AGN/Nephrotic; RF vs RHD

GROUP-6
Members
1. Pawar Maheshwari
2. Haide E. Salcedo
3. Premkumar Aneesh
4. Shilpa Resmi Satheesh
5. Manikanta Ruddirala
6. Eirene Sacla
7. Andrei Rivera
ACUTE GLOMERULONEPHRITIS

PAWAR, MAHESHWARI
Overview
➔ Common in children and postinfectious complication of AGN
➔ Classic example of Acute Nephritic Syndrome with sudden onset
of:
◆ Hematuria
◆ Edema
◆ Hypertension
◆ Renal insufficiency
➔ Major cause of morbidity in group A β-hemolytic streptococcal
infections.
Etiology and Epidemiology
➔ Infection of throat or skin (Nephritogenic strains) of group A
β-hemolytic streptococci.
➔ 97% cases occur in less-developed countries
➔ Epidemics of nephritis have been described in association
with throat (Serotypes: M1,M4,M25 AND M12) and skin
(Serotype 49) infections.
➔ Mostly sporadic
Pathology and Pathogenesis
➔ Glomeruli appears enlarged and bloodless showing diffuse
mesangial cell proliferation
➔ Crescents and interstitial inflammation in severe cases
➔ Immunofluorescence microscopy reveals pattern of
“Lumpy-Bumpy” deposits of immunoglobulin and
mesangium.
➔ “Humps” on epithelial side of glomerular basement
membrane can be seen on electron microscopy.
Clinical Manifestations

➔ Most common in 5-12 years old children

➔ Uncommon in 3 years old
➔ Typically, Acute Nephritic syndrome occurs after:
◆ 1-2 weeks in antecedent streptococcal pharyngitis
◆ 3-6 weeks after streptococcal pyoderma
➔ History is not specific sometimes due to mild or early resolved
symptoms without medical treatment.
➔ Severity of kidney involvement varies from asymptomatic
microscopic hematuria with normal renal function to gross
hematuria with acute renal failure.
Clinical Manifestations

➔ Also, It can develop various degrees of edema, hypertension and

oliguria in child
➔ Risk for encephalopathy and heart failure secondary to
hypertension (shows symptoms like blurred vision, severe HA,
altered mental status or seizures) or hypervolemia.
➔ Respiratory distress following orthopnea, cough leading to
pulmonary edema and heart failure.
➔ Peripheral edema from salt and water retention.
Clinical Manifestations

➔ Non-specific symptoms:
◆ Malaise
◆ Lethargy
◆ Abdominal pain
◆ Flank pain
➔ Acute phase resolves: within 6-8 weeks
➔ Urinary retention normalizes: 4-6 weeks
➔ Persistent microscopic hematuria can persist upto 1-2 years after
initial presentation.
Diagnostics
➔ Urinalysis
➔ Confirmation of dx requires clear evidence of a prior streptococcal
infection
➔ Throat culture
➔ BEst antibody titer to document cutaneous streptococcal infection:
Antideoxyribonuclease B level
➔ Serologic evidence
➔ MRI
➔ Chest X-ray
Treatment
➔ Treating acute effects of renal insufficiency:
◆ 10 days course of systemic antibiotics therapy with penicillin
◆ Sodium restriction, diuresis (usually with IV furosemide)
➔ Pharmacotherapy for hypertension (gold standard):
◆ calcium channel antagonists
◆ Vasodilators
◆ Angiotensin converting enzyme inhibitors.
PREVENTION
➔ Early systemic antibiotics therapy for ➔
skin and throat infections does not
limit risk for AGN.
➔ Family members (young children) : ➔
considered at high risk for infection
➔ Family pets are also reported as
carriers.
PROGNOSIS
Complete recovery occurs in >95% of
children with AGN
➔ Recurrence are extremely rare
Mortality in acute phase can be
avoided by appropriate management
of acute renal failure, cardiac failure
and hypertension
➔ Acute phase is severe and leads to
Glomerulosclerosis and chronic renal
disease in <2% of affected children.
NEPHROTIC SYNDROME

Premkumar Aneesh

Shilpa Resmi Satheesh

Manikanta Ruddirala
 NEPHROTIC SYNDROME
➔ Clinical manifestations of glomerular disease associated with heavy
(nephrotic-range) proteinuria.
➔ Clinical triad of nephrotic syndrome:
◆ Large urinary loss of protein (Hypoalbuminemia ≤ 2.5% g/dL)
◆ Edema
◆ Hyperlipidemia (Cholesterol >200 mg/dL)
➔ 1-3 per 100,000 children <16 years of age mostly from infection
➔ 80% of children responds to corticosteroids therapy.
➔ Standard therapy- Glucocorticosteroid therapy
➔ Early referral to a pediatric nephrologist is recommended for initial
management of nephrotic syndrome.
1). IDIOPATHIC NEPHROTIC SYNDROME
● 90% children
● associated with primary glomerular
disease without an identifiable causative
disease or drug.
● Histologic types:
■ Minimal Change Disease,
■ Mesangial Proliferation,
■ Focal Segmental
Glomerulosclerosis,
■ Membranous Nephropathy,
■ Membranoproliferative
Glomerulonephritis
Pathology:
Minimal Change Disease
○ 85% of total cases of nephrotic
syndrome in children
○ Glomeruli-minimal increase in
mesangial cells and matrix.
○ Immunofluorescence microscopy
are typically negative,
○ Electron microscopy simply
reveals effacement of the
epithelial cell foot processes
○ Corticosteroid therapy respond
to 95% Children
1). IDIOPATHIC NEPHROTIC SYNDROME
Pathology:
Mesangial Proliferation
● Increase in mesangial cells and matrix on
light microscopy.
● Immunofluorescence microscopy reveal
trace to 1+ mesangial IgM and/or IgA
staining.
● Electron microscopy reveals increased
numbers of mesangial cells and matrix as
well as effacement of the epithelial cell
foot processes.
● 50% of patients respond to corticosteroid
therapy.
Pathology:
Focal Segmental Glomerulosclerosis (FSGS)
1. Focal (present only in a proportion of glomeruli)
2. Segmental (localized to ≥1 intraglomerular tufts).
Immunofluorescence microscopy- positive for IgM
and C3 staining in areas of segmental sclerosis.
Electron microscopy- segmental scarring of the
glomerular tuft with obliteration of the glomerular
capillary lumen.
20% of patients with FSGS respond to prednisone.
Involving all glomeruli, and leads to end-stage renal
disease in most patients.
MINIMAL CHANGE NEPHROTIC SYNDROME
Clinical Manifestation
MCNS:
● More common in boys than in girls (2 : 1)
● Appears between the ages of 2 & 6 yr
● Early as 6 mo of age and throughout adulthood.
● Childhood: 85-90% ( <6 yr of age).
● Adolescents: 20-30%
The more common cause of idiopathic nephrotic syndrome in this older age group is
FSGS.
Incidence of FSGS increasing;
● More common in African-American, Hispanic, and Asian patients.
 Mild edema, noted around the eyes and in
the lower extremities.
Clinical Manifestation of MCNS
Misdiagnosed as an allergic disorder
because of the periorbital swelling that
decreases throughout the day.

With time, the edema becomes generalized,

with the development of;
● Ascites,
● Pleural Effusions,
● Genital Edema

Common Signs & Symptoms are;

● Anorexia,
● Irritability,
● Abdominal Pain,
● Diarrhea

***Important features of MCNS -absence of hypertension and gross

hematuria (the so-called nephritic features).
Differential Diagnosis

With marked edema includes;

● protein-losing enteropathy,
● hepatic failure,
● heart failure,
● acute or chronic
● glomerulonephritis,
● protein malnutrition.

A diagnosis other than MCNS should be considered;

● children <1 yr of age,
● with a positive family history of nephrotic syndrome,
● presence of extrarenal findings (e.g., arthritis, rash, anemia), hypertension or
pulmonary edema, acute or chronic renal insufficiency, and gross hematuria
 Diagnosis Recommendations for the Initial Evaluation of Children
with Nephrotic Syndrome Confirming the Diagnosis of Nephrotic Syndrome.

urinalysis with first morning urine protein :

● creatinine ratio and serum electrolytes, blood urea nitrogen, creatinine, albumin, and cholesterol levels;

Evaluation to rule out secondary forms of nephrotic syndrome (children ≥10 yr):
● complement C3 level, antinuclear antibody, double-stranded DNA and hepatitides B and C, and HIV in
high-risk populations; and kidney biopsy (for children ≥12 yr, who are less likely to have MCNS).

The urinalysis reveals 3+ or 4+ proteinuria, and microscopic hematuria is present in 20% of children.

A spot urine protein : creatinine ratio should be >2.0. The serum creatinine value is usually normal, but it may
be abnormally elevated if there is diminished renal perfusion from contraction of the intravascular volume.

The serum albumin level is <2.5 g/dL, and serum cholesterol and triglyceride levels are elevated.

Serum complement levels are normal. A renal biopsy is not routinely performed if the patient fits the standard
clinical picture of MCNS.
TREATMENT OF MCNS
● Mild to moderate edema- outpatients.
● The child’s parents must be able to recognize the signs and symptoms of the complications of
the disease and may be taught how to use a dipstick and interpret the results to monitor for
the degree of proteinuria.
● Children with onset of uncomplicated nephrotic syndrome between 1 and 8 yr of age are likely
to have steroid-responsive MCNS, and steroid therapy may be initiated without a diagnostic
renal biopsy.
● Children with features that make MCNS less likely (gross hematuria, hypertension, renal
insufficiency, hypocomplementemia, or age <1 yr or >12 yr) should be considered for renal
biopsy before treatment.
TREATMENT OF MCNS
● Use of Corticosteroids to Treat Minimal Change Nephrotic Syndrome
● Treatment of Initial Episode of Nephrotic Syndrome

Drug Dosage Period of time

Prednisone 60 mg/m2/day or 2 mg/kg/day 4-6 wk

Alternate-day prednisone 40 mg/m2 qod or 1.5 mg/kg 8 wk to 5 mo

qod)

● Steroid therapy
○ 80-90% of children respond
○ Response is defined as the attainment of remission within the initial 4 wk of
corticosteroid therapy.
○ Remission consists of a urine protein : creatinine ratio of <0.2 or <1+ protein on urine
dipstick for 3 consecutive days. The vast majority of children who respond to prednisone
therapy do so within the first 5 wk of treatment.
Managing the Clinical Sequelae of Nephrotic Syndrome
1. Edema:
● severe symptomatic edema, including;
○ large pleural effusions,
○ ascites, or severe genital edema,
● water/fluid restriction may be necessary if the child is hyponatremic.
● swollen scrotum may be elevated with pillows to enhance fluid removal by gravity
● Diuresis- loop diuretics(furosemide, orally/intravenously)
● severe generalized edema with evidence of;
○ intravascular volume depletion (e.g., hemoconcentration,hypotension, tachycardia),
○ IV administration of 25% albumin (0.5-1.0 galbumin/kg) as a slow infusion followed
by furosemide (1-2 mg/kg/ dose IV) is sometimes necessary.
○ Mandates close monitoring of volume status, blood pressure, serum electrolyte
balance, and renal function.
2. Dyslipidemia:

● Managed with a low-fat diet.

○ Dietary fat intake <30% of calories with saturated fat intake <10% calories.
○ Dietary cholesterol intake be <300 mg/day.
● Recommend the use of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors
routinely in children with dyslipidemia.

3. Infections:

● Families of children with nephrotic syndrome should be counseled regarding the signs and
symptoms of infections such as cellulitis, peritonitis, and bacteremia.
● Blood culture- empiric antibiotic therapy.
● In the case of spontaneous bacterial peritonitis, peritoneal fluid should be collected if there is
sufficient fluid to perform a paracentesis and sent for cell count, Gram stain, and culture.
● A 3rd-generation cephalosporin is a common choice of IV antibiotic.
4. Thromboembolism.

● Evaluated by appropriate imaging studies to confirm the presence of a clot.

● Anticoagulation therapy in children with thrombotic events appears to be effective heparin,
low-molecular-weight heparin, and warfarin are therapeutic options.

5. Obesity and Growth.

● OVERWEIGHT-Glucocorticoids may increase the body mass index in children who are overweight when
steroid therapy is initiated
● Anticipatory dietary counseling is recommended.
● Growth affected in children who require long-term corticosteroid therapy.

6. Relapse of Nephrotic Syndrome.

● Relapse of nephrotic syndrome is defined as a urine protein : creatinine ratio of >2 or ≥3+ protein on urine
dipstick testing for 3 consecutive days.
● Triggered by upper respiratory or gastrointestinal infections.
● Daily high-dose prednisone is given until the child has achieved remission, and the regimen is then
switched to alternate-day therapy.
● The duration of alternate day therapy varies depending on the frequency of relapses of the individual
child.
7. Steroid Resistance: Defined as the failure to achieve remission after 8 wk of corticosteroid therapy.

Further evaluation, including;

● diagnostic kidney biopsy,

● evaluation of kidney function,
● quantitation of urine protein excretion (in addition to urine dipstick testing).

Alternative Therapies to Corticosteroids in the Treatment of Nephrotic Syndrome.

● Cyclophosphamide- (2 mg/kg) is given as a single oral dose for a total duration of 8-12 wk.
● Alternate-day prednisone therapy
● Calcineurin inhibitors (cyclosporine or tacrolimus)
○ Mycophenolate can maintain remission in children with steroid-dependent or frequently
relapsing nephrotic syndrome.
○ Levamisole, an antihelmintic agent with immunomodulating effects that has been shown to
reduce the risk of relapse in comparison to prednisone.
○ Rituximab, the chimeric monoclonal antibody against CD20, in children with steroid-dependent
and/or steroid-resistant nephrotic syndrome.

Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers may be helpful as adjunct
therapy to reduce proteinuria in steroid-resistant patients.
2). SECONDARY NEPHROTIC SYNDROME
❏ Nephrotic syndrome can occur as a secondary feature of many forms of
glomerular disease.
❏ the syndrome develops because of other causes, such as diseases that
affect other parts of the body, infections, and medicines
❏ Secondary nephrotic syndrome should be suspected in patients > 8 yr and
those with hypertension, hematuria, renal dysfunction, extrarenal
symptoms (e.g., rash, arthralgias, fever), or depressed serum complement
levels.
CAUSES
3. CONGENITAL NEPHROTIC SYNDROME
● Congenital nephrotic syndrome is defined as nephrotic syndrome
manifesting at birth or within the 1st 3 mo of life.
● Congenital nephrotic syndrome may be classified as primary or as
secondary to a number of etiologies such as in utero infections
(cytomegalovirus, toxoplasmosis, syphilis, hepatitides B and C, HIV),
infantile systemic lupus erythematosus, or mercury exposure.
● Primary congenital nephrotic syndrome is due to a variety of
syndromes inherited as autosomal recessive disorders.
● A number of structural and functional abnormalities of the glomerular
filtration barrier causing congenital nephrotic syndrome have been
elucidated
● children with congenital nephrotic syndrome, 85% carried disease-causing
mutations in 4 genes (NPHS1, NPHS2, WT1, and LAMB2), the first 3 of
which encode components of the glomerular filtration barrier.
● The Finnish type of congenital nephrotic
syndrome is caused by mutations in the
NPHS1or NPHS2 gene, which encodes nephrin
and podocin, critical components of the slit
diaphragm.
● Denys-Drash syndrome is caused by mutations
in the WT1 gene,which results in abnormal
podocyte function.
● Mutations in the LAMB2 gene, seen in Pierson
syndrome, lead to abnormalities of β2-laminin,
a critical component of glomerular and ocular
basement membranes.
● Secondary congenital nephrotic syndrome
can resolve with treatment of the underlying
cause, such as syphilis
MANAGEMENT
● The management of primary congenital nephrotic syndrome includes
intensive supportive care with intravenous albumin and diuretics, regular
administration of intravenous γ-globulin, and aggressive nutritional support
(often parenteral)
● while attempting to pharmacologically decrease urinary protein loss with
angiotensin-converting enzyme Inhibitors, angiotensin II receptor inhibitors,
and prostaglandin synthesis inhibitors, or even unilateral nephrectomy.
● If conservative management fails and patients suffer from persistent
anasarca or repeated severe infections, bilateral nephrectomies are
performed and chronic dialysis is initiated.
● Renal transplantation is the definitive treatment of congenital nephrotic
syndrome,
RHEUMATIC FEVER

Haide E. Salcedo
Etiology and Epidemiology
● Annual Incidence: exceeds 50 per 100,000 children.
● Very high rates are seen in ethnic minority populations within Australia and New
Zealand.
● Most common in children 6-15 years of age.
● It is due to an immunological reaction that is a delayed sequelae of group A
beta-hemolytic streptococcal infections of the pharynx.
● A family history of rheumatic fever and lower socioeconomic status are additional
factors.
Pathogenesis
● Cytotoxicity theory: GAS toxin
○ GAS produces an enzymes such as streptolysin: has a direct cytotoxic effect on
mammalian cells in tissue culture.
○ Major problem: inability to explain the substantial latent period (usually 10-21 days)
between GAS pharyngitis and onset of acute RF.
● Immune-mediated pathogenesis
● Pathogenetic hypothesis: antibody response to collagen
Clinical Manifestations and Diagnosis
Clinical Manifestations and Diagnosis
1. Initial attack : 2 major manifestations, or 1 major and 2 minor manifestations, plus
evidence of recent GAS infection. Recurrent attack : 2 major, or 1 major and 2 minor, or 3
minor manifestations (the latter only in the Moderate/High-Risk population), plus evidence of
recent GAS infection.

2. Low-Risk population is defined as acute rheumatic fever (ARF) incidence <2 per 100,000
school-age children per year, or all-age rheumatic heart disease (RHD) prevalence of <1 per
1,000 population. Moderate/High-Risk population is defined as ARF incidence >2 per
100,000 school-age children per year, or all-age RHD prevalence of >1 per 1,000 population.
The 5 Major Criteria
● Migratory Polyarthritis
○ Approximately 75% of patients
○ Typically involves larger joints, particularly the knees, ankles, wrists, and elbows.
● Carditis
○ Subclinical carditis: defined as without a murmur of valvulitis but with
echocardiographic evidence of valvulitis.
○ Clinical carditis: with a valvulitis murmur
● Chorea
○ Approximately 10-15% of patients
○ Usually presents as an isolated, frequently subtle, movement disorder.
○ Emotional lability, incoordination, poor school performance, uncontrollable movements,
and facial grimacing are characteristic, all exacerbated by stress and disappearing with
sleep.
The 5 Major Criteria
● Erythema Marginatum
○ Approximately 1% of patients
○ It consists of erythematous, serpiginous, macular lesions with
pale centers that are not pruritic.
○ It occurs primarily on the trunk and extremities.
● Subcutaneous Nodules
○ Rare (≤1% of patients with acute RF)
○ Consist of firm nodules approximately 0.5-1 cm in diameter
along the extensor surfaces of tendons near bony prominences.
Minor Criteria
● 2 clinical minor criteria
○ 1st: defined as polyarthralgia in Low-Risk populations and monoarthralgia in
Moderate/High-Risk populations.
○ 2nd: defined as at least 38.5°C in Low-Risk populations and at least 38.0°C in
Moderate/High-Risk populations.
● 2 laboratory minor criteria:
○ (1) elevated acute-phase reactants, defined as erythrocyte sedimentation rate (ESR)
at least 60 mm/hr and/or C-reactive protein (CRP) at least 3.0 mg/dL (30 mg/L) in
Low-Risk populations, and ESR at least 30 mm/hr and/or CRP at least 3.0 mg/dL (30
mg/L) in Moderate/High-Risk populations.
○ (2) prolonged P-R interval on ECG (unless carditis is a major criterion).
Treatment
● Antibiotic Therapy
○ Penicillin or Amoxicillin: administered orally > 10 days
○ Benzathine penicillin: single intramuscular injection
○ If penicillin allergic: 10 days of erythromycin, 5 days of azithromycin, or 10 days of
clindamycin
● Anti Inflammatory Therapy
○ Anti inflammatory agents : e.g., salicylates, corticosteroids
○ Acetaminophen: control pain and fever
Treatment
● The usual dose of aspirin is 50-70 mg/kg/day in 4 divided doses orally for 3-5 days >
followed by 50 mg/kg/day in 4 divided doses PO for 2-3 wk and half that dose for another
2-4 wk.
● The usual dose of prednisone is 2 mg/kg/day in 4 divided doses for 2-3 wk, followed by
half the dose for 2-3 wk and then tapering of the dose by 5 mg/24 hr every 2-3 days.
○ When prednisone is being tapered, aspirin should be started at 50 mg/kg/day in 4
divided doses for 6 wk to prevent rebound of inflammation.
RHEUMATIC HEART DISEASE

Eirene Sacla
Introduction
● Rheumatic involvement of the valve is the most important sequelae of
acute rheumatic fever
● The mitral valve is the most affected followed by the aortic valve
● Repeated episodes of rheumatic fever results in rheumatic heart disease
● The valvular lesions begin as small verrucae composed of fibrin and blood
cells along the borders of one or more of the heart valves
Patterns of Valvular Disease
MITRAL INSUFFICIENCY

Pathophysiology

-The result of structural changes that usually include some loss of valvular substance and shortening
and thickening of chordae tendineae

● Regurgitation of blood into LA during systole

● LA dilatation and hypertrophy
● Increased left atrial pressure-> pulmonary congestion and symptoms of left-sided heart failure
● In chronic mitral insufficiency, pulmonary atrial pressure becomes elevated-> RV and atrial
pressure becomes elevated->enlargement
● Right sided heart failure
Patterns of valvular disease
Clinical Manifestation

Mild

-Signs of heart failure is not present

-The precordium is quiet, and auscultation reveals a high pitched holosystolic

murmur at the apex that radiates to the axilla

-The ECG and chest X-ray are normal

Patterns of valvular disease
Clinical Manifestation

Severe

-The heart is enlarged, with a heaving apical left ventricular impulse and often an apical systolic thrill

-The 2nd heart sound may be accentuated if pulmonary hypertension is present

-A third heart sound is prominent

-A holosystolic murmur is heard at the apex with radiation to the axilla

-A short mid diastolic rumbling murmur is caused by increased BF across the mitral valve as a result of the insufficiency

-ECG shows prominent bifid P waves, signs of left ventricular hypertrophy if pulmonary hypertension is present

-ECG shows enlargement of the LA and ventricle, abnormally thickened mitral valve

-Doppler studies demonstrate severity of the mitral regurgitation

Patterns of valvular disease
Complications

-Severe may result in cardiac failure that may be precipitated by progression of rheumatic process

Right ventricular failure and atrial and ventricular arrhythmias

Treatment

-Prophylaxis against recurrences of RF

-Afterload-reducing agents (ACE inhibitors or angiotensin receptor blockers)--> reduce regurgitant

volume and preserve LV function

-Surgical-persistent heart failure, dyspnea with moderate activity, and progressive cardiomegaly,
often with pulmonary hypertension
Patterns of valvular disease
Mitral Stenosis
Pathophysiology
● Results from fibrosis of the mitral ring, commissural adhesions, and
contracture of the valve leaflets, chordae and papillary muscles
● Increased pressure and hypertrophy of the LA, pulmonary venous
hypertension, increased pulmonary vascular resistance, and pulmonary
hypertension
● RV hypertrophy and RA dilatation then followed by RV dilatation, tricuspid
regurgitation→ Right sided heart failure
Patterns of valvular disease
Clinical Manifestation

● Mild-asymptomatic

-ECG and roentgenograms- normal

● More severe-Exercise intolerance and dyspnea

-Prominent and notched P waves and varying degrees of RV hypertrophy is evident

-Roentgenographic signs of left atrial enlargement and prominence of pulmonary

artery and right sided heart chambers; calcification is noted in the region of the mitral valve

-ECG shows thickening of the mitral valve

● Critical lesions-orthopnea, PND, overt pulmonary edema and atrial arrhythmias

Patterns of valvular disease
● Treatment

-Surgical valvotomy or balloon catheter mitral valvuloplasty

-Valve replacement is avoided unless absolutely necessary

Patterns of valvular disease
Aortic Insufficiency

● Sclerosis of the aortic valve results in distortion and retraction of the cusps
● Regurgitation of blood leads to volume overload with dilation and hypertrophy of the left
ventricle

Clinical manifestation

-Palpitation

-Sweating and heat intolerance-excessive vasodilation

-Dyspnea→orthopnea and pulmonary edema

-Angina during heavy exercise

Patterns of valvular disease
Clinical manifestation

-Pulse pressure is wide with bounding peripheral pulse

-SBP is elevated;DBP is lowered

-Heart is enlarged, with left ventricular apical heave

-Diastolic thrill

-Typical murmur sound begins with 2nd heart sound and continuous until late in diastole; upper and midleft sternal border with radiation to
the apex and upper right sternal border

-Austin flint murmur

-Chest X-ray→ enlargement of the left ventricle and aorta

-ECG→ left ventricular hypertrophy and strain with prominent P mitral valve flutter or oscillation caused by regurgitant flow hitting the valve
leaflet

-Doppler studies aortic run-off into the left ventricle

Patterns of valvular disease
Treatment

-Afterload reducers and prophylaxis against recurrence of ARF

-Surgical intervention (valve replacement)-advance of the onset

-Surgery-early symptom are present

-ST-T wave changes are seen in ECG

-Evidence of decreasing LV ejection fraction

Patterns of valvular disease
Tricuspid Valve Disease

-Rare after RF

-Secondary to RV dilatation from unrepaired left-sided lesions

-Signs: prominent pulsations of the jugular veins, systolic pulsation of the liver,
and a blowing holosystolic murmur at the lower left sternal border that
increases during inspiration

-Tricuspid valvuloplasty may be required in rare cases

Patterns of valvular disease
Pulmonary Valve Disease

-Occur on functional basis secondary to pulmonary hypertension

-Graham Steell murmur-similar to aortic insufficiency, but peripheral arterial

signs are absent

-Diagnosis: confirmed by two-dimensional ECG and doppler studies

RF vs RHD

Andrei Rivera
RF vs RHD
REFERENCE

Md, R. K. M., & Geme, J. S. W., III MD. (2019). Nelson Textbook of Pediatrics, 2-Volume Set
(Nelson Pediatrics) (20th ed.). Elsevier.
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