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By Dr.Nuha Abdel Ghaffar
The importance of History and Physical examination cannot be overemphasized. Laboratory evaluation
Eventual treatment or reassurance.
Maternal illness Drugs Prematurity Presence of cyanosis Respiratory distress
Timing of the first presentation
Symptoms of CHF are age specific
Feeding difficulties Frontal sweating Respiratory distress
In older children:
Exercise intolerance Nocturnal dyspnoea& orthopnoea
Cyanosis Chest pain
. So FH of muscular dystrophy or Relatives with CHD.Cardiac disease may be a manifestation of a known cong. PH of Recurrent attacks of chest infection. Malformation or of a generalized disorder affecting the heart as well as other organ systems.
General Physical Examination Assessment of growth Dysmorphic features. Proper cardiac examination. Respiratory distress. Clubbing. BP Signs of congestive heart failure. JVP Pulse & its character. .
Laboratory investigations Chest X ray: Cardiac size and shape . .lung field ECG Echocardiography Cardiac catheterization.
Fetal Circulation The right & left ventricles exist in a parallel circuit. The lungs do not provide gas exchange & vessels in the pulmonary circulation are vasoconstricted. 3 structures unique to the fetus : Ductus venosus Ductus arteriosus Foremen ovale. . Gas & metabolite exchange are provided for by the placenta.
. Genetic factors Association with chromosomal abnormalities. Environmental or adverse Maternal conditions (Maternal illness & drugs).5 -0.Congenital Heart disease Incidence: Occurs in 0.8% of live birth. Aetiology: Multifactorial inheritance.
Acynotic CHD Left to Right shunt Its classified according to the predominant physiologic load they placed on the heart into: 1. Regurigitant valve &cardiomyopathy. AV canal defect. VSD.Volume overload: -Examples are ASD. PDA. .
Pressure overload: -Obstruction to normal blood flow -Examples are Pulmonary & aortic stenosis. Cor triatriatum . 2. Tricuspid or Mitral stenosis . COA .-Communication between the systemic & pulmonary sides of the circulation result in shunting of the fully oxygenated blood back into the lungs.
VSD It is the most common malformation accounting for 25% 0f CHD. . Definition: Is a developmental defect of the interventricular septum whereby a communication exists between the cavities of the two ventricles.
right shunt are: The physical size of the defect Level of pulmonary vascular resistance If the size of the defect is small (Maladie de Roger)( <0.5cm2 restrictive type): the cardiac chambers & pulmonary vascular bed are normal. .Pathophysiology The major determinants of the size of the leftto.
In large non restrictive defects (>1cm2). . there are 2 adverse effects: Altered hemodynamic leading to : Left ventricle volume overload “LV dilation & hypertrophy with left atrial enlargement Pulmonary over circulation Compromise of systemic cardiac output Altered pulmonary vascular bed –leading to pulmonary hypertension.
feeding difficulties. recurrent chest infection. poor growth.Clinical Manifestations Symptoms & signs relate to the size of the defect & the magnitude of the Lft –to Rt shunt. heart failure. O/E: Loud lower left parasternal pan systolic murmur frequently accompanied by thrill. . Patients with small defects are asymptomatic Large defects are responsible for dyspnoea . profuse sweating .
.Diagnosis CXR Cardiomegaly. prominence of both ventricles &left atrium &pulmonary artery with normal Pul.vascularity Biventricular hypertrophy ECG Echocardiography Cardiac catheteriztion.
.Prognosis & complications Ranged from spontaneous closure to congestive heart failure to death in early infancy depending on the size of the defect Small defect (30-50%) will close spontaneously during the first year of life .rare in children older than 2 years Large defects rarely close –end with pulmonary hypertension Infective endocarditis occur in <2% Treatment : Surgical correction prophylaxis against infective endocarditis.
ASD It accounts 6-8% of CHD It can occur in any portion of the atrial septum (Secundum. primum or sinus venosus) Ostium Secundum defect: Most common form of ASD Occurs in the region of the fossa ovalis Females outnumbers males 3:1 .
. the pulmonary arterial pressure remains normal because of the absence of a high pressure communication between the pulmonary &systemic circulation Left ventricle &aorta are normal in size. Despite the large pulmonary blood flow .Pathophysiology The shunting of oxygenated blood from the left to the right atrium leads to enlargement of the right atrium and ventricle & dilation of the pulmonary artery.
Clinical manifestation Most often asymptomatic Various degree of exercise intolerance Loud first heart sound Fixed splitting of the second heart sound Ejection systolic murmur at left upper sternal border due to increase right ventricular volume O/E: .
ECG: Rt axis deviation & Rt bundle branch block Echo Secundum is well tolerated & symptoms appear in the 3rd decade or later.Diagnosis CXR: Rt vent +atria + pulmonary artery enlargement. Prognosis: . Pulmonary hypertension . atrial dysrythmias & heart failure are late manifestation.
Infective endocarditis is extremely rare. . Surgery for symptomatic &asymptomatic prior to the entry into school. Treatment Atrioventricular septal defects (Ostium primum and AV canal defect): They are grouped together because they represent a spectrum of embryologic abnormality: a deficiency of the Atrioventricular septum.
trans -ventricular & AV valve sufficiency Pulmonary hypertension is common .Ostium primum defect: Situated in the lower portion of the atrial septum Continuous atrial & ventricular septal defects with abnormal AV valves. common in Down syndrome AV canal defect: Pathophysiology: Ostium primum is very similar to that of Ostium Secundum AV canal defect : The shunt is both trans-atrial .
O/E: . recurrent pneumonias since birth & failure to thrive Fixed splitting of the 2nd heart sound Diastolic murmur at lower left sternal border Apical pan systolic murmur due to mitral insufficiency.Clinical manifestations Ostium primum may be asymptomatic or have a history of exercise intolerance or recurrent pneumonias AV canal defect present with congestive heart failure.
↑ pulmonary vascularity ECG: left axis deviation .Diagnosis: CXR large heart .Rt vent conduction delay Echo & catheterization If left untreated : death in infancy from congestive heart failure Pulmonary hypertension Surgical correction. Prognosis & complications Treatment: .
. artery . The extend of the shunt depends on the size of the ductus & the ratio of Pul to systemic vascular resistance.Patent ductus arteriosus Functional closure of the ductus normally occurs soon after birth Females outnumber males 2:1 It is associated with maternal rubella & common in premature infants Pathophysiology: Blood flow through the ductus from the aorta to the Pul.
O/E: .Clinical features Small PDA : produce no symptoms Large PDA : result in congestive heart failure in infancy Wide pulse pressure with bounding arterial pulses Enlarged heart with apical impulse There is machinery murmur localized to the 2nd left intercostal space accompanied by thrill.
Or systemic embolisation & Pul. hypertension Surgical correction Prognosis & complications Treatment : .Diagnosis: CXR :prominent Pul.Pul. Artery ECG: biventricular hypertrophy Echo& catheterization Small PDA : have a normal live span Large PDA: CHF in early infancy Infective endocarditis .
Coarctation of the aorta (COA) Localized narrowing of the descending aorta . May occur at any point but more commonly 98% just below the origin of the left subclavian artery “at the origin of the ductus arteriosus” More common in males It is a feature of turner syndrome .
Pathophysiology: Arterial blood bypasses the obstruction reaching the lower part of the body through collaterals vessels. Left ventricle hypertrophies to overcome the obstruction –heart failure. Neonates or infants with severe COA: CHF Asymptomatic or weakness or pain in legs after exercise. Clinical features: . Systolic blood pressure in the upper part of the body is elevated.
significant hypertension in the upper limbs. Short systolic murmur along the left sternal border at the 3rd and 4th intercostal space and on the back .O/E: The classical signs of COA is a disparity in pulsations & blood pressure of the arms and legs The peripheral pulses are weak in contrast to the bounding pulses of the arms There is radio-femoral delay.
Diagnosis: CXR : cardiac enlargement mainly left ventricle Rib notching due to enlarged intercostal arteries ECG: Left ventricular hypertrophy Treatment: surgery .
truncus arteriosus .Tricuspid atresia. single ventricle .Cyanotic CHD Divided into two: Cyanotic with decreased Pul blood flow: Examples are : TOF.Total anomalous PUL venous return with obstruction Cyanotic with increased Pul blood flow: Examples are : TGA.Total anomalous Pul venous return without obstruction . Pul atresia.
Evaluation of the critically ill neonate with cyanosis & respiratory distress : Δ / Δ: Cardiac disease CNS disease like intracranial hemorrhage Pulmonary disease like RDS Hyperoxia test: Administration of 100% oxygen will usually not raise the arterial PaO2 in CHD .
VSD Dextroposition of the aorta with septal overriding Rt ventricular hypertrophy. .Tetralogy of Fallot (TOF) Consists of the combination of : Obstruction of the Rt ventricular outflow “Pul stenosis.
the severity of cyanosis and the degree of Rt ventricular hypertrophy. the blood is shunted across the VSD into the aorta Persistent arterial desaturation & cyanosis result The degree of Rt ventricular outflow obstruction determines the timing of onset of symptoms . .Pathophysiology: Systemic venous return to the Rt atrium & Rt ventricle is normal When the Rt ventricle contracts in the presence of marked Pul stenosis .
Clinical manifestations: Cyanosis is not often present at birth Dyspnoea on exertion . Paroxysmal hyercyanotic attacks or blue spells: Occurs more frequently in the morning or following episodes of vigorous crying The child become hyperpneic & restless .characteristically they assume a squatting position for the relief of dyspnoea.cyanosis increased Syncope may follow It may last from minutes to few hours They are rarely fatal but can be followed by convulsions or hemi paresis The spells are associated with reduction of already compromised Pul blood flow---hypoxia & metabolic acidosis Poor growth & development .
Diagnosis: CXR: narrow base . bacterial endocarditis.Rt vent hypertrophy Echo.O/E: Systolic murmur along left sternal border due to turberalence flow across the Rt ventricular outflow tract accompanied by thrill. produced by hypertrophied Rt ventricle" boot shape” ECG: Rt axis deviation . CHF is not a feature of TOF .rounded apical shadow situated higher above the diaphragm . brain abscess. Catheterization Complications: Cerebral thrombosis due to polycythemia.
Blalock Taussig shunt : anastomosis of a subclavian artery to the homolateral branch of the Pul artery Total correction : relieve of the obstruction to the Rt ventricle + closure of the VSD.1mg/kg/SC Beta blocker 0.Management Cyanotic spells Placement of the child on the abdomen in the chest – knee position Oxygen Morphia 0.2mg/kg. Surgical correction of TOF: .
ductus arteriosus or VSD Clinical presentation: According to the anomaly found Progressive cyanosis & heart failure may follow .Transposition of the great arteries (TGA) The aorta arise from the Rt Vent & the Pul artery from the Lft Vent The systemic & Pul circulation consist of two parallel circuits The only mean of survival is provided by the foramen ovale .
Investigation: CXR : heart looks like an egg on side Catheterization Treatment : A communication between systemic & Pul circulation is urgently needed Arterial switch procedure should be carried out in the first 2 weeks of life. .