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-- Surgical repair generally before 1 year if possible

-3.4.2. Cyanotic Heart Diseases

An example of D-transposition of the great arteries in an infant is shown. A: From the subcostal view, the pulmonary artery (PA) can again be seen to arise from the anatomic left ventricle (LV). B: By demonstrating

bifurcation of the great artery that arises from the posterior left ventricle, ventriculoarterial discordance is confirmed. C: A short-axis view at the base of the heart demonstrates the parallel course of the great arteries with an anterior aortic valve (AV). D: The right ventricle (RV) is seen anterior and rightward of the left ventricle. It is dilated and hypertrophied. Ao, aorta; LA, left atrium.

-Definition: Cyanotic heart disease is a heart defect, present at birth (congenital), that results in low blood oxygen levels (< 90 % even with oxygen).

-

-Common lesions

-decreased flow to the lungs (does not cause heart failure)

-• Tetralogy of fallot

-• Pulmonary atresia

-- Increased flow to the lungs (does cause heart failure and failure to thrive):

-• Transposition of great vessels (TGA)

-• Truncus arteriosus

-• Single ventricle / Tricuspid atresia

-Tetralogy of Fallot

-

-Long-axis image from a patient with tetralogy of Fallot demonstrates the overriding aorta (Ao) and a large subaortic ventricular septal defect (asterisk). Right ventricular hypertrophy is also present. LA, left atrium; LV, left ventricle; RV, right ventricle.

-Definition: Tetralogy of Fallot refers to a type of congenital heart defect comprising of:

-- Large ventricular septal defect

-- Narrowing of the pulmonary outflow tract (pulmonary stenosis)

-- Overriding aorta

-- Right ventricular hypertrophy

-Signs and Symptoms

-- Progressive cyanosis with pulmonary systolic murmur

-- digital clubbing occurs after long time

-- Hallmark: Paroxysmal hyper cyanotic attacks (blue spells) with the following manifestations

-• Hyperpnea and restlessness

-• Increased cyanosis

-• Gasping respiration

-• Syncope or convulsions

-•Spontaneous squatting position is frequent (in older children)

-• Heart murmur disappears

-Complications

-- delayed development/growth

-- Polycythemia

-- Hypercyanotic attack, sometimes associated with seizures and death

-Infective endocarditis

-Brain abscess

-Investigations

-- Chest x-ray

-- Complete blood count (CBC)

-- Echocardiogram

-- Electrocardiogram (ECG)

-Management

-- Avoid dehydration and stress

-- Propanolol 0.5-1mg/kg every 6 hours to prevent hypercyanotic attacks

-- Iron 5mg/kg /day to prevent microcytosis

-- Surgical repair, urgent as soon as spells begin

-- In case of Hypercyanotic attacks

-• Squatting position (hold the infant with the legs flexed on the abdomen)

-• Oxygen 6l/min with mask

-• Diazepam 0.3mg/kg IV or 0.5mg PR if convulsing

-• normal saline 10-20ml/kg/ 30 minutes

-• Sodium bicarbonate 8.5% 1ml/kg to correct acidosis

-• Morphine 0.1mg/kg IV if persistent attacks (but risk of

-respiratory depression)

-• Propranolol IV 0.1 – 0.2 mg/kg slowly then continue oral maintenance to relax the infundibular spasms

-Recommendations

-- All children with cyanotic heart diseases who come with diarrhea and vomiting should be admitted for closer observation.

-Furosemide is contra-indicated.

-- All new born babies with suspected cyanotic heart disease should be referred to a cardiologist/tertiary hospital immediately.

-- Common causes of heart failure in Neonates:

-

-3.5. Acquired Heart Diseases

-3.5.1. Acute Rheumatic fever

-

-

-

-Definition: This is an acute, systemic connective tissue disease in

-children related to an immune reaction to untreated group A Beta

-haemolytic streptococcus infection of the upper respiratory tract. The initial attack of acute rheumatic fever occurs in most cases between the ages of 3 and 15 years.

-Cause

-- Auto-immune disease

-Signs and Symptoms (Revised Jones Criteria)

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