Early management of congenital heart diseases

1. Earlymanagement of congenital heart diseases Jameel A. AL-Ata Consultant & assistant professor of pediatrics & pediatric cardiology.

2. Introduction • Outcome of CHD has improved mainly due to improved Surgical & Interventional care, specially for neonates. • In KSA overall CHD surgical mortality in 4 large centers is 3—6 %. • Pre-surgical morbidity & mortality remains high for many different reasons.

3. Introduction • Poor early recognition.( pre , natal & postnatal ). • Delayed presentation. • None familiarity of pathophysiology and natural history of CHD. • Delayed initiation of treatment. • Limited NICU / PICU facilities. • Limited PGE availability . • Limited medivac services. • Others.

4. Pediatricians can make the difference by ; • Early recognition. • Categorizing into type & severity. • Timely initiation of proper medical treatment. • Timely referral for interventional or surgical treatment. = EARLY MANAGEMENT

5. Early management of secondum Atrial Septal Defect ; • Confirm DX and size of ASD. • Most pts will not need medical treatment. • Assure parents and inform them of high likelihood of spontaneous closure. • Watch for development of PHTN at F/U. • Look for none cardiac associations.

6. ASD • No limitation of activity. • SBE prophylaxis not usually recommended. • Screen the family. • Follow every 6—12 months. • Refer for intervention or surgery at age 3-5 y. if size remains > 5 mm.

7. Early management of VSD ; • Confirm DX , type of VSD & size. • Examine for presence or development of coarctation or aortic insufficiency. • Medical therapy ( diuretics +/- ACE ) usually needed for > 5mm defects. Digoxin not usually needed. • Treat respiratory infections aggressively.

10. VSD • Ensure optimum caloric intake. • High risk of development of PHTN. • Large VSDs can be silent. ( PHTN ) • No limitation of activity. • SBE prophylaxis is a must.

11. VSD • Follow monthly < 4 m.o & every 2—4 m. for age > 4 m.o. • Refer to surgery or intervention if ; 1) FTT ,CHF 2) PHTN 3) AI 4) Endocarditis. ( usual age 6—12 months ) • Small < 5mm muscular & Pm VSDs have a good chance for spontaneous closure, so assure parents but follow the Pm VSD for AI. • Inlet & Sub arterial VSDs do not close spontaneously.

12. Early management of PDA • Confirm DX and size. • Spontaneous closure is the rule in the 1st year of life , so assure parents. • Limitation of activity not needed. • Medical therapy ( diuretics +/- ACE ) can be needed usually if size > 2 mm.

16. PDA • Large PDA > 3 mm act like large VSDs. • Look for associations cardiac or non cardiac. • Small PDAs can be referred for intervention if still patent at age > 1 year whether symptomatic or not.

17. Early management of aortic stenosis ; • Confirm DX and severity. • Look for aortic insufficiency and other associations. • Mild to moderate AS. do not require medical therapy. Avoid hypotensive agents. • Assure strict 6 m. f/u by echocardiography for grading of severity & LVH + function. • Limit activity only if moderate to severe stenosis , no need to limit usual daily activity but only strenuous exercise and competitive sports.

18. AS . ; • Strict SBE prophylaxis & dental hygiene. • Admit the child with AS. and chest pain & obtain urgent cardiac consultation. • Refer for balloon valvuloplasty if severe except for sub aortic stenosis which should be referred earlier to prevent aortic insufficiency. • Critical AS is an emergency that presents with CHF & may PDA dependant.

19. Early management of Pulmonary stenosis • Confirm DX & severity. • Look for associations. • Even severe PS usually does not require medical therapy. • Limitation of activity is usually not required.

20. PS . ; • SBE prophylaxis is controversial. • Yearly F/U for mild to moderate PS & 6 m. for moderate to severe by echocardiography. • Refer for balloon valvuloplasty if severe. • Critical PS can present with RV failure & or cyanosis and may be PDA dependant.

21. Early management of Tetralogy of Fallot • A surgical cyanotic CHD where our role is to get the child to surgery safely at ag 6-9 m • Excellent physical growth. • CHF is rare. • Accept saturation > 70% in room air • Prevent aneamia. • Prevent dehydration. ( no LASIX ).

22. TOF • Prevent endocarditis. • Advice to avoid high altitude • Prevent and treat hypercyanotic spells. • Refer to earlier than 6 m if developed spells.

24. Early management of PDA dependant CHD • Severe acute cyanosis < 70 % or circulatory collapse in the 1st week of life indicate cyanotic or Lt sided obstruction PDA dependant CHD respectively. • Examples are pulmonary atresia and d-TGA for cyanotic and critical COA or IAA. For obstructive Lt sided CHD. • The PDA provides the needed PBF, MIXING ,or SBF. for these lesions.

27. PDA dependant CHD • Our aims in these pts are ; 1) Keep ductal patency by PGE through a secure venous line. 2) Maintane saturation 75—80 % in RA even if ventilated to avoid induction of CHF 2nd to increased PBF with decreasing PVR. 3) Avoid pulmonary vasodilation.

28. PDA dependant CHD 4) Avoid fluid overload . 5) Avoid infection. 6) Early intervention or surgery within 2-7 d. 7) Provide a mixing or loading site.( d-TGA & HLHS ). 8) Aggressive correction of metabolic acidosis.

29. Conclusion • Knowing the pathophysiology and natural history of outcome is essential in the management of CHD. • Most CHD pts can be managed as OPD in the community provided there is a clear plan set between the primary pediatrician and the cardiologist. • More exposure of ped. Trainees to CHD medical therapy & surgery and to the ICU care these pts need will help in increasing the successful early management of CHD BY the pediatrician.

30. THANK YOU