Follow-Up for Common Congenital Heart Defects: Non-Surgical

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7 th Annual Northern Nevada Heart Conference Follow-Up for Common Congenital Heart Defects: Non-Surgical Katrinka T Kip, MD Associate Professor of Pediatrics University of Nevada Reno, School of Medicine

Part II Valves and Other

T. S. Followed in our prac:ce un:l the age of 4 years with a diagnosis of mild aor:c stenosis (mean gradient of 30 mmhg across aor:c valve). Family lost insurance and were unaware that they qualified for state assistance. Next follow-up at 12 years. Gradient is now 120 mmhg across aor:c valve.

Pulmonary Valve Stenosis Mild PS, gradient less then 30 mmhg, is not likely to progress. Follow-up is recommended every 5 years. Mild to moderate PS, gradient between 30 and 50 mmhg s:ll not likely to progress. Follow-up is recommended every 2-5 years. ASer surgery or balloon procedure degree of PI determines. Follow-up should be at least every 5 years.

Aor:c Stenosis/BAV Follow-up should be yearly, even in pa:ents with no significant stenosis or insufficiency. As well as aor:c valve dysfunc:on there is a high incidence of ascending aor:c enlargement. Follow-up will be more frequent depending on degree of insufficiency or stenosis of valve. Follow-up also allows reitera:on to parents of family transmission of aor:c valve disease.

N. A. Infant with an interrupted aor:c arch A (extreme coarcta:on) and VSD repaired at birth ReadmiYed in CHF 3 months later. Cardiac cath showed residual gradient at repaired arch. The balloon angioplasty was unsuccessful. Next follow-up was at age 10 aser ER visit.

Coarcta:on 80 percent of individuals will have an aor:c valve abnormality as well as a coarcta:on. Whether interven:on done or not, follow-up should be every year.

Severe Coarcta:on

Surgery for Coarcta:on

Stents

Coarcta:on ASer Stent

Mitral and Tricuspid Valve Disease These lesions were hard to find recommenda:ons on follow-up. Our prac:ce has been to recommend follow-up on pa:ents with more then mild regurgita:on. The follow-up has been every 1-2 years. Mild regurgita:on is normal as children approach teens. Tricuspid stenosis alone is rare for our pa:ents and I have no thoughts.

Kawasaki Disease New Guidelines out in March 2017. Follow-up recommended every 1-3 years in pa:ents who have small aneurysms or persistent dila:on of coronary greater then z- score of > 2.5 every 1-3 years. Follow-up not recommended for dila:on less then z-score of 2 with regression or normal coronary aser 1 year.

More Kawasaki Disease These recommenda:ons are from newest AHA (March 2017) discussion of Kawasaki s. In prac:ce cardiologists are more conserva:ve. Follow-up has been recommended every 5 years, with dila:on or not. Educa:on about disease is key for pa:ents approaching adulthood.

Last thoughts on Kawasaki Controversy around follow-up appears to come from some vascular studies sugges:ng abnormal reac:vity of blood vessels (like brachial arteries) in pa:ents with a history of Kawasaki disease. Reac:vity more impaired in pa:ents with abnormal coronary arteries ini:ally. But clinically no increased risk for CAD in pa:ents without persistent aneurysms or dila:on.

Conclusion Follow-up should be no longer then every 5 years for most lesions (repaired and unrepaired VSD, except muscular). ASD Device closure every 1-5 years. Most people discharge PDA, coiled or ligated, PFO and small muscular VSD s. Kawasaki (normal coronaries) every 5 years. Abnormal aor:c valve, coarcta:on every 1 year.

Diversity in Follow-up An email survey of CHC physicians (14) was done. There was a poor response (50%) to survey. Most extreme prac:ce: No pa:ent with a lesion (including PFO) gets discharged. Other respondents do discharge pa:ents: PDA aser liga:on or coiling, PFO.

Follow-up, Does It Happen? The focus of this talk was when our pa:ents should be seen for follow-up. In an ideal world the recommenda:ons outlined would be followed. The world is not ideal. Mackie et al report only 39% adults with CHD were followed by age 25. Given that, educa:on of parents, pa:ents needs to be complete, especially for teens becoming adults.

Final Thoughts on Follow-up My recommenda:ons come primarily for AHA/ACA consensus statements. I agree with Journal of Thoracic Diseases editorial from December 2016 which concludes that more studies are needed on follow-up of simple congenital heart disease. Almost all recommenda:ons were Level of Evidence-C. Only a few were even B.

References (not in any order) Zuin,M et al: Systema:c long-term follow-up programs in pa:ents with simple congenital heart diseases: how long is long? Journal of Thoracic Disease /vol 8.No 12 (December 2016. Videbaek J et al: Long-Term Na:onwide Follow-Up Study of Simple Congenital Heart Disease Diagnosed in Otherwise Healthy Children Circula:on 2016;133:474-83.

References Tierney, ES, et al: Vascular Health in Kawasaki Disease. Journal of the American College of Cardiology. Vol62, issue 12, 17 September 2013 p1114=1121. Cheung YF, et al: Novel and tradi:onal cardiovascular risk factors in Children aser Kawasaki disease: implica:ons for premature atherosclerosis. JACC 2004 Jan7;43(1):120-4.

References For references for VSD and AI see Nadas Pediatric Cardiology : second edi:on, Keane et al. For most informa:on about follow-up see ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease. hyp://circ.ahajounals.org/contents/ 118/23/2395

References Mackie AS, et al. Children and Adults with congenital heart disease lost to follow-up. Who and When? Circula:on 2009;120:302-9. McCrindle B, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scien:fic Statement for Health Professionals From the American Heart Associa:on. Circula:on online March 29.2017.