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Interactive Cases in Clinical Medicine (SPHMMC production) Episode 01

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An interactive case where we discuss the diagnosis and management of Acute Rheumatic Fever, Rheumatic Heart Disease and Heart Failure in general. …

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

Published in Health & Medicine
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  • 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
  • 42. 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
  • 43. • 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
  • 44. • 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
  • 45. • Before discharge, what further instructions are you going to give W/o Tsehay about her daughter?
  • 46. 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
  • 47. 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)
  • 48. • 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
  • 49. • 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
  • 50. • 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*
  • 51. A few years down the road…….
  • 52. 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
  • 53. • 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
  • 54. • 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
  • 55. 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..”
  • 56. • 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
  • 57. • At this point, what further questions about the patients history would you like to ask the overly enthusiastic intern?
  • 58. 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
  • 59. • While the overly enthusiastic intern continues his narrations, you perform a quick physical exam and discover the following
  • 60. 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
  • 61. 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
  • 62. Abdominal ◦ RUQ tenderness, with liver palpable to 5 cm below costal margin Extremities ◦ 2+ pedal edema ◦ cool peripheral extremities
  • 63. 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
  • 64. • 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
  • 65. • 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?
  • 66. 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.
  • 67. 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).
  • 68. Etiology
  • 69. Epidemiology • Rheumatic heart disease remains a major cause of HF in Africa and Asia, especially in the young.
  • 70. 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.
  • 71. 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
  • 72. 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?
  • 73. Ix CBC, Electrolytes, Organ Function Tests ◦ BUN, Creatinine, ◦ Liver enzymes ◦ TSH Urinalysis Blood glucose and lipid profile ECG CXR Echo
  • 74. • 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
  • 75. Lab Results CBC, Electrolytes, Organ Function Tests ◦ BUN, Creatinine, ◦ Liver enzymes ◦ TSH Urinalysis Blood glucose and lipid profile
  • 76. 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.
  • 77. 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.
  • 78. TTE Echo • Sever Mitral Stenosis (with calculated valve area of <1cm2)
  • 79. Rheumatic Heart Disease • RHD is the long term sequelea of poorly treated ARF • 60% of patients with ARF progress to RHD
  • 80. 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)
  • 81. 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
  • 82. DDX of Murmur of MS MR AS ASD LA Myxoma
  • 83. • Overly enthusiastic intern draws you back from your contemplations and asks you what he should do next?
  • 84. • Management of this patient has two aspects ◦Management of Heart Failure ◦Management of MS
  • 85. 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
  • 86. Mx of MS • 2ory prevention of RF • IE prophylaxis (?) • Thromboembolism • A-Fib
  • 87. 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
  • 88. • 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?
  • 89. • Reasons have to do with • Algorism • Titration • Combination
  • 90. • In light of the fact that this patient has MS, you ask overly enthusiastic intern what other medications he would order?
  • 91. • 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
  • 92. • 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?
  • 93. • 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
  • 94. • 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
  • 95. • 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?
  • 96. 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.
  • 97. 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).
  • 98. • 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.
  • 99. However…… • Like any good story, ours also should end with a happy ending
  • 100. • 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.
  • 101. • 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
  • 102. • 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
  • 103. • 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
  • 104. Cast and Characters • Alem Gezahegn as The Patient • Tsehay Abebayehu as The Mother • Overly Enthusiastic Intern as The Intern • Professor Abraham as Himself
  • 105. And last but by no means least YOU As The caring, companionate and community oriented doctor SPHMMC trained you to be
  • 106. The End