Ravilya_Pediatrics_ Cardiology_ Hypoplastic_Heart Syndrome

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A case presentation of hypoplastic left heart syndrome (important)

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A case presentation of hypoplastic left heart syndrome (important): a newborn appears in 1st week of life. dyspnea, poor feeding. Examination reveals grayish blue color of skin, weak peripheral pulses or hepatomegaly, cardiomegaly, (R) ventricular heave, or a nondescript systolic murmur. (Important for examination)

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CHEST x-RAY

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MOST LIKELY DIAGNOSIS: hypoplastic left heart syndrome CHEST x-RAY: moderate-to-severe cardiomegaly, increased pulmonary vasc

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EKG
:HYPEROXIA

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EKG: (R) ventricular hypertrophy, small (L)ventricular pattern, prominent P waves HYPEROXIA TEST: no improvement

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DIAGNOSIS CONFIRMED BY:
PREOPERATIVE EVALUATION

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DIAGNOSIS CONFIRMED BY: echocardiogram PREOPERATIVE EVALUATION: genetic, neurologic, and opthalmologic consultations should be performed before surgery.

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TREATMENT:

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TREATMENT:
(a)
MEDICAL: PGE, to keep duct open, so blood flows from pulmonary artery to aorta. Sodium bicarbonate for metabolic acidosis, intravenous glucose to prevent hypoglycemia, andavoid hypothermia.
(b)
SURGICAL:procedure of choice is Norwood operation.
(i) NORWOOD STAGE I: atrial septectomy, transection and ligation of distal pulmonary artery, proximal pulm artery is connected to hypoplastic aortic arch forming a neo-aorta, repair of coarcted segment; a synthetic ao pulmonary shunt connecting aorta to pulmonary artery at bifurcation to control pulmonary blood flow. NORWOOD STAGE II: Glenn anastomosis is followed by modified Fontan operation. Glenn anastom represents connection between superior vena cava and pulmonary arteries.
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(li) HEART TRANSPLANTATION

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Key words:

PROGNOSIS:

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PROGNOSIS: verypoor,high mortality.
Key words: grayish blue of the skin, weak or absent peripheral pulses, right ventricular heave or hypertrophy, small
ventricular pattern in EKG