Ravilya_Pediatrics_ Cardiology_ As

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Williams syndrome

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Bicuspid aortic valve

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A case presentation of congenital aortic stenosis:
CHEST X-RAY
EKG
DIAGNOSIS CONFIRMED BYTREATMENT
:Key words

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Most common cardiac anomaly with Williams syndrome

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Williams syndrome:

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Williams syndrome: supravalvuIar aortic stenosis, pulmonary arterial branch stenosis, elfin face, mental retardation, and idiopathic hypercalcemia

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Bicuspid aortic valve:

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Bicuspid aortic valve: found in coarctation of aorta (70% ofcases) and in Turner syndrome <33-50% of cases).

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Most common type ofcongenital aortic stenosis

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Most common type of congenital aortic stenosis: valvular stenosis. (Other types are subvalvular and supravalvular.) A bicuspid aortic valve is one of most common congenital heart lesions and found in 2% of adults

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Complication of aortic valvotomy in neonate in congenital aortic stenosis:

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Complication of aortic valvotomy in neonate in congenital aortic stenosis: residual obstruction and aortic incompetence

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A child with history of sudden death was brought to emergency room. This child is a known cardiac patient and waiting for surgery to be done next month. (Very important for Board examination) MOST LIKELY ds

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A child with history of sudden death was brought to emergency room. This child is a known cardiac patient and waiting for surgerytobedone next month. (Very important for Board examination) MOST LIKELY ds :congenital aortic stenosis.

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A case presentation of congenital aortic stenosis:

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A case presentation of congenital aortic stenosis: a child with normal growth and development, asymptomatic, came for routine physical.

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Examination reveals

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Examination reveals systolic ejection murmur with thrill present in aortic area radiating towards neck
(R)
side and down (L) sternal border towards apex.

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clinica

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Most congenital aortic stenosis are asymptomatic, except in critical aortic stenosis which presents in early infant age with
(L)
ventricular failure (dyspnea, rales, and tachypnea), cardiomegaly, pulmonary edema, weak peripheral pulses, pale or
grayish skin color, systolic ejection murmur in right upper sternal border, anddecreased urine output. Sudden death occurs
in patient" waiting for surgery or untreated.

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CHEST X-RAY
EKG:

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CHEST X-RAY: cardiomegaly, prominent ascending aorta; calcification ofvalve in older children.
EKG: normal or (1) ventricular hypertrophy.

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DIAGNOSIS CONFIRMED BY: TREATMENT:

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DIAGNOSIS CONFIRMED BY: echocardiogram.
TREATMENT:
(a)
Children with moderate-to-severe stenosis: balloon valvuloplasty is procedure of choice; if this fails, then surgical treatment. Patients usually develop aortic insufficiency or calcification several years later, for which reoperation and often aortic valve replacement needed. Ross procedure (pulmonary valve replaces aortic valve, and homograft is placed in pulmonary valve area) are used.
(b)
NewbornwithAS:bothsurgicalandcatheterapproachareused,butballoonvalvuloplastyisdifficulttoperform.
(c)
Discrete subaortic stenosis: surgical resection of subaortic stenosis without damageto aortic valve.
(d)
Supravalvular stenosis: surgical correction.

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Key words:

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Key words: systolic ejection murmur in right upper sternal border (aortic area) radiating towards neck (R) side and down (1)
sternal border toward" apex.

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Most common cardiac anomaly with Williams syndrome:

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Most common cardiac anomaly with Williams syndrome: supravalvular aortic stenosis with hypercalcemia. (Characteristic features are: round face with full cheeks and lips, strabismus, stellate pattern in iris, and mental retardation.) Patients are very friendly