An interactive case where we discuss the diagnosis and management of Acute Rheumatic Fever, Rheumatic Heart Disease and Heart Failure in general. Presented at Saint Paul's Hospital Millennium Medical College (SPHMMC), Addis Ababa, Ethiopia

1. Interactive Cases in Clinical
Medicine
AN SPHMMC PRODUCTION

2. Episode 01
P R E S E N T E D B Y
AHMED I

3. The Setting
 You are a recently graduated General
Practitioner of Saint Paul’s Hospital
Millennium Medical College who, as part
of the newly amended 5 year mandatory
public service, are enthusiastically
working at the newly renovated Modjo
Zonal Hospital *sarcasm*

4.  It’s a very hot Friday morning and you are in the Poly Clinic attending
to OPD patients when in walk Mother and Child
 You notice the young child (whom you guess to be no older than 10)
looks unwell
 She was protectively holding her right arm and winced in pain when
the mother touched it while trying to lead her into the OPD
 After greeting the mother, she narrates the following story to you

5. The
Patient
Alem Gezahegn
8 year old child
Parents are
• Gezahegn Birhanu (Farmer) and
• Tsehay Abebayehu (Housewife)
Monthly income is 500 birr
Live on the outskirts of Modjo Town
House is a 2 room-ed mud hut with only
one window and poor lighting
A total of 9 people + 2 pets live together

6. And this is what
happened….
• 4 weeks back, Alem started complaining of pain in her
right knee that progressively increased in severity in the
following 2 days
◦ Knee was hot, swollen and painful when touched
• That pain resolved itself in the subsequent week
• But then she experienced similar pain in her left hip
followed by her right elbow
• During this time, she also developed Fever which her
mother described as persistent and high grade

7. • What pertinent questions would you
like to ask Alem or her mother?

8. Other pertinent in her
history
• Recalls having soar throat in the weeks preceding
her illness
• Mother denies
◦ Pain in other area
◦ Recent trauma
◦ Previous episode of similar illness in the past
◦ Family Member with Similar Illness
◦ Other systemic complaints on review of systems

9. On physical
examination, which
body systems would
you focus on?

10. Physical Examination is
significant for
• Vital signs are
◦ temperature 39.3°C,
◦ pulse 120 bpm,
◦ respirations 12/minute,
◦ blood pressure 110/70, and oxygen saturation, 95%.
• three firm, symmetric, and painless
subcutaneous nodules over the olecranon
processes of both arms

11. • Red, swollen and tender right elbow
• erythematous, nonpruritic plaques
with a pale center on the trunk

12. When auscultating the
heart….
• You hear the following
• 2/6 blowing holosystolic murmur that is
heard best at the apex and radiates to the
axilla

13. In Summery
8 year old female patient presents with
◦ 4 week history of migrating joint pain and
◦ associated high grade fever
◦ History of soar throat 2 weeks preceding symptoms
Physical exam remarkable for
◦ temperature 39.3°C, pulse 120 bpm, respirations 12/minute, blood
pressure 110/70, and oxygen saturation, 95%.
◦ three firm, symmetric, and painless subcutaneous nodules over the
olecranon processes
◦ erythematous, nonpruritic plaques with a pale center on the trunk
◦ Red, swollen and tender right elbow
◦ 2/6 blowing holosystolic murmur that is heard best at the apex and
radiates to the axilla

14. • After returning to your seat, what
differential diagnosis go thru your mind?

15. Diseases with Fever and
Joint Pain
ARF
Infectious Arthritis
Reactive and Post-infectious arthritis
Infective Endocarditis, Viral Myocarditis/Pericarditis
Other AFI’s
◦ Malaria, Typhoid/Typhus, Relapsing Fever
Systemic rheumatologic diseases (SLE, RA)
Trauma
Tumor – local or systemic

16. ARF
• delayed, nonsuppurative sequela of
a pharyngeal infection with the
group A streptococcus (GAS).

17. Epidemiology
• ARF and RHD are diseases of economy
• “You show me a country with a per-capital
income above 12000 dollars a year, I’l show you
a country with no Mitral Stenosis”
• Dr Conrad Fisher

18. • ARF is mainly a disease of children aged
5–14 years and RHD peaks between 25
and 40 years.
• There is no clear gender association for
ARF, but RHD more commonly affects
females

19. 3%
97%
Susceptible to ARF Not Susceptible to ARF
60%
40%
Will Subsequently Develop RHD
Will Subsequently Not Develop RHD
Susceptibility and Sequelea

20. Clinical Features
• preceding GAS infection is commonly subclinical
• latent period of ~3 weeks
• most common clinical presentation of ARF is
polyarthritis and fever.
◦ Polyarthritis 60–75%
◦ Carditis 50–60%.
◦ Chorea <2% to 30%.
◦ Erythema marginatum and subcutaneous nodules <5%

21. • What further tests would you order to
confirm your diagnosis?

22. Lab Ix
CBC
ESR, CRP
Blood Film
Blood Culture
Throat Swab for Culture, ASO Titer or Rapid Antigen Tests
Chest X-Ray, Elbow X-ray
Echo, ECG
Autoimmune Markers
Joint Aspirate

23. Lab Results Come Back As
Follows
• CBC – Mild Leukocytosis
• ESR and CRP elevated 4x
• No Hemoparasites on Blood Film
• Blood Culture did not grow any organisms
• Throat Swab for Culture and ASO Titer were not available
• Rapid Antigen Test was positive for GAS
• Chest X-Ray was normal
• Echo shows mild mitral regurgitation
• ECG is normal

24. • Based on the history, physical examination
and lab results, what is the working
diagnosis?

25. Diagnosis
J ♥ N E S
Criteria

26. ♥-eart Involvement
• PAN-carditis
• Valvular damage is the hallmark of rheumatic carditis with Aschoff
Nodule
• early-regurgitation, late-stenosis
• Manifestaitons
• Sinus Tachy
• Mitral/Aortic Regurg Murmer
• Pericardial Friction Rub
• CHF, S3 Gallop
• Cardiomegally
• Prolonged PR
• ECG of pericarditis

27. Erythema Marginatum

28. Sydenham’s Chorea

29. Minor Features
• Fever – High Grade (>39o)
• Polyarthralgia
• CRP & ESR - dramatically elevated
• Leukocyte Count – elevated
• ECG – Prolonged PR
• Evidence of a Preceding GAS Infection
◦ swab culture, rapid antigen test or serologic test

30. World Health Organization Criteria for the Diagnosis of Rheumatic Fever and Rheumatic Heart Disease (Based on the 1992
Revised Jones Criteria)
Primary episode of rheumatic fever Two major or one major and two minor manifestations plus
evidence of preceding group A streptococcal infection
Recurrent attack of rheumatic fever in a patient without
established rheumatic heart disease
Two major or one major and two minor manifestations plus
evidence of preceding group A streptococcal infection
Recurrent attack of rheumatic
fever in a patient with
established rheumatic heart
diseas
Two minor manifestations plus
evidence of preceding group A
streptococcal infection
Rheumatic chorea Insidious onset rheumatic carditis Other major manifestations or evidence of group A
streptococcal infection not required
Chronic valve lesions of rheumatic
heart disease (patients presenting
for the first time with pure mitral
stenosis or mixed mitral valve
disease and/or aortic valve disease)
Do not require any other criteria to
be diagnosed as having rheumatic
heart disease

31. What is the next step in management?

32. Mx
• There is no treatment for ARF that has been
proven to alter the likelihood of developing, or
the severity of, RHD
• Principles
• antibiotic therapy,
• anti-inflammatory therapy

33. • ANTIBIOTICS
◦ Penicillin PO or IM, Erythromycin, Azithromycin
• Salicylates and NSAIDs
◦ Asprin
◦ Only given once diagnosis is confirmed
• Glucocorticoids & IVIg
• Haloperidol, Carbamazepine or Sodium Valproate

34. • After explaining to W/ro Tsehay about her child’s
illness, you admit Alem to the Ward and start her on
◦ Oral Penicillin V, 250mg TID for 10 days
◦ Aspirin, 100 mg/kg/day in 4 divided doses PO for 3–5 days, followed
by 75 mg/kg/day in 4 divided doses PO for 4 wk
◦ Prednison, 2 mg/kg/day in 4 divided doses for 2–3 wk followed by a
tapering of the dose that reduces the dose by 5 mg/24 hr every 2–3
days

35. • The subsequent day while on morning
rounds, you find Alem in a worse state
• Her mother tells you that after admission, Alem
progressively developed labored breathing and
fatigue
• She was unable to lay in her bed and her mother
had to prop her up with 2 pillows

36. • What other pertinent questions would you ask
next?

37. • Alem tells you she feels her heart pounding
• She denies PND but states she did not sleep the whole night
• She denies fever
• She denies abdominal pain, jaundice
• She went to the toilet once during the night and denies
dysuria, oliguria, hematuria

38. On physical examination…
• Vitals
◦ BP is 120/90, RR is 60, PR is 130, Temp is 37.2o
• Respiratory
◦ Flaring of ala nasea and use of accessory muscles
◦ There are scattered rales and wheezes on lung exam
• CVS
◦ There is 9 cm of JVD
◦ She has marked peripheral edema
◦ In addition to previous findings, auscultation now reviles
S3 and S4

39. You order an urgent chest x-ray

40. Why do you think Alem is deteriorating?
What Mx should be given?

41. Mx of Acute Heart Failure
IV Diuretics
IV Nitrates
Ionotropes
Vasoconstrictors

43. Just to digress…….
• What are the 4 most common causes of
JVD>6cm?
Cardiac Tamponade,
Constrictive Pericarditis,
CHF (biventricular or isolated right heart failure), and
superior vena cava syndrome

44. • You put Alem on
◦ supplemental O2 by facemask and
◦ IV furosemide (lasix) 40mg bolus, which is repeated 2 more times
during the day
• By nightfall, Alem’s dyspnea and physical signs of fluid
collection have resolved
• You switch her over to PO furosemide 20mg/day while
monitoring her with input/output and daily weight
measurments

45. • 2 weeks later, you are on your morning rounds and make
your way to Alem
• You find all of Alem’s symptoms have resolved and she is
back to being the playful child she use to be
• You decide she no longer requires in-patient treatment
and tell mother and child they will be returning home in
the afternoon

46. • Before discharge, what further instructions are
you going to give W/o Tsehay about her
daughter?

47. Prevention
1o
◦ timely and complete treatment of GAS soar throat
2o
◦ long-term penicillin prophylaxis to prevent recurrences
◦ benzathine penicillin G delivered every 4 weeks
◦ Sulfonaminde or Erythromycin

48. Duration
Rheumatic fever with carditis and
residual heart disease
(persistent valvular disease*)
10 years or until 40 years of age
(whichever is longer);
sometimes lifelong prophylaxis
Rheumatic fever with carditis but no
residual heart disease
(no valvular disease*)
10 years or until 21 years of age
(whichever is longer)
Rheumatic fever without carditis
5 years or until 21 years of age
(whichever is longer)

49. • W/o Tsehay tells you she understands your
instructions and promises to bring her child
every month for a penicillin shot
• True to her word, W/o Tsehay brings little Alem
to the Health Center every month for the
subsequent 7 mo

50. • However, she fails to show up on her 8th appointment
• After waiting a week, you get worried and decide to visit
the family home to find out why she has not shown up
• You arrive at the family home and find it abandoned. You
ask the neighbors and are told the father, Ato Gezahegn
had found work in a factory in Hawassa and thus had
moved the whole family with him

51. • Suddenly, a frightening realization comes to you
• W/ro Tsehay may not take Alem to a health
facility to get her monthly shot any more
• *que sound effects in the back ground*

52. A few years down the
road…….

53. The story continues
• After completing your compulsory 5 year public
service, you return to (the now) Saint Paul’s
Hospital Millenium Medical University to start a
3 year residency in Internal Medicine followed
by a 2 year Cardiology fellowship, from which
you graduate with honors
• Upon graduation, you are hired at SPHMMU as
Consultant Cardiologist

54. • A year later, you are
in your office when
paged to the ER for
a consult
• Upon arrival at the
ER, an eager Intern
gives an overly
respectful greeting
and leads you to the
patient

55. • You see a female patient who looks to be in her early
twenties. She is lying in bed propped up with 5 large
pillows. She has labored breathing (even while breathing
thru her face mask) and you notice she’s using her
accessory muscles
• She had initially seemed familiar to you. As the intern
starts narrating the history, you realize it is little Alem
• The overly enthusiastic intern starts narrating the
following history

56. The History
• “This is Alem Gezahegn, a 19 year old female
who presents with
◦ shortness of breath and dry cough that has been
progressively worsening over the last 6 months.
• Initially she experienced dyspnea only after brisk
walks. She now has dyspnea even at rest.
• Lying down worsens symptoms, and she often
needs three to four pillows to fall asleep..”

57. • In the past month, she has had multiple episodes
of severe shortness of breath and coughing that
awoke her from sleep and states she feels her
heart pounding
• In addition, she has had a couple episodes of
coughing up bright red blood
• She also reports swelling of lower extremities
that gradually comes on during the day

58. • At this point, what further questions about
the patients history would you like to ask
the overly enthusiastic intern?

59. Associated with this, she
reports
• RUQ pain, anorexia, nausea, and early satiety
• Denies
◦ Fever
◦ Chest pain, Productive sputum
◦ Alteration in urine frequency or amount, dysuria,
urgency, change in color of urine
◦ Change in color, character or frequency of stool, jaundice

60. • While the overly enthusiastic intern continues
his narrations, you perform a quick physical
exam and discover the following

61. Physical Examination
• Vitals
◦ Blood pressure is 125/80, and pulse is 110 bpm and regular.
◦ Respiratory rate 30/min, Temp 37.2o
• Respiratory
◦ cyanosis of the lips and nails with clubbing
◦ dullness to percussion and scattered rales and wheezes heard widely
over both lung fields.
• Venous
◦ 9 cm of JVD with positive abdominojugular reflux

62. Precordial
• Precordial
◦ Soft apical impulse
◦ On auscultation, you hear the following
◦ Loud S1, P1 accentuated, OS heard
◦ low-pitched, rumbling, diastolic murmur, heard best at the apex with the
patient in the left lateral recumbent position

63. Abdominal
◦ RUQ tenderness, with liver palpable to 5 cm
below costal margin
Extremities
◦ 2+ pedal edema
◦ cool peripheral extremities

64. In Summery
• 19 y/o female presents with
◦Dyspnea and dry cough of 6 mo duration
◦Orthopnea, PND, Hemoptysis of 1 mo
duration
◦Extremity Swelling
◦RUQ Pain, N/V, early satiety

65. • Physical Exam Significant for
◦ Tachycardia (110/min), Tachypnea (30/min)
◦ Central cyanosis,
◦ Sings of consolidation in the lung
◦ Raised JVP
◦ Soft apical impulse
◦ Loud S1 with P1 accentuation
◦ low-pitched, rumbling, diastolic murmur, heard best at
the apex
◦ Tender hepatomegaly
◦ 2+ bilateral extremity edema

66. • Overly enthusiastic intern still hasn’t
finished narrating the overly detailed
history he has taken.
• What is the most likely diagnosis that you
consider in the patient?

67. Heart Failure
• Heart failure (HF) is a clinical syndrome that
occurs in patients who, because of an inherited
or acquired abnormality of cardiac structure
and/or function, develop a constellation of
clinical symptoms (dyspnea and fatigue) and
signs (edema and rales) that signify the inability
of heart to meet the needs of the body.

68. Classification
1) HF with a depressed EF (commonly
referred to as systolic failure) or
(2) HF with a preserved EF (commonly
referred to as diastolic failure).

69. Etiology

70. Epidemiology
• Rheumatic heart disease remains
a major cause of HF in Africa and
Asia, especially in the young.

71. Diagnosis – Modified Framingham
Criteria
Major Criteria Minor Criteria
Orthopnea Bilateral Lee Edema
PND Night Cough
Elevated JVP Dyspnea on Ordinary Exertion
Pulmonary Rales Hepatomegaly
S3 Gallop Pleural Effusion
Cardiomegaly on CXR Tachycardia (>=120/min)
Pulmonary Edema on CXR Weight loss >=4.5kg in 5 days
Weight loss >=4.5kg in 5 days of Tx
The diagnosis of heart failure requires that 2 major or 1 major and 2 minor criteria cannot be
attributed to another medical condition.

72. DDx
• (1) conditions in which there is
circulatory congestion secondary to
abnormal salt and water retention
but in which there is no disturbance
of cardiac structure or function
• (2) noncardiac causes of pulmonary
edema

73. Back to Alem……
• By the time all this information flashes in your
mind, the overly enthusiastic intern has finished
narrating his overly detailed history and physical
examination
• He asks what investigations he should order
• Which investigations would you suggest to him?

74. Ix
CBC,
Electrolytes,
Organ Function Tests
◦ BUN, Creatinine,
◦ Liver enzymes
◦ TSH
Urinalysis
Blood glucose and lipid profile
ECG
CXR
Echo

75. • Overly enthusiastic intern sends for
the investigations and comes back to
your office in the afternoon to show
you the results
• Pertinent results are as follows

76. Lab Results
CBC,
Electrolytes,
Organ Function Tests
◦ BUN, Creatinine,
◦ Liver enzymes
◦ TSH
Urinalysis
Blood glucose and lipid profile

77. ECG
• Signs of left atrial enlargement
◦ P wave that becomes broader (duration in lead
II>0.12 sec), is of increased amplitude, and is
notched (due to the delay in left atrial
activation). This is termed "P-mitrale." The left
atrial changes also produce a prominent
negative terminal portion of the P wave in lead
V1.

78. CXR
• The earliest changes are
• straightening of the upper left border of the cardiac silhouette,
• prominence of the main pulmonary arteries,
• dilation of the upper lobe pulmonary veins, and
• posterior displacement of the esophagus by an enlarged LA.

79. TTE Echo
• Sever Mitral Stenosis (with
calculated valve area of <1cm2)

80. Rheumatic Heart Disease
• RHD is the long term sequelea of
poorly treated ARF
• 60% of patients with ARF progress to
RHD

82. MS
• Rheumatic fever is the leading cause of mitral
stenosis
• Pure or predominant MS occurs in approximately
40% of all patients with rheumatic heart disease
and a history of rheumatic fever
• mitral commissures fuse, the chordae tendineae
fuse and shorten, the valvular cusps become
rigid (fish-mouth)

83. SYMPTOMS
◦ Dyspnea
◦ Hemoptysis
◦ Embolic Event
◦ Chest Pain
◦ Right side heart
failure
◦ Hoarseness and
Dysphagia
◦ Mitral Face
SIGNS
◦ Loud S1, P1
accentuated
◦ S3 and S4 are rare
◦ OS
◦ Chx-istic murmur
CF of MS

84. DDX of Murmur of MS
MR
AS
ASD
LA Myxoma

85. • Overly enthusiastic intern draws you
back from your contemplations and
asks you what he should do next?

86. • Management of this patient has
two aspects
◦Management of Heart Failure
◦Management of MS

87. Mx of Heart Failure
Symptoms
◦ Diuretics
Progression
◦ ACE Inhibitors/ARB
◦ Beta Blockers
◦ Aldosteron Antagonists
Special Cases
◦ Digoxin
◦ Anti-platelet and Anti-coag
◦ Anti-arrythmics
◦ Device Therapy

88. Mx of MS
• 2ory prevention of RF
• IE prophylaxis (?)
• Thromboembolism
• A-Fib

89. You tell overly enthusiastic intern to admit the
patient and start her on treatment
Considering this as a very important teaching
moment for overly enthusiastic intern (and in a bid
to show off a little bit yourself), you decide to ask
him what treatment he would like to put this
patient on for

90. • He replies he will immediately start her on
• IV Furosemide 40mg bid +
• Enarapril 10mg bid +
• Bisoprolol 10mg qid +
• Spironolactone 25mg qid+
• Valsartan 160mg bid
• You tell him he is wrong, why?

91. • Reasons have to do with
• Algorism
• Titration
• Combination

94. • In light of the fact that this patient has MS, you
ask overly enthusiastic intern what other
medications he would order?

95. • Penicillin prophylaxis of group A hemolytic
streptococcal infections
• Anti-coag for Thromboembolism (Warfarin, with
target INR of 2-3)
• Mx of AF
• Stable Vs Unstable Patients

96. • Lastly, in a bid to show off how you keep up to
date with medical advances, you ask overly
enthusiastic intern whether he would advise the
patient to take IE prophylaxis before undergoing
medical procedures in the future?

97. • IE is much more likely to result from frequent
exposure to random bacteremias associated
with daily activities (eg, tooth brushing) than
from bacteremia caused by a
dental, gastrointestinal, or genitourinary
procedure.
• Thus, recent recommendations do not support
IE prophylaxis for patients with RHD

98. • Its 6 weeks later and overly enthusiastic intern
returns to your office to update you on Alem’s
status.
• He tell you she has been put on
• Furosemide 20mg bid +
• Enarapril 10mg bid +
• Bisoprolol 10mg qid +
• Spironolactone 25mg qid

99. • All sings of fluid collection have resolved, labs are
normal and she has improved to the point where
she is able to take short walks in the hospital
compound without disabling symptoms
• Overly enthusiastic intern asks you if he should
discharge her, what's your reply?

100. Criteria for Discharge
• Criteria for discharge should include at least 24 h
of stable fluid status, blood pressure, and renal
function on the oral regimen planned for home.

101. Prognosis
• Despite many recent advances in the evaluation
and management of HF, the development of
symptomatic HF still carries a poor prognosis.
• Community-based studies indicate that 30–40%
of patients die within 1 year of diagnosis and 60–
70% die within 5 years, mainly from worsening
HF or as a sudden event (probably because of a
ventricular arrhythmia).

102. • Although it is difficult to predict prognosis in an
individual, patients with symptoms at rest [New York
Heart Association (NYHA) class IV] have a 30–70% annual
mortality rate, whereas patients with symptoms with
moderate activity (NYHA class II) have an annual
mortality rate of 5–10%.
• Studies carried out before the development of mitral
valvotomy revealed that once a patient with MS became
seriously symptomatic, the disease progressed
continuously to death within 2–5 years.

103. However……
• Like any good story, ours also should end with a
happy ending

104. • MS is a purely mechanical problem
• Thus, unless there is a contraindication, mitral
valvotomy is indicated in
• symptomatic [New York Heart Association
(NYHA) Functional Class II–IV] patients with
isolated MS, whose effective orifice (valve area)
is < ∼1 cm2/m2 body surface area, or <1.5 cm2 in
normal-sized adults.

105. • On her follow up visit to your Cardiology Clinic,
you tell Alem Gezahegn that a much more
permanent solution to her condition has
recently been made available at the hospital

106. • A certain colleague of yours,
• one who amongst other
things had a tendency to
“admire nature” during his
undergraduate years (pun
intended)
• has recently returned from
Israel after completing a
fellowship in Cardiothoracic
Surgery and has started
practice in the hospital

107. • Professor Abraham does a Percutaneous Mitral
Balloon Valvotomy
• Alem survives the procedure and has a
remarkable improvement in her symptoms
• She is now 80 and is happily married with 3
children and 8 grandchildren

108. Cast and Characters
• Alem Gezahegn as The Patient
• Tsehay Abebayehu as The Mother
• Overly Enthusiastic Intern as The Intern
• Professor Abraham as Himself

109. And last but by no means least
YOU
As
The caring, companionate and community oriented doctor
SPHMMC trained you to be

110. The End