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-dizziness and syncope

-Increased risk of sudden death Diagnosis

-ECG: LV hypertrophy

-Chest X-ray: Mild cardiomegaly

-Echocardiogram: LV hypertrophy, ventricular outflow tract gradient

-doppler flow studies may demonstrate diastolic dysfunction before the development of hypertrophy Management

-Prohibit competitive sports and strenuous physical activities

-Propranolol 0.5 -1mg/kg/day devised in 3 doses or Atenolol

-Implantable cardioverter-defibrillator if documented arrhythmias or a history of unexplained syncope

-Open heart surgery for septal myotomy: rarely indicated

-3.6.3. Restrictive Cardiomyopathy

-

-Apical four-chamber view recorded in a patient with an idiopathic restrictive cardiomyopathy. This was a 70-year-old patient with refractory congestive heart failure and atrial fibrillation.

Note the marked biatrial enlargement and

normal right and left ventricular sizes. Left ventricular systolic function was normal. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

-Definition: Restrictive cardiomyopathy refers to a group of disorders in which the heart chambers are unable to properly fill with blood because of stiffness in the heart muscle. Its prognosis is poor, and clinical deterioration can be rapid.

-Causes

-- Idiopathic, Systemic disease (scleroderma, amyloidosis, or sarcoidosis)

-- Mucopolysaccharidosis

-- Hypereosinophilic syndrome; malignancies

-- Radiation therapy

-- Isolated non compaction of the left ventricular myocardium

-Signs and Symptoms

-- dyspnea

-- Edema and ascites

-- Hepatomegaly with increased venous pressure

-- Pulmonary congestion

-Complications

-- Arrhythmias

-- Mitral regurgitation

-- Progressive heart failure

-- Tricuspid regurgitation

-Investigations

-- ECG: Prominent P waves, ST segment depression, T-wave inversion

-- Chest X-ray: mild to moderate cardiomegaly

-- Echocardiogram: markedly enlarged atria and small to normal-sized ventricles with often preserved systolic function but highly abnormal diastolic function

-Management

-- Lasix 2mg/kg divided in 2 doses

-- Aldactone 1-2mg/kg devised in 2 doses

-- Antiarrhythmic agents / biventricular pacing are used as required

-- Aspirin or Warfarin in case of non compaction LV with an increased risk of mural thrombosis and stroke

-- Cardiac transplantation where possible and indicated

-

3.7.

 

Pericarditis/Pericardial Effusion

Definition: Pericarditis is the inflammation of the pericardium.

-Pericardial effusion is the abnormal build-up of excess fluid that develops between the pericardium, the lining of the heart, and the heart itself.

-Causes

-- Infection such as viral, bacterial (tuberculosis)

-- Inflammatory disorders, such as lupus

-- Cancer that has spread (metastasized) to the pericardium

-- Kidney failure with excessive blood levels of nitrogen

-- Heart surgery (postpericardectomy syndrome).

-Signs and Symptoms

-- Pericardial tamponade

-- Chest pressure or pain and signs of congestive heart failure which can sometimes lead to shock.

-

-Investigations

-- ECG

-• Small complexes tachycardia, diffuse T wave changes

-- Chest X-ray: “water bottle” heart, or triangular heart with smoothed out borders

-- Echocardiogram

-- Tuberculin skin test

-- diagnostic pericardiocentesis

-• In all patients with suspected bacterial or neoplastic pericarditis and patients whom diagnosis is not readily obtained

-- Cell count and differential, culture, gram stain, PCR

-Management

-Non-pharmacological

-Semi-sitting position if tamponnade suspected

- • Pericardiocentesis

-→ preferably under ultrasound guidance

-→ Performed by an experienced person

-→ Indicated in children with symptomatic pericardial effusion

-Pharmacological

-• If hypotensive, rapidly administer intravenous fluids 20ml/kg of Normal saline over 30min to 1 hour

-• If suspected TB pericarditis: standard anti TB treatment + steroids

-• In case of purulent pericarditis: cloxacillin, IV 50 mg/kg/dose 6 hourly for 3 – 4 weeks + ceftriaxone, IV, 100 mg/kg as a single daily dose, to adapt according to culture results.

-• Treat heart Failure (See section on heart failure)

-Recommendation

-- All patients with pericardial effusion should be referred to a cardiologist

-3.8. Hypertension in children

-Definition: Hypertension is defined as systolic and/or diastolic blood

-pressure ≥ the 95th percentile for gender, age and height percentile on at least three consecutive occasions.

-A sustained blood pressure of > 115/80 is abnormal in children between 6 weeks and 6 years of age.

-Causes

-

- Signs and Symptoms

-

-- Headache

-- Convulsions, coma and visual symptoms

-- Oedema, haematuria, proteinuria

-- Acute heart failure and pulmonary oedema

-- Some children may be asymptomatic

-Blood pressure in children correlates with body size and age

-

-

-Investigations

-- Urea, creatinine, electrolytes (Na+, K+)

-- Fundoscopy

-- ECG

-- Echocardiogram

-- Abdominal ultrasound (focused on kidneys)

-- Others according to the suspected etiology

-Management

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