Congenital Heart Disease in Adult

1. Congenital Heart Disease in Adult 陳震寰醫師 台北榮民總醫院心臟科 國立陽明大學醫學系

2. SVC Ao RPA LPA PV PV MPA LA MV PV AoV RA LV TV RV IVC Biventricular Circulation:Series Circulation Systemic circulation Pulmonary circulation

3. Arterial end of heart 1st aortic arch 22nd DAY 21st DAY 1st aortic arch Bulbus cordis Fusing cardiac tubes Ventricle Unfused cardiac tubes Atrium Venous end of heart 24th DAY Truncus arteriosus Truncus arteriosus Bulbus cordis Bulbus cordis Ventricle Atrium Ventricle Sinus venosus Atrium Common cardinal v Sinus venosus Umbilical v Vitelline v 23rd DAY

4. Fetal Development of the Arterial Trunk and Brachiocephalic Arteries RIC REC LEC LIC I I I I II II II II III III III III IV IV IV IV RV Left Aortic Arch V V DA VI VI RS RPA LPA PT Desc Aorta

5. Arch of aorta Superior vena cava Ductus arteriosus Lungs Pulmonary trunk Pulmonary veins Foramen ovale Left atrium Right atrium Inferior vena cava Ductus venosus Descending aorta Left hepatic vein Portal sinus Portal vein Gut High oxygen Saturation in blood Umbilical vein Kidney Medium oxygen Saturation in blood Umbilicus Umbilical arteries Low oxygen Saturation in blood Urinary bladder Superior vesical artery Internal iliac artery Placenta Legs • Blood oxygenated in the placenta • Returns to the heart through the umbilical veins • Umbilical venous blood to IVC via the ductus venosus • Inferior vena cava blood shunted to the LA through the foramen ovale • To LV and ejected to upper body • Upper extremity return is diverted to RV • RV pumps blood into the main pulmonary artery • Most flows through the ductus arteriosus into the aorta • Deoxygenated blood then passes to the placenta through two umbilical arteries

6. SVC RA LA PV MV IVC Valve of the foramen ovale Foramen Ovale Closure • Following birth, SVR increases • Inflation of lungs decreases PVR and increases pulmonary flow • Reduction in systemic venous return through foramen ovale • When LA pressure > RA pressure the valve closes

7. ASD VSD

8. Ao SVC RPA PAB LPA PV MPA PV LA MV PV AoV RA LV TV RV IVC Pulmonary Artery Banding • Band around main pulmonary artery to create a stenosis • Prevent excessive pulmonary blood flow (congestive heart failure) • Non restrictive VSDs • Transposition of the great arteries

9. Ductus Arteriosus Closure PDA • Changes in systemic and pulmonary vascular resistance • Aortic pressure exceeds the pulmonary artery pressure • Flow through the ductus reverses and flows from the aorta into the pulmonary arteries • Begins to constrict and usually closes off within 1-4 days after birth. SVC DA RPA LPA Ao PV PV LA MPA MV PV AoV RA LV TV RV IVC

10. Eisenmenger’s Syndrome • A large communication between the two circulations at the aortopulmonary, ventricular, or atrial levels • Bidirectional or predominantly right-to-left shunts because of high-resistance and obstructive pulmonary hypertension • No specific treatment has proved beneficial • Single lung transplantation and intracardiac defect repair • Total heart-lung transplantation

11. SVC SVC Ao Ao RPA LPA LPA RPA PV PV PV MPA PV LA MPA LA MV MV PV AoV RA AoV PV LV RA LV TV RV RV IVC IVC Tricuspid Atresia:Parallel circulation, Cyanosis

12. SVC Ao RPA LPA PV PV MPA LA MV PV AoV RA LV TV RV IVC Superior Vena Cava to Right Pulmonary Communication SVC SRC Ao RPA LPA PV PV MPA LA MV AoV LV RV RA IVC

13. Normal TGA TF

14. TGA TF

15. Blalock-Taussig Shunt • “Blue baby operation” • Connection between pulmonary and systemic circulations • Conduit anastomosis between subclavian and proximal right pulmonary artery • Improves oxygenation to pulmonary blood flow SVC Ao RPA LPA PV PV MPA LA MV PV AoV RA LV TV RV IVC

16. Congenital Heart Disease, Adult • Cyanotic vs. acyanotic heart disease • Patients who have had palliative or corrective cardiac surgery or interventional cardiac catheterization • Those who have never had intervention

17. Adults Patients withCyanotic Heart Disease • May have survived without corrective surgery/interventional catheterization • Eisenmenger’s syndrome • Ebstein’s anomaly • Balanced complex lesions with adequate pulmonary blood flow and normal pulmonary artery pressure (e.g., tetralogy of Fallot with moderate pulmonary stenosis)

18. Adult Patients withCyanotic Heart Disease • Who have had surgery/interventional catheterization who may remain cyanotic • Status post fenestrated Fontan procedure with right-to-left shunt • Glenn alone • Arterial shunt

19. Adult Patients withAcyanotic Heart Disease • Who have not had surgery/interventional catheterization • Atrial septal defect (ASD) • Small, medium-sized ventricular septal defect (VSD) with left-to-right shunt • Bicuspid aortic valve • Coronary artery fistula • Sinus of Valsalva aneurysm • Partial anomalous pulmonary venous return • Mild pulmonary stenosis

20. Adult Patients withAcyanotic Heart Disease • Who have had surgery/interventional catheterization • Aortic valve disease, valvotomy or replacement, Ross procedure • Pulmonary stenosis, valvotomy • ASD, VSD • Fontan surgery for complex congenital heart disease • Transposition of great arteries, atrial redirection, or arterial switch • Tetralogy of Fallot • Aortopulmonary window • Mitral valve disease, valvotomy or replacement

21. Medical considerations Congestive heart failure Cyanosis Infective endocarditis Electrophysiologic problems Pregnancy Genetic counceling Psycosocial aspect Employment Insurance Psycosocial development Contraception Exercise, sports Surgical considerations Reoperation Inevitable reoperation Heart/lung transplantation Noncardiac operations Potential Problems

22. Epidemiology • Incidence/Prevalence: increasing • As the number of patients who have had corrective or palliative surgery has increased and survival improves. • Etiology: unknown, multifactorial • Genetic: Holt-Oram syndrome with autosomal-dominant transmission of an ASD; Williams syndrome with supravalvular aortic stenosis; and Down’s syndrome with atrioventricular canal defects • Environmental: 2%, e.g., congenital rubella syndrome • Genetic counseling should be provided for all potential parents with congenital heart disease, both male and female

23. Pregnancy • Patients with cyanosis are at high risk of early spontaneous abortion. • Elevated pulmonary vascular resistance from Eisenmenger’s syndrome or primary pulmonary hypertension is a contraindication to pregnancy. • Patients with Marfan’s syndrome and some patients with bicuspid aortic valve are at risk for aortic rupture. • Patients with severe aortic stenosis are at high risk for aortic rupture. • Patients with aortic coarctation are at risk for aortic rupture and cerebral hemorrhage as well as increased fetal risk of compromised placental blood supply

24. Pregnant Women with Prosthetic valves • Warfarin until the 35th week of pregnancy, then iv/sc heparin • Risk of warfarin embryopathy: 3.4% • A warfarin dose of 5 mg/day lowers risk of fetal complication. • Heparin is more difficult to regulate and is associated with a greater risk of thromboembolism in the mother.

25. Signs and Symptoms • History • History of murmur in childhood • History of palliative surgery in early infancy • History of corrective surgery in early infancy or childhood • History of arrhythmia • History of endocarditis

26. Cyanotic Secondary to Uncorrected Cardiac Defect • Effort-induced dyspnea • Increased cyanosis with exercise • Effort induced fatigue

27. Cyanotic Secondary to Pulmonary Vascular disease • Eisenmenger’s Syndrome • Hemoptysis (late) • Decrease in intensity of childhood murmur • Palpable P2, right ventricular heave • Clubbing of fingers and toes, perioral cyanosis, hypertrophic osteopathy

28. Acyanotic • Range from asymptomatic to significant limitation of activities of daily living with symptoms at rest secondary to heart failure

29. Laboratory Procedures • Cyanotic • Hemoglobin/hematocrit elevagted secondary to elevated erythropoietin levels (Hb 16-23 g, Hct 55-80) • Platelet count low to markedly reduced • Blood urea nitrogen/creatinine may be elevated • Uric acid elevated secondary to inappropriately low renal excretion • Acyanotic • Depends on the nature of the underlying lesion

30. Imaging Studies • Chest X-ray • Cyanotic secondary to pulmonary vascular disease • Decreased pulmonary blood flow • Prominent pulmonary arteries at the hilum with rapid tapering • Enlarged right ventricular silhouette • Acyanotic: depends on the underlying lesion • Cardiac catheterization • Primary diagnostic tool to evaluate coronary artery atherosclerotic disease • Evaluate ventricular filling pressures • Delineate patterns of collateral blood flow • Delineate pulmonary anatomy

31. Imaging Studies • Transthoracic echocardiography • Color flow imaging for • Following the course of arteries and veins • Detecting small atrial or ventricular septal defects • Detecting small aortic-to-pulmonary connections • Delineating stenotic and regurgitant lesions • Doppler studies for identification and quantification of • Valcular, supravalvular, and subvalvular stenosis • Valcular regurgitation • Extracardiac and intracardiac conduit obstructions • Pulmonary and systemic venous stenoses • Septal defects • Surgical shunts

32. Imaging Studies • Transesophageal echocardiography • Evaluate anatomic areas not easily demonstrated by transthoracic echo. • Monitor therapeutic interventions in the cardiac catheterization laboratory • Intraoperative imaging during cardiac surgery

33. Special Tests • Cardiovascular/pulmonary anatomy • Magnetic resonance imaging (MRI) • Magnetic resonance angiography • Arrhythmia • Holter • Event monitor • Electrophysiology study • Functional capacity and cardiac ischemia • Standard Bruce protocol exercise stress testing • Thallium and sestamibi nuclear stress testing

34. Treatment • General measures • Nonchemotherapeutic prophylaxis for endocarditis • Meticulous oral hygiene, skin and nail care

35. Surgical Measures • Indications for operation in adults with congenital heart disease include: • Repair of primary congenital heart defect: ASD, aortic stenosis, pulmonary stenosis, Ebstein’s anomaly, coarctation of the aorta, tetralogy of Fallot, etc. • Inevitable reoperation: bioprosthetic heart valve, extracardiac conduit • Residual defects after repair: mitral valve regurgitation and/or subvalvular aortic stenosis after AV canal defect repair • New/recurrent defects after corrective surgery: subaortic stenosis recurrence, pulmonary regurgitation after repair of tetralogy of Fallot

36. Surgical Measures • Indications for operation in adults with congenital heart disease include: • Staged repair of complex defect: Fontan procedure • Late complication: infective endocarditis • Patient with acquired heart disease: coronary atherosclerotic disease • Uncorrectable congenital heart disease: heart or heart/lung transplant • Insertion of permanent pacemaker and/or defibrillator • Mapping and ablation of arrhythmias

37. Admission/Discharge Criteria • Symptomatic arrhythmia for drug treatment or electrophysiology study (ablation or intracardiac defibrillator placement) • Exacerbation of congestive heart failure • Surgery or interventional/diagnostic cardiac catheterization • Endocarditis • Cerebral abscess or stroke in cyanotic patient

38. Medications • Drug(s) of choice • Patients with left-sided ventricular dysfunction benefit from established medical therapy for congestive heart failure (digoxin, angiotensin-converting enzyme inhitor, directic, beta-blockade) • Prior to dental work or invasive procedures patients must receive endocarditis prophylaxis • Phlebotomy recommended for secondary erythrocytosis only if patient is significantly symptomatic with Hct of >65% • Iron repletion in cyanotic patients who are iron deficient (close monitoring of hematocrit required as blood counts may increase quickly with iron therapy)

39. Medications • Drug(s) of choice • Colchicine for treatment of gouty arthritis in cyanotic patients • Anticoagulation and chronic pulmonary vasodilator therapy for primary pulmonary hypertension • Avoid aspirin and other antithrombotic medications in patients with Eisenmenger’s syndrome because these patients are at an increased risk of bleeding • Antiarrhythmic therapy

40. Patient Monitoring • Close regular visits with both primary care physician and a cardiologist trained in congenital heart disease. • The frequency of visits would depend on the functional class of the patient and the expected natural history of the congenital cardiac lesion.

41. Expected Course and Prognosis • Depends on the severity of the underlying lesion

42. Patient Education • The patient must be educated as to the particular risks and limitations of his or her type of congenital heart disease. • The degree of exercise that is safe for an individual patient depends on the nature of the underlying congenital lesion.

43. Risk for Strenuous Exercise • Aberrant coronary artery that crosses between the aorta and the right ventricular outflow track • Greater than moderate coarctation of the aorta, Marfan’s syndrome, or bicuspid aortic valve with aortic root dilatation (patients should especially avoid isotonic exercise) • Moderate to severe aortic stenosis • Moderate to severe pulmonary stenosis • Balanced tetralogy of Fallot