This document summarizes congenital heart disease and infective endocarditis. It discusses the main types of congenital heart defects including ventricular septal defects, atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, and obstructive defects. It describes the pathophysiology, clinical features, and complications associated with each type of defect. The document also provides details on infective endocarditis including causative organisms, pathology, diagnostic criteria, and comparisons to other forms of endocarditis.

1. CARDIOVASCULAR
SYSTEM…

3. Congenital Heart Disease
• Congenital heart diseases are abnormalities of the heart
or great vessels that are present at birth.
• Most such disorders arise from faulty embryogenesis
during gestational weeks 3 through 8, when major
cardiovascular structures develop.
Pathogenesis:
• Cause is unknown in almost 90% of cases.
• Environmental factors, such as congenital rubella
infection, are causal in many instances.
• Genetic factors are also clearly involved, as evidenced by
familial forms of congenital heart disease and by well-
defined associations with certain chromosomal
abnormalities (e.g., trisomies 13, 15, 18, and 21 and
Turner syndrome).

4. • Congenital heart diseases subdivided into 3 groups:
1. Malformations causing a left-to-right shunt: increase
pulmonary blood flow, (no cyanosis atleast initially) ->
Increased pressure and volume in pulmonary circulation ->
pulmonary hypertension -> Right ventricular hypertrophy
and shunt reversal -> cyanosis. Eg: VSD, ASD, PDA
2. Malformations causing a right-to-left shunt: pulmonary
circulation is bypassed -> poorly oxygenated blood enters
the systemic circulation -> dusky blueness of the skin
(cyanosis) Eg: cyanotic congenital heart diseases -
tetralogy of fallot, transposition of great arteries, tricuspid
atresia, total anomalous pulmonary venous connection,
persistent truncus arteriosus.

5. • Malformations causing obstruction:
Eg: Coarctation of aorta, aortic stenosis,
pulmonary stenosis.

6. 1. VENTRICULAR SEPTAL DEFECT
• Incomplete closure of ventricular septum, allowing
free communication of blood between the left to
right ventricles, most common form of congenital
cardiac anomaly
• Most VSDs are associated with other congenital
cardiac anomalies such as tetralogy of Fallot
• About 90% involve the region of the membranous
interventricular septum (membranous VSD).
• The remainder lie below the pulmonary valve
(infundibular VSD) or within the muscular septum.
• Although most VSDs are single, those in the muscular
septum may be multiple (so-called "Swiss-cheese"
septum).
Left-to-right shunts “ACYANOTIC”

8. • i) Volume hypertrophy of the right ventricle.
• ii) Enlargement and haemodynamic changes in tricuspid and
pulmonary valves.
• iii) Endocardial hypertrophy of right ventricle.
• iv) Pressure hypertrophy of the right atrium.
• v) Volume hypertrophy of the left atrium and left ventricle.
• vi) Enlargement and haemodynamic changes in the mitral ,
aortic valves

9. 2. ATRIAL SEPTAL DEFECT
• An abnormal, fixed opening in the atrial septum caused
by incomplete tissue formation that allows
communication of blood between left and right atria
• ASDs are usually asymptomatic until adulthood
• Three major types of ASDs
• Secundum ASDs (90%) - deficient or fenestrated oval
fossa near the center of the atrial septum.
• Primum anomalies (5%) adjacent to the AV valves.
• Sinus venosus defects (5%) located near the entrance of
the superior vena cava

10. • i) Volume hypertrophy of the right atrium and right ventricle.
• ii) Enlargement and haemodynamic changes of tricuspid and
pulmonary valves.
• iii) Focal or diffuse endocardial hypertrophy of right atrium
and right ventricle.
• iv) Volume atrophy of the left atrium and left ventricle.
• v) Small-sized mitral and aortic orifice

12. 3. Patent Ductus Arteriosus
• PDA results when the ductus arteriosus, an essential fetal
structure that normally spontaneously closes, remains open after
birth and shunts blood from the aorta to pulmonary artery.
• Persistence after 3 months of age is considered abnormal
• i) Volume hypertrophy of the left atrium and left ventricle.
• ii) Enlargement and haemodynamic changes of mitral and
pulmonary valves.
• iii) Enlargement of the ascending aorta

13. LEFT TO RIGHT SHUNTS

14. Right-to-left shunts “CYANOTIC”
1. Tetralogy of Fallot
• Four cardinal features of TOF are
(1) VSD
(2) obstruction of the right ventricular outflow tract
(pulmonary stenosis)
(3) an aorta that overrides the VSD
(4) right ventricular hypertrophy
• Most common cyanotic congenital heart disease
• Heart is often enlarged and may be "boot-shaped" due to
marked right ventricular hypertrophy, particularly of the
apical region.
• The VSD is usually large

15. • An ASD may be present – pentalogy of fallot
• If the pulmonary stenosis is mild, the abnormality
resembles an isolated VSD, and the shunt may be left-to-
right, without cyanosis (so-called pink tetralogy).
• As the obstruction increases in severity, there is greater
resistance to right ventricular outflow -> right-sided
pressures approach or exceed left-sided pressures -> right-
to-left shunt develops -> cyanosis (classic TOF).
• Most infants with TOF are cyanotic from birth or soon
thereafter

16. RIGHT TO LEFT
SHUNT TOF

18. TRANSPOSITION OF GREAT
ARTERIES TGA
• Aorta arises from Rt. Ventricle.
• Pulm. Artery arises from Lt. ventricle.
• Separation of systemic and pulmonary circulation
incomplete. Degree of mixing of blood.
• Immediate intervention required.
• Without VSD, with VSD

20. PERSISTENT TRUNCUS
ARTERIOSUS
• the arch that normally separates aorta from
pulmonary artery fails to develop.
• This results in a single large common vessel
receiving blood from the right as well as left
ventricle.
• often an associated VSD.

22. TRICUSPID ATRESIA &
STENOSIS
• Single functional ventricle , absence of tricuspid orifice
and, tricuspid ring is small and the valve cusps are
malformed
• Complete occlusion.
• Interatrial defect “R-L shunting”
• Cyanosis at birth.
• High mortality.

24. OBSTRUCTIVE CONGENITAL
ANOMOLY
• COARCTATION OF AORTA:
• Narrowing, constriction.
• More in male, more in Turners syndrome.
• 2 Forms: 1. “Infantile”/Preductal- 2. “Adult” /Postductal
• Symptoms depend on severity of narrowing.
• Associated with PDA, symptoms appear early.
• Without PDA, adulthood. Murmur. Hypertension in
upper limbs, low pressure in lower limbs- claudication,
cold limbs.

26. PULMONARY STENOSIS AND
ATRESIA
• Obstruction at Pulmyonar Valve. Mild to severe.
• Isolated defect or complex with Fallots
• Rt. Ventricular hypertrophy, obstruction to blood outflow
from Rt ventricle
• Cyanosis.

27. AORTIC STENOSIS AND ATRESIA
• Narrowing an obstruction of Aortic valve.
• 3 Types. 1. Valvular-aortic valve cusps are malformed
• and are irregularly thickened. The aortic valve may
• have one, two or three such maldeveloped cusps.
• Sub valvular - thickening under aortic V.
• Supra valvular-fibrous constriction above valve.

29. Infective Endocarditis
Infective endocarditis (IE) characterized by colonization or
invasion of the heart valves or mural endocardium by
microbe.
vegetations composed of thrombotic debris and
organisms, often associated with destruction of underlying
cardiac tissues.
Classified into acute and subacute forms.
Acute IE - infection of previously normal heart valve by
highly virulent organism that produces necrotizing,
ulcerative, destructive lesions.
Subacute IE - caused by organisms of lower virulence;
insidious infections of deformed valves that are less
destructive

30. Etiology
Bacterial – most common
1. Staph. aureus – acute IE, IV drug users
2. Strep Viridans – most common etiology of
subacute IE
3. Staph epidermidis – prosthetic valves
4. HACEK group

31. Morphology
The hallmark of IE is presence of friable, bulky, potentially
destructive vegetations containing fibrin, inflammatory
cells, and bacteria on heart valves.
The aortic and mitral valves are the most common sites of
infection, although the valves of the right heart may also be
involved, particularly in intravenous drug abusers
May form emboli -> septic infarct
Microscopically, the vegetations of typical subacute IE often
have granulation tissue indicative of healing at their bases.
With time, fibrosis, calcification, and a chronic
inflammatory infiltrate can develop.

32. B
Infective (bacterial)
endocarditis. A: Endocarditis
of mitral valve (subacute,
caused by Streptococcus
viridans). The large, friable
vegetations are denoted
by arrows.
B: Histologic appearance of
vegetation of endocarditis
with extensive acute
inflammatory cells and fibrin

33. Diagnostic criteria for IE – Duke’s Criteria
I. Pathologic criteria
• Microorganisms, demonstrated by culture or histologic
examination, in a vegetation, embolus from vegetation, or
intracardiac abscess
• Histologic confirmation of active endocarditis in vegetation
or intracardiac abscess
II. Clinical criteria
Major
• Blood culture(s) positive for a characteristic organism or
persistently positive for an unusual organism
• Echocardiographic identification of a valve-related or
implant-related mass or abscess, or partial separation of
artificial valve
• New valvular regurgitaion

34. Minor
• Predisposing heart lesion or intravenous drug use
• Fever
• Vascular lesions, including arterial petechiae,
subungual/splinter hemorrhages, emboli, septic infarcts,
mycotic aneurysm, intracranial hemorrhage, Janeway
lesions
• Immunological phenomena, including glomerulonephritis,
Osler nodes, Roth spots, rheumatoid factor
• Microbiologic evidence, including a single culture positive
for unusual organism
• Echocardiographic findings consistent with but not
diagnostic of endocarditis, including worsening or changing
of a preexistent murmur

35. • Diagnosis by these guidelines, called the Duke
Criteria, requires either pathologic or clinical
criteria; if clinical criteria are used, 2 major, 1
major + 3 minor, or 5 minor criteria are required
for diagnosis.

36. Comparison of the 4 major forms of vegetative endocarditis. RHD is marked by small,
warty vegetations along the lines of closure of the valve leaflets. Infective endocarditis
(IE) is characterized by large, irregular masses on the valve cusps that can extend onto
the chordae. Nonbacterial thrombotic endocarditis (NBTE) typically exhibits small,
bland vegetations, usually attached at the line of closure. One or many may be
present. Libman-Sacks endocarditis (LSE) has small or medium-sized vegetations on
either or both sides of the valve leaflets.

37. Rheumatic Heart Disease (RHD)
Acute Rheumatic Fever (RF)
Def: Acute Post-streptococcal systemic, immune-mediated,
inflammatory disease –affects tendons, joints, muscle, heart,
arteries, brain
Incidence:0.3 to 3 %.
Age: Children and young adults. 5-15 yrs
Sex: Female – more.
Socio-economic status: Poor , overcrowding.

38. • Etiology: Post streptococcal pharyngitis
• Beta Hemolytic streptococci Group A Type
• Mortality and morbidity improved with rapid diagnosis &
treatment

39. Pathophysiology
• Group A streptococcal pharyngitis
• Host immune response to group A streptococcal antigens
that cross-react with host proteins
• Abs , CD4 +T cells directed against M proteins recognise
cardiac Ags
• Complement activation & cytokine production
damage

41. Clinical features
Modified Jones criteria:
The Major diagnostic criteria include –
• Carditis,
•
• Polyarthritis,
• Chorea- Sydenhams
• Subcutaneous nodules-attached to deeper structures
• like tendons, ligaments, fascia or periosteum ; Characteristic
locations are extensor surfaces of the wrists, elbows, ankles ,
knees.
• Erythema marginatum.

42. The Minor diagnostic criteria include
• Fever,
• Arthralgia,
• Previous h/o RF
• Prolonged PR interval on the ECG
• Elevated acute phase reactants (increased
ESR), presence of C-reactive protein, and
leukocytosis.

43. Lab evidence of previous group A streptococcal
pharyngitis is must to diagnose rheumatic fever.
One of the following must be present:
• Positive throat culture or rapid streptococcal
antigen test
• Elevated or rising streptococcal antibody titer
• History of previous rheumatic fever or rheumatic
heart disease
The Jones criteria require the presence of 2 major or 1
major and 2 minor
+ Lab evidence

44. Morphology: Aschoff bodies
• Pathognomonic of RHD
• In interstitial tissue of myocardium , endocardium
• 3-4 weeks to develop.
• Consist T lymphocyte , plasma cells , & macrophages
• Anitschkow cell- cardiac histiocyte/ macrophage
• Cells with abundant cytoplasm , round ovoid nucleus
with slender wavy chromatin condensation(Cater pillar –
longitudinal section)

45. Anitschkow cell
Caterpillar

46. Anitschkow cell
Owl’s eye

47. Aschoff nodule

48. Subcutaneous nodule

49. Pathology
• ARF: diffuse inflammation & Aschoff bodies lead
to Pancarditis
• Early stage- Fibrinoid degeneration
• Intermediate stage- Proliferative
• Late stage- Healing
• Inflammation of Endocardium & Left side valves
lead to Fibrinoid necrosis

50. Fibrinous Pericarditis.

51. • Overlying necrotic foci and along lines of closure
, small vegetations / verrucae form
• Mac Callum plaques
• Mitral V cardinal changes: leaflet thickening ,
commisural fusion & shortening & thickening
fusion of tendinous cords

52. Valvular lesions
• Mitral stenosis: RF – common cause of Mitral
stenosis, 90%
• Pathology: Mitral valve leaflets, along lines of
closure margins. Diffuse thickening. Fibrosis, fish
mouth or button hole configuration.
• Mitral valves. Most affected
• Mitral + Aortic Valves
• Tricuspid , pulmonary V-rare

53. Small verrucous vegetations

54. Mitral stenosis

55. Mitral stenosis - Fish mouth
appearance

57. Diagnosis of RF, RHD
• Clinical
• Electrocardiogram and Imaging studies.
• Laboratory diagnosis:
• Throat swab.
• Culture.
• test for Streptococcal antigen
• ESR, TLC, CRP
• Anti Streptolysin - S and O Rising titer.
• Anti M antibodies.
• Histological findings

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