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MRCP PACES by Jansen Koh (http://jansenkoh.com/MRCPnotes.htm )

CARDIO

LOWER LIMBS (NEURO)

  • 1. Mitral regurgitation 2

  • 51. Lower limbs overview 116

  • 2. Mitral valve prolapse 7

  • 52. Flaccid paraparesis 118

  • 3. Mitral stenosis 8

  • 53. Spastic paraparesis 123

  • 4. Aortic regurgitation 10

  • 54. Peripheral neuropathy 129

  • 5. Aortic stenosis 12

  • 55. Charcot‟s Joint 131

  • 6. Mixed mitral valve disease 14

  • 56. Proximal myopathy 133

  • 7. Mixed aortic valve disease 15

  • 57. Brown-sequard syndrome 135

  • 8. Prosthetic heart valves 16

  • 58. Footdrop 136

 
  • 9. VSD 18

  • 59. Hemiparesis/Hemiplegia 137

10.

ASD 20

  • 60. Gait assessment 140

11.

HOCM 22

  • 61. Parkinsonism 141

12.

Approach to central cyanosis and clubbing 24

13.

Dextrocardia 25

RHEUMATO

 
  • 62. Rheumatoid arthritis 144

 

RESPI

  • 63. Gouty hands 146

14.

Bronchiectasis 26

  • 64. Psoriatic hands 149

15.

Interstitial lung disease 30

  • 65. OA of the hands 151

16.

COPD 33

  • 66. Scleroderma 153

17.

Pleural effusion 35

  • 67. Ankylosing Spondylitis 155

18.

Collapse 38

  • 68. Marfan syndrome 157

19.

Consolidation 40

  • 69. Dupytren‟s contractures 160

20.

Lobectomy/Pneumonectomy 44

  • 70. Clubbed fingers 160

21.

Approach to lateral thoracotomy scar 45

  • 71. Painful/swollen knee joint 161

22.

Lung transplant 46

  • 72. Still‟s disease/Juvenile chronic arthritis 161

 
  • 73. Enteropathic arthropathy 161

 

ABDO

  • 74. Old rickets 162

23.

Chronic liver disease 48

24.

Hepatomegaly 54

ENDOCRINE

25.

Splenomegaly 56

  • 75. Acromegaly 163

26.

Hepatosplenomegaly 58

  • 76. Cushing‟s syndrome 165

27.

Ascites 60

  • 77. Goitre 168

28.

Unilateral enlarged kidney 63

  • 78. Paget‟s disease 174

29.

Bilateral enlarged kidney 64

  • 79. Panhypopituitarism (Simmond‟s disease) 176

30.

Transplanted kidney 67

  • 80. Addison‟s disease 177

 
  • 81. Gynaecomastia 177

 

NEURO

31.

Approach to examination of the face 70

DERM

32.

Cranial nerve syndromes 71

  • 82. Dermatology overview 178

33.

Isolated III nerve palsy 74

  • 83. Psoriasis 181

34.

Isolated VI nerve palsy 76

  • 84. Lichen planus 183

35.

VII nerve palsy 79

  • 85. Neurofibromatosis 185

36.

Myasthenia gravis 82

  • 86. Purpura 187

37.

Approach to examination of the eyes 85

  • 87. Dermatomyositis 189

38.

Gaze palsies 86

39.

Unilateral ptosis 87

EYE

40.

Bilateral ptosis 89

  • 88. Diabetic retinopathy 191

III, IV and VI cranial nerve palsies (33, 34, 35)

  • 89. Hypertensive retinopathy 195

41.

Assessment of higher cortical function 90

  • 90. Optic atrophy 197

 
  • 91. Papilloedema 199

UPPER LIMBS (NEURO)

  • 92. Central and branch retinal vein occlusion 201

42.

Upper limbs overview 92

  • 93. Central retinal artery occlusion 203

43.

Radial nerve palsy 94

  • 94. Retinitis pigmentosa 204

44.

Median nerve palsy 96

  • 95. Visual field defects 205

45.

Ulnar nerve palsy 99

  • 96. Visual acuity 207

46.

Wasted hands 101

  • 97. Cataracts 207

Peripheral neuropathy (54)

  • 98. Nystagmus 207

47.

Syringomyelia 104

  • 99. Pupillary defects 208

48.

Dystrophica myotonica 106

49.

Cerebellar signs 109

OTHERS

50.

Chorea 114

100. History-taking station 209

Parkinsonism (61)

CARDIO!

1. Mitral Regurgitation

Presentation

Sir, this gentleman has mitral regurgitation that is moderately sever e 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 so und 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

  • 6 th

IC at the anterior axillary line.

This is complicated by pulmonary hypertension as evidenced b y 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 puls e 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 temperatu re 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 fibrillatio n and pulmonary hypertension. There are no complications of heart failure or infective endocarditis. My differential diagnoses include IH D, MVP and Rh heart disease. (If thoracotomy scar, think of mitral valvotomy for MS)

Questions How do you grade the severity of mi tral 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

Anterior leaflet: radiates to axilla and back

Left ventricular dilatation, cardiomyopathy, Marfan‟s, Rheumatoid, AS

Acute causes: MI, IE, Trauma, Surgery, spontaneous rupture

 

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 systoli c 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 murmu r?

Mitral Regurgitation

 
 

Triscupid Regurgitation PSM heard best at the LLSE

PSM heard best at Apex 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 JVP unless complication

Normal pulse character Giant V wave with pulsatile liver

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 Soft S1

Harsh, low pitched 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
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 acros s 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 displace d

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

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

Indications

Types of surgery

Treatment of underlying cause eg IHD, dilated CMP (Rx of CCF and afterload reduction)

Treatment of complications eg AF, IE, CCF

Surgical

Symptomatic or

EF<60% or

LV end-systolic dimension >45mm

Mitral valve repair if technical feasible is b est

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

Minor

Positive echocardiogram: vegetations, abscesses, valve perforation, dehiscence

New valvular regurgitation

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 cri teria

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 endoca rditis

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

Prosthetic valves esp if unstable or early(<60 days) or cau sed by S aureus

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 dis
Risk stratify
Highest risk: prosthetic valves, both bioprosthetic and mechanical; previous IE; congenital cyanotic heart dis ease; 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 se ptal 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 azithromyc in 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 genitourina ry
tract procedures are for preventing enterococcal endocarditis. They are as follows:

High Risk regimens recommended by the Americ an 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 penicillin s 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 Asymptomatic regurgitation is a serious disease with a 5 -yr death rate of 22-33% form cardiac adverse events

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 crescen do-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 accent uate 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 sy stole

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 MV P?

o

o

Myxomatous degeneration of the mitral valve tissue

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

o

leaflets from prolapsing into the LA Hence the systolic click occurs earlier with a longer duration of the systolic murmur

How do patients with MVP present?

o

o

o

o

Asymptomatic Symptomatic o Palpitations

Anxiety Atypical chest pain Light-headedness Complications of MR

o

o

o

CCF fatigue and dyspnea o IE o Arrhythmias

o

Embolic phenomenon Sudden death

o

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

o

o

Symptomatic

Rx underlying cause or associations

Palpitations Rx with beta blockers (benign ventricular ectopy)

Rx complications such as MR with CCF, IE or AF or TIA

o

Surgical

o

As for MR

3. Mitral Stenosis

Presentation

Sir, this patient has mitral stenosis which is severe in nature with complications of infective endocarditis and congestive cardia c 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 lon g 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 5 th intercostal space.

There is associated pulmonary hypertension with a palpable pulmonary component of the second heart sound with left parasterna l 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 S teel 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 lesion s on the palms and Osler‟s nodes noted on the pulp of t he 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 org anism 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 a s taking the patient‟s BP.

In summary, this patient has MS that is severe in nature with complications of atrial fibrillation, pulmonary hypertension co ngestive cardiac failure and infective endocarditis secondary to MSSA. The possible causes for MS incl ude 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 ventricu lar contraction

What causes presystolic accentuation of the murmur?

Occurs in sinus rhythm only during atrial systole whic h 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 dyspne a, PND, orthopnea Less frequently with right sided heart failure, hemoptysis and hoarseness of voice, although this symptoms are more specific

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

o

Primary prevention: Rx with IM Benzathine Pen G 1 dose or 10 days of Pen V

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

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 o f 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 6 th 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 bilat eral peal edema.

Peripheral examination showed no evidence of IE. The pulse is bounding and collapsing in nature at a rate of 90 b pm 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 si gns 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 ca uses 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 o f 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

Tapping apex beat, which is not displaced

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, pulmonar y 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?

4% develop symptoms, CCF or both annually

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 systoli c murmur a/w with a systolic thrill. It is severe as there is an ea rly 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 he art sound. The apex beat is heaving in nature and is disp laced, located at the 6 th 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 ed ema 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 ta rdus in nature. There are no features suggestive of haemolytic anaemia with no c onjunctival 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 angi na, 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. Th ere is no evidence of infective endocarditis or haemolytic anae mia. 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 p ulmonary, 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

4 th 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

Dyspnea (Most impt)

2

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 p erson

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, 1 st degree heart block, LBBB

 

CXR Calcified aortic valve, cardiomegaly, pulmonary conges tion

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

o

o

Antibiotic prophylaxis

o

Rx complications such as arrythmias and CCF (caution with antihypt to avoid reducing preload)

Statins may have a role in reducing calcification of the aortic valve

Surgical treatment

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

o

o

Can be isolated or associated with Williams syndrome

It is an inherited disorder, autosomal dominant, Ch 7

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

Note: There is no official definition but studies usually measures the pulse pressure as a continuum

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 accent uated 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 lo ud 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

o

o

o

Apex beat is not displaced Pulmonary hypertension, CCF IE, AF, over-anticoagulation

Mitral facies and Ortner‟s

o

o

o

No lateral thoracotomy scars to suggest previous operation for MS Requests 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 sign s of IE. The most likely cause in this patient is rheumatic heart disease.

o

o

o

o

o

o

o

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 al so 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. Apex CCF, Pulmonary hypt IE, AF Lateral thoracotomy scar

Marfan‟s and SLE

Requests

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 involvemen t in rheumatic heart disease?

o

MV 80%

o

AV 50%

o

Mixed MV and AV 20%

o

TV 10%

o

PV -1%

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 undis placed 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 ar ea that radiates towards the carotids. It is a grade 4/6/ murmur and it is

o

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 secon d heart sound is soft; I could not detect a paradoxial splitting of the second heart sound. 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

o

murmur and is not associated with any diastolic thrill. (skip the severity markers for AR) 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

o

dyspnea. 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.

o

There is also no mdm at the paex to suugest an Austin Flint murmur. 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

o

associated with any systolic thrill. (skip the severity markers for AS) 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

o

Biscupid aortic valve

8. Prosthetic Heart Valves

Sir, this patient has got mechanical mitral/aortic valve which has been done for an underlying mitral/aortic stenosis/regurgi tation.

I say this because there presence o f 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 n o 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 6 th 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). Ther e 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 pul m 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 anticoagulati on, 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 malfun ction?

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 struc tural 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 S tarr-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

>5mm Fibrinolysis (if high operative mortality) or valve replacement

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 fi rst 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 assoc iated mid-diastolic apical rumble at the apex to suggest a fl ow murmur at mitral valve which can be a/w VSD.

Apex beat is displaced and is located at the anterior axillary line at the 6 th IC. It is thrusting in nature.

No evidence of Eisenmenger‟s syndrome such as central cyanosis, clubbing. There is evidence of p ulmonary 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 oxy gen.

There is no peripheral stigmata of IE. The pulse is regular at 70 bpm and character of the pulse is normal. There are no feat ures to suggest Down syndrome or (Turner syndrome - if female).

  • I would like to complete my examination by looking at the pa tient‟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 I E. 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? MR PSM at apex radiates towards the axilla, soft S1 TR PSM heard at the triscupid area, louder on inspiration; usually secondary to pulmonary hypertension or seen in IVDAs; Giant V wave, pulsatile liver VSD PSM heard at the LLSE which is louder on expiration

o

o

o

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

o

Upper membranous

Lower muscular (<5%)

o

Different morphology

o

o

o

o

Maladie de Roger

Swiss cheese

Large

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

o

IE

Does the loudness of the murmur correlate with severity?

o

No; in fact, a small VSD results in a lo uud 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

o

o

Normal in small defects

LVH, RVH, p mitrale

Pulmonary hypertension P pulmonale, RAD

o

CXR

o

o

o

o

Normal in small defects

o

o

o

Cardiomegaly, LA and LVH

Pulmonary plethora initially

Pulmonary hypertension later with prominent pulmonary tru nks, rapid tapering of the peripheral pulmonary

arteries and oligaemic lung fields CCF

o

Echocardiogram

Diagnostic

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

Percutaneous transcatheter

10. ASD

Presentation

Sir, this patient has atrial septal defect as evidenced by presence of a wide and fixed spli tting 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 thri ll.

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 5 th 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 e vidence 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. Th ere 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

o

o

o

o

common congenital heart conditi on

Most remain asymptomatic

If small <2 cm, normal life expectancy with no symptoms

Larger defects may present in the second or third decades with dyspnea or fatigue

defect in the fossa ovalis with no involvement of the AV valves

o

Ostium primum type

o

10%

o

o

Failure of fusion of the septum primum with the endocardial cushions

AV valves affected MR, TR and VSD

o

Sinus venosus type Defect in the septum just below the entrance of the SVC (inverted P waves in the inferior leads)

o

How do patients present?

o

Secundum

o

o

Asymptomtic

Symptomatic

Fatigue, dyspnea

Right heart failure

AF

Recurrent pulmonary infections

Paradoxical emboli

o

Primum

o

In addition to the above

IE

Failure to thrive, poor development

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

o

through the pulmonary and triscupid valve respectively due to left to right shunting of blood via the ASD MS murmur means acquired Rh heart disease affecting the mitral valve in Lutembacher‟s syn drome

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

o

shunt, producing a widely split S2, with the pulmonary valve closing much later cf to the aortic valve 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 suc h 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

o

o

o

Secundum Partial RBBB, RAD

Primum LBBB, LAD, low atrial rhythm

Sinus venosus inverted P in inferior leads

Pulmonary hypertension p pulmonale, RVH

o

CXR

o

o

Cardiomegaly

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 Diagnosis - demonstrate the defect

o

o

Cardiac catheterisation Determine the severity and direction of shunt

o

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

o

o

Early childhood closure is recommended at 5 -10 years of age to prevent complications

Small ASDs can be left alone(5 mm or less)

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

o

and mortality both to fetus and mother Routine closure before pregnancy as complications of progressive pulmonary vascular disease may develop

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 f irst heart sound is soft and there is no associated third or fourth heart sounds.

The apex beat is not displaced located at the 5 th 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 accentu ate 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 ass ociated 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;

o

autosomal dominant 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 Angina, syncope, dyspnea, palpitations Sudden death (Ventricular fibrillation) (overall annual mortality in 1%) Complications of CCF, AF, IE and thromboembolic stroke

o

o

o

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

o

o

o

Normal in 25%

Tall QRS in precordial leads with ST-T changes, Q in inf and lateral leads

LAD

AF

o

CXR

o

o

Normal

LA enlargement, LVH

o

Echocardiogram

o

Diagnostic

Asymmetrical septal hypertrophy

Systolic anterior motion of the anterior mitral valve leaflet

Diastolic dysfunction

o

Severity

o

Septal thickness >18mm

Complications

Outflow tract gradient > 40mmHg as rest

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

 

o

increase death especially after first few doses Beta blockers and disopyramide 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

o

Surgical septal myomectomy (Gold standard)

12. Approach to Central cyanosis and Clubbing

Examination

o

On detecting this, concentrate on

o

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 s hunt)

o

Shunt scar (BT shunt)

o

On auscultation determine if

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 restri ctive)

No pulmonary hypertension but has RVH

Presentation o Sir this patient has got VSD/ASD/PDA complicated by Eisenmenger‟s complex. Eisenmenger‟s because - clubbed and central cyanosis and Pulmonary Hypertension

o

o

The underlying cause is ASD/VSD/PDA becaus e - 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) No PS murmur to suggest ToF

o

o

o

o

o

o

Apex beat

CCF

IE stigmata

Pulse

Peripheral presentation for ASD/VSD/PDA

o

(For Eisenmenger‟s syndrome, will have pulmonary hypertension and therefore look for TR and PR murmur)

o

o

o

Also state complications of polycythaemia, venesection marks

Requests

Summary

o

o

Sir this patient has ToF with a BT shunt done previously

Clubbed and centrally cyanosis

o

Presence of PS murmur and shunt murmur

o

o

PS murmur ESM heard best at the pulmonary area 4/6 and systolic thrill

Shunt murmur (continuous murmur)

o

(No VSD murmur as it is large and non -restrictive)

o

o

o

o

o

o

o

o

o

o

RVH with left parasternal heave

Apex beat

No pulmonary hypertension (no loud P2)

Cor pulmonale raised JVP, pedal oedema, no lung crepitation

No IE

Pulse

Presence of a thoracotomy scar with a weak left radial pulse suggesting BT shunt

Venesection marks, polycythemia

Requests

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

o

Collapsing pulse PDA MR with AR VSD with AR

o

o

o

No collapsing pulse BT shunt Venous hum (right of the sternum , children, disappears when lie flat or right JVP occluded)

o

o

What is ToF?

o

Congenital heart condition comprising of VSD, RVH, Overriding aorta , PS

o

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 5 th 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 crepitati ons 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 fo r 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

RESPI!
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 hemo ptysis associated with clubbing.

Chest excursion was reduced bilaterally with a normal percussion note and vocal resonance. Trachea is central and the apex be at is not displaced. There are no signs to suggest presence of COPD.

(There is concomitant COPD with a reduced chest excursio n 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 no t 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 wit h 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 o f respiratory failure (he does not require any supplemental oxygen and there is no central cyanosis; there is also no flapping trem or of the hands and no bounding pulse). There is also no nicotine staining of the fingers, patient is not cachexic looking and n o 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 kyphosc oliosis.

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 abdomin al examination to look for splenomegaly from amyloidosis which can result from bronchiectasis. A neurological examination is useful to screen for de ficit 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.

Questions What are your differential diagnoses for a patient that is clubbed and has crepitations?

o

 

Bronchiectasis

o

Pulmonary fibrosis

o

Mitotic lung lesion

o

Abscess

What is bronchiectasis?

 

o

Definition: permanent dilatation of the bronchi

o

Pathology: Retained secretions and chronic inflammation

o

Clinical course: Chronic, progressive wit h recurrent infective exacerbations

o

Clinical: Symptoms - productive purulent cough, dyspnea and hemoptysis and Signs: coarse late inspiratory crepitations with a 3 layered purulent sputum

What are the causes of bronchiectasis?

o

Focal

o

Luminal blockage FB, broncholith