Early management of congenital heart diseases

Jameel A. AL-AtaJameel A. AL-Ata

Consultant & assistant professor of Consultant & assistant professor of pediatrics & pediatric cardiology.pediatrics & pediatric cardiology.

Introduction

Outcome of CHD has improved mainly due to Outcome of CHD has improved mainly due to improved Surgical & Interventional care, specially improved Surgical & Interventional care, specially for neonates. for neonates.

In KSA overall CHD surgical mortality in 4 large In KSA overall CHD surgical mortality in 4 large centers is 3—6 %.centers is 3—6 %.

Pre-surgical morbidity & mortality remains high Pre-surgical morbidity & mortality remains high for many different reasons. for many different reasons.

Introduction

Poor early recognition.( pre , natal & postnatal ).Poor early recognition.( pre , natal & postnatal ). Delayed presentation.Delayed presentation. None familiarity of pathophysiology and natural None familiarity of pathophysiology and natural

history of CHD.history of CHD. Delayed initiation of treatment.Delayed initiation of treatment. Limited NICU / PICU facilities.Limited NICU / PICU facilities. Limited PGE availability .Limited PGE availability . Limited medivac services.Limited medivac services. Others.Others.

Pediatricians can make the difference by ; Early recognition.Early recognition.

Categorizing into type & severity.Categorizing into type & severity.

Timely initiation of proper medical treatment.Timely initiation of proper medical treatment.

Timely referral for interventional or surgical treatment. Timely referral for interventional or surgical treatment.

= = EARLY MANAGEMENTEARLY MANAGEMENT

Early management of secondum Atrial Septal Defect ; Confirm DX and size of ASD.Confirm DX and size of ASD.

Most pts will not need medical treatment.Most pts will not need medical treatment.

Assure parents and inform them of high likelihood Assure parents and inform them of high likelihood of spontaneous closure.of spontaneous closure.

Watch for development of PHTN at F/U.Watch for development of PHTN at F/U. Look for none cardiac associations.Look for none cardiac associations.

ASD

No limitation of activity.No limitation of activity.

SBE prophylaxis not usually recommended.SBE prophylaxis not usually recommended.

Screen the family.Screen the family.

Follow every 6—12 months.Follow every 6—12 months.

Refer for intervention or surgery at age 3-5 y. if size Refer for intervention or surgery at age 3-5 y. if size remains > 5 mm.remains > 5 mm.

Early management of VSD ;

Confirm DX , type of VSD & size.Confirm DX , type of VSD & size.

Examine for presence or development of coarctation or Examine for presence or development of coarctation or aortic insufficiency.aortic insufficiency.

Medical therapy ( diuretics +/- ACE ) usually needed Medical therapy ( diuretics +/- ACE ) usually needed for > 5mm defects. for > 5mm defects.

DigoxinDigoxin not usually needed. not usually needed.

Treat respiratory infections aggressively.Treat respiratory infections aggressively.

VSD

Ensure optimum caloric intake.Ensure optimum caloric intake.

High risk of development of PHTN.High risk of development of PHTN.

Large VSDs can be silent. ( PHTN )Large VSDs can be silent. ( PHTN )

No limitation of activity.No limitation of activity.

SBE prophylaxis is a must.SBE prophylaxis is a must.

VSD Follow monthly Follow monthly < < 4 m.o & every 2—4 m. for age 4 m.o & every 2—4 m. for age >> 4 4

m.o.m.o. Refer to surgery or intervention if ;Refer to surgery or intervention if ; 1) FTT ,CHF 2) PHTN 3) AI 4) Endocarditis. 1) FTT ,CHF 2) PHTN 3) AI 4) Endocarditis. ( ( usual ageusual age 6—12 months ) 6—12 months ) Small < 5mm muscular & Pm VSDs have a good Small < 5mm muscular & Pm VSDs have a good

chance for spontaneous closure, so assure parents but chance for spontaneous closure, so assure parents but follow the Pm VSD for AI.follow the Pm VSD for AI.

Inlet & Sub arterial VSDs do not close spontaneously. Inlet & Sub arterial VSDs do not close spontaneously.

Early management of PDA

Confirm DX and size.Confirm DX and size.

Spontaneous closure is the rule in the 1st Spontaneous closure is the rule in the 1st year of life , so assure parents.year of life , so assure parents.

Limitation of activity not needed.Limitation of activity not needed.

Medical therapy ( diuretics +/- ACE ) can Medical therapy ( diuretics +/- ACE ) can be needed usually if size > 2 mm.be needed usually if size > 2 mm.

PDA

Large PDA > 3 mm act like large VSDs.Large PDA > 3 mm act like large VSDs.

Look for associations cardiac or non Look for associations cardiac or non cardiac.cardiac.

Small PDAs can be referred for intervention Small PDAs can be referred for intervention if still patent at age > 1 year whether if still patent at age > 1 year whether symptomatic or not.symptomatic or not.

Early management of aortic stenosis ;

Confirm DX and severity.Confirm DX and severity.

Look for aortic insufficiency and other associations.Look for aortic insufficiency and other associations.

Mild to moderate AS. do not require medical therapy. Mild to moderate AS. do not require medical therapy. Avoid hypotensive agents. Avoid hypotensive agents.

Assure strict 6 m. f/u by echocardiography for grading Assure strict 6 m. f/u by echocardiography for grading of severity & LVH + function.of severity & LVH + function.

Limit activity only if moderate to severe stenosis , no Limit activity only if moderate to severe stenosis , no need to limit usual daily activity but only strenuous need to limit usual daily activity but only strenuous exercise and competitive sports.exercise and competitive sports.

AS . ;

Strict SBE prophylaxis & dental hygiene.Strict SBE prophylaxis & dental hygiene.

Admit the child with AS. and chest pain & obtain Admit the child with AS. and chest pain & obtain urgent cardiac consultation.urgent cardiac consultation.

Refer for balloon valvuloplasty if severe except Refer for balloon valvuloplasty if severe except for sub aortic stenosis which should be referred for sub aortic stenosis which should be referred earlier to prevent aortic insufficiency.earlier to prevent aortic insufficiency.

Critical AS is an emergency that presents with Critical AS is an emergency that presents with CHF & may PDA dependant. CHF & may PDA dependant.

Early management of Pulmonary stenosis

Confirm DX & severity.Confirm DX & severity.

Look for associations.Look for associations.

Even severe PS usually does not require medical Even severe PS usually does not require medical therapy.therapy.

Limitation of activity is usually not required.Limitation of activity is usually not required.

PS . ;

SBE prophylaxis is controversial.SBE prophylaxis is controversial.

Yearly F/U for mild to moderate PS & 6 m. for Yearly F/U for mild to moderate PS & 6 m. for moderate to severe by echocardiography.moderate to severe by echocardiography.

Refer for balloon valvuloplasty if severe.Refer for balloon valvuloplasty if severe.

Critical PS can present with RV failure & or Critical PS can present with RV failure & or cyanosis and may be PDA dependant. cyanosis and may be PDA dependant.

Early management of Tetralogy of Fallot A surgical cyanotic CHD where our role is A surgical cyanotic CHD where our role is

to get the child to surgery safely at ag 6-9 mto get the child to surgery safely at ag 6-9 m Excellent physical growth.Excellent physical growth. CHF is rare.CHF is rare. Accept saturation > 70% in room airAccept saturation > 70% in room air Prevent aneamia.Prevent aneamia. Prevent dehydration. ( no LASIX ).Prevent dehydration. ( no LASIX ).

TOF

Prevent endocarditis.Prevent endocarditis.

Advice to avoid high altitudeAdvice to avoid high altitude

Prevent and treat hypercyanotic spells.Prevent and treat hypercyanotic spells.

Refer to earlier than 6 m if developed spells. Refer to earlier than 6 m if developed spells.

Early management of PDA dependant CHD Severe acute cyanosis < 70 % or circulatory collapse Severe acute cyanosis < 70 % or circulatory collapse

in the 1in the 1stst week of life indicate cyanotic or Lt sided week of life indicate cyanotic or Lt sided obstruction PDA dependant CHD respectively.obstruction PDA dependant CHD respectively.

Examples are pulmonary atresia and d-TGA for Examples are pulmonary atresia and d-TGA for cyanotic and critical COA or IAA. For obstructive cyanotic and critical COA or IAA. For obstructive Lt sided CHD.Lt sided CHD.

The PDA provides the needed PBF, MIXING ,or The PDA provides the needed PBF, MIXING ,or SBF. for these lesions.SBF. for these lesions.

PDA dependant CHD Our aims in these pts are ; Our aims in these pts are ; 1) 1) Keep ductal patency by PGE through a secure Keep ductal patency by PGE through a secure

venous line.venous line. 2) 2) Maintane saturation 75—80 % in RA even if Maintane saturation 75—80 % in RA even if

ventilated to avoid induction of CHF 2ventilated to avoid induction of CHF 2ndnd to increased to increased PBF with decreasing PVR.PBF with decreasing PVR.

3) 3) Avoid pulmonary vasodilation.Avoid pulmonary vasodilation.

PDA dependant CHD4) 4) Avoid fluid overload .Avoid fluid overload . 5)5) Avoid infection. Avoid infection. 6)6) Early intervention or surgery within 2-7 d. Early intervention or surgery within 2-7 d.

7)7) Provide a mixing or loading site.( d-TGA & HLHS ). Provide a mixing or loading site.( d-TGA & HLHS ).

8)8) Aggressive correction of metabolic acidosis. Aggressive correction of metabolic acidosis.

Conclusion

Knowing the pathophysiology and natural history of Knowing the pathophysiology and natural history of outcome is essential in the management of CHD.outcome is essential in the management of CHD.

Most CHD pts can be managed as OPD in the Most CHD pts can be managed as OPD in the community provided there is a clear plan set between community provided there is a clear plan set between the primary pediatrician and the cardiologist.the primary pediatrician and the cardiologist.

More exposure of ped. Trainees to CHD medical More exposure of ped. Trainees to CHD medical therapy & surgery and to the ICU care these pts need therapy & surgery and to the ICU care these pts need will help in increasing the successful early will help in increasing the successful early management of CHD BY the pediatrician.management of CHD BY the pediatrician.

THANK YOUTHANK YOU