Condition	Pathology	Clinical features	Morphology	
Congestive Heart Failure	•	Impaired cardiac function that render the heart unable to maintain an sufficient cardiac output.
•	Progressive deterioration of myocardial contractile function due to ischemic injury, pressure or volume overload or dilated cardiomyopathy.
•	Failures can result from an inability of the heart chambers to relax sufficiently during diastole.
•	Can occur with left ventricular hypertrophy, myocardial fibrosis, deposition of amyloid or constrictive pericarditis.	•	Characterized by diminished cardiac output or damming back of blood in the venous system.
•	Compensatory changes ultimately constitute further burdens on cardiac function.

Compensatory changes:
•	Hypertrophy makes myocytes vulnerable to injury.
•	Ventricular dilation (Frank-Starling law).
•	Blood volume expansion by salt & water retention.
•	Tachycardia.	•		
Left-sided Heart failure	Causes:
•	Ischemic heart disease
•	Hypertension
•	Aortic & mitral valve disease
•	Myocardial disease	•	Manifested by pulmonary congestion & edema secondary to impairment of lung vascular outflow.
•	Reduced cardiac output results in reduced renal perfusion.
•	CNS perfusion is reduced, leading to hypoxic encephalopathy.

Results of reduced renal perfusion:
•	Further salt & water retention.
•	Ischemic acute tubular necrosis.
•	Impairment of waste excretion, causing prerenal azotemia.	•		
Right-sided Heart failure	•	Typically a consequence of left-sided failure.

Caused by:
•	Intrinsic disease of the lungs.
•	Pulmonary vasculature causing functional right ventricular outflow obstruction.
•	Tricuspid or pulmonary vulvar disease.	•	Port, systemic & dependent peripheral congestion & edema & effusions.
•	Congestive splenomegaly with sinusoidal dilation, focal hemorrhages, hemosiderin deposits & fibrosis.
•	Renal congestion, hypoxic injury & acute tubular necrosis.

Hepatomegaly:
•	Centrilobular congestion.
•	Atrophy of central hepatocytes, producing a nutmeg appearance.
•	Severe hypoxia leads to centrilobular necrosis.
•	High right-sided pressure: sinusoidal rupture causes central hemorrhagic necrosis.
•	Subsequent central fibrosis creates cardiac sclerosis.	•		
Ischemic heart disease	Caused by:
•	Reduced coronary blood flow; often a combination of coronary atherosclerosis with vasospasm, thrombosis, or both.
•	Increased myocardial demand exceeding vascular supply.	•	Angina pectoris: paroxysmal substernal or precordial pain or discomfort.
•	Myocardial infarction is marked by death of cardiac muscle.
•	Chronic ischemic heart disease is seen typically in elderly patients; may result from postinfarction cardiac decompensation or slow ischemic myocyte degeneration.
•	Sudden cardiac death: unexpected death within 1 hour of onset of symptoms.

Causes of sudden cardiac death:
•	Aortic valvular stenosis.
•	Hereditary or acquired conduction system abnormalities.
•	Electrolyte derangements.
•	Mitral valve prolapse.
•	Dilated or hypertropic cardiomyopathy.
•	Ultimate mechanism of death is a fatal arrhythmia. 	•	Myocardium has variable myocyte atrophy.
•	Perinuclear deposition of lipofuscin, myocytolysis of single cells or clusters.
•	Diffuse perivascular & interstitial fibrosis.
•	Patchy to confluent replacement fibrosis.	
Myocardial infarction	Types of MI:
•	Transmural infarct: infarction of full thickness of ventricular wall, caused by severe coronary atherosclerosis worsened by plaque disruption & superimposed occlusive thrombosis.
•	Subendocardial infarct: limited to inner one third to one half of ventricular wall.

Typical sites of plaques:
•	Proximal 2cm of the left anterior descending & left circumflex coronary arteries.
•	Proximal & distal thirds of the right coronary artery.

Pathogenesis:
•	Nearly all transmural MIs affect left ventricle; 15% simultaneously involve right ventricle; isolated right ventricle infarction occurs in 1-3% of cases.
•	Initial event in transmural MI: erosion, ulceration, fissuring, rupture or hemorrhagic expansion.
•	Thrombosis follows acute plaque change.
•	Time interval between onset of complete myocardial ischemia & initiation of irreversible injury: 20-40 minutes.
•	Thrombi may lyse spontaneously, reestablishing flow.

Causes of Subendocardial infarcts:
•	Diffuse coronary atherosclerosis.
•	Global borderline perfusion made critical by increased demand, vasospasm, or hypotension.
•	Disrupted plaque with overlying thrombus. 	•	Chest pain
•	Nausea
•	Diaphoresis
•	Dyspnea
•	ECG changes
•	Serum elevation of myocardial enzymes, e.g. creatine kinase-MB isozyme.
•	25% of patients experience sudden death after infarction, secondary to a fatal arrhythmia.
•	80-90% of survivors develop complications.

Complications:
•	Arrhythmias
•	Congestive heart failure
•	Cardiogenic shock
•	Ventricular rupture within first 10 days – rupture of free wall causing pericardial hemorrhage & tamponade & rupture of septum producing a left-to-right shunt with right-sided heart volume overload.
•	Papillary muscle infarction with or without rupture.
•	Fibrinous-to-hemorrhagic pericarditis, common 2-3 days postinfarction.
•	Mural thrombosis with risk of peripheral embolization.	Gross:
•	6-12 hours: lesions is usually inapparent.
•	18-24 hours: infarcted tissue is readily apparent – pale to cyanotic.
•	First week: lesions becomes progressively more sharply defined, yellow, & softened.
•	7-10 days: a circumferential rim of hyperemic granulation tissue appears & progressively expands.
•	6 weeks: white fibrous scar is established.

Histology:
•	1 hour: intercellular edema, & myocytes at edge of infarct become wavy & buckled.
•	12-72 hours: neutrophilic infiltration into necrotic tissue, with myocyte coagulative necrosis; dead myocytes become hypereosinophilic with loss of nuclei.
•	7-10 days: granulation tissue progressively replaces necrotic tissue, generating a dense fibrous scar.	
Hypertensive Heart Disease	
Systemic (left-sided) hypertensive heart disease	•	Myocyte hypertrophic enlargement as a response to increased work.
•	Thickened myocardium reduces left ventricle compliance, impairing diastolic filling while increasing oxygen demand.
•	Individual myocyte hypertrophy increases distance for oxygen & nutrient diffusion from adjacent capillaries.	•	Increases risk of sudden cardiac death.
•	Remainder die of renal disease, stroke, or unrelated causes.	•	Thickened left ventricle wall with increased heart weight (>2cm wall thickness, heart weight > 500gm).
•	Myocytes & nuclei are enlarged.
•	Diffuse interstitial fibrosis & focal myocyte atrophy & degeneration may develop, with left ventricle chamber dilation & wall thinning.	
Pulmonary (right-sided) heart disease (Cor Pulmonale)	•	Right ventricle hypertrophy is secondary to pulmonary hypertension caused by disorders affecting lung structure or function.
•	Acute cor pulmonale: right ventricle dilation after massive pulmonary embolization.
•	Chronic cor pulmonale: result of chronic right ventricle pressure overload.
•	Vasoconstriction in pulmonary vasculature incident to hypoxemia & acidosis exacerbates pulmonary hypertension.	•		•	Right ventricle hypertrophy, to 1cm or more in thickness; dilation, or both.
•	Right ventricle dilation may lead to tricuspid regurgitation.
•	Pulmonary arteriolar wall thickening.
•	Left side of heart is normal.	
Valvular Heart Disease	
Degenerative calcific aortic valve stenosis	•	Age-related lesions clinically important in patients in their 70s & 80s.
•	Remainder are congenital bicuspid valves.	•	Angina
•	Syncope
•	Congestive heart failure	•	Heaped-up subendothelial rigid calcific masses within sinuses of Valsalva cause thickening & immobility of the valve cusps with narrowing of orifice.
•	There is usually concentric left ventricle hypertrophy from chronic pressure overload.	
Mitral Annular Calcification	•	Degenerataive, noninflammatory calcific deposits within mitral annulus, usually in the elderly.
•	Regurgitation may occur owing to inadequate sytolic contraction of the mitral valve ring.
•	Leaflets may be unable to open over bulky deposits, causing stenosis.
•	Nodular calcific deposits may cause arrhythmias by impinging on the conduction pathways.	•		•		
Mitral valve prolapse	•	One or both mitral valve leaflets are enlarged, myxomatours & floppy, & they balloon back into left atrium during systole, causing midsystolic click & mitral valve insufficiency.
•	Common in Marfan syndrome.	•	Generally asymptomatic.
•	Discovered only as a midsystolic click on auscultation.
•	Atypical chest pain.
•	Dyspnea
•	Fatigue
•	Psychiatric manifestations: depression, anxiety.

Patients have increased risk of:
•	Infective endocarditis
•	Slow, progressive mitral valvular insufficiency that may produce congestive heart failure.
•	Atrial & ventricular arrhythmias
•	Sudden death	Gross:
•	Interchordal ballooning (>4cm) of mitral valve leaflets.
•	Elongated, attenuated, or ruptured chordae tendineae.
•	Fibrous thickening of valve leaflets.
•	Thickened left ventricular endocardium.
•	Atrial thrombosis
•	Calcification of mitral annulus.

Histology:
•	Thinning & degeneration of fibrosa layer.
•	Myoxomatous thickening of spongiosa.	
Rheumatic Fever & Rheumatic heart disease	•	An acute, recurrent inflammatory disease that occurs 1-5 weeks after group A streptococcal infection.
•	Occurs mainly in children between 5-15 years of age. 
•	Secondary to host antistreptococcal antibodies that are cross reactive to cardiac antigens.

Chronic rheumatic heart disease is more likely to occur:
•	when first attack is in early childhood.
•	when first bout of rheumatic fever is severe.
•	with recurrent attacks.	Changes secondary to tight mitral stenois:
•	Left atrial hypertrophy & enlargement, occasionally with mural thrombi.
•	Chronic congestive changes in the lungs.
•	Right ventricle hypertrophy
•	Congestive heart failure

Complications:
•	Increased risk of developing infective endocarditis.
•	Atrial fibrillation secondary to atrial dilation.

Diagnosis requires presence of 2 of 5 major Jones criteria:
•	Erythema marginatum: macular skin lesions with erythematous rims & central clearing.
•	Sydenham chorea: neurologic disorder with rapid, involuntary, purposeless movements.
•	Carditis
•	Subcutaneous nodules
•	MIgratory large joint polyarthritis: 90% of adults, less common in children.	Histology:
•	Aschoff bodies are foci of fibrinoid necrosis surrounded by lymphocytes, macrophages & slowly replaced by fibrous scar.
•	Inflammatory valvulitis induces formation of beady fibrinous vegetations; aschoff bodies may be present in inflamed valves.
•	Subendocardial collections of Aschoff nodules in left atrium usually induced thickenings called MacCallum plaques.

Chronic valve shows:
•	Fibrous thickening of leaflets.
•	Bridging fibrosis across commissures.
•	Thickened, fused, & shortened mitral valve chordae.
•	Calcification deep in fibrous leaflets.	
Vegetative Endocarditis	
Infective Endocarditis	•	Colonization of heart valves with microbiologic organisms leads to formation of friable, infected vegetations & valve injury.
Acute infective endocarditis:
•	Caused by highly virulent organisms, e.g. S. aureus.
•	Seeding a previously normal valve & producing a necrotizing, ulcerative, & invasive infection.

Subacute infective endocarditis:
•	Caused by an organism of moderate to low virulence, e.g. Streptococcus viridans.
•	Seeding an abnormal or previously injured valve, causing less valvular destruction than acute infective endocarditis.

Predisposing factors:
•	Cardiac congenital abnormalities: tight shunts or stenoses with jet stream.
•	Mitral valve prolapse
•	Degenerative calcific stenosis
•	BIcuspid aortic valve
•	Prosthetic valves
•	Indwelling catheters
•	Neutropenia
•	Immunosuppressed states
•	Intravenous drug abuse	Acute infective endocarditis:
•	Rapidly developing fever with rigors, malaise, & weakness.
•	Larger vegetations often cause embolic complications.
•	Splenomegaly is frequent.
•	Even with treatment, death occurs in days to weeks in 50-60% of patients.

Subacute infective endocarditis:
•	Insidious onset with malaise, low-grade fever, weight loss & flulike syndrome.
•	Embolic complications are less frequent due to smaller vegetations.
•	Tends to have a protracted course & is less fatal than acute form.

Clinical consequences:
•	Direct injury to valves.
•	Emboli to spleen, kidneys, heart & brain with infarction or metastatic infection.
•	Renal injury: embolic infarction or glomerulonephritis.

Diagnosis is confirmed by blood cultures.	•	Friable, 0.5-2.0cm, microbe-laden vegetations are found on one or more valves.
•	Acute infective endocarditis is associated with bulky vegetations that cause erosions or perforations of leaflets, invading adjacent myocardium to produce abscesses.
•	Subacute infective endocarditis has smaller vegetations that rarely erode or penetrate leaflets.
•	Nonvalvular vegetations are found on downstream margin of a jet lesion.
•	Ring abscess is present with prosthetic valves.
•	With intravenous drug abuse, vegetations are often acute & or on right-sided valves. 	
Nonbacterial Thrombotic Endocarditis	Occurs in settings of disseminated intravascular coagulation or other hypercoagulable state:
•	Cancer: visceral adenocarcinomas
•	Renal failure
•	Chronic sepsis		•	Small (1-5mm), sterile, bland fibrin & platelet thrombi loosely adherent to valve leaflets.	
Endocarditis associated with SLE	•	Valvulitis may appear in systemic lupus erythematous & antiphospholipid syndrome.
•	Mitral & tricuspid valves are most often affected with fibrinoid necrosis, mucoid degeneration & subsequent development of small, fibrinous, sterile vegetations on either side of valve leaflets.		•		
Carcinoid heart disease	•	Elaboration of argentaffinomas of bioactive products – serotonin, kallikrein, histamine & prostaglandins.
•	Predominantly right-sided heart lesions.		•	Plaquelike intimal thickenings of endocardium of tricuspid valve, right ventricular flow tract & pulmonic valve.
•	Left side of heart is usually unaffected.
•	Use of fenfluramine & phentermine, appetite suppressants used to treat obesity associated with similar left-sided valve lesions.
	
Complications of artificial valves	•	Paravalvular leak: separation of sewing ring from valve annulus.
•	Thrombosis, thromboembolism, or both.
•	Infective endocarditis.
•	Structural deterioration.
•	Occlusion as a result of tissue overgrowth.
•	Hemolysis from mechanical trauma to erythrocytes.		•		
Cardiomyopathy	
Dilated cardiomyopathy	•	Gradual four-chamber hypertrophy & dilation.
•	May occur at any age as slow, progressive congestive heart failure.

Causes:
•	Genetic defect
•	Alcohol toxicity
•	Peripartum cardiomyopathy
•	Postviral myocarditis	Arrhythmogenic right ventricular cardiomyopathy:
•	Heart failure & various rhythm disturbances.
•	Ventricular tachycardia & sudden death.
•	Death occurs secondary to progressive congestive heart failure, embolism of mural thrombi, or fatal arrhythmias.	•	Cardiomegaly (up to 900gm) & flabby heart.
•	Poor contractile function & stasis lead to mural thrombi.
•	Milde to moderate focal endocardial thickening in ventricles.
•	Right ventricular wall is severely thinned, with excessive fatty infiltration, loss of myocytes & interstitial fibrosis.	
Hypertrophic cardiomyopathy	•	Heavy, muscular, hypercontractile, poorly compliant heart with poor diastolic relaxation.
•	Autosomal dominant inheritance: mutations of gene for myosin heavy chain & other contractile proteins.
•	Increased risk of sudden death.	Clinical symptoms:
•	Dyspnea
•	Angina
•	Near-syncope
•	Congestive heart failure

Complications:
•	Atrial fibrillation with mural thrombus
•	Embolization
•	Infective endocarditis
•	Congestive heart failure
•	Sudden death	•	Cardiomegaly, owing to hypertrophy, often with atrial dilation.
•	Disproportionate thickening of the septum versus left ventricle free wall (asymmetrical septal hypertrophy)
•	Left ventricle cavity compressed into a banana-like configuration.
•	Septum shows helter-skelter myocyte disarray, accompanied by myofilament disorganization within muscle cells.
•	Patchy replacement fibrosis as a result of focal ischemic injury & abnormal thick-walled arteries of unknown origin.	
Restrictive cardiomyopathy	•	Restriction of ventricular filling & reduced cardiac output.
•	Interstitial myocardial fibrosis is usually present.
•	Endomyocardial fibrosis: found typically in young children & adults in Africa.
•	Endocardial fibroelastosis: common in patients younger than 2 years old, one-third with congenital abnormalities.		Endomyocardial fibrosis:
•	Ventricular subendocardial fibrosis.
•	Mural thrombus formation.

Loeffler endocarditis:
•	Endomyocardial fibrosis with large mural thrombi.
•	Peripheral eosinophilia.
•	Eosinophilic infiltration of multiple organs, including heart (rapid fatal course).

Endocardial fibroelastosis:
•	Focal-to-diffuse, cartilage-like fibroelastic thickening of endocardium.
•	Affects left ventricle more.
	
Myocarditis	Infections:
•	Viruses: coxsackievirus, ECHO, influenza, HIV, CMV.
•	Chlamydia: C. psittaci
•	Rickettsia
•	Bacteria: Corynebacterium, meningococcus, Borrelia.
•	Fungi: Candida
•	Protozoa: Trypanosoma cruzi, toxoplasmosis
•	Helminths: trichinosis

Immune-mediated reactions:
•	Postviral
•	Poststreptococcal (rheumatic fever)
•	Systemic lupus erythematous
•	Drug hypersensitivity: methyldopa, sulfonamides
•	Transplant rejection

Unknown:
•	Sarcoidosis
•	Giant cell myocarditis	•	Asymptomatic
•	Abrupt onset of arrhythmia
•	Congestive heart failure
•	Sudden death
•	Most patients recover quickly without sequelae.	Gross:
•	Flabby ventricular myocardium
•	Four-chamber dilation
•	Patchy, diffuse hemorrhagic mottling.
•	Mural thrombi arise in dilated chambers.

Histology:
•	Myocardial inflammatory infiltrate with associated myocyte necrosis or degeneration.
•	Lesions are typically focal.
•	Myocarditis associated with viral infections: isolated myofiber necrosis with interstitial edema & mononuclear cell infiltrate.
•	Chagas disease: trypanosomes parasitize myocytes & produce acute & chronic inflammation, including eosinophils.
•	Idiopathic giant cell myocarditis: focal myocyte necrosis associated with granulomatous inflammation, including multinucleated giant cells.
•		
Other myocardium diseases	•	Doxorubicin & Daunorubicin induce a dose-dependent cardiotoxicity due to lipid peroxidation of myofiber membranes.
•	Iron overload with hemosiderin deposits in myofibers occur in hereditary hemochromatosis & hemosiderosis from multiple blood transfusions.
•	Amyloidosis: patchy & perivascular hyaline deposits.
•	Catecholamines induce tachycardia & vasomotor constriction resulting in diffuse patchy ischemic necrosis. 	•		•	Myofiber swelling
•	Fatty change
•	Individual cell lysis
•	Mitochondrial abnormalities
•	Smooth endoplasmic reticulum swelling & fragmentation
•	Myofibril lysis
•	Delicate interstitial fibrosis & focal replacement scarring occur with time. 	
Pericardial Disease	
Pericardial effusion	•	Serous form is most common.
•	Fluid accumulates slowly till a large volume compromises diastolic filling.
•	Most common causes are congestive heart failure & hypoproteinemia.	•		•		
Hemopericardium	•	Accumulation of pure, clotted blood in pericardium without an inflammatory component.
•	200-300ml may cause tamponade.

Causes:
•	Traumatic perforation.
•	Myocardial rupture after a transmural MI.
•	Rupture of intrapericardial aorta.
•	Hemorrhage from an abscess or tumor metastasis.	•		•		
Acute Pericarditis	Serous:
•	Consists of 50-200ml of slowly accumulating exudate.
•	Produced by rheumatic fever, SLE, tumors, uremia & primary viral infection.
•	Scant epicardial & pericardial acute & chronic inflammatory infiltrate.

Fibrinous:
•	Most common clinical form.
•	Seen with MI
•	Associated with a pericardial rub.
•	Exudate may be completely resolved or organized leaving delicate, stringy adhesions.

Purulent:
•	Bacterial, fungal or parasitic infection which has reached pericardium by direct seeding, hematogenous or lymphatic spread.
•	Common causes: gram-positive staphylococci, streptococci & pneumococci.
•	400-500ml of thin-to-creamy pus with erythematous, granular serosal surfaces.
•	Presents with fever, rigors & a friction rub.

Hemorrhagic:
•	Exduate of blood mixed with fibrinous-to-suppurative effusion.
•	Follows cardiac surgery.
•	Associated with tuberculosis or malignancy.
•	Usually organizes without calcification.

Caseous: due to tuberculosis or mycotic infection.	•		•		
Chronic Pericarditis	Healing of acute lesions lead to:
•	Resolution
•	Pericardial fibrosis ranging from a thick, nonadherent epicardial plaque to thin, delicate adhesions.

Adhesive mediastinopericarditis:
•	Pericardial sac is obliterated.
•	Parietal layer is tethered to mediastinal tissue.
•	Heart contract against surrounding attached structures, with subsequent hypertrophy & dilation.

Constrictive pericarditis:
•	Thick, dense fibrous obliteration.
•	Calcification of pericardial sac.
•	Limit diastolic expansion & restricting cardiac output.
•	Common cause: tuberculosis.	•		•		
Rheumatoid heart disease	•	Rheumatoid arthritis in 20-40% of severe chronic cases.
•	Pericarditis, marked by a mixture of fibrin & necrotic debris derived from pericardial rheumatoid granulomas.
•	Progress to form dense, fibrous, & restrictive adhesions.	•		•		
Tumors	
Myxomas	•	Most common primary cardiac tumors in adults.
•	90% arise in atria in region of fossa ovale.
•	Cause symptoms by physical obstruction or trauma to atrioventricular valves.
•	Probably hamartomas.	•		•	Composed of stellate or globular multipotential mesenchymal myxoma cells.
•	Admixed with endothelia, smooth muscle & inflammatory cells.	
Lipomas	•	Circumscribed but poorly encapsulated large polypoid accumulations of adipose tissue.
•	Probably hamartomas.	•		•		
Papillary fibroelastomas	•	Commonly found on right-sided valves in children & left-sided valves in adults.
•	Composed of 2-55mm hairlike projections.
•	Probably derived from organized thrombi.	•		•	Filaments have a core of myxoid connective tissue with smooth muscle cells & fibroblasts, covered by endothelium.	
Rhabdomyomas	•	Most common primary heart tumor in children.
•	May cause valvular or outflow tract obstruction.
•	Probably hamartomas.	•		•	Composed of large, rounded or polygonal cells rich in glycogen & containing myofibrils.
•	Cytoplasmic strands radiating from central nucleus to plasma membrane create spider cells.	
Congenital heart disease:
•	Most critical juncture is embryologic cardiac development in gestational weeks 3 through 8.
•	Complications: failure to thrive, retarded development, cyanosis, increased risk of chronic illness & of infective endocarditis.	
Shunts	•	Abnormal communication between heart chambers, vessels, or between chambers & vessels.	Secondary findings:
•	Clubbing of fingers & toes
•	Hypertrophic osteoarthropathy
•	Polycythemia	•		
Left-to-Right Shunts: Late Cyanosis	
Atrial septal defect	•	Abnormal opening in the atrial septum that allows free communication of blood. 
•	Most common congenital cardiac anomaly in adults.
•	Primum type: 5%; common in Down syndrome.
•	Secundum type: 90%, occurs at foramen ovale.
•	Sinus venosus type: 5%; occurs high in septum & near superior vena cava entrance.	•	Usually asymptomatic until adulthood.
•	Right-sided heart failure.
•	Right-sided hypertrophy.
•	Surgical correction early in life prevents pulmonary vascular changes & paradoxic embolism.	•		
Ventricular septal defect	•	Abnormal opening in ventricular septum that allows free communication between left & right ventricles.
•	Most common congenital cardiac anomaly.
•	Associated with other structural anomalies such as tetralogy of Fallot; 30% are isolated.	•	Fulminant congestive heart failure.
•	Late cyanosis.
•	Asymptomatic holosystolic murmurs.
•	Spontaneous closure.
•	Increased risk of infective endocarditis.
•	Surgical correction needed before right-sided heart overload & pulmonary vascular disease develop.	•		
Patent ductus arteriosus	•	In fetus, ductus arteriosus permits blood flow between aorta & pulmonary artery. 
•	85-90% isolated defects.
•	Associated left ventricle hypertrophy & pulmonary artery dilation.	•	Initially asymptomatic with prominent heart murmur.
•	Pulmonary hypertension with right ventricle hyperthropy.
•	Early closure with prostaglandin or surgery.	•		
Right-to-Left Shunts: Early Cyanosis	
Tetralogy of Fallot	•	Due to embryologic anterosuperior displacement of infundibular septum.
Four cardinal features:
•	Ventricular septal defect
•	Dextroposed aorta overriding the ventricular septal defect
•	Pulmonary stenosis with right ventricle outflow obstruction
•	Right ventricle hypertrophy	•	Cyanosis is present from birth or soon after. 	•		
Transposition of great arteries	•	The aorta arises from right ventricle & pulmonary artery from the left.
•	Common in children of diabetic mothers.	•	Prognosis depends on severity of tissue hypoxia & ability of right ventricle to maintain aortic flow.
•	Untreated, most children die within a few months.	•		
Truncus arteriosus	•	Associated with numerous cardiac defects.
•	A developmental failure of the aorta & pulmonary artery to separate.
•	Results in an infundibular ventricular septal defect with a single vessel receiving blood from both right & left ventricles.	•		•		
Obstructive Congenital Anomalies	
Coarctation of aorta	•	Constriction of aorta with cardiomegaly.

Preductal coarctation:
•	Manifests early in life & may be rapidly fatal.
•	Lower body cyanosis
•	Involves a 1-5cm segment of aortic root.
•	Associated with fetal right ventricle hypertrophy & early right-sided heart failure.

Postductal coarctation:
•	Generally asymptomatic unless severe.
•	Leads to upper extremity hypertension.
•	Low flow & hypotension in lower extremities.
•	Arterial insufficiency: claudication & cold sensitivity.
•	Collateral flow: internal mammary & axillary artery dilation.	•	Untreated, mean life span is 40 years.

Death secondary to:
•	Congestive heart failure.
•	Aortic dissection proximal to coarctation.
•	Intracranial hemorrhage.
•	Infective endocarditis at site of narrowing.	•		
Pulmonary valve stenosis	•	Occurs in isolation or in association with other anomalies.
•	Complete pulmonic atresia: hypoplastic right ventricle & an atrial septal defect with blood entering lungs via a patent ductus arteriosus.	•	Mild stenosis is generally asymptomatic.	•		
Aortic valve stenosis & atresia	•	Congenital complete aortic atresia is rare & incompatible with neonatal survival.	•	Infective endocarditis.
•	Left ventricle hypertrophy.
•	Poststenotic dilation of aortic root.
•	Sudden death (rare).	•