❤️Rheumatic Heart Disease - Proforma

I. Presenting History

Start by elucidating the chief complaints in chronological order. In RF/RHD, children may present either with an acute attack of rheumatic fever (Jones criteria) or with chronic valvular complications (heart failure).

Exhaustive List of Complaints & Follow-Up Questions:

• Fever & Sore Throat (Preceding Infection):
  • Follow-up: Was there a history of high-grade fever and severe sore throat 1 to 5 weeks prior to the onset of current symptoms? (The latent period between Group A Beta-hemolytic streptococcal infection and RF is typically 1-5 weeks).
• Joint Pain & Swelling (Migratory Polyarthritis):
  • Follow-up: Which joints are involved? (Typically large joints: knees, ankles, elbows, wrists). Is the pain fleeting/migratory (moving from one joint to another in quick succession)? Are the joints red, hot, swollen, and exquisitely tender even on slight movement? Did the pain respond dramatically within 12-24 hours to analgesics (salicylates)?
• Abnormal Involuntary Movements (Sydenham's Chorea):
  • Follow-up: Are there purposeless, jerky, non-repetitive movements of the hands, feet, or face? Do the movements disappear during sleep and worsen with stress/excitement? Did the mother notice emotional lability, aggressive behavior, or deterioration in handwriting (macrographia) prior to the movements? (Note: Chorea manifests late, typically 3-6 months after the onset of RF).
• Palpitations / Fast Heartbeat:
  • Follow-up: Is it present at rest or on exertion? Does it limit the child's physical activities (e.g., school absenteeism)? (Indicates arrhythmias, volume overload from regurgitant lesions, or active myocarditis).
• Difficulty in Breathing (Dyspnea / Orthopnea / PND):
  • Follow-up: Grade the dyspnea (NYHA Class I-IV). Does the child wake up suddenly at night gasping for air (Paroxysmal Nocturnal Dyspnea) or require multiple pillows to sleep (Orthopnea)? (Hallmarks of left ventricular failure and pulmonary venous congestion, classically seen in Mitral Stenosis and severe regurgitant lesions).
• Chest Pain:
  • Follow-up: Is the pain sharp, stabbing, and radiating to the left shoulder (suggestive of pericarditis)? Is it squeezing/heaviness aggravated by exertion (suggestive of ischemic pain/angina due to severe Aortic Stenosis)?
• Edema & Decreased Urine Output (Oliguria):
  • Follow-up: Is there swelling over the feet or facial puffiness? (Indicates right heart failure/systemic venous congestion secondary to long-standing left heart lesions or pulmonary hypertension).

II. Negative History (3C 1D Framework)

Use the 3C 1D framework to systematically rule out conditions based on Causes, Complaints, Complications, and Differentials.

III. Other Relevant History

• Past History: Note the exact age of the first attack of rheumatic fever. Inquire about previous hospitalizations for joint pains, chorea, or heart failure. Recurrence risk is extremely high (up to 50%) if secondary prophylaxis was missed.
• Treatment / Immunization History (Critical for RHD): Ask specifically: Is the child receiving secondary prophylaxis (Injection Benzathine Penicillin G every 21 days or oral Penicillin V daily)? What is the compliance? Has the child received Infective Endocarditis prophylaxis prior to dental procedures?
• Birth & Developmental History: Usually normal in acquired heart diseases, differentiating it from congenital heart defects.
• Socioeconomic History: Elicit the modified Kuppuswamy scale score. Note overcrowding and poor ventilation, which facilitate the spread of streptococcal pharyngitis.

IV. History Summary Template

"A [Age, e.g., 12]-year-old [Gender] child, belonging to lower socioeconomic status, presented with a history of progressive exertional dyspnea, palpitations, and orthopnea for the past [Duration]. The child had a documented episode of migratory polyarthritis and high-grade fever [X] years ago, suggestive of acute rheumatic fever, for which secondary penicillin prophylaxis is irregular. Currently, the history points towards chronic left heart failure with possible pulmonary venous congestion. There is no history suggestive of cyanotic heart disease, active infective endocarditis, or embolic cerebrovascular events."

V. General & Head-to-Toe Examination

1. Vitals (Crucial for hemodynamics):

• Pulse: Detail rate, rhythm, volume, and character.
  • Water-hammer/Collapsing pulse: Classic for Aortic Regurgitation (AR).
  • Pulsus parvus et tardus (slow rising, low volume): Classic for Aortic Stenosis (AS).
  • Irregularly irregular rhythm: Points to Atrial Fibrillation (common in advanced Mitral Stenosis).
  • Pulsus alternans: Indicates severe left ventricular failure.
• Blood Pressure: Look for a wide pulse pressure (>50-60 mmHg) with a low diastolic in AR, or a narrow pulse pressure with systolic decapitation in AS.
• Respiratory Rate (RR) & Temperature: Tachypnea indicates failure; fever indicates active carditis or IE.

2. Anthropometry: Check for undernutrition/cardiac cachexia due to chronic failure.

3. Signs of Active Rheumatic Fever & Chorea (Examiner Favorites):

• Subcutaneous Nodules: Painless, firm, 0.2–2 cm nodules over bony prominences (extensor surfaces of elbows, knees, spine, occiput). Strongly associated with severe carditis.
• Erythema Marginatum: Evanescent, non-pruritic pink macules with serpiginous margins on the trunk and proximal limbs. Worsens with heat.
• Chorea Signs:
  • Darting / Jack-in-the-box tongue: Inability to keep the tongue protruded.
  • Dinner fork sign: Hyperextension of MCP joints with wrist flexion when arms are outstretched.
  • Hung up reflex: Delayed relaxation phase of the knee jerk.
  • Milkmaid's grip: Rhythmic squeezing when the patient grasps the examiner's fingers.

4. Peripheral Signs of Aortic Regurgitation (Must actively state): Look for De Musset's sign (head nodding), Corrigan's sign (dancing carotids), Quincke's sign (capillary pulsations in nail beds), Muller's sign (pulsating uvula), Traube's sign (pistol shot sounds over femorals), Duroziez's murmur (to-and-fro murmur over compressed femoral artery), and Hill's sign (Lower limb systolic BP >20 mmHg higher than upper limb).

5. Markers of Infective Endocarditis: Osler's nodes (tender, finger pads), Janeway lesions (painless, palms/soles), Splinter hemorrhages, Roth spots (fundus), and severe dental caries (nidus for infection).

VI. Systemic Examination (Cardiovascular System)

1. Inspection

• Shape of Chest: Precordial bulge (indicates cardiomegaly onset in early childhood while the chest wall was compliant).
• Apical Impulse: Note the exact position. Shifted downwards and outwards in LV volume overload (MR, AR).
• Venous Prominence / JVP: Prominent 'a' waves in pulmonary hypertension/TS; absent 'a' waves in atrial fibrillation.

2. Palpation

• Apical Impulse Character:
  • Tapping: Palpable loud S1, hallmark of Mitral Stenosis.
  • Hyperdynamic: Forceful, ill-sustained, indicates volume overload (Mitral Regurgitation, Aortic Regurgitation).
  • Heaving: Forceful, sustained thrust, indicates pressure overload (Aortic Stenosis).
• Parasternal Heave: Grade I-III left parasternal lift indicates Right Ventricular Hypertrophy (RVH) secondary to pulmonary hypertension (common in advanced MS/MR).
• Thrills:
  • Diastolic thrill at apex: Mitral Stenosis (felt best in left lateral position).
  • Systolic thrill at apex: Mitral Regurgitation.
  • Systolic thrill at aortic area radiating to carotids: Aortic Stenosis.

3. Auscultation (The Core Granular Breakdown)

RHD predominantly affects valves in this order: Mitral > Aortic > Tricuspid. Pulmonary is almost never involved.

4. Other Systemic Highlights:

• Respiratory: Bilateral basal fine crepitations or wheezing (Cardiac Asthma) indicating pulmonary venous congestion/pulmonary edema.
• Abdomen: Tender hepatomegaly (Right heart failure secondary to prolonged pulmonary hypertension).
• CNS: Thoroughly examine for focal deficits (hemiplegia) indicating cardio-embolic stroke from left atrial thrombus in MS/Atrial Fibrillation.

VII. Final Summary & Diagnosis

Summary Template: "To summarize, this is a Age-year-old Gender presenting with progressive exertional dyspnea, orthopnea, and palpitations. General examination reveals a thin-built child with a collapsing pulse, wide pulse pressure, and positive peripheral signs of aortic run-off including Corrigan's and Quincke's signs. There is no pallor, icterus, or markers of infective endocarditis. Systemic cardiovascular examination shows an apical impulse shifted downwards and outwards that is hyperdynamic in character. Auscultation reveals a soft S1, an S3 gallop, a high-pitched early diastolic decrescendo murmur at the left sternal border radiating to the apex, and an Austin Flint murmur at the apex. The chest is clear, and the liver is not palpable."

Exact Format for Stating the Final Diagnosis: To satisfy strict academic requirements, your diagnosis string MUST logically flow through these 6 checkpoints:

1. Etiology (Acquired, Rheumatic)
2. Anatomical/Valvular Lesion (Dominant lesion first, then secondary)
3. Severity of the lesion
4. Cardiac Rhythm (Sinus Rhythm vs. Atrial Fibrillation)
5. Functional Status / Heart Failure status (NYHA Class)
6. Current Complications / Disease Activity (Active carditis, IE, Pulmonary Hypertension)

Final Diagnosis String:

"My provisional diagnosis is an Acquired Rheumatic Heart Disease, with severe Aortic Regurgitation and moderate Mitral Regurgitation, currently in Normal Sinus Rhythm, presenting with Congestive Cardiac Failure (NYHA Class III), with clinical evidence of Pulmonary Arterial Hypertension, and currently NO clinical evidence of active Rheumatic fever or Infective Endocarditis."