MRCP PACES by Jansen Koh
(http://jansenkoh.com/MRCPnotes.htm) CARDIO
1. Mitral regurgitation 2 2. Mitral valve prolapse 7 3. Mitral stenosis 8 4. Aortic regurgitation 10 5. Aortic stenosis 12
6. Mixed mitral valve disease 14 7. Mixed aortic valve disease 15 8. Prosthetic heart valves 16 9. VSD 18
10. ASD 20 11. HOCM 22
12. Approach to central cyanosis and clubbing 24 13. Dextrocardia 25
RESPI
14. Bronchiectasis 26 15. Interstitial lung disease 30 16. COPD 33
17. Pleural effusion 35 18. Collapse 38 19. Consolidation 40
20. Lobectomy/Pneumonectomy 44 21. Approach to lateral thoracotomy scar 45 22. Lung transplant 46
ABDO
23. Chronic liver disease 48 24. Hepatomegaly 54 25. Splenomegaly 56 26. Hepatosplenomegaly 58 27. Ascites 60
28. Unilateral enlarged kidney 63 29. Bilateral enlarged kidney 64 30. Transplanted kidney 67 NEURO
31. Approach to examination of the face 70 32. Cranial nerve syndromes 71
33. Isolated III nerve palsy 74 34. Isolated VI nerve palsy 76 35. VII nerve palsy 79 36. Myasthenia gravis 82
37. Approach to examination of the eyes 85 38. Gaze palsies 86
39. Unilateral ptosis 87 40. Bilateral ptosis 89
III, IV and VI cranial nerve palsies (33, 34, 35) 41. Assessment of higher cortical function 90 UPPER LIMBS (NEURO)
42. Upper limbs overview 92 43. Radial nerve palsy 94 44. Median nerve palsy 96 45. Ulnar nerve palsy 99 46. Wasted hands 101 Peripheral neuropathy (54) 47. Syringomyelia 104 48. Dystrophica myotonica 106 49. Cerebellar signs 109 50. Chorea 114 Parkinsonism (61)
LOWER LIMBS (NEURO) 51. Lower limbs overview 116 52. Flaccid paraparesis 118 53. Spastic paraparesis 123 54. Peripheral neuropathy 129 55. Charcot‟s Joint 131 56. Proximal myopathy 133 57. Brown-sequard syndrome 135 58. Footdrop 136 59. Hemiparesis/Hemiplegia 137 60. Gait assessment 140 61. Parkinsonism 141 RHEUMATO 62. Rheumatoid arthritis 144 63. Gouty hands 146 64. Psoriatic hands 149 65. OA of the hands 151 66. Scleroderma 153 67. Ankylosing Spondylitis 155 68. Marfan syndrome 157 69. Dupytren‟s contractures 160 70. Clubbed fingers 160
71. Painful/swollen knee joint 161
72. Still‟s disease/Juvenile chronic arthritis 161 73. Enteropathic arthropathy 161 74. Old rickets 162 ENDOCRINE 75. Acromegaly 163 76. Cushing‟s syndrome 165 77. Goitre 168 78. Paget‟s disease 174
79. Panhypopituitarism (Simmond‟s disease) 176 80. Addison‟s disease 177 81. Gynaecomastia 177 DERM 82. Dermatology overview 178 83. Psoriasis 181 84. Lichen planus 183 85. Neurofibromatosis 185 86. Purpura 187 87. Dermatomyositis 189 EYE 88. Diabetic retinopathy 191 89. Hypertensive retinopathy 195 90. Optic atrophy 197 91. Papilloedema 199
92. Central and branch retinal vein occlusion 201 93. Central retinal artery occlusion 203
94. Retinitis pigmentosa 204 95. Visual field defects 205 96. Visual acuity 207 97. Cataracts 207 98. Nystagmus 207 99. Pupillary defects 208 OTHERS 100. History-taking station 209
2 CARDIO!
1. Mitral Regurgitation Presentation
Sir, this gentleman has mitral regurgitation that is moderately severe in nature.
There is a pansystolic murmur heard best at the apex which radiates to the axilla. (If it radiates to the carotids – posterior mitral leaflet rupture) This is a grade 3/6 murmur and is not associated with a systolic thrill. The first heart sound is soft and there is presence of a third heart sound(S3). I did not detect any mid-diastolic murmur. The apex is thrusting and displaced, located at the 6th IC at the anterior axillary line.
This is complicated by pulmonary hypertension as evidenced by a palpable and loud pulmonary component of the second heart sound associated with a left parasternal heave. There are no clinical signs of heart failure.
On the peripheral examination, patient is in atrial fibrillation with an irregularly irregular pulse which is rate controlled at 80 beats per min. There is no bruising to suggest overanticoagulation. There are also no stigmata of infective endocarditis.
To complete the examination, I would like to take the patient‟s blood pressure, as well as temperature chart for any fever. (Mention abdominal examination, urine dipstick and fundoscopy if clinically suggestive of IE)
In summary, this gentleman has mitral regurgitation that is moderately severe in nature, with complication atrial fibrillation and pulmonary hypertension. There are no complications of heart failure or infective endocarditis. My differential diagnoses include IHD, MVP and Rh heart disease. (If thoracotomy scar, think of mitral valvotomy for MS)
Questions
How do you grade the severity of mitral regurgitation clinically? Mild – No Pulm hypt
Moderate – Pulmonary hypertension Severe – LVF, S3
What are the causes of mitral regurgitation?
Common causes are MVP, IHD, Rh heart disease and endocarditis Left ventricular dilatation, cardiomyopathy, Marfan‟s, Rheumatoid, AS Acute causes: MI, IE, Trauma, Surgery, spontaneous rupture
Anterior leaflet: radiates to axilla and back Posterior leaflet: radiates to carotids Mitral valvotomy if a thoracotomy scar seen
If elderly and mild to moderate, typically due to annular calcification What are the differential diagnoses for a pansystolic murmur?
MR TR VSD
What congenital conditions can be associated with MR? Corrected TGA
Partial AV canal
Ostium primum atrial defect (cleft mitral valve) What causes a third heart sound?
Rapid filling of the left ventricle from the large volume of blood from the left atrium occurring in early diastole Why is the pulse jerky?
Pulse is sharp and abbreviated due to lack of sustained forward stroke volume with a reduced systolic ejection time because of regurgitant leak into the left atrium
How do you differentiate an MDM from severe MR vs MS? MS has an opening snap
Severe MR associated with S3 MS murmur is longer
MS has loud S1
How do you differentiate between MR and TR murmur?
Mitral Regurgitation Triscupid Regurgitation
PSM heard best at Apex PSM heard best at the LLSE
Radiates towards the axilla Radiates towards the right of sternum
Louder on expiration Louder on inspiration
Displaced apex beat Apex beat not displaced
Jerky pulse character Normal pulse character
How do you differentiate between an MR murmur and that of a VSD?
MR VSD
Loudest at the apex Loudest at the LLSE
High pitched Harsh, low pitched
Soft S1 Normal S1
What are the types of dynamic manoeuvres that you are aware of and what are their uses? Respiration
Murmurs on the right side louder on inspiration due to increased venous return and blood flow to the right side of heart Converse is true
Valsalva manoeuvre (decrease preload) 3 phases
Phase 1 – beginning of maneuver
Rise in intrathoracic pressure and a transient increase in LV output Phase 2 – Straining phase
Systemic return falls
Reduced filling of the right and left heart chambers SV and BP drops while HR increases
Most murmurs become softer and shorter except
MVP – Systolic click and murmur begins earlier (LV size is smaller), ie longer and louder HOCM – murmur is louder as LV volume is reduced
Phase 3
Release of maneuvre
Right heart murmurs becomes louder followed by left heart murmurs Squatting (increases venous return and systemic arterial resistance)
Most murmurs are louder
MVP – click occurs later and murmur is shorter because LV size increased
HOCM – LV size increased which reduced the obstruction to outflow and systolic murmur is softer Standing
Most murmurs are softer except
MVP – louder and longer and HOCM - louder Isometric exercises (increases afterload)
AS – Softer murmur as there is reduction of pressure gradient across the valve MVP – click occurs later and murmur is shorter because LV size increased
HOCM – LV size increased which reduced the obstruction to outflow and systolic murmur is softer MR/AR/VSD louder
Amyl Nitrite inhalation
Initial relative hypotension MR/AR/VSD decrease
AS increases because of increased stroke volume Later tachycardia phase
MS and right murmurs increase
Can use to differentiate Austin Flint from MS What are the signs of severity for MR?
Presence of S3 Short MDM
Apex thrusting and displaced Pulmonary hypertension CCF
What is the pathophysiology of MR? MR leads to LV overload
Compensatory LV dilatation
Eventually, decompensate resulting in heart failure and increased risk of sudden death
Also, regurgitation into the LA leads to enlargement of LA with AF and elevated pulmonary pressures Should all murmurs be investigated?
All should be Ix except 1. mid-systolic, grade 2 or < murmurs with no associated findings or symptoms 2. continuous murmurs of venous hum or mammary soufflé of pregnancy
4 How would you investigate this patient?
ECG
LA enlargement – P mitrale (II – P >0.12s, Limb; Bifid P waves in limb leads with inter-peak > 0.04s, terminal P negativity in V1)
LVH – Sokolow & Lyon Criteria (S in V1 and R in V5 or 6 >35mm) AF
Pulmonary hypertension CXR
CCF – pulmonary congestion, enlarged heart Left atrial enlargement
Pulmonary artery enlargement Echocardiogram
Dx of MR
Severity – EF <60% and LV end-systolic diameter >45mm Cause
Complications eg IE Cardiac catheterisation
Not indicated in most patients but useful if there is discrepancy between echocardiographic and clinical findings Useful to stenosis, regurgitation and intracardiac shunting
How would you manage this patient? Education
Medical therapy
Antibiotic prophylaxis
Treatment of underlying cause eg IHD, dilated CMP (Rx of CCF and afterload reduction) Treatment of complications eg AF, IE, CCF
Surgical Indications Symptomatic or EF<60% or LV end-systolic dimension >45mm Types of surgery
Mitral valve repair if technical feasible is best
Mitral valve replacement if technically not feasible provided EF >30% Controversial
Varied causes for MR
If due to IHD or dilated CMP, then Sx is controversial If due to MVP, timing of surgery
Indicated if symptomatic, AF, pulmonary hypt, EF<60% or ESD>45 If asymptomatic, risk stratify according to regurgitant orifice(doppler)
<20mm2 20-39mm2
>40mm2 (this affects Px and closer follow up necessary) How do you diagnose infective endocarditis?
Duke‟s criteria
2 Major, 1 Major 3 Minor or 5 minor Major
Persistently positive blood c/s with typical organism Persistently positive blood c/s
2 or more positive c/s > 12h apart 3 or more positive c/s each 1 hr apart if 4 or more taken, >70% positive Typical organism
Strep viridans, Strep bovis, enterococci, Staph aureus
HACEK: Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella Endocardial involvement
Positive echocardiogram: vegetations, abscesses, valve perforation, dehiscence New valvular regurgitation
Minor
Predisposing heart condition Fever
Vascular phenomena: arterial emboli, septic pulmonary emboli, mycotic aneurysm, ICH, Janeway lesion Immunologic phenomena: GN, Osler‟s nodes, Roth spots, Rh factor
Positive blood c/s not satisfying major criteria Positive echocardiogram not satisfying major criteria
What are the types of endocarditis? Native valve endocarditis
Strep, enterococci, Staph
Rh, Cong HD, MVP with murmur and degenerative valvular disease Prosthetic
Early(<60 days): Staph aureus or S. epidermis Late(>60 days): Similar to native endocarditis Fungal :IVDA and ICU
IVDA: TV involvement, AV also; Staph aureus, MRSA, fungi, Strep, GNB How would you investigate?
Bloods
Blood C/S (as above)
FBC (NCNC anaemia, raised TW with left shift), ESR, CRP 2D echo
CXR, ECG
How do you treat infective endocarditis? General measures
Eg oxygen, treat fever Antibiotics
IV CP 12-18MU/d 4H for 4 weeks
Can also add IV gentamicin 1mg/kg 8H for first 2 weeks
If allergic, use vancomycin 30mg/kg/d in 2 divided doses for 4 weeks HACEK organism: IV ceftriaxone
MSSA: IV cefazolin or nafcillin or cloxacillin Surgery
Heart failure
Failure of medical therapy
Presence of fever and inflammatory syndrome after 1 week of appropriate and adequate antibiotics Presence of mobile vegetation >10mm with 1 major embolism 1 week A/B
Presence of mobile vegetation >15mm with 1 week of A/B
Valvular complication eg valvular abscess, valvular obstruction, rupture into the pericardium, septal formation, fistula Fungal endocarditis
6 When and how should you prophylax against IE? (3 steps: Risk stratify, Type of Procedure and Type of antibiotics)
Risk stratify
Highest risk: prosthetic valves, both bioprosthetic and mechanical; previous IE; congenital cyanotic heart disease; or surgically produced systemic/pulmonary shunts.
Moderate risk: (1) all other congenital cardiac conditions, except isolated secundum atrial septal defects and surgical repairs of an atrial septal defect or patent ductus arteriosus or ventricular septal defect more than 6 months ago; (2) acquired valvular dysfunction (eg, rheumatic heart disease, calcific aortic stenosis); or (3) hypertrophic cardiomyopathy and mitral valve prolapse with valvular regurgitation and/or thickened leaflets. Thickening of the anterior leaflets of the mitral valve correlates with significant mitral insufficiency, especially in men older than 45 years.
Low risk: mitral valve prolapse without significant regurgitation or thickened leaflets on echocardiography, implanted cardiac PMs, implanted defibrillators, implanted coronary stents, or "innocent" murmurs. An important caveat is that in elderly individuals, an innocent murmur may not be hemodynamically significant but may signify the presence of a calcified leaflet that is susceptible to infection during a transient bacteremia.
Procedures that require antibiotic prophylaxis in high-to-moderate risk patients are as follows: Invasive manipulation of the respiratory tract (eg, tonsillectomies, rigid bronchoscopy)
Gastrointestinal surgery, biliary tract surgery, sclerotherapy of esophageal varices, dilatation of esophageal strictures, and endoscopic retrograde cholangiopancreatography in the presence of biliary obstruction
Prostate surgery, cystoscopy, and urethral dilatation
Generally, hysterectomies, vaginal delivery, cesarean delivery, urethral catheterizations, dilation and curettage, therapeutic abortions, sterilization procedures, insertion or removal of intrauterine devices, cardiac catheterizations, angioplasties, or endoscopies with or without biopsies do not require prophylaxis.
Adult antimicrobial IE preventive regimens for dental, oral, respiratory tract, or esophageal procedures recommended by the American Heart Association to prevent streptococcal IE from oral-dental sources are as follows:
Administer amoxicillin at 2 g orally 1 hour before the procedure or ampicillin at 2 g IM or IV 30 minutes before the procedure.
If the individual is allergic to penicillin, clindamycin at 600 mg, cephalexin at 2 g, or azithromycin at 500 mg orally 1 hour before the procedure are alternatives.
If the individual is allergic to penicillin and is unable to take oral medication, clindamycin at 600 mg IV or cefazolin at 1 g IM or IV should be given 30 minutes before the procedure.
Adult IE prophylactic regimens for individuals undergoing lower gastrointestinal tract surgery or instrumentation of genitourinary tract procedures are for preventing enterococcal endocarditis. They are as follows:
High Risk regimens recommended by the American Heart Association are ampicillin at 2 g IM or IV plus gentamicin at 1.5 mg/kg (not to exceed 120 mg) within 30 minutes of the procedure, followed by ampicillin at 1 g IM, IV, or orally 6 hours later.
High-risk individuals who are allergic to penicillins should receive vancomycin at 1 g IV over 1-2 hours plus gentamicin at 1.5 mg/kg IV or IM (not to exceed 120 mg) within 30 minutes of starting the procedure.
For moderate-risk patients, amoxicillin at 2 g orally 1 hour before the procedure or ampicillin at 2 g IM or IV within 30 minutes of starting the procedure is recommended.
The alternative for patients who are allergic to penicillin who are at moderate risk is vancomycin at 1 g IV over 1-2 hours, completed 30 minutes before the procedure.
What is the prognosis?
Depends on underlying cause
If IHD or dilated CMP, Px dependent on the underlying disease If due to MVP
2. Mitral Valve Prolapse (Floppy MV, Barlow‟s syndrome, Click-murmur syndrome) Presentation
Sir, this patient has got a MR that is severe and secondary to a mitral valve prolapse.
I say this because there is a presence of a mid-systolic click associated with a late systolic crescendo-decrescendo murmur heard best at the apex.
This murmur radiates towards the axilla and is a grade…..(present as for MR)
I would like to complete my examination by asking the patient to perform the valsalva manoeuvre as well as to stand to accentuate the murmur; take BP and temperature chart as well as a neurological examination for signs of stroke.
Questions
What causes a mid-systolic click?
o Inability of the papillary muscles or the chordae tendinea to tether the mitral valves in the late stages of systole
o The prolapsing of the valve leaflet into the LA and sudden tensing of the mitral valve apparatus causes the mid-systolic click
What are the differential diagnoses for a systolic murmur? o AS, PS, MVP, HOCM
What are the causes and associations of a MVP? o Myxomatous degeneration of the mitral valve tissue o Associated with
o Heart conditions
ASD (Secundum type) Cardiomyopathy Myocarditis o Systemic conditions
Marfan‟s syndrome Ehlers danlos syndrome Osteogenesis Imperfecta Polycystic Kidney disease SLE
What manoeuvres can accentuate the findings of an MVP and why? o Valsalva manoeuvre and standing
o Decrease preload
o Reduction in the cardiac volume
o Further imparing the papillary muscles or chordae tendinea from maintaining tension on the leaflets and preventing the leaflets from prolapsing into the LA
o Hence the systolic click occurs earlier with a longer duration of the systolic murmur How do patients with MVP present?
o Asymptomatic o Symptomatic
o Palpitations o Anxiety
o Atypical chest pain o Light-headedness o Complications of MR
o CCF – fatigue and dyspnea o IE
o Arrhythmias
o Embolic phenomenon o Sudden death
How would you Ix?
o Echocardiogram: Confirm Dx, Complications of MR How would you manage?
o Education and reassurance o Medical
o Antibiotic prophylaxis
Only if associated with MR
Otherwise not necessary if just MVP o Symptomatic
Palpitations Rx with beta blockers (benign ventricular ectopy) o Rx underlying cause or associations
o Rx complications such as MR with CCF, IE or AF or TIA o Surgical
8 3. Mitral Stenosis
Presentation
Sir, this patient has mitral stenosis which is severe in nature with complications of infective endocarditis and congestive cardiac failure.
There is presence of a mid diastolic murmur heard best at the apex and is accentuated in the left lateral position. It is a grade 3/6 murmur and is not associated with any diastolic thrill. It is severe as it is associated with an early opening snap and a long mid diastolic murmur. The first heart sound is also loud. The apex beat is tapping in nature and is not displaced, located just medial to the mid-clavicular line in the 5th intercostal space.
There is associated pulmonary hypertension with a palpable pulmonary component of the second heart sound with left parasternal heave. There is a loud pulmonary component of the second heart with a presence of a functional TR as evidence of PSM at the LLSE that is louder with inspiration associated with a giant V wave which is elevated at 5cm. There is no associated Graham Steel murmur (PR).
This is associated with congestive cardiac failure with bilateral basal crepitations and bilateral pedal edema.
Examination of the peripheries reveals evidence of stigmata of infective endocarditis. Patient is clubbed with Janeway lesions on the palms and Osler‟s nodes noted on the pulp of the fingers. There are also splinter haemorrhages with presence of conjunctival pallor. There is presence of a peripherally inserted central catheter suggesting use of long term antibiotics. The patient is on IV cloxacillin suggesting that the infective organism is MSSA. There is complication of atrial fibrillation. The HR is irregularly irregular with a rate of 84 bpm. There is a characteristic pulsus parvus pulse. There are no bruises to suggest overanticoagulation.
I did not notice any mitral facies. The patient‟s voice is also not hoarse which may suggest Ortner‟s syndrome. (mention the lateral thoracotomy scar with possible mitral valvotomy as intervention)
I would like to complete the examination by looking at the patient temperature chart as well as taking the patient‟s BP.
In summary, this patient has MS that is severe in nature with complications of atrial fibrillation, pulmonary hypertension congestive cardiac failure and infective endocarditis secondary to MSSA. The possible causes for MS include rheumatic heart disease or congenital parachute valves.
Questions
How do you grade the severity of MS clinically? Mild – no PHT
Moderate – PHT Severe - CCF
What are your differentials for a mid-diastolic murmur? MS
Atrial myxoma Ball-valve thrombosis Flow across the TV in ASD
MR with increased flow through the mitral valve during diastole Austin Flint murmur (in severe AR)
What are the causes of mitral stenosis? Common
Rheumatic heart disease Congenital parachute valve Rare
Calcification of mitral annulus and leaflets CTDs: SLE, RA
Carcinoid (malignant) What causes a tapping apex beat?
An accentuated first heart sound What causes an opening snap?
Opening of a stenosed mitral valve and indicates that leaflets are pliable Why is the first heart sound loud?
The mitral valve is held open during diastole by the transmitral gradient. The valve is suddenly slammed shut during ventricular contraction
What causes presystolic accentuation of the murmur?
Occurs in sinus rhythm only during atrial systole which increases flow from the LA to the LV through the stenotic valve How do patients present?
Asymptomatic
Women, may have h/o RH heart disease
Symptoms ppt especially during pregnancy or development of AF Usually left-sided heart failure: exertional dyspnea, PND, orthopnea
How do you diagnose rheumatic fever? Duckett-Jones criteria
2 major or 1 major + 2 minor Major
Carditis, Sydenham‟s chorea, SC nodules, erythema marginatum, arthritis Minor
Past H/o RHD, fever, arthralgia, prolonged PR, ESR, CRP
+ a h/o streptococcal infection (ASOT raised, recent scarlet fever, +GpA Strep throat c/s or kits for GpA Strep with high specificity but low sensitivity. How do you manage Rheumatic fever?
Prevention
o Primary prevention: Rx with IM Benzathine Pen G 1 dose or 10 days of Pen V
o Secondary prevention: all patient with history of Rh fever should receive prophylaxis; IM Pen G once/month or PO Pen V daily bd
What are the signs of severity for MS? Early opening snap
Long MDM
Pulmonary hypertension CCF
Pulsus parvus
What is Ortner‟s syndrome?
Hoarseness of voice from compression of the recurrent laryngeal nerve from an enlarged left atrium What is Lutembacher‟s syndrome?
Association of MS with ASD How would you investigate?
ECG P mitrale
P pulmonale, RVH, RAD, AF
CXR
Calcified mitral valve
Enlarged LA (double silhoutte sign, straightening of the left heart border, filling of the pulmonary bay by enlarged LA, horizontalization of the left bronchus)
Prominent pulmonary trunk
Pulmonary congestion (Upper lobe diversion, Kerly B lines) Echocardiogram
Dx
Assess severity (Valve area calculation and transmitral gradient) Look for complications (eg IE)
Assessment suitability for balloon valvotomy
What is the normal cross-sectional area of the valve? 4-6 cm2
What is a significantly stenosed mitral valve? <1 cm2 and >10 mmHg gradient across the valve How would you manage?
Education Medical treatment
Antibiotic prophylaxis Treat complications
AF – anticoagulation and rate control
CCF – symptomatic treatment vs improvement of mortality Surgery
Indications
Symptomatic (limit activity) with significant stenosis (<1 cm2 and >10mmHg) Pulmonary hypertension
Hemoptysis
Recurrent thromboembolic events despite adequate anticoagulation Type
Valvotomy Closed
Closed mitral valvotomy
Balloon valvuloplasty (procedure of choice) but need to satisfy Good mobility of valves
Minimal calcification No or mild MR
Minimal subvalvular disease Open valvotomy
Mitral replacement
In which trimester does pregnancy results in symptomatic MS? Second trimester due to increase in blood volume
10 4. Aortic Regurgitation
Examination
Proceed as per cardiovascular examination
On detecting AR, to examine eyes for Argyll-Robertson pupil and auscultate the femorals for pistols shots (Traube sign) and Duroziez sign.
Presentation
Sir, this patient has aortic regurgitation that is severe. My findings are:
Presence of a high-pitched early diastolic murmur heard best at the left lower sternal edge and is loudest at end expiration with the patient sitting forwards. It is a grade 4/6 murmur and is associated with a diastolic thrill. It is severe as the murmur is of a long duration associated a soft second heart sound with a S3 present. There is also an Austin Flint murmur with presence of a mid diastolic murmur heard at the apex not associated with an opening snap.
The apex beat is displaced at the 6th IC anterior axillary line and is thrusting in nature.
This is associated with evidence of CHF with bibasal crepitations, raised JVP at 4 cm with a prominent V wave as well as bilateral peal edema.
Peripheral examination showed no evidence of IE. The pulse is bounding and collapsing in nature at a rate of 90 bpm in SR. There is no RR or RF delay to suggest coarctation of the aorta. In addition, quinke‟s sign was negative.
There was no conjunctival pallor but Corrigan‟s sign and brachial dance were present. Muller‟s sign, Duroziez and Traube‟s signs were not detected.
In terms of etiology, there is no evidence of symmetrical deforming polyarthropathy to suggest RA and patient does not have a Marfanoid habitus or a high arched palate. There is no Argyll-Robertson pupil to suggest lewitic disease.
To complete my examination, I would like to take patient‟s BP looking for wide pulse pressure and severe hypertension. I would also want to look at the patient‟s temperature chart.
In summary, this patient has got AR that is severe with complication of CCF. Possible causes for this patient‟s AR are Rh heart disease, infective endocarditis or congenital bicuspid valve.
Questions
What are the signs of severity of AR? S3
Austin Flint murmur (functional mdm at the apex due to regurgitant jet striking the anterior leaflet of the MV, therefore obstructing flow from the LA into the LV)
Soft S2
Duration of the decrescendo murmur and loudness of murmur (cf with AS) Apex beat displaced and thrusting
CCF
Wide pulse pressure Hill‟s sign
What are the characteristic signs of AR? Collapsing pulse
Brachial dance
Quinke‟s sign (visible capillary pulsation in the nail bed) Corrigan‟s sign (Visible Carotid pulsation in the neck) De Musset‟s sign (head nodding in time with the heart beat Muller‟s sign (pulsation of the uvula)
Traube‟s sign(pistol shots) and Duroziez sign(to and fro murmur on sl compression of the femoral artery) What are the causes of a collapsing pulse?
AR PDA
An aortopulmonary window
A ruptured aneurysm of the aortic sinus Active Paget‟s
High fever Severe Anaemia Pregnancy
What would you expect to find on taking this patient‟s blood pressure? Wide pulse pressure
Severe hypertension (with functional AR)
UL and LL discrepancy with systolic in LL>UL = Hill‟s sign
How do you differentiate an Austin Flint murmur from mitral stenosis? Opening snap
Loud S1
What are the causes of AR? Valvular
Rh, IE and congenital biscupid valve (associated with CoA) Aortic root dilatation
Syphilis, RA, AS, Marfan, severe hypertension Acute causes
IE, trauma, Aortic dissection, rupture of sinus of valsalva How would you investigate?
ECG – LVH with diastolic overload pattern – deep but narrow Q, isoelectric ST, and tall T waves in left praecordial leads CXR – valvular calcification, cardiomegaly, pulmonary congestion, widened aorta
2D echo Confirm Dx Assess cause Severity Complications
How would you manage this patient? Education
Medical
Antibiotic prophylaxis Treat underlying cause
Treat complications such as CCF, IE Vasodilators – ACE and CCB Surgical
Indications
Symptomatic – CCF, angina and severe AR LV ESD >55mm
Aortic root >55mm
Reduction of EF >5% on exercise Types of surgery
What is the prognosis?
12 5. Aortic Stenosis
Presentation
Sir, this patient has Aortic stenosis that is severe in nature. My findings include:
Presence of an ejection systolic murmur heard best at the aortic area and radiates to the carotids. It is a grade 4/6 systolic murmur a/w with a systolic thrill. It is severe as there is an early ejection click a/w a long systolic murmur with delayed peaking of the murmur. I could not detect an S4 and the second heart sound is soft. There was also no paradoxical splitting of the second heart sound.
The apex beat is heaving in nature and is displaced, located at the 6th IC space at the just lateral to the mid-clavicular line.
This is associated with signs of congestive cardiac failure as evidenced by presence of bibasal crepitations, raised JVP at 3 cm with prominent V wave and bilateral pedal edema but she does not require supplemental oxygen.
Peripheral examination does not reveal any stigmata of IE. The pulse is regular at 84bpm and is anacrotic/pulsus parvus et tardus in nature. There are no features suggestive of haemolytic anaemia with no conjunctival pallor and patient is not jaundice.
I would like to complete my examination by taking the patient‟s blood pressure to look for a narrow pulse pressure as well as his temperature chart. I would also like to enquire on patient‟s symptoms of angina, syncope and dyspnea as these are important prognostic markers.
In summary, this patient has got aortic stenosis that is severe in nature with complication of congestive cardiac failure. There is no evidence of infective endocarditis or haemolytic anaemia. The most likely causes include Rh heart disease, calcified biscupid aortic valve or degenerative calcified aortic valves.
Questions
What are the differential diagnoses for an ejection systolic murmur? AS
PS HOCM MVP/MR Coarctation
How do you differentiate between them? AS and PS – expiration and inspiration AS and HOCM – Valsalva, squatting AS and MVP – location and clicks AS and Coarctation – differential pulse
What are the types of pulses associated with aortic stenosis?
Pulsus parvus et tardus – means low volume pulse with delayed upstroke due to a reduction in systolic pressure and a gradual decline in diastolic pressure
Anacrotic pulse – small volume pulse with a notch on the upstroke What does a normal pulse volume in AS mean?
The travsvalvular gradient is <50 mmHg What does a palpable systolic thrill implies?
It means that the transvalvular gradient is > 40mmHg
What does the second heart sound indicate about the aortic stenosis? Soft second heart sound means poorly mobile and stenotic valve
Reversed splitting means mechanical or electrical prolongation of ventricular systole; S2 is normally created by the closure of the aortic valve followed by the pulmonary valve, if the closure of the aortic valve is delayed enough, it may close after the pulmonary, creating an abnormal paradoxical splitting of S2.
Single second heart sound implies fibrosis and fusion of the leaflets Normal second heart sound implies insignificant stenosis
What is Gallavardin phenomenon?
Systolic murmur may radiate towards the apex, which may be confused with a MR murmur How can haemolytic anaemia result from aortic stenosis? MAHA from severely calcified aortic valve What are the causes of aortic stenosis?
Rheumatic heart disease (<60), Degenerative calcification (>75), Calcified biscupid (60-75, males) What are the severity markers?
Early ejection click Long Systolic murmur Late peaking of the murmur 4th heart sound
Paradoxical splitting of S2
Heaving apex beat which is displaced Systolic thrill
Pulsus parvus et tardus Narrow pulse pressure
Symptoms (ASD) Px
Angina 5 years
Syncope 3 years
How do you differentiate AS from aortic sclerosis? No severity signs as above
ESM which is localised to aortic area with a normal S2 in elderly person How do patients present?
Asymptomatic and incidental finding Angina
o Increase oxygen requirement for hypertrophied LV with hypoperfusion of the subendocardial myocardium Syncope
o Cardiac arrythmias
o Peripheral vasodilatation eg post exercise without concomitant increase in CO o Transient elctromechanical dissociation
Dyspnea
o Implies LV dysfunction and heart failure How would you investigate?
ECG – LVH with strain, 1st degree heart block, LBBB
CXR – Calcified aortic valve, cardiomegaly, pulmonary congestion 2D echo
o Dx o Severity
LVH, EF
Severity Area Transvalvular gradient
Mild >1.5 <25
Moderate 1-1.5 25-50
Severe <1 50-80
Critical <0.7 >80
o Complications eg IE How would you manage?
Education Medical
o Antibiotic prophylaxis
o Rx complications such as arrythmias and CCF (caution with antihypt to avoid reducing preload) o Statins may have a role in reducing calcification of the aortic valve
Surgical treatment o Indications
Symptomatic and severe Asymptomatic but has
Area<0.6
LV systolic dysfunction Hypotension on exercise VT
LVH>15mm
Moderate AS but going for Sx for CABG, MVR or aortic root surgery o Options
Valve replacement (Sx of choice) Valvuloplasty (for moribund patients)
What are your thoughts on a young person with AS murmur but a normal aortic valve? Supravalvular stenosis
o Can be isolated or associated with Williams syndrome o It is an inherited disorder, autosomal dominant, Ch 7
o Features of elfin facies, hypertension and mental retardation with other cardiac lesions such as PS Subvalvular stenosis
What abdominal condition is associated with AS? Angiodysplasia of the colon (PR bleed) What is pulse pressure?
Difference between systolic and diastolic pressure Normal – 40mmHg
Wide - >60 mmHg Narrow - <25mmHg
14 6. Mixed Mitral Valve disease
Sir, this patient has mixed mitral valve disease and the predominant lesion is 1. Mitral stenosis
Early opening snap with long diastolic murmur Loud S1
No S3
Tapping apex beat that is not displaced Pulsus parvus pulse
2. Mitral Regurgitation
Displaced apex beat that is thrusting in nature Soft S1
S3
Jerky pulse
Mdm is a short diastolic murmur with no opening snap My findings are:
o Presence of a MDM heard best at the apex and accentuated at the left lateral position. It is a grade 4/6 murmur as it is associated with a diastolic thrill. It is severe as it is associated with an early opening snap and a long mdm. There is a loud first heart sound. There is also a MR murmur as evidenced by a PSM heard best at the apex. It is a grade 3/6 murmur and is not associated with any systolic thrill. There is also no third heart sound.
o Apex beat is not displaced o Pulmonary hypertension, CCF o IE, AF, over-anticoagulation o Mitral facies and Ortner‟s
o No lateral thoracotomy scars to suggest previous operation for MS o Requests
o In summary, this patient has mixed mitral valves disease with the predominant lesion being MS. This is complicated by pulmonary hypertension and AF. He is not in heart failure and signs of IE. The most likely cause in this patient is rheumatic heart disease.
o Presence of a PSM heard best at the apex; 4/6 murmur and is associated with a systolic thrill. There is a soft first hearts sound and a presence of a third heart sound. There is also a MS as evidenced by MDM heard best at the apex at the left lateral position. It is associated with a late opening snap and short mdm.
o Apex
o CCF, Pulmonary hypt o IE, AF
o Lateral thoracotomy scar o Marfan‟s and SLE o Requests o In summary Questions
What is the significance of a third heart sound in mixed mitral valve disease? o Presence of S3 implies no significant MS
What are the causes?
o Rheumatic heart disease
o MS with valvotomy done that has been complicated by MR
What are the frequencies of valvular involvement in rheumatic heart disease? o MV – 80%
o AV – 50%
o Mixed MV and AV – 20% o TV – 10%
7. Mixed Aortic Valve Disease
Sir, this patient has got mixed aortic valve disease and the predominant lesion is Aortic Stenosis
1. small volume pulse
2. heaving and undisplaced apex beat 3. Loud and harsh systolic murmur 4. associated with systolic thrill Aortic regurgitation
1. Collapsing pulse
2. Displaced and thrusting apex beat 3. Soft systolic murmur
4. No systolic thrill My findings are:
o Presence of an ESM heard best at he aortic area that radiates towards the carotids. It is a grade 4/6/ murmur and it is associated with a systolic thrill. It is severe as it is associated with an early ejection click with a long systolic murmur with late peaking. There is no S4 detected and the second heart sound is soft; I could not detect a paradoxial splitting of the second heart sound.
o There is also an EDM heard bset at the LLSE and is loudest in expiration with the patient sitting forwards. It is a grade 3/6 murmur and is not associated with any diastolic thrill. (skip the severity markers for AR)
o Apex o CCF
o IE, SR and small volume, haemolytic anaemia
o Request BP especially for narrow pulse pressure, Temperature chart and enquire symptoms of angina, syncope and dyspnea.
o In summary
o Presence of an EDM heard best at the LLSE and is loudest in expiration with the patient sitting forwards. It is a grade 3/6 murmur as it is not asssociatd with ay diastolic thrill. The second heart sound is soft and there is no third hear sound. There is also no mdm at the paex to suugest an Austin Flint murmur.
o There is also an ESM heard best at the aortic area that radiates towards the carotids. It is a grade 2/6/ murmur and is not associated with any systolic thrill. (skip the severity markers for AS)
o Apex o CCF
o IE, SR, collapsing, brachial dance and Corrigan‟s o No quinke, muller‟s de Musset‟s, Duroziez and Traube‟s o No Marfans, AS or RA
o Requests for BP especially for a wide pulse pressure, temperature chart. o In summary
Question
What are the causes of a mixed aortic valvular lesion? o Rheumatic heart disease
16 8. Prosthetic Heart Valves
Sir, this patient has got mechanical mitral/aortic valve which has been done for an underlying mitral/aortic stenosis/regurgitation. I say this because there presence of a mid-line sternotomy scar associated with audible metallic clicks to the unaided ear.
There is presence of a mitral valve replacement with a metallic first heart sound and a normal second heart sound. There is no pan-systolic murmur to suggest a valve leakage.
(There is presence of an aortic valve replacement as evidenced by a normal first heart sound followed by a metallic click and a metallic second heart sound. There is no early diastolic murmur or a collapsing pulse to suggest a valve leakage.)
(There are both mitral and aortic valve replacement as evidenced by dual metallic heart sounds. There is no pan-systolic murmur to suggest a mitral valvular leakage or an early diastolic murmur which indicates an aortic valve leakage.)
Ther metallic sounds are crisps (no valvular thrombosis) and there is no conjunctival pallor or jaundice to suggest hemolytic anaemia. The apex beat is displaced at the 6th IC at the ant axillary line. (Displaced and MVR = MR; undisplaced and MVR = MS; Displaced and AVR = AR). There is no evidence of pulmonary hypt(MVR). Patient is in CCF as evidenced by presence of bibasal crepitations, raised JVP of 3 cm and bipedal edema.
Patient is not in AF(MVR) and pulse is not collapsing in nature (mention this if AVR for leakage). There is no peripheral stigmata of IE such as clubbing, Janeway‟s lesion, Osler‟s nodes or splinter haemorrhages. This is associated with bruises which suggest overanticoagulation.
There is no evidence of any Marfan‟s, RA, AS or Syphilis (mention this if AVR for AR or MVR for MR)
I would like to complete my examination by taking the BP of the patient and looking at his temperature chart and neurological examination for strokes.
In summary, this patient has got MVR/AVR or both which is most likely done for MR/MS/AR/AS (which is due to underlying Marfan‟s syndrome). There is no clinical evidence of valvular leakage, thrombosis or haemolytic anaemia. There is also no pulm hypt but pt is in heart failure and in AF. There are no signs of IE or overanticoagulation.
Questions
What are the indications of a mitral/aortic valve replacement? o See respective MS/MR/AS/AR
What are the types of prosthetic valves? Mechanical valves
Ball and cage valve (Starr-Edwards) Single tilting disc (Bjork-Shiley) Double tilting disc (St Jude) Bioprosthetic – Homograft or heterograft What are their differences?
Duration
Mechanical valves last 20-30yrs Bioprosthetic may fail within 10-15 years Thrombogenecity
Mechanical require lifelong anticoagulation (Starr-Edwards>single disc>double disc) Bioprosthetic does not require lifelong anticoagulation
Therefore in the young and those who already require long term anticoagulation, mechanical valves preferred And in the elderly(lifespan <10-15 years) or those that cannot tolerate anticoagulation, bioprosthetic valve preferred What are the complications?
Complications of prosthesis
Valve leakage (mild- hemolytic anaemia, severe – CHF) Valve thrombosis
Valve strut failure (rare, acute presentation with high mortality, Bjork-Shiley)
Hemolytic anaemia (from valvular leakage due to partial dehiscence; Rx with Fe, folate, transfusions, B blockers or if fit for op, repair of valve replacement)
Complications of valvular heart disease Infective endocarditis
Congestive cardiac failure
Thromboembolism (rule out IE and thrombosis) Complications of management
Overanticoagulation Bleeding
What are the causes of anaemia in such patients? Bleeding from anticoagulant
Hemolytic anaemia Infective endocarditis
How do you tell clinically that the valve has malfunction? New murmur
Change in characteristic of a preexisting murmur Change in intensity or characteristic of an audible sound
How would you investigate a patient suspected of having valve dysfunction? Cinefluoroscopy – rapid, fast ad inexpensive for structural integrity
TTE – often difficulty study due to reverberations from the metal TEE – useful for assessing MV prosthesis but limited in AV prosthesis Can MRI be done for a patient with mechanical heart valves?
Yes it is safe except those with pre 6000 Starr-Edwards prosthesis (1960-64) Valve thrombosis
Up to 5% per patient-year
Factors – inadequate anticoagulation and mitral location Manisfest as
pulmonary congestion, poor peripheral perfusion or systemic embolisation, acute deterioration Change in audible sounds or murmur
Ix shows reduced movement of the disc or poppet, reduced orifice area, increased regurgitation or transvulvular pressure Mx
<5mm – IV heparin
18 9. VSD
(Clue: Young patient; look for associated conditions of Down and Fallot‟s Tetrology) Presentation
Sir, this patient has got a Ventricular Septal Defect that is hemodynamically significant as evidenced by:
Presence of a pan-systolic murmur heard best at the left lower sternal edge with radiation towards the right side of the sternum. This murmur can be heard at the apex but there is no radiation to the axilla. It is louder on expiration. It is a grade of 5/6 murmur and is associated with a systolic thrill. The first heart sound is not soft. I did not detect any third heart sound.
I did not detect any early diastolic murmur at the left lower sternal edge to suggest an associated AR. This is also no associated mid-diastolic apical rumble at the apex to suggest a flow murmur at mitral valve which can be a/w VSD.
Apex beat is displaced and is located at the anterior axillary line at the 6th IC. It is thrusting in nature.
No evidence of Eisenmenger‟s syndrome such as central cyanosis, clubbing. There is evidence of pulmonary hypertension such as palpable or loud P2, no parasternal heave.
There are no signs of CCF such as bilateral pedal edema, no basal crepitations or raised JVP; she is comfortable at rest with a RR of 14 bpm and does not require any supplemental oxygen.
There is no peripheral stigmata of IE. The pulse is regular at 70 bpm and character of the pulse is normal. There are no features to suggest Down syndrome or (Turner syndrome - if female).
I would like to complete my examination by looking at the patient‟s temperature chart and taking his blood pressure.
In summary, this young man has got a VSD that is severe with a displaced apex beat and is complicated by pulmonary hypertension. Clinically, there is no heart failure or Eisenmenger‟s syndrome or IE. The most likely cause is congenital VSD. Dy/Dx – MR, TR, VSD – For VSD, murmur radiates to the right of the sternum, young patient and a palpable thrill
Questions
What are your differential diagnoses for a PSM?
o MR – PSM at apex radiates towards the axilla, soft S1
o TR – PSM heard at the triscupid area, louder on inspiration; usually secondary to pulmonary hypertension or seen in IVDAs; Giant V wave, pulsatile liver
o VSD – PSM heard at the LLSE which is louder on expiration What are the causes of a VSD?
o Congenital o Acquired: MI How common is VSD?
o The most common congenital heart condition o 2 per 1000
o Usually in the membranous portion (can also be found in the muscular) o Small defects close spontaneously in early childhood in about 50% What are the types of VSD?
o Supracristal (above the crista supraventricularis) o Infracristal o Upper membranous o Lower muscular (<5%) o Different morphology o Maladie de Roger o Swiss cheese o Large
o Gerbode defects (opens into the RA) What are the conditions in which VSD is part of?
o Fallot‟s tetralogy o Truncus arteriosus o AV canal defects o DORV (double outlet RV) What are the complications of VSD?
o AR
o Pulmonary Hypt o Eisenmenger‟s complex o CCF
Does the loudness of the murmur correlate with severity?
o No; in fact, a small VSD results in a louud murmur and the converse is true. What is Maladie de Roger?
o A term used to describe a small VSD that is hemodynamically insignificant with normal heart size, ECG and CXR; it is a loud murmur on ausculatation
How do you differentiate an isolated VSD with one that is associated with Fallot‟s tetralogy? o Pulmonary thrill, PS murmur
o Clubbed and central cyanosis (but could be VSD with Eisenmenger‟s) How do you differentiate VSD from HOCM?
o ESM rather than PSM
o Apex is not displaced, double apical impulse o Jerky pulse
How would you Ix? o ECG
o Normal in small defects o LVH, RVH, p mitrale
o Pulmonary hypertension – P pulmonale, RAD o CXR
o Normal in small defects o Cardiomegaly, LA and LVH o Pulmonary plethora initially
o Pulmonary hypertension later with prominent pulmonary trunks, rapid tapering of the peripheral pulmonary arteries and oligaemic lung fields
o CCF o Echocardiogram
o Diagnostic
o Determine severity and direction of shunt via color doppler How would you manage?
o Counsel o Medical
o Antibiotic prophylaxis o Rx complications of CCF o Surgical
o Small, asymptomatic and normal pulmonary pressure do not need surgery o Indications
Evidence of pulmonary hypertension or CCF Right ventricular pressure >50 mmHg
Right to left flow ratio or pulmonary to systemic resistance ratio >1.5 Recurrent IE
Cx by AR
Acquired cause eg rupture of septum form MI o Contraindication
Development of Eisenmenger o Types
Surgery
20 10. ASD
Presentation
Sir, this patient has atrial septal defect as evidenced by presence of a wide and fixed splitting of the second heart sound.
There is presence of an ejection systolic murmur over the pulmonary area which is louder on inspiration, implying presence of a pulmonary systolic murmur. This is a grade 3/6 murmur and there is no associated systolic thrill.
There is no associated mid-diastolic flow murmur to suggest relative tricuspid stenosis or Lutembacher‟s syndrome (Acquired MS and ASD). There was also no associated PSM to suggest an ostium primum defect (TR, MR, VSD).
The apex beat is not displaced and is located in the 5th IC space just medial to the mid-clavicular line.
There is no complication of Eisenmenger‟s syndrome; there is no evidence of pulmonary hypertension; is not clubbed and no central cyanosis. There is also no evidence of congestive cardiac failure.
There are no stigmata of infective endocarditis. Patient is in atrial fibrillation with an irregularly irregular pulse and is rate controlled at a rate of 84 bpm; there are also no bruises to suggest over-anticoagulation.
There is no evidence of any thumb defects to suggest Holt-Oram syndrome. The patient also does not features of Down‟s syndrome.
I would like to complete my examination by examining patient‟s chest for pneumonia as patients are prone to recurrent chest infections as well as a neurological examination to look for evidence of stroke due to paradoxical embolus.
In summary, this patient has got an ASD with complications of AF. There are no complications of pulmonary hypertension, heart failure or Eisenmenger‟s syndrome. There is also no infective endocarditis. This patient has ASD is most likely due to an ostium secundum atrial septal defect which is a congenital heart condition.
Questions
What are the types of ASDs? o Ostium secundum type
o 90%
o common congenital heart condition o Most remain asymptomatic
o If small <2 cm, normal life expectancy with no symptoms
o Larger defects may present in the second or third decades with dyspnea or fatigue o defect in the fossa ovalis with no involvement of the AV valves
o Ostium primum type o 10%
o Failure of fusion of the septum primum with the endocardial cushions o AV valves affected – MR, TR and VSD
o Sinus venosus type
o Defect in the septum just below the entrance of the SVC (inverted P waves in the inferior leads) How do patients present?
o Secundum
o Asymptomtic o Symptomatic
Fatigue, dyspnea Right heart failure AF
Recurrent pulmonary infections Paradoxical emboli
o Primum
o In addition to the above
Failure to thrive, poor development IE
Syncope (heart block) What are the complications of ASD?
o Pulmonary hypertension, heart failure, Eisenmenger‟s o AF, IE (primum defects)
o Recurrent chest infection, paradoxical emboli
What are the various types of murmurs that can be associated with ASD and what do they mean?
o Pulmonary ejection systolic murmur and mid-diastolic murmur at the triscuspid area implies increased flow of blood through the pulmonary and triscupid valve respectively due to left to right shunting of blood via the ASD
o MS murmur means acquired Rh heart disease affecting the mitral valve in Lutembacher‟s syndrome o MR, TR or VSD murmur implies that ASD is of the ostium primum type
What is the mechanism of a split second heart sound?
o A split S2 is caused physiologically during inspiration because the increase in venous return overloads the right ventricle and delays the closure of the pulmonary valve
Why is there wide and fixed splitting of the second heart sound in ASD?
o With an atrial septal defect, the right ventricle can be thought of as continuously overloaded because of the left to right shunt, producing a widely split S2, with the pulmonary valve closing much later cf to the aortic valve
o It is fixed because the atria are linked via the defect, inspiration produces no net pressure change between them, and has no effect on the splitting of S2
How do you differentiate between a flow mumur through the pulmonary valve vs a PS murmur? o PS murmur is a/w P2 that is soft, delayed and varies with respiration
What are the conditions that can cause a wide splitting of the second heart sound? o Increase RV volume – ASD, VSD, PR
o Increase RV pressure – PS o RV conduction delay –RBBB o Increase LV emptying – MR, VSD What is Eisenmenger‟s syndrome?
o It implies a reversal of a left to right shunt as a result of the development of pulmonary hypertension o This occurs in conditions such as ASD, VSD or PDA
o Patients are markedly clubbed and deeply cyanosed
o The defect must not be repaired once this complication occur due to high mortality risk What is Lutembacher‟s syndrome?
o Acquired Rh MS o ASD
What is Fallot‟s trilogy? o ASD
o RVH o PS
What is Holt-Oram syndrome? o ASD secundum type
o Hypoplastic thumb with accessory phalanx o Autosomal dominant
How would you investigate? o ECG
o Secundum – Partial RBBB, RAD o Primum – LBBB, LAD, low atrial rhythm o Sinus venosus – inverted P in inferior leads o Pulmonary hypertension – p pulmonale, RVH o CXR
o Cardiomegaly
o Pulmonary hypertension
Prominent pulmonary trunk Enlarged RA and RV
o Shunt vascularity/pulmonary plethora (well visualised pulmonary arteries in the periphery of the lung o Small aortic knob
o Echocardiogram
o Diagnosis - demonstrate the defect o Cardiac catheterisation
o Determine the severity and direction of shunt How would you manage?
o Counsel o Medical
o No antibiotic prophylaxis required, repaired or unrepaired o Treatment of complications such as heart failure and AF
o May consider anticoagulation if there is evidence of bidirectional shunting to prevent strokes from paradoxical emboli
o Surgery
o Early childhood – closure is recommended at 5-10 years of age to prevent complications o Small ASDs can be left alone(5 mm or less)
o Large ASDs or pulmonary to systemic flow ratio>1.5
o Closed surgically or transcatheter button or clam-shell devices
o Closure prevents pulmonary hypertension and RHF but does not alter incidence of AF How would you counsel a patient with ASD who intends to get pregnant?
o Pregnancy is well tolerated in patients with small and hemodynamically insignificant ASD
o For large defects with pulmonary hypertension, Eisenmenger‟s syndrome, avoid pregnancy as there is increase morbidity and mortality both to fetus and mother
22 11. HOCM
Presentation
Sir, this patient has got Hypertrophic obstructive cardiomyopathy.
There is presence of a ESM heard best at the LLSE. It is a grade 3/6 murmur as it is not associated with any systolic thrill. In addition, there is presence of a MR mumur with a PSM heard bst at the ap3ex beat and radiates to the axilaa. It is a grade 4/6/ murm,ru as it is associated with a systolic thrill. The first heart sound is soft and there is no associated third or fourth heart sounds. The apex beat is not displaced located at the 5th IC space just medial to the midclavicular line. It has a double apical impulse (say this only if not if AF).
There are no complications of congestive cardiac failure. However the JVP is raised at 3 cm with a prominent „a‟ wave.
Examination of the peripheries did not show any stigmata of infective endocarditis. He is is SR at a pulse rate of 84 bpm and has a characteristic bifid pulse (only if not in AF; if in AF, say shrap, rising and jerky pulse)
I did not notice any clinical features to suggest Friederich‟s ataxia.
I would like to complete my examination by taking performing the valsalva manoeuvre or standing to accentuate the murmurs as well as take the patient‟s blood pressure and look for fever from the temperature chart. A neurological examination would be useful to screen for any signs of stroke.
In summary, this patient has got a HOCM with an ESM and MR murmur associated with a double apical impulse, bifid pulse and a raised JVP with prominent „a‟wave. There are no complications of heart failure, AF or IE. This is a genetic condition.
Questions What is HOCM?
o Hypertrophic Cardiomyopathy
o Genetic cardiac disorder caused by missense mutation in the genes that encode proteins of the cardiac sarcomere; autosomal dominant
o Resulting in hypertrophy of the ventricular septum with LV outflow tract obstruction o 1 in 500, male:female 1:1
o Variable penetrance o Variable expression
o Asymptomatic (majority) o Symptomatic
o Angina, syncope, dyspnea, palpitations
o Sudden death (Ventricular fibrillation) (overall annual mortality in 1%) o Complications of CCF, AF, IE and thromboembolic stroke
Why is there a „double apical impulse‟?
o Presence of a LV heave with a prominent presystolic pulse caused by atrial contraction o A differential diagnosis is LV aneurysm
Why is there a prominent „a‟ wave?
o Due to forceful atrial contraction against a non-compliant right ventricle What is Brockenbrough-Braunwauld-Morrow sign?
o Reduced pulse pressure in the post-extrasystolic beat o Occurs in HOCM and AS
What are the causes of HOCM? o Familial
o Friederich‟s Ataxia o Idiopathic
How would you investigate? o ECG
o Normal in 25%
o Tall QRS in precordial leads with ST-T changes, Q in inf and lateral leads o LAD o AF o CXR o Normal o LA enlargement, LVH o Echocardiogram o Diagnostic
Asymmetrical septal hypertrophy
Systolic anterior motion of the anterior mitral valve leaflet Diastolic dysfunction
o Severity
Septal thickness >18mm
Outflow tract gradient > 40mmHg as rest o Complications
MR IE o TMX for those with angina
o Holter monitoring looking for arrythmias especially presence of VT How would you predict poor outcome?
o Family history of sudden death o History of syncope, cardiac arrest o Poor BP response to exercise
o Holter monitoring with ventricual arrythmias detected, esp spontaneous VT o Echo findings
How would you treat?
o Education and counselling with screening of first degree relatives o 50% chance of being affected
o Screen with ECG and 2D echo
Annually for adolescent (12-18) And 5 yearly
o Treatment is directed at symptom relief and prevention of sudden cardiac death o Relief symptoms
Beta blockers
If cannot tolerate, verapamil but caution I patients with sever symptomatic obstruction because of increase death especially after first few doses
Beta blockers and disopyramide o Rx complications
Rx CCF Rx AF
Rx and prevention of IE Prevention of sudden death
Amiodarone
Pacing (dual-chamber pacing) o Septal ablation with alcohol or surgery
24 12. Approach to Central cyanosis and Clubbing
Examination
o On detecting this, concentrate on
o Differential cyanosis and clubbing (ULs vs LLs or right LL vs others where the LLs are cyanosed and clubbed) o Look for weak L radial pulse (BT shunt)
o Shunt scar (BT shunt) o On auscultation determine if
o Eisenmenger
ASD, VSD, PDA No PS
Has pulmonary hypertension (loud and palpable P2) and RVH Check single (VSD) or fixed splitting (ASD)
o Fallot‟s tetralogy
PS murmur (No VSD murmur as this is non restrictive) No pulmonary hypertension but has RVH
Presentation
o Sir this patient has got VSD/ASD/PDA complicated by Eisenmenger‟s complex. o Eisenmenger‟s because - clubbed and central cyanosis and Pulmonary Hypertension o The underlying cause is ASD/VSD/PDA because - second heart sound is crucial
o ASD – fixed spitting second heart sound o VSD – Single second heart sound
o PDA – reversed splitting second heart sound (split on expiration) o No PS murmur to suggest ToF
o Apex beat o CCF o IE stigmata o Pulse
o Peripheral presentation for ASD/VSD/PDA
o (For Eisenmenger‟s syndrome, will have pulmonary hypertension and therefore look for TR and PR murmur) o Also state complications of polycythaemia, venesection marks
o Requests o Summary
o Sir this patient has ToF with a BT shunt done previously o Clubbed and centrally cyanosis
o Presence of PS murmur and shunt murmur
o PS murmur – ESM heard best at the pulmonary area 4/6 and systolic thrill o Shunt murmur (continuous murmur)
o (No VSD murmur as it is large and non-restrictive) o RVH with left parasternal heave
o Apex beat
o No pulmonary hypertension (no loud P2)
o Cor pulmonale – raised JVP, pedal oedema, no lung crepitation o No IE
o Pulse
o Presence of a thoracotomy scar with a weak left radial pulse suggesting BT shunt o Venesection marks, polycythemia
o Requests o In summary Questions
How do you differentiate ToF vs Eisenmenger‟s syndrome? o ToF has PS murmur with systolic thrill and a soft P2
o ToF no pulmonary hypertension (CXR ToF has small pulmonary aretrial trunks) What are the characteristic findings of a PDA?
o Collapsing pulse
o Continuos murmur heard best just below the left clavicle and radiates to the back What are the differential diagnoses for a continuous murmur?
o Collapsing pulse o PDA o MR with AR o VSD with AR o No collapsing pulse o BT shunt
o Venous hum (right of the sternum, children, disappears when lie flat or right JVP occluded) What is ToF?
o Congenital heart condition comprising of o VSD, RVH, Overriding aorta, PS
13. Dextrocardia Examination
After the routine examination
Request to examine the abdomen for a liver on the left side of the abdomen for situs inversus Presentation
Sir this patient has dextrocardia as evidenced by: o Right apex beat
o Heart sounds that are better heard on the right than on the left The heart sounds are normal and there are no murmurs detected.
Apex is not displaced located at the right 5th IC just medial to the midclavicular line and has a normal characteristic. She is in SR with a rate of 84bpm
On examination of his lungs posteriorly, there was no evidence of coarse late inspiratory crepitations to suggest bronchiectasis and patient does not have a nasal voice to suggest sinusitis. (Katargener‟s syndrome)
There is no evidence of Turner‟s syndrome. (mention this only if female!)
I would like to complete my examination examining the abdomen for a left sided liver for situs inversus. In summary this patient has got dextrocardia and is well clinically and is of congenital etiology.
Questions
What is the significance of situs inversus in patients with dextrocardia? o It usually implies that there is no significant cardiac malformation What conditions is dextrocardia associated with?
o Kartagener‟s syndrome – a type of immotile ciliary syndrome o Triad of
Bronchiectasis
Sinusitis, otitis media and dysplasia of the frontal sinuses Infertility
o Turner‟s syndrome
o Asplenia – PBF may show Heinz bodies and Howell-Juoly bodies What is situs inversus?
o Right sided apex and right descending aorta o Left lung having 3 lobes and right lung with 2 lobes o Left sided liver and right sided stomach
o Right descending colon What is dextroversion?
o Right sided apex and left sided descending aorta o Left sided stomach
What is levoversion?
o Left sided apex and right sided descending aorta o Right sided stomach
26 RESPI!
14. Bronchiectasis Presentation
Sir, this patient has got bronchiectasis affecting both lower lobes as evidenced by late, coarse inspiratory crepitations heard best posteriorly in the lower one third bilaterally. Patient has a productive cough with large volume of purulent sputum with hemoptysis associated with clubbing.
Chest excursion was reduced bilaterally with a normal percussion note and vocal resonance. Trachea is central and the apex beat is not displaced.
There are no signs to suggest presence of COPD.
(There is concomitant COPD with a reduced chest excursion bilaterally, hyperinflation of the chest associated with hyperresonance on percussion with loss of liver and cardiac dullness. There is presence of ronchi and a prolonged expiratory phase. Vocal resonance is normal. Trachea is central and apex beat is not displaced.)
There is complication of pulmonary hypertension with a loud and palpable component of the second heart sound associated with a left parasternal heave. There is also cor pulmonale with a raised JVP of 3 cm with prominent a wave associated with bilateral pedal oedema. Clinically there are no signs of polycythemia such as plethoric facies or conjunctival suffusion.
He is not in respiratory distress (with a RR of 14 bpm without use of accessory muscles of respiration). There are no signs of respiratory failure (he does not require any supplemental oxygen and there is no central cyanosis; there is also no flapping tremor of the hands and no bounding pulse). There is also no nicotine staining of the fingers, patient is not cachexic looking and no enlarged Cx LNs.
With regards to aetiology, there is no dextrocardia or a nasal voice to suggest possible Kartagener‟s syndrome. In addition, there is no symmetrical deforming polyarthropathy to suggest RA or any cutaneous signs of SLE. There is no kyphoscoliosis.
With regards to treatment, patient has a steroid metered-dose inhaler, salbutamol and ipratropium metered-dose inhalers by the bed side.
I would like to complete the examination by looking at the temperature chart for fever as well as an abdominal examination to look for splenomegaly from amyloidosis which can result from bronchiectasis. A neurological examination is useful to screen for deficit as patients are prone to brain abscesses.
In summary, this patient has bronchiectasis affecting both lower lobes with complications of pulmonary hypertension and cor pulmonale. There is no concomitant COPD and no polycythemia. He is clinically not in respiratory failure. The possible causes for this patient‟s bronchiectasis are post infective causes such as post viral, bacterial, TB or ABPA, connective tissue disease such as RA or SLE, congenital conditions such as cystic fibrosis, Kartagener‟s syndrome or hypogammaglobulinemia.
