Adult Congenital Heart Disease: What Every Practitioner Should Know

Transcription

1 Adult Congenital Heart Disease: What Every Practitioner Should Know Sabrina Phillips, MD FACC FASE Associate Professor of Medicine Director of Adult Congenital Heart Disease Services The University of Oklahoma

2 No Disclosures

3 SCOPE Cardiac malformations occur in 8 per 1000 live births. 32,000 new cases in the United States per year. 80% of new cases will survive one year. 80% of 1-year survivors will survive to adulthood 1,000,000 adults with congenital heart disease in the U.S.

4 No Lesion is Cured by Surgery Physiologic repair Palliation

5 Who Needs to be Seen? Any new diagnosis Anyone with a named operation Anyone who is cyanotic Anyone with a previous diagnosis who is considering pregnancy Anyone with a complete repair who has not seen a specialist since becoming an adult or who has new symptoms

6 Case 1

7 26-Year-Old Male Coarctation repair (end to end) age 4 No medical visits in 10 years No symptoms, physically active Contacted to participate in a research study re: intra-cranial aneurysm

8 MRA Brain 2mm Aneurysm at Origin of Right Choroidal Artery

9 Neurology Consultation BP 120/70 (RUE) 124/74 (LUE) Neurologic exam normal

10 Management Options? 1. Repeat MRA 6 months 2. Repeat MRA 6 months and have patient see cardiology at that time 3. Repeat MRA 6 months and have patient see cardiology now 4. No follow up needed

11 Management Options? 1. Repeat MRA 6 months 2. Repeat MRA 6 months and have patient see cardiology at that time 3. Repeat MRA 6 months and have patient see cardiology now 4. No follow up needed

12

13 26-Year-Old Prior Coarctation Repair

14 26-Year-Old Prior Coarctation Repair

15 26-Year-Old, Prior Coarctation Repair

16 Take Home Points Patients with coarctation of the aorta have an increased risk of intracranial aneurysm

17 5-Fold Increase in ICA Risk in Patients With Coarctation Mayo Clinic Proceedings 2003

18 Take Home Points Patients with congenital heart disease are often lost to follow up during and after childhood

19 Patients Lost To Follow Up During Childhood Mackie A et al. Circulation 2009;120: Copyright American Heart Association

20 Patients in Contact with the Healthcare System Mackie A et al. Circulation 2009;120: Copyright American Heart Association

21 Predictors of Lack of Cardiology Follow Up Male gender Non-severe lesion Treatment outside a university hospital system Mackie A et al. Circulation 2009;120:

22 Case 2

23 32 year old Woman Maternal lithium use during pregnancy Cyanotic at birth Diagnosed with Ebstein anomaly in the neonatal period Followed routinely in childhood told to not have surgery on the valve Presents for routine follow-up No complaints

24 ECHO Report Ebstein anomaly of the tricuspid valve Marked tethering of the anterior leaflet with multiple fenestrations Right ventricular enlargement with reduced systolic function RVSP 35 mmhg LV EF 65%

25 Exercise Testing 53% FAC No desaturation No arrhythmias

26 Surgery Tricuspid valve replacement: 35 mm CE Right reduction atrioplasty Resection (inferior wall) of atrialized right ventricle

27

28 6 month Follow-up Marked subjective improvement in exercise capacity FAC 94% predicted

29 Take Home Points The tricuspid valve can be replaced with good results Patients often don t recognize their exercise limitations Don t wait for significant right heart failure to operate

30 Case 3

31 Can I Run A Marathon? 25 year old woman with tetralogy of Fallot Initial repair in infancy PVR age 20 RV mildly enlarged, normal systolic function, mildmod PR, mild PS Peak VO2 72% of predicted

32 Peak VO2 ml/kg/min Peak VO2 of Heart Failure Patients versus CHD Patients Congenital Heart Disease Patients (mean age 33 years) Heart Failure Patients (mean age 59 years) 0 Age Matched Controls NYHA I NYHA II NYHA III Diller et al. Circ 2005; 112:

33 Freedom from hospitalization or death (%) VO2 Predicts Clinical Outcome Quartile 1; VO2 <15.5 ml/kg/min Quartile 2-3;VO ml/kg/min Quartile 4; VO2>27 ml/kg/min Days Diller et al. Circ 2005; 112:

34 Can I Run a Marathon? A. Yes B. No

35 Outcome Patient met with exercise physiologist to discuss training One year later completed one marathon Peak VO2: 124% of predicted

36

37 CHD Patients Can Improve Fitness Level with Training Holloway et al. International Journal of Cardiology Volume 150, Issue

38 Take Home Points CHD patients can and should exercise CHD patient need guidance for exercise Exercise improves fitness and quality of life scores

39 Case 4

40 32 Year Old Woman Born at 28 weeks gestation Lung disease of prematurity Found to have severe pulmonary HTN age 5 large PDA On triple therapy including IV Flolan Presents to establish care Functional status stable

41 32 Year Old Woman with Eisenmenger PDA Using no contraception Seeing a blood doctor

42

43 Eisenmenger Syndrome New equilibrium Hematologic Issues Tissue hypoxia O 2, sat erythropoietin Improved tissue oxygenation number RBCs RBC mass Negative feedback Bone marrow Erythroid cell proliferation Courtesy of Dr. Heidi Connolly

44 Iron Deficiency Anemia blood viscosity O 2 carrying capacity fragility RBC survival Cause Risk Usually phlebotomy stroke Treatment Iron therapy risk of stroke Courtesy of Dr. Heidi Connolly

45 Phlebotomy Perform for hyperviscosity symptoms if no dehydration or iron deficiency: - Long bone pain - Headache - Altered mentation Preoperatively to improve hemostasis Crystalloid volume replacement must be given if phlebotomy is performed

46 Avoid Pregnancy Counsel early Consider permanent contraception Avoid estrogens but can use Depo, mini-pill, Mirena IUD

47 Take Home Points Don t routinely phlebotomize patients who are cyanotic with intra-cardiac shunts Cyanotic patients with relative anemia may improve with cautious iron replacement You must be up to date on contraceptive choices if you are counselling a patient

48