Adults with Congenital Heart Disease Michael E. McConnell MD, Wendy Book MD Teresa Lyle RN NNP
Outline History of CHD Statistics Specific lesions (TOF, TGA, Single ventricle) Erythrocytosis Pregnancy
History of Congenital Heart Disease Surgery 1938 Closure of a patent arterial duct by Robert Gross (Boston) 1944 Repair of coarctation of the aorta by Clarence Crafoord (Sweden) 1945 Palliation of tetralogy of Fallot by Alfred Blalock (Baltimore) 1948 Opening of a stenotic mitral valve by Charles Bailey (Philadelphia)
History of Congenital Heart Disease Surgery (continued) 1952 Closure of an ASD using hypothermic cardiac arrest by F. John Lewis, Minneapolis 1954 Closure of a VSD using controlled cross circulation by Walt Lillehei, Minneapolis 1954 Repair of tetralogy of Fallot using controlled cross circulation by Walt Lillehei, Minneapolis
Adults with Congenital Heart Disease Over 1,000,000 adults with congenital heart disease living in North America 26,000 infants are born with CHD annually ~ 300 CHD patients in the Atlanta area reach adulthood each year
Adults with Congenital Heart Disease 50-75% have had corrective or palliative surgery ~30% will require additional surgery Many will have improved survival if cared for in a comprehensive adult CHD clinic
Adults with Congenital Heart Disease Pediatric cardiologists Internal medicine cardiologists with additional training in adults with congenital heart disease Med/Peds trained cardiologists EP/Surgeons/Interventional cardiologists/high risk OB/congenital cardiac anesthesiologists
Tetralogy of Fallot
Tetralogy of Fallot
Shunts for patients with limited pulmonary blood flow
Surgical correction of tetralogy of Fallot
Consequences of a transannular patch
Transannular patch (cont d)
Enlarged RV after a transannular patch
Improved RV after pulmonary valve replacement
Improved RV after pulmonary valve replacement
Pulmonary Valve Replacement Changes in RV size and function after pulmonary valve replacement in 26 adults with tetralogy of Fallot From Vliegen H et al Circulation 106 (13) Sept 2002 1703-1707.
Pulmonary Valve Replacement Impact of Pulmonary Valve Replacement on Arrhythmia Propensity Late After Repair of Tetralogy of Fallot Therrien, J et al. Circulation Volume 103(20) 22 May 2001 pp 2489-2494
Transposition of the Great Vessels
Transpositions
Transposition of the Great Vessels
Transposition of the Great Vessels
Transposition of the Great Vessels
Transposition of the Great Vessels
Transposition of the Great Vessels
Transposition of the Great Vessels
Transposition of the Great Vessels
Transposition of the Great Vessels FIGURE 1. Percent distribution of ability index (Warnes) and patient's perception index scores as determined by answers to the health screening questionnaire. From: Ebenroth: Am J Cardiol, Volume 89(3).February 1, 2002.353-356
Transposition of the Great Vessels Surgery for the failing right ventricle 11 patients with failing RV had PA Banding, plus one other who had PS 4 had reoperation to tighten the band 6 had ASO, 2 died 4 had biventricular failure and had transplantation 2 awaiting ASO Mavroudis C and Backer CL. Annals of Thoracic Surgery. 69(3):851-7, 2000 Mar
Single Ventricle LV angiography in a patient with tricuspid atresia and a very small right ventricle
Single Ventricle Right ventriculography in a patient with a dominant right ventricle and transposition
Single Ventricle Palliation
The Fontan operation is doomed to fail Dr. Andrew Reddington, American College of Cardiology Annual Meeting March 2003 At Egleston Children s Hospital we perform about 30 Fontan operations yearly The total number of adult heart transplants performed in Georgia in 2003 was 30.
Erythrocytosis NOT Polycythemia!!! A physiologic consequence of chronic hypoxia RBC s increased, WBC low normal, platelets low
Erythrocytosis (cont d) Compensated and Decompensated Compensated- High (stable) hematocrit, replete iron stores NO MAGIC NUMBER! Decompensated- an acute rise in Hct from baseline May be secondary to acute illnesspneumonia, sepsis
Erythrocytosis (cont d) Phlebotomy is RARELY indicated Erythrocytosis complications Microcytosis (usually iatrogenic) Increased uric acid production Cholelithiasis
Pregnancy Outcome depends on the lesion and the pre pregnancy symptom status Lesions usually associated with good outcomes Left to right shunts Valve regurgitation
Pregnancy (cont d) Lesions that generally do poorly with pregnancy Stenotic lesions (AS, PS, MS) Cyanotic patients Patients with pulmonary hypertension
Pregnancy (cont d) Results from Loyola Adult Congenital Heart Clinic (Dr. Frank Cetta) 106 women with 258 pregnancies No maternal mortality 2 pacemaker malfunctions 1 atrial fibrillation 1 SVT 1 balloon mitral valvuloplasty at 32 weeks
Pregnancy (cont d) 21% spontaneous abortions 14% premature deliveries 6 sets of twins 3 neonatal deaths
Pregnancy (cont d) Loyola 46 women told not to get pregnant 19 (41%) had a total of 44 pregnancies No maternal mortality 25% spontaneous abortions 16% premature births 1 neonatal death
Summary There are many adult patients with congenital heart disease Many will require additional surgery Long term complications often include dysrrhythmias and ventricular failure, but interventions are often unique to the population
Variables PVR p Value Before (n = 13) After (n = 13) RV end-diastolic volume (ml) 149 ± 62 113 ± 44 <0.01 RV end-systolic volume (ml) 102 ± 48 69 ± 38 <0.01 LV end-diastolic volume (ml) 139 ± 57 151 ± 37 0.25 LV end-systolic volume (ml) 75 ± 49 84 ± 35 0.48 RV ejection fraction (%) 32 ± 11 40 ± 16 0.11 LV ejection fraction (%) 46 ± 22 45 ± 16 0.89 RV systolic pressure (mm Hg) 53 ± 16 41 ± 11 <0.005 Data are expressed as mean ± SD.