolytes not measured New runs of SVT New syncope/near syncope Rapid increase in hypoxia Hemoptysis Rapid increase in heart size New widening QRS,Arizona Adults with Congenital Heart Disease,Establish a standard of care Recognition of a referral center Staff Multidisciplinary consultants Patient population Diagnostic and interventional facilities training and education,
