What do I need to know about my adult patient with congenital heart disease?

1. What do I need to know about my adult patient with congenital heart disease? www.anaesthesia.co.in anaesthesia.co.in@gmail.com

2. Adult Congenital Heart Disease

3. 38 y/o male with Tetralogy of Fallot and Pulmonary Atresia, B-T shunt. Works (janitorial), married, two children. Looks blue but otherwise normal. Hx brain abscess age 18. Periodic phlebotomies by hematologist. At “other” hospital: admit for “simple” urologic procedure, profound hypotension after anesthesia induction with worsening hypoxemia. Procedure terminated. • BP labile post, some “unobtainable” though patient asymptomatic, some normal. TIA day 2 post-op. Treated with aspirin. • Feeling “poorly, weak” after discharge. Saw primary MD, blood work “OK” including electrolytes, LFTs, TFTs, CBC (hgb 12.9, hct 39). Referred to RI Adult Congenital Heart Program

4. Adult Congenital Heart Disease is an epidemic! • Over 2 million in USA • Most common birth defect • Most (over 85%) now living to adulthood • Most common presenting in adulthood: bicuspid aortic valve, coarctation of aorta, atrial septal defect • Most common post-op seen as adults: tetralogy of Fallot, Transposition of Great Arteries (TGA)

5. Adults with congenital heart disease may look like your other patients. They’re not… Don’t be taken by surprise. • Responses to noncardiac surgery are different – risks are increased, may be life-threatening even with minor procedures… • Patients with CHD have special pregnancy issues…be prepared to address them. • Infection is an ongoing risk for many patients with CHD… • Cyanotic patients with CHD require special management… • Jobs, school, and sports may raise special questions for patients with CHD…

6. Noncardiac surgery in patients with CHD… • Risks: arrhythmias, complications of cyanosis, anticoagulation issues, adverse effects on shunts leading to hypotension, hypoxemia, paradoxical embolus, infection; endocarditis • Our patient: anesthesia effect worsening shunting with hypotension and hypoxemia; later TIA due to unnecessary IV without filter and paradoxical embolus • Management: understand anatomy and physiology,anticipate potential for complications and treatment. (our patient – nurses didn’t know about BT shunt, erroneous BP readings…). • Pre-op studies if needed – echo, EKG, monitor, CBC, coags, etc. • Stratify risk, communication between anesthesia, surgeon, patient’s physicians. Choose your anesthesiologist! • General steps: optimize hemodynamics, prophylactic antibiotics if needed, meticulous IV line care, hematologic management, maintain hydration and oxygenation (atelectasis), rhythm monitoring, hemodynamic monitoring in select cases.

7. Doctor, I think I’m pregnant… • Birth Control: thrombogenic potential of BCPs esp with higher estrogen content; IUDs with risks at time of insertion, ongoing risk of infection, menorrhagia; surgical sterilization; TOP in high-risk pregnancies (pulmonary hypertension, etc.) • Counseling: risk stratification for mother and baby, management during pregnancy, risk of CHD in baby • Low risk: valve regurgitation with good function, mild-mod valve stenosis, small to moderate left to right shunts (VSD, ASD), post-op CHD with good function, minimal/no hypoxemia • High risk: ventricular dysfunction, pulmonary hypertension (esp. Eisenmenger’s Syndrome), hypoxemia, severe left heart obstructive lesions, aortic root dilation (Marfan’s), thrombus/embolus potential (mechanical valves), dangerous ventricular arrhythmias • Hemodynamic stresses: increased blood volume (32 wks), CO (20-24 wks), HR; decreased SVR and PVR. L &D: CP increase, SVR and BP decrease; smaller changes in lateral recumbent position.

8. Pregnancy… • Complications: CHF, aortic dissection or rupture, worsening right to left shunt with hypoxemia; thromboemboli; syncope, sudden death, endocarditis, fetal and maternal risks of anticoagulation ( and too little anticoagulation) • Management: understand anatomy and physiology, anticipate complications and treatment. Best to plan with patient before pregnancy, follow with team (high-risk OB, ACHD center, etc.). • Minimize anxiety, judicious diuretics for CHF, avoid isometric exercise, make sure Hct appropriate, prompt treatment of infections, meticulous leg care; normal vaginal delivery usual, CX for intractable CHF, R to L shunt with fetal distress, Marfan’s with dilated root. Vasodilation with epidural, monitor O2; antibiotics • Fetal echo 18-20 weeks. Risks of CHD in baby: no FH<1%, one parent 2-4%, more with maternal (up to 13.5%, highest with AS, VSD, PS), 1.5-3% for father, slight increase with parental sibling. • Anticoagulation: high-risk, controversial. Heparin 12 weeks, coumadin, then heparin for L&D. No consensus…

9. Patients with CHD may be at risk for infection… • Increased risk with prosthetic materials (valves, conduits, baffles), shunts, abnormal surfaces such as bicuspid valve. Unusual locations for endocarditis (RV in patients with VSD) • Lifestyle issues: body piercing, tattoos; need for good dental hygiene and regular care • Prophylaxis, prompt treatment of potential bacterial infections, avoid unnecessary IVs in hospital (avoid hospitals!) • Patient education is the most important tool! They must know they are at risk, and remind caregivers…

10. Cyanotic patients with CHD require special management... • Erythrocytosis is a normal response to normalize tissue oxygenation. Increased blood viscosity is rarely a problem, equilibrium is usually achieved. Bleeding more common than clotting (avoid platelet inhibitors). • Phlebotomy is rarely needed, except: symptomatic with Hct>65 (usually neurologic, may be hemorrhagic). No more than one unit at a time, replace with isotonic saline. • Think about Fe-deficiency! Worsened by phlebotomy, leads to impaired O2 delivery due to RBC abnormalities (our patient). Careful replacement! • INR measurement needs citrate adjustment in tube due to lower plasma volume! • High urate levels common – don’t treat unless gout

11. Functional status issues may require special attention in patients with CHD… • Jobs, school, sports, insurability, planning for the future… • Integrate knowledge of: specific conditions, subjective reports from patient, objective information from testing and exam • Stress testing (especially metabolic stress testing with measurement of oxygen consumption) useful. Often divergence of patient report and objective findings • Sports risks: high in connective tissue disease with aortic involvement (Marfan’s), coarctation, bicuspid aortic valve with aortic root dilation: avoid isometric stresses. Hypertrophic CMP risk of sudden death; obstructive lesions. • Advise patients about potential insurability issues before they find out the hard way…

12. What setting is most appropriate for follow-up of adults with congenital heart disease? • Primary care and cardiologist without special ACHD training: isolated mild PS, small restrictive VSD (endocarditis is primary risk); successfully closed ASD (late atrial arrhythmias); isolated aortic valve disease (same management as acquired) • ACHD Center: complex CHD; post-op other than above (most operations are palliative with late sequelae – TOF, TGA, Fontan, etc.- an unfolding story); cyanotic CHD. • Resources: see handout

13. Adults with Congenital Heart Disease… Coming to your office soon!

14. Adults with Congenital Heart Disease… On the Web... cachnet.org (Canadian Adult Congenital Heart Network) excellent and up to date source of information, including management guidelines and information for patients Pediheart.org good source of material for patients; anatomy and descriptions of specific defects for practitioners (requires MD registration for physician use) Review Text: Perloff JK, Childs JS, eds. Congenital heart disease in adults. 2nd ed. Philadelphia: W.B. Saunders, 1998:15-53. Review Articles: Medical Progress: Congenital Heart Disease in Adults— First of Two PartsBrickner M. E., Hillis L. D., Lange R. A. N Engl J Med 2000; 342:256-263, Jan 27, 2000. Medical Progress: Congenital Heart Disease in Adults— Second of Two PartsBrickner M. E., Hillis L. D., Lange R. A. N Engl J Med 2000; 342:334-342, Feb 3, 2000. PROCEEDINGS OF THE 32ND BETHESDA CONFERENCE CARE OF THE ADULT WITH CONGENITAL HEART DISEASE JACC Vol. 37, 2001: 1161-98 Recommendations for Determining Eligibility for Competition in Athletes with Cardiovascular Abnormalities: Bethesda Conference 26 (Revision of Bethesda Conference #16), January 6-7, 1994. (J Am Coll Cardiol 1994;24:845-99) www.anaesthesia.co.in anaesthesia.co.in@gmail.com