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PATHOLOGY CONGENITAL HEART DISEASE IN CHILDREN

Congenital heart diseases (CHDs) arise from genetic and environmental factors, with various types classified as acyanotic or cyanotic based on blood flow patterns. Common defects include atrial septal defect and ventricular septal defect, which can lead to complications such as pulmonary hypertension and heart failure. The document details mechanisms, hemodynamics, and clinical manifestations of several key heart defects, emphasizing the importance of early diagnosis and intervention.

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CONGENITAL HEART DISEASES
• Congenital heart diseases result from interaction between genetic and environmental factors. • GENETIC • Single classic Mendelian mutant genes account for 3% of congenital heart diseases; 5% are caused by gross chromosomal aberrations, 3% by known environmental factors (e.g. rubella, fetal alcohol syndrome), and the rest by multifactorial gene effects or single gene effects modulated by random events. • Single mutant genes (autosomal dominant or recessive or X-linked) usually cause congenital heart disease as part of a complex of abnormalities. • Chromosomal abnormalities also cause congenital heart diseases as part of a complex of lesions
• Environmental factors: • Fetal environment: Women taking lithium salts and Retinoic acid used to treat acne during pregnancy may cause several types of congenital heart lesions. • Diabetic women or those taking progesterone in pregnancy have an increased risk. • Alcoholic mothers risk their babies having CHD (usually left-to-right shunts). • Infections: TORCHES. Rubella embryopathy is often associated with peripheral pulmonic stenosis, patent ductus arteriosus, and valvar pulmonic stenosis, coxsackieviruses and Mumps virus is responsible for endocardial fibroelastosis.
Classification • Congenital heart diseases are customary divided into noncyanotic and cyanotic types. • By convention, patients who have right-to-left shunts fall into the cyanotic category whether or not they have readily recognizable cyanosis. • Patients who do not have right-to-left shunts-even if they are cyanotic for other reasons, such as low cardiac output-are placed in the Acyanotic category.
Classification • Acyanotic: according to the predominant physiologic load placed on the heart. • Cyanotic: based on pathophysiology. • Volume load: L-R shunts-ASD,VSD,PDA. • Pressure load: Ventricular outflow obstruction; Pulmonary, aortic valve lesions, aortic coarctation & pulmonary stenosis. • Decreased pulmonary blood flow: TOF, Pulmonary atresia, Tricuspid atresia, Single ventricle with pulmonic stenosis. • Increased pulmonary blood flow: Transposition of great vessels,Truncus arteriosus.
Heart defects • Patent Ductus Arteriosus • Atrial Septal Defect • Ventricular Septal Defect • Tetralogy of Fallot • Transposition of the Great Arteries • Coarctation of the Aorta • Anomalous Venous Return • Truncus Arteriosus • Hypoplastic Left-Heart Syndrome
Acyanotic congenital heart diseases • Left-to-Right Shunt Lesions i) Atrial Septal Defect (ASD) ii) Ventricular Septal Defect (VSD) iii) Atrioventricular Septal Defect (AV Canal) iv) Patent Ductus Arteriosus (PDA)
Atrio-Septal defect(ASD)
• ASD is an opening in the atrial septum permitting free communication of blood between the atria. Seen in 10% of all CHD and 6-10% of all births (1 of 1500 live births) • 2 times more common in females than males. • In 1950 most children with ASD did not reach the first grade. • Today, first year surgery facilitates normal growth and development
• There are 3 major types: • Sinus Venosus ASD – high in the atrial septum, associated with partial anomalous venous return & the least common. • Secundum ASD – at the Fossa Ovalis, most common. • Primum ASD – lower in position & is a form of Atrial Septal Ventricular Defect (ASVD), MV cleft.
Hemodynamics • Small shunts may remain asymptomatic throughout life, or they may be discovered in pregnancy due to volume overload. • Large defects cause left to right shunts during late ventricular systole or early diastole. • This leads to diastolic overload of the right ventricle and increased pulmonary blood flow causing increased pulmonary vascular resistance and pulmonary hypertension that may cause a reversal of shunt the Eisenmenger syndrome
Ventricular septal defect
• VSD – is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles. Accounts for 25% of CHD. • There are 4 Types • Perimembranous (or membranous) – Most common. • Infundibular (subpulmonary or supracristal VSD) – involves the RV outflow tract. • Muscular VSD – can be single or multiple. • AVSD – inlet VSD, almost always involves AV valvular abnormalities
Hemodynamics • The left to right shunt occurs secondary to PVR being < SVR, not the higher pressure in the LV. • This leads to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV. • Increased pulmonary overflow causes pulmonary oedema and eventually increases pulmonary vascular resistance leading to pulmonary hypertension. • Elevated Pulmonary Vascular Resistance causes reversal of the shunt to right-to-left shunt
• Clinical Signs & Symptoms Depend on size. Ranges from Asymptomatic, growth, failure, recurrent LRTI, congestive heart, failure, cyanosis and loud holosystolic murmurs. • Small - moderate VSD, 3-6mm, are usually asymptomatic and 50% will close spontaneously by age 2yrs. • Moderate – large VSD, almost always have symptoms and will require surgical repair. • Today almost all VSD can be closed successfully, even in small babies. • Large defects lead to pulm hypertension-Eissenmenger syndrome.
Atrioventricular Septal Defect • AVSD results from incomplete fusion the endocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves. • They account for 4% OF ALL CHD. • Common in downs Syndrome
Types of AVSD Complete Form • Low primum ASD continuous with a posterior VSD. • Cleft in both septal leaflets of Tricuspid Valve/Mitral Valve. • Results in a large L to R shunt at both levels, TR/MR, Pulm HTN with increase in PVR. Incomplete Form • Any one of the components may be present. • Most common is primum ASD, cleft in the MV & small VSD. • Hemodynamics are dependent on the lesions.
Symptoms • Incomplete AVSD maybe indistinguishable from ASD - usually asymptomatic. • Congestive heart failure in infancy. • Recurrent pulmonary infections, Failure to thrive, Exercise intolerance, easy fatigability. • Late cyanosis from pulmonary vascular disease with R to L shunt. Clinical • Hyperactive precordium • Normal or accentuated 1st heart sound • Wide, fixed splitting of S2 • Pulmonary systolic ejection murmur w/thrill • Holosystolic murmur @ apex with radiation to axilla • Mid-diastolic rumbling murmur @ LSB • Marked cardiac enlargement on CX- Ray
PDA • The Ductus Arteriosus Connects Pulmonary Artery to Aorta Shunts blood away from lungs in fetal circulation. • Maintained patent by low oxygen pressures and presence of prostaglandins • Closes at Birth secondary to Increase in PaO2 and Decrease in level of prostaglandins. • If this shunt persists post delivery then its called PDA
PDA • Normally closes in the 1st wk of life, within 48hrs post delivery. • 5-10% of all births (1 of 2000 live births) occurs in 80% of premature babies • Accounts for 10% of all CHD, seen in 10% of other congenital heart lesions and can often play a critical role in some lesions. Often associated with coarctation & VSD. • 2-3 times more common in females than males. • 5th or 6th most common congenital cardiac defect. • May be desirable with some defects. Morbidity/Mortality related to degree of blood flow through PDA.
Hemodynamics of PDA • As a result of higher aortic pressure and low pulmonary pressure, blood shunts L to R through the ductus from Aorta to PA into the lungs. This causes pulmonary overflow and systemic hypoperfussion. • The pulmonary veins return excess blood to the left atria into left ventricle thus increasing CO but with decreased systemic output and hypoxia causing a heamodynamic paradox. • Increased pulmonary overflow causes pulmonary oedema and eventually increases pulmonary vascular resistance leading to pulmonary hypertension. • Increased pressures in the right will eventually reverse the flow and the condition becomes cyanotic.
PDA • With increase LV output and low systemic perfussion, hypoxia triggers cardiac support neuro-humoral effects i.e the sympathetic system and RAAS which cascades to promote heart failure. • There will be left atria and ventricular enlargement, aortic and pulmonary vein and arteries dilatation with pulmonary hypertension. • Cerebral and systemic hypoxia lead to cerebral damage and multi- organ failure.
PDA Small PDA’s are usually asymptomatic Large PDA’s can result in symptoms of Congestive Heart Failure, growth restriction, Failure to Thrive. Bounding arterial pulses Widened pulse pressure Enlarged heart, prominent apical impulse Grade 1-2 Classic continuous machinery systolic murmur (washing Machine) Mid-diastolic murmur at the apex
Obstructive Heart Lesions • Pulmonary Stenosis • Aortic Stenosis • Coarctation of the Aorta
Pulmonary Stenosis • Pulmonary Stenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract. • Accounts for 7-10% of all CHD. • Most cases are isolated lesions • Maybe biscuspid or fusion of 2 or more leaflets. • Can present w/or w/o an intact ventricular septum.
Hemodynamics • Right Ventricular volume overload, RV pressure hypertrophy ->Right Ventricular failure. • Right Ventricular pressures maybe more than systemic pressure. • Post-stenotic dilation of main Pulmonary Artery. • In severe stenosis without intact septum lead to R-L shunt through Pertent foramen ovale causing cyanosis. • Cyanosis is indicative of Critical PS.
Pulmonary Stenosis Clinical Signs & Symptoms • Depends on the severity of obstruction. • Asymptomatic with mild PS < 30mmHg. • Mod-severe: 30-60mmHg, > 60mmHg • Prominent jugular a-wave, RV lift • Split 2nd heart sound with a delay • Ejection click, followed by systolic murmur. • Heart failure & cyanosis seen in severe cases
Aortic Stenosis • Aortic Stenosis is an obstruction to the outflow from the left ventricle at or near the aortic valve that causes a systolic pressure gradient of more than 10mmHg. Accounts for 7% of CHD. • 3 Types • Valvular – Most common. • Subvalvular(subaortic) – involves the left outflow tract. • Supravalvular – involves the ascending aorta is the least common.
Hemodynamics • Pressure hypertrophy of the LV and LA with obstruction to flow from the LV. • Mild AS 0-25mmHG • Moderate AS 25-50mmHg • Severe AS 50-75mmHg • Critical AS > 75mmHg
Aortic Stenosis Symptoms • Mild AS may present with exercise intolerance, easy fatigabiltity, but usually asymptomatic. • Moderate AS – Chest pain, dypsnea on exertion, dizziness & syncope. • Severe AS – Weak pulses, left sided heart failure, Sudden Death. Clinical Signs • LV thrust at the Apex. • Systolic thrill @ right base/suprasternal notch. • Ejection click, III-IV/VI systolic murmur @ Right sternal border (RSB)/Left Sternal Border (LSB) with radiation to the carotids.
Coarctation of the Aorta • Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation. • 98% of coarctations are juxtaductal. • Male: Female ratio 3:1. • Accounts for 7 % of all CHD • Associated with Bicuspid Aortic Valve and Turner’s Syndrome
Location of Coarctation Pre-Ductal • Less common but more serious • Associated with VSD, PDA, Transposition Post-Ductal • More common • Often associated with collateral circulation beyond coarctation, which minimizes effect. • Diagnosed by a difference in blood pressure between lower extremities and upper ones. • Pressure in upper extremities > lower
Hemodynamics • There is obstruction of left ventricular outflow -> severely reduced systemic blood flow and pressure hypertrophy of the LV. • Increased work on the heart leading to CHF and cardiovascular collapse.
Rib Notching
Cyanotic Heart Diseases • Tetralogy of Fallot • Complete Transposition of the Great Arteries. • Anomalous Venous Return • Truncus Arteriosus • Hypoplastic Left Heart Syndrome
Tetralogy of Fallot • Most common of the cyanotic cardiac diseases. • Mortality increases with age (1 year- old has a 25% mortality, 40 year-old has 95%). • Four Defects • Pulmonary infandibular Stenosis (determinant factor related to severity) i.e narrowing of RV outflow • VSD (usually large) • Overriding Aorta • RV hypertrophy
TOF hemodynamics • Due narrowed pulmonary infandibula and overriding Aorta, both RV and LV ejects blood into the Aorta. • VSD is restrictive thus no gradient across the chambers • Systolic pressure in LV and RV are the same • RV persists its dorminance as in fetal life preferentially ejects into the aorta. With increased flow it dilates giving a boot shape on X-ray. • RV pressure never exceeds systemic pressure. • With the narrowed pulmonary infandibula, very minimal blood is delivered to the lungs for oxygenation, thus the cyanosis and finger clubbing.
Complete Transposition of the Great Arteries • Second most common form of CHD. Causes blue baby syndrome • Aorta arises from RV and Pulmonary Arteries from LV. • Without an abnormality, life would not be possible. • ASD • VSD (30-40%) • PDA
Hemodynamics • Oxygen poor blood returns to the RA, to RV then into the misconnected Aorta back to the system. • Oxygen rich blood returns to the LA from lungs into the LV then into the PA back to the lungs • Two separate circutes are formed, one of oxygen poor and oxygen rich blood. • Other heart defects associated with TGA help mitigate the problem. PDA, VSD and ASD facilitate mixing of this blood thus allowing some oxygen to reach tissues.
Anomalous Venous Return • Return of pulmonary venous blood to the right atrium instead of the left or in the systemiv veins. Can also be partial or complete. • ASD is present to sustain life.
Hemodynamics of TVR • Pulmonary veins pours into the RA causing volume overload and thus pressure overload to thr RA and RV. • There is incread blood flow to the lungs leading to pulm oedema, and increased pulm vascular resistance then pulmonary hypertension. • Without a ASD, life is not sustainable. • LA and LV receive less and pump less. Reduced CO triggers neuro- humoral cardiac response which cascades to CHF
Truncus Arteriosus • Defect in which one large vessel( usually pulmonary trunk) arises from right and left heart over a large VSD. • Cyanosis is often present. • CHF common
• Outflow from both ventricles is directed to this one trunk. • Resistances to flow is determined by both systemic and pulmonary resistance. • Subnormal arterial oxygen concentration, pulmonary flow is elevated causing over circulation and deprived systemic circulation. • This caused increased myocardial workload and failure.
Hypoplastic left heart syndrome • It’s a condition where the left heart does not form normally during embryogenesis. • Features are: • -under developed LV • Unformed or underdeveloped mitrial valve • Unformed or underdeveloped Aortic valve • Underdeveloped or very small ascending aorta • Atrioseptal defect
hemodynamics • In the first few days of life, the right side pumps blood to both the lungs and the system via the PDA. • Pulmonary venous return drains into the right heart via ASD • Closure of the two is fatal therefore they must be kept open using prostaglandins.
Dextrocardia • Dextrocardia is a radiographic term used when the heart is on the right side of the chest. • When dextrocardia occurs with reversal of position of the other important organs of the chest and abdomen (e.g. liver, lungs, and spleen), the condition is called situs inversus totalis and the heart is usually completely normal. • When dextrocardia occurs with otherwise normal organs (situs solitus), the heart usually has severe defects. • Rarely, the abdominal organs and lungs are neither in situs solitus or situs inversus, these states are called situs ambiguous. • The liver is central and anterior in the upper abdomen with the stomach pushed behind • There may be bilateral right-sidedness (asplenia syndrome) or bilateral left- sidedness (polysplenia syndrome), but in virtually all cases of situs ambiguous, severe congenital heart disease is present.
Cardiomyopathy • Its an acquired or hereditary progressive disease of the myocardium. • The muscle weakens and it is unable to pump blood to the systems • There are 3 types of cardiomyopathy • i) Dilated • ii) Restrictive • Iii) Hypertrophic • Treatment depends on the type.
Dilated Cardiomyopathy-Pathology • Gradual four chamber hypertrophy and dilation. can occur at any age as slow progressive CHF but mostly affects middle aged people. • Leads to impaired myocardial contractility and CHF. • The following gross changes may be observed • The heart is flabby. • Cardiomegaly (to 900gm) is typical, although wall thickness may not reflect the degree of hypertrophy due to dilation • Poor contractile function and stasis can predispose to mural thrombi • Valves and coronary arteries are generally normal
Hypertrophic cardiomyopathy • Characterised by abnormal thickening of the myocardium • Affected people have Fhx of the disease, linked to genetic mutations. • characterized by heavy, muscular, hypercontractile, poorly compliant hearts with poor distolic relaxation. Has increased risk of Sudden death.
Pathology of Hypertrophic cardiomyopathy • marked cardiomegaly owing to hypertrophy, left ventricle greater than right ventricle, often with atrial dilation. • Classically, there is disproportionate thickening of the interventricular septum (asymmetric septal hypertrophy) although many cases have concentric or symmetric hypertrophy • The left ventricular cavity is compressed into a banana-like configuration by the symmetric bulging of the septum • Septal thickening at the level of the mitral valve compromises left ventricular systolic outflow by contact of the anterior mitral leaflet with the septum.
• Microscopically, there is marked myofibre hypertrophy; 25-50% of the septum classically shows helter-skelter myocyte disarray, accompanied by myofilament disorganization within muscle cells • There are abnormal thick-walled intramyocardial arterioles, as well aspatchy replacement fibrosis, the later presumably due to focal ischemic injury.
Restrictive Cardiomyopathy • Relatively rare. The myocardium becomes rigid and less elastic so it cant expand to fill with blood in diastole. Often occurs in old age. • It is characterized by ventricular subendocardial fibrosis extending from the apex to the ventricular inflow tracts, often with superiposed mural thrombus • The atrioventricular valves may be involved • Restrictive physiology results with reduced ventricular chamber volume and reduced CO
Arrythmogenic Right ventricular cardiomyopathy • Arrythmogenic right ventricular cardiomyopathy is a recently recognized cardiomyopathy with distinct presentation and morphology • It is typically a familial disorder characterized by predominantly right sided failure (occasionally left sided) and various rythmic disturbances, particularly ventricular tarchcardia and sudden death. • Morphologically the right ventricular wall is severely thinned with myocyte loss and profound fatty infiltration. • Death occurs secondary to progressive CHF, embolism of mural thrombi of fatal arrythmias.
Myocarditis • It is characterized by myocardial inflammation as the principle feature • Most cases are thought to be of viral origin (e.g. coxsackie A and B, echovirus). Cardiac involvement occurs days to weeks after a primary viral infection, which can be in the heart or solely at another site • The cardiac involvement can be a direct infection or an immunological reaction to a myocardial antigen triggered by the infectious agent • Rare cases are secondary to bactereamia e.g. staphylococci, tuberculosis trypanosome cruzi
pathology • Gross • Gross manifestations include a flabby heart often with four chamber dilation and patchy heamorrhagic mottling. • Mural thrombi can form in dilated chambers • Endocardium and valves are unaffected • After the acute stage, there may be residual dilation or hypertrophy
• Microscopically • There is a myocardial inflammatory infiltrate with associated myocyte necrosis or degeneration. Lesions are typically focal and may be missed by routine endomyocardial biopsy. • In viral infections, associated myofibre necrosis is seen with interstitial edema and a mononuclear cell infiltrate. After the acute stage, inflammatory lesions may resolve, leaving either no residual or variable interstitial and replacement fibrosis • Depending on cause, bacteria, other organisms and cellular infiltrates may be demonstrated.
Read about: • Pericarditis • Tumours of the heart

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PATHOLOGY CONGENITAL HEART DISEASE IN CHILDREN

  • 1.
  • 2.
    • Congenital heartdiseases result from interaction between genetic and environmental factors. • GENETIC • Single classic Mendelian mutant genes account for 3% of congenital heart diseases; 5% are caused by gross chromosomal aberrations, 3% by known environmental factors (e.g. rubella, fetal alcohol syndrome), and the rest by multifactorial gene effects or single gene effects modulated by random events. • Single mutant genes (autosomal dominant or recessive or X-linked) usually cause congenital heart disease as part of a complex of abnormalities. • Chromosomal abnormalities also cause congenital heart diseases as part of a complex of lesions
  • 3.
    • Environmental factors: •Fetal environment: Women taking lithium salts and Retinoic acid used to treat acne during pregnancy may cause several types of congenital heart lesions. • Diabetic women or those taking progesterone in pregnancy have an increased risk. • Alcoholic mothers risk their babies having CHD (usually left-to-right shunts). • Infections: TORCHES. Rubella embryopathy is often associated with peripheral pulmonic stenosis, patent ductus arteriosus, and valvar pulmonic stenosis, coxsackieviruses and Mumps virus is responsible for endocardial fibroelastosis.
  • 4.
    Classification • Congenital heartdiseases are customary divided into noncyanotic and cyanotic types. • By convention, patients who have right-to-left shunts fall into the cyanotic category whether or not they have readily recognizable cyanosis. • Patients who do not have right-to-left shunts-even if they are cyanotic for other reasons, such as low cardiac output-are placed in the Acyanotic category.
  • 5.
    Classification • Acyanotic: accordingto the predominant physiologic load placed on the heart. • Cyanotic: based on pathophysiology. • Volume load: L-R shunts-ASD,VSD,PDA. • Pressure load: Ventricular outflow obstruction; Pulmonary, aortic valve lesions, aortic coarctation & pulmonary stenosis. • Decreased pulmonary blood flow: TOF, Pulmonary atresia, Tricuspid atresia, Single ventricle with pulmonic stenosis. • Increased pulmonary blood flow: Transposition of great vessels,Truncus arteriosus.
  • 6.
    Heart defects • PatentDuctus Arteriosus • Atrial Septal Defect • Ventricular Septal Defect • Tetralogy of Fallot • Transposition of the Great Arteries • Coarctation of the Aorta • Anomalous Venous Return • Truncus Arteriosus • Hypoplastic Left-Heart Syndrome
  • 7.
    Acyanotic congenital heartdiseases • Left-to-Right Shunt Lesions i) Atrial Septal Defect (ASD) ii) Ventricular Septal Defect (VSD) iii) Atrioventricular Septal Defect (AV Canal) iv) Patent Ductus Arteriosus (PDA)
  • 8.
  • 9.
    • ASD isan opening in the atrial septum permitting free communication of blood between the atria. Seen in 10% of all CHD and 6-10% of all births (1 of 1500 live births) • 2 times more common in females than males. • In 1950 most children with ASD did not reach the first grade. • Today, first year surgery facilitates normal growth and development
  • 10.
    • There are3 major types: • Sinus Venosus ASD – high in the atrial septum, associated with partial anomalous venous return & the least common. • Secundum ASD – at the Fossa Ovalis, most common. • Primum ASD – lower in position & is a form of Atrial Septal Ventricular Defect (ASVD), MV cleft.
  • 11.
    Hemodynamics • Small shuntsmay remain asymptomatic throughout life, or they may be discovered in pregnancy due to volume overload. • Large defects cause left to right shunts during late ventricular systole or early diastole. • This leads to diastolic overload of the right ventricle and increased pulmonary blood flow causing increased pulmonary vascular resistance and pulmonary hypertension that may cause a reversal of shunt the Eisenmenger syndrome
  • 12.
  • 13.
    • VSD –is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles. Accounts for 25% of CHD. • There are 4 Types • Perimembranous (or membranous) – Most common. • Infundibular (subpulmonary or supracristal VSD) – involves the RV outflow tract. • Muscular VSD – can be single or multiple. • AVSD – inlet VSD, almost always involves AV valvular abnormalities
  • 14.
    Hemodynamics • The leftto right shunt occurs secondary to PVR being < SVR, not the higher pressure in the LV. • This leads to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV. • Increased pulmonary overflow causes pulmonary oedema and eventually increases pulmonary vascular resistance leading to pulmonary hypertension. • Elevated Pulmonary Vascular Resistance causes reversal of the shunt to right-to-left shunt
  • 15.
    • Clinical Signs& Symptoms Depend on size. Ranges from Asymptomatic, growth, failure, recurrent LRTI, congestive heart, failure, cyanosis and loud holosystolic murmurs. • Small - moderate VSD, 3-6mm, are usually asymptomatic and 50% will close spontaneously by age 2yrs. • Moderate – large VSD, almost always have symptoms and will require surgical repair. • Today almost all VSD can be closed successfully, even in small babies. • Large defects lead to pulm hypertension-Eissenmenger syndrome.
  • 16.
    Atrioventricular Septal Defect •AVSD results from incomplete fusion the endocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves. • They account for 4% OF ALL CHD. • Common in downs Syndrome
  • 17.
    Types of AVSD CompleteForm • Low primum ASD continuous with a posterior VSD. • Cleft in both septal leaflets of Tricuspid Valve/Mitral Valve. • Results in a large L to R shunt at both levels, TR/MR, Pulm HTN with increase in PVR. Incomplete Form • Any one of the components may be present. • Most common is primum ASD, cleft in the MV & small VSD. • Hemodynamics are dependent on the lesions.
  • 18.
    Symptoms • Incomplete AVSDmaybe indistinguishable from ASD - usually asymptomatic. • Congestive heart failure in infancy. • Recurrent pulmonary infections, Failure to thrive, Exercise intolerance, easy fatigability. • Late cyanosis from pulmonary vascular disease with R to L shunt. Clinical • Hyperactive precordium • Normal or accentuated 1st heart sound • Wide, fixed splitting of S2 • Pulmonary systolic ejection murmur w/thrill • Holosystolic murmur @ apex with radiation to axilla • Mid-diastolic rumbling murmur @ LSB • Marked cardiac enlargement on CX- Ray
  • 19.
    PDA • The DuctusArteriosus Connects Pulmonary Artery to Aorta Shunts blood away from lungs in fetal circulation. • Maintained patent by low oxygen pressures and presence of prostaglandins • Closes at Birth secondary to Increase in PaO2 and Decrease in level of prostaglandins. • If this shunt persists post delivery then its called PDA
  • 20.
    PDA • Normally closesin the 1st wk of life, within 48hrs post delivery. • 5-10% of all births (1 of 2000 live births) occurs in 80% of premature babies • Accounts for 10% of all CHD, seen in 10% of other congenital heart lesions and can often play a critical role in some lesions. Often associated with coarctation & VSD. • 2-3 times more common in females than males. • 5th or 6th most common congenital cardiac defect. • May be desirable with some defects. Morbidity/Mortality related to degree of blood flow through PDA.
  • 21.
    Hemodynamics of PDA •As a result of higher aortic pressure and low pulmonary pressure, blood shunts L to R through the ductus from Aorta to PA into the lungs. This causes pulmonary overflow and systemic hypoperfussion. • The pulmonary veins return excess blood to the left atria into left ventricle thus increasing CO but with decreased systemic output and hypoxia causing a heamodynamic paradox. • Increased pulmonary overflow causes pulmonary oedema and eventually increases pulmonary vascular resistance leading to pulmonary hypertension. • Increased pressures in the right will eventually reverse the flow and the condition becomes cyanotic.
  • 22.
    PDA • With increaseLV output and low systemic perfussion, hypoxia triggers cardiac support neuro-humoral effects i.e the sympathetic system and RAAS which cascades to promote heart failure. • There will be left atria and ventricular enlargement, aortic and pulmonary vein and arteries dilatation with pulmonary hypertension. • Cerebral and systemic hypoxia lead to cerebral damage and multi- organ failure.
  • 23.
    PDA Small PDA’s areusually asymptomatic Large PDA’s can result in symptoms of Congestive Heart Failure, growth restriction, Failure to Thrive. Bounding arterial pulses Widened pulse pressure Enlarged heart, prominent apical impulse Grade 1-2 Classic continuous machinery systolic murmur (washing Machine) Mid-diastolic murmur at the apex
  • 24.
    Obstructive Heart Lesions •Pulmonary Stenosis • Aortic Stenosis • Coarctation of the Aorta
  • 25.
    Pulmonary Stenosis • PulmonaryStenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract. • Accounts for 7-10% of all CHD. • Most cases are isolated lesions • Maybe biscuspid or fusion of 2 or more leaflets. • Can present w/or w/o an intact ventricular septum.
  • 26.
    Hemodynamics • Right Ventricularvolume overload, RV pressure hypertrophy ->Right Ventricular failure. • Right Ventricular pressures maybe more than systemic pressure. • Post-stenotic dilation of main Pulmonary Artery. • In severe stenosis without intact septum lead to R-L shunt through Pertent foramen ovale causing cyanosis. • Cyanosis is indicative of Critical PS.
  • 27.
    Pulmonary Stenosis Clinical Signs& Symptoms • Depends on the severity of obstruction. • Asymptomatic with mild PS < 30mmHg. • Mod-severe: 30-60mmHg, > 60mmHg • Prominent jugular a-wave, RV lift • Split 2nd heart sound with a delay • Ejection click, followed by systolic murmur. • Heart failure & cyanosis seen in severe cases
  • 28.
    Aortic Stenosis • AorticStenosis is an obstruction to the outflow from the left ventricle at or near the aortic valve that causes a systolic pressure gradient of more than 10mmHg. Accounts for 7% of CHD. • 3 Types • Valvular – Most common. • Subvalvular(subaortic) – involves the left outflow tract. • Supravalvular – involves the ascending aorta is the least common.
  • 29.
    Hemodynamics • Pressure hypertrophyof the LV and LA with obstruction to flow from the LV. • Mild AS 0-25mmHG • Moderate AS 25-50mmHg • Severe AS 50-75mmHg • Critical AS > 75mmHg
  • 30.
    Aortic Stenosis Symptoms • MildAS may present with exercise intolerance, easy fatigabiltity, but usually asymptomatic. • Moderate AS – Chest pain, dypsnea on exertion, dizziness & syncope. • Severe AS – Weak pulses, left sided heart failure, Sudden Death. Clinical Signs • LV thrust at the Apex. • Systolic thrill @ right base/suprasternal notch. • Ejection click, III-IV/VI systolic murmur @ Right sternal border (RSB)/Left Sternal Border (LSB) with radiation to the carotids.
  • 31.
    Coarctation of theAorta • Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation. • 98% of coarctations are juxtaductal. • Male: Female ratio 3:1. • Accounts for 7 % of all CHD • Associated with Bicuspid Aortic Valve and Turner’s Syndrome
  • 32.
    Location of Coarctation Pre-Ductal •Less common but more serious • Associated with VSD, PDA, Transposition Post-Ductal • More common • Often associated with collateral circulation beyond coarctation, which minimizes effect. • Diagnosed by a difference in blood pressure between lower extremities and upper ones. • Pressure in upper extremities > lower
  • 33.
    Hemodynamics • There isobstruction of left ventricular outflow -> severely reduced systemic blood flow and pressure hypertrophy of the LV. • Increased work on the heart leading to CHF and cardiovascular collapse.
  • 34.
  • 35.
    Cyanotic Heart Diseases •Tetralogy of Fallot • Complete Transposition of the Great Arteries. • Anomalous Venous Return • Truncus Arteriosus • Hypoplastic Left Heart Syndrome
  • 36.
    Tetralogy of Fallot •Most common of the cyanotic cardiac diseases. • Mortality increases with age (1 year- old has a 25% mortality, 40 year-old has 95%). • Four Defects • Pulmonary infandibular Stenosis (determinant factor related to severity) i.e narrowing of RV outflow • VSD (usually large) • Overriding Aorta • RV hypertrophy
  • 37.
    TOF hemodynamics • Duenarrowed pulmonary infandibula and overriding Aorta, both RV and LV ejects blood into the Aorta. • VSD is restrictive thus no gradient across the chambers • Systolic pressure in LV and RV are the same • RV persists its dorminance as in fetal life preferentially ejects into the aorta. With increased flow it dilates giving a boot shape on X-ray. • RV pressure never exceeds systemic pressure. • With the narrowed pulmonary infandibula, very minimal blood is delivered to the lungs for oxygenation, thus the cyanosis and finger clubbing.
  • 38.
    Complete Transposition ofthe Great Arteries • Second most common form of CHD. Causes blue baby syndrome • Aorta arises from RV and Pulmonary Arteries from LV. • Without an abnormality, life would not be possible. • ASD • VSD (30-40%) • PDA
  • 39.
    Hemodynamics • Oxygen poorblood returns to the RA, to RV then into the misconnected Aorta back to the system. • Oxygen rich blood returns to the LA from lungs into the LV then into the PA back to the lungs • Two separate circutes are formed, one of oxygen poor and oxygen rich blood. • Other heart defects associated with TGA help mitigate the problem. PDA, VSD and ASD facilitate mixing of this blood thus allowing some oxygen to reach tissues.
  • 40.
    Anomalous Venous Return •Return of pulmonary venous blood to the right atrium instead of the left or in the systemiv veins. Can also be partial or complete. • ASD is present to sustain life.
  • 41.
    Hemodynamics of TVR •Pulmonary veins pours into the RA causing volume overload and thus pressure overload to thr RA and RV. • There is incread blood flow to the lungs leading to pulm oedema, and increased pulm vascular resistance then pulmonary hypertension. • Without a ASD, life is not sustainable. • LA and LV receive less and pump less. Reduced CO triggers neuro- humoral cardiac response which cascades to CHF
  • 42.
    Truncus Arteriosus • Defectin which one large vessel( usually pulmonary trunk) arises from right and left heart over a large VSD. • Cyanosis is often present. • CHF common
  • 43.
    • Outflow fromboth ventricles is directed to this one trunk. • Resistances to flow is determined by both systemic and pulmonary resistance. • Subnormal arterial oxygen concentration, pulmonary flow is elevated causing over circulation and deprived systemic circulation. • This caused increased myocardial workload and failure.
  • 44.
    Hypoplastic left heartsyndrome • It’s a condition where the left heart does not form normally during embryogenesis. • Features are: • -under developed LV • Unformed or underdeveloped mitrial valve • Unformed or underdeveloped Aortic valve • Underdeveloped or very small ascending aorta • Atrioseptal defect
  • 45.
    hemodynamics • In thefirst few days of life, the right side pumps blood to both the lungs and the system via the PDA. • Pulmonary venous return drains into the right heart via ASD • Closure of the two is fatal therefore they must be kept open using prostaglandins.
  • 46.
    Dextrocardia • Dextrocardia isa radiographic term used when the heart is on the right side of the chest. • When dextrocardia occurs with reversal of position of the other important organs of the chest and abdomen (e.g. liver, lungs, and spleen), the condition is called situs inversus totalis and the heart is usually completely normal. • When dextrocardia occurs with otherwise normal organs (situs solitus), the heart usually has severe defects. • Rarely, the abdominal organs and lungs are neither in situs solitus or situs inversus, these states are called situs ambiguous. • The liver is central and anterior in the upper abdomen with the stomach pushed behind • There may be bilateral right-sidedness (asplenia syndrome) or bilateral left- sidedness (polysplenia syndrome), but in virtually all cases of situs ambiguous, severe congenital heart disease is present.
  • 47.
    Cardiomyopathy • Its anacquired or hereditary progressive disease of the myocardium. • The muscle weakens and it is unable to pump blood to the systems • There are 3 types of cardiomyopathy • i) Dilated • ii) Restrictive • Iii) Hypertrophic • Treatment depends on the type.
  • 48.
    Dilated Cardiomyopathy-Pathology • Gradualfour chamber hypertrophy and dilation. can occur at any age as slow progressive CHF but mostly affects middle aged people. • Leads to impaired myocardial contractility and CHF. • The following gross changes may be observed • The heart is flabby. • Cardiomegaly (to 900gm) is typical, although wall thickness may not reflect the degree of hypertrophy due to dilation • Poor contractile function and stasis can predispose to mural thrombi • Valves and coronary arteries are generally normal
  • 49.
    Hypertrophic cardiomyopathy • Characterisedby abnormal thickening of the myocardium • Affected people have Fhx of the disease, linked to genetic mutations. • characterized by heavy, muscular, hypercontractile, poorly compliant hearts with poor distolic relaxation. Has increased risk of Sudden death.
  • 50.
    Pathology of Hypertrophiccardiomyopathy • marked cardiomegaly owing to hypertrophy, left ventricle greater than right ventricle, often with atrial dilation. • Classically, there is disproportionate thickening of the interventricular septum (asymmetric septal hypertrophy) although many cases have concentric or symmetric hypertrophy • The left ventricular cavity is compressed into a banana-like configuration by the symmetric bulging of the septum • Septal thickening at the level of the mitral valve compromises left ventricular systolic outflow by contact of the anterior mitral leaflet with the septum.
  • 51.
    • Microscopically, thereis marked myofibre hypertrophy; 25-50% of the septum classically shows helter-skelter myocyte disarray, accompanied by myofilament disorganization within muscle cells • There are abnormal thick-walled intramyocardial arterioles, as well aspatchy replacement fibrosis, the later presumably due to focal ischemic injury.
  • 52.
    Restrictive Cardiomyopathy • Relativelyrare. The myocardium becomes rigid and less elastic so it cant expand to fill with blood in diastole. Often occurs in old age. • It is characterized by ventricular subendocardial fibrosis extending from the apex to the ventricular inflow tracts, often with superiposed mural thrombus • The atrioventricular valves may be involved • Restrictive physiology results with reduced ventricular chamber volume and reduced CO
  • 53.
    Arrythmogenic Right ventricular cardiomyopathy •Arrythmogenic right ventricular cardiomyopathy is a recently recognized cardiomyopathy with distinct presentation and morphology • It is typically a familial disorder characterized by predominantly right sided failure (occasionally left sided) and various rythmic disturbances, particularly ventricular tarchcardia and sudden death. • Morphologically the right ventricular wall is severely thinned with myocyte loss and profound fatty infiltration. • Death occurs secondary to progressive CHF, embolism of mural thrombi of fatal arrythmias.
  • 54.
    Myocarditis • It ischaracterized by myocardial inflammation as the principle feature • Most cases are thought to be of viral origin (e.g. coxsackie A and B, echovirus). Cardiac involvement occurs days to weeks after a primary viral infection, which can be in the heart or solely at another site • The cardiac involvement can be a direct infection or an immunological reaction to a myocardial antigen triggered by the infectious agent • Rare cases are secondary to bactereamia e.g. staphylococci, tuberculosis trypanosome cruzi
  • 55.
    pathology • Gross • Grossmanifestations include a flabby heart often with four chamber dilation and patchy heamorrhagic mottling. • Mural thrombi can form in dilated chambers • Endocardium and valves are unaffected • After the acute stage, there may be residual dilation or hypertrophy
  • 56.
    • Microscopically • Thereis a myocardial inflammatory infiltrate with associated myocyte necrosis or degeneration. Lesions are typically focal and may be missed by routine endomyocardial biopsy. • In viral infections, associated myofibre necrosis is seen with interstitial edema and a mononuclear cell infiltrate. After the acute stage, inflammatory lesions may resolve, leaving either no residual or variable interstitial and replacement fibrosis • Depending on cause, bacteria, other organisms and cellular infiltrates may be demonstrated.
  • 57.