The patient is an 18-year-old male who presented with shortness of breath, lower limb edema, and headache for the past 3-4 months. On examination, he showed features of biventricular heart failure that had improved with medication. Echocardiogram revealed an enlarged heart. The provisional diagnoses were rheumatic heart disease, congenital heart disease, dilated cardiomyopathy, or infective endocarditis. Further testing showed anemia and elevated liver enzymes. Echocardiogram demonstrated all chambers were dilated with mild mitral regurgitation and tricuspid regurgitation.

1. Case presentation
Presenter : Zahra khan

2. PATIENT’S PARTICULARS
 Name: B.A.Z
 Age: 18 years
 Sex: Male
 Referral from Amana Hosp.
 3/12 post admission
 Residence: Chanika
 Occupation: form four graduate
 DOA: 24th September 2014

3. MAIN COMPLAINTS
 Shortness of breath 4/12
 Lower limb edema 4/12
 Headache 3/12

4. HPI
The pt was apparently well until 4/12 prior to
admission when he started experiencing:
 SOB
 Lower edema
 Headache

5. HPI
SOB – 4 months
• Gradual onset
• Progressively increasing in severity with time
• Initially experienced at exertion, but then at
rest
• a/c difficulty in breathing on lying flat, at
home he uses about two pillows
• a/c easy fatigability on exertion, heartbeat

6. HPI
• No h/o migratory joint pain, swellings
underneath skin or sore throat
• No h/o bluish discoloration of lips/tongue or
squatting down to rest with exertion
• No known h/o recurrent chest infection or fever
• There is no Hx of Failure To thrive or Poor
growth

7. HPI
Lower limb edema - 4months
 gradual onset, bilateral, worsening with time
 not painful
 Increased in severity on sitting
 not accompanied by facial , abdominal or
scrotal swelling
 reduced on raising limbs

8. HPI
 Has no hx of reduced urine output
 No discomfort during micturition
 No hx of blood in urine
 neither yellowish discoloration of skin/eye nor
body itching
 No Hx of rashes or abnormal body
movements

9. HPI
Headache- 3months
 Gradual onset
 Unilateral, on right side
 Squeezing , not pulsating
 mild or moderate intensity
 aggravated by routine physical activity but
not by eating chocolate , drinking tea or

10. HPI
 Radiating to the right neck posteriorly
 no nausea/vomiting/ running nose/ear ache
or discharge
 no aura, light or sound sensitivity
 No loss or blurring of vision
 No Hx dizziness, LOC or convulsions
 No weakness or numbness of the limb

11. ROS
 GIT
 HX of vomiting 3rd day post admission
 No h/o dysphagia, dyspepsia, epigastric
pain, hematemesis
 No HX of constipation , diarrhoea or
melena
 No h/o bleeding from the nose, gums
 No h/o prolonged bleeding after minor
cuts

12. HPI
 He was then taken to Amana hospital ,was
admitted a week and was Rx for Pneumonia
with minimal improvement
 Few days later his symptoms worsened and
went back to Amana Hosp.
 At Amana blood tests, CXR and ECHO
performed and was told heart has problem
(enlarged) hence referred to MNH

13. PROGRESS IN THE WARD
In the ward he developed fever, abd.pain and
vomiting which was presumed to be malaria
and IE was Rx with Artemether ; Gentamycin
and Xpen with improvement
 On cardiac symptoms he was treated with
 Valsartan , carvedilol, furosemide and
aldactone
 His symptoms improved from NYHA IV on

14. PMHx
 2nd admission – 1st was at Amana for a
week
 No hx of trauma to the chest
 No Hx of surgery or BT
 No HTN , DM or Asthma

15. FSHX
 Orphan staying with guardian
 Parents died of HIV at the age of 9yrs
 Form four leaver – withhold his further studies
 Not alcoholic/smoker/drug user/risk sexual
behavior
 No FHx of congenital or inherited disease
 No Hx of DM, HTN in the family
 staying with aunty h/wife and uncle works –
petty trader

16. DIETARY HISTORY
Mainly 3 meals a day;
 Breakfast: tea with chapati/ uji /mtori
 Lunch: Ugali/ rice with beans or meat ±
green vegetables
 Dinner: same as lunch
 Conclusion: Adequate in quality and
quantity

17. SUMMARY
 BAZ,18yrs, male orphan from chanika, referral
from Amana who has been in ward for 3/12
 Came with Hx of : SOB, LL edema suggestive
of Biventricular HF in class IV on admission
but currently NYHA class II after RX
 Non-migraine headache , no h/o head injury
 No positive HX suggestive of acute RHD or
Congenital HD

18. Examination finds on 24/9/14
 O/E : alert , febrile ,dyspnoeic ,pale ,not
cyanosed
 Had features of Biventricular Heart Failure
and systolic murmur
 Vitals :RR 30cpm
PR 100bpm , regular ,good Cx and
volume

19. EXAMINATION FINDINGS ON
17/11/14
3/12 POST ADMISSION

20. GENERAL EXAMINATION
 Awake , afebrile 37.5⁰c
 Not dyspnoeic , not cyanosed
 Normal hair texture and distribution
 No neck stiffness, de’musset sign, scalp swelling
or tenderness
 Pale , not jaundiced
 No oral thrush or high arched palate /uvula
central and non pulsating and no dental caries or

21. GENERAL EXAMINATION
 No lymphadenopathy
 No finger clubbing , arachnodactyly , wrist sign
 Arm span to height ratio is normal
 no skin hardening or subcutaneous nodules
 No purpura, scars or marks seen
 No sacral or LL edema
 Bwt and height : 45 kg and 160cm
 BMI: 17.5

22. CVS
 PR 86bpm , regular with good volume, normal
character
 BP : Left -100/50mmhg
Right - 96/50mmHg
 No Pulse Discrepancy or Radial-femoral delay
 Raised JVP 4cm of H2O above sternal angle ( v-
wave)
 Precordial hyperactivity but no bulging

23. CVS
 Left parasternal heave
 Apex beat at 7th ICS lateral to MCL
 Normal S1 and S2 heard
 No added sound or murmur
 No pericardial rub

24. RS
 RR - 18breaths/min
 No chest wall deformity
 No prominent veins or obvious mass
 Trachea central
 No areas of tenderness

25. RS
 Symmetrical chest expansion
 Normal TVF
 Resonant percussion note
 Vesicular breath sounds
 Bilateral crepitation
 No wheeze

26. PA
 Scaphoid abdomen
 Soft
 No palpable mass
 Tender RUQ
 Positive Hepatojugular reflux
 Liver span is 12cm, no pulsation
 No arterial bruits
GENITALIA - normal male external genitalia

27. PA
DRE URINE DEEP STICK
 Normal anal verge,
sphincter tone
 No palpable mass
 Gloves stained with
yellowish brown stool
 Clarity - clear
yellowish
 Protein - absent
 Glucose - absent
 Ketones - absent
 Blood/Hb - absent
 Urobillinogen - absent
 Nitrite - absent
 PH - 6.0
 Specific gravity - 1.020

28. CNS
 Fully conscious GCS 15/15
◦ Oriented to TPP
◦ Normal speech
◦ Intact short and long-term memory
 Conclusion: Intact higher centers

29. CNS cont.
 Can smell normally
 Can see normally
 Normal and equal size pupils with reaction to
light
 Normal visual fields
 Can discriminate colors
 Can move eyes in all directions
 VA 6/6
 Fundoscopy – normal
 Refraction test - normal

30. CNS cont…
 Can clench teeth with temporalis prominence
 Has normal facial sensation
 Normal corneal reaction
 Can frown
 Can close eyes against resistance
 Can balloon the mouth against resistance
 Can whistle, show teeth without deviation
 Can hear normally

31. CNS cont.
 Can say aahh without uvula deviation
 Can shrug shoulders and turn the head
against resistance
 Tongue exam:
◦ No fasciculation
◦ No wasting
◦ Can protrude without deviation

32. CNS cont.
Upper Limbs
Motor System
 Normal muscle bulkiness B/L symmetrical
 No fasciculation
 Normal tone
 Power 5/5: all groups of muscles
 Reflexes: Normal

33. CNS cont.
Lower limbs
 muscle bulk normal
 No fasciculation
 Normal tone
 Power 5/5: All groups of muscles
 Reflexes: Deep and Superficial reflexes
Normal

34. CNS cont.
 Coordination : Normal
 Sensation: intact
 Gait : normal
Conclusion: Normal motor and sensory
function

35. MSS
 No deformities noted
 Not Wasted
 No joint or muscle tenderness

36. SUMMARY
 BAZ,18yrs, Male ,Orphan from Chanika,
Referal from Amana who has been in ward for
3/12
 Came with Hx of : SOB, LL oedema suggestive
of Biventricular HF in class IV on admission
but currently class 2 after Rx
 Non-migraine headache , no h/o head injury
 No positive Hx suggestive of Acute RHD

37. SUMMARY
 Presented with features of HF on admission but
currently modest improvement after being on
anti-failure drugs, though the headache still
persist
 O/E : alert, afebrile T 37.5c , pale ,no dyspnoeic
, no features suggestive of AR or Marfans
syndrome, no LL edema

38. SUMMARY
 Systemic examination revealed BVF and
cardiomegaly with BP of L- 100/50mm and R-
94/50mmHg , PR-98b/min,regular ,goodvolume,
↑JVP (v-wave),with +ve HJ reflux, PMI at 7th
ICSLMCL, Left parastenal heave ,normal -S1-
S2,no murmur or added sounds , RR 18c/min,
vesicular BS and B/L basal crepitation , and tender
RUQ with liver span of 12cm but no LL edema

39. PROVISIONAL DIAGNOSIS
 HEART FAILURE SECONDARY TO:
- Rheumatic Heart Ds
- Congenital Heart Ds
- Dilated cardiomyopathy
- Infective Endocarditis
 TENSION HEADACHE

40. BIVENTRICULAR HF
RIGHT SIDED
 Liver congestion
 LL edema
 RUQ abd. Pain
 ↑ JVP and positive
HJR
LEFT SIDE
 Dyspnoea (PND)
 Fatigue
 Basal creptation

41. RHD
Yes
 Age
 Developing country
 Systolic murmur
No
 Sore throat
 Migratory polyarthritis
 Sydenham chorea
 Erythema marginatum
 Subcutaneous
nodules

42. CONGENITAL HD
YES
 Age

43. DILATED
CARDIOMYOPATHYyes
 Displaced apex
 Idiopathic ?viral
 No secondary risk
factors
◦ Alcohol
◦ Hiv
◦ Diabetes
◦ HTN
◦ Drug use
◦ malnutrition
No
 Age
 Family hx

44. INFECTIVE
ENDOCARDITIS
Yes
 Fever
 Headache
 Pallor
 Rales
 Weight loss
No
 Petechiae
 Splinter hemorrhage
 Osler nodes
 Roth spots
 Pleural or percardial
rub

45. TENSION HEADACHE
YES
 Unilateral (10 -
20%ppl)
 constant
 Tight /pressure
 Non pulsatile
 No aura
 No sensitivity
 Cause : stress and
NO
 Often bilateral
 Scalp muscle
tenderness
 Lacrimation
 Normal findings on
general examination

46. FBP and DIFFERENTIAL
21/9/14 21/10/14 17/11/14 N/RANGES
WBC 04.91 05.75 5.17 4-11 K/UL
NEUTROPHI
L
31.1 58.8 59.8 37-80%
LYMPHOCY
TES
49.3 24.8 39.2 10-50%
MONOCYTE
S
16.4 13.9 12.0 0-12%
HB 9.03 9.34 8.49 14.6 -17.8
RBC 4.88 4.59 4.76 4.69-6.07M/UL
HEMATOCRI
T
31.1 27.4 29.4 40.8 – 51.9%
MCV 63.7 59.7 59.3 80-97FL
MCHC 29.0 30.9 30 31.8-35.4G/DL

47. INVESTIGATI
ON 2014
25/9 1/10 6/10 7/10 20/11 NORMAL
RANGE
ALT/SGPT 22 24 16 0 - 55U/L
AST/SGOT 32 39 23 5 - 34U/L
D/BILIRUBIN 12.8 7.9 0 – 8.6Umol/L
TOTAL BIL 22 18.0 3.4 – 20.5umo
ALK 157 145 148 40 - 150U/L
GAMMA GT 169 140 160 12 - 64U/L
CREATININE 79.4 70.8 78.5 92.1 131.5 62- 115umol/L
BUN 6.0 6.2 6.0 6.8 7.0 3.2 – 7.4mmo
ALBUMIN 32 32 40 35 – 50g/L
CHLORIDE 102 95 96 98 98 - 107mmol
SODIUM 135 132 133 136 136 – 145mm
PATASSIUM 4.5 3.8 3.8 3.6 3.5 – 5.5mmo

48. MICROBIOLOGY
INVESTIGATION RESULTS
BLOOD CULTURE NO GROWTH FOR BOTH AEROBES ,
ANAEROBES, FUNGI
HIV ELISA NEGATIVE
VDRL NEGATIVE
BS FOR MPS NEGATIVE
ASOT NEGATIVE

49. Urine analysis
COLOUR YELLOW
CLARITY CLEAR YELLOWISH
PROTEIN ABSENT
GLUCOSE ABSENT
KETONES ABSENT
BLOOD/HB ABSENT
UROBILINOGEN ABSENT
NITRITE ABSENT
BILIRUBIN ABSENT
PH 6.0
SPECIFIC GRAVITY 1.020
LEUKOCYTES CASTS ABSENT
GRANULOCYTES CASTS ABSENT

50. CXR

51. ECG

53. ECHO - PSLA

54. ECHO – PSLAx

56. ECHO PSSAx

57. ALL CHAMBERS DILATED

58. 4CHAMBER VIEW – GRADE 1MR

59. 4 CHAMBER VIEW -TR

60. SAX - AORTA

61. AORTIC CASPS AND ?MASS

62. SIMPSON BIPLANE

63. CT CHEST

64. CT chest (coroner sec)

65. CT BRAIN

66. Final diagnosis
 Aneurysm of Valsalva sinus with a
thrombus
 Biventricular heart failure, probably
due to Cardiomyopathy (idiopathic)

67. Discussion

68. Anatomy
 An aortic sinus is one of the anatomic dilations of
the ascending aorta which occurs just above the
aortic valve
 There are generally three aortic sinuses:
i. The left aortic sinus gives rise to the LCoA
ii. The right aortic sinus gives rise to the RCoA
iii. Usually, no vessels arise from the posterior aortic
sinus, which is therefore known as the non-
coronary sinus.

69. Each aortic sinus can also be referred to as
the Sinus of valsalva

70. Sinus of Valsalva aneurysm (SVA)
 SVA is also known as Aneurysm of the aortic sinus
 The malformation consists of a separation, or lack of
fusion, between the media of the aorta and the annulus
fibrosus of the aortic valve
 Uncommon cardiac anomaly- congenital or acquired
 Male to female ratio 4:1, rupture and unruptures
When present, it is usually
◦ 65 to 85% of SVA originate from right SVA
◦ Non-coronary 10 – 30%
◦ Left sinuses < 5% which is very rare

71. Causes of SVA
 Primary causes - Congenital
 Secondary causes
 Atherosclerosis
 Syphilis
 Marfan syndrome
 Blunt or penetrating chest injury
 Infective endocarditis
 Associated congenital defects
 Ventricular septal defect
 Aortic insufficiency
 Coarctation

72. Presentation
 The true natural history of SVA is unclear
 It is associated with deficiency of normal
elastic tissue and abnormal development
of the bulbus cordis .
 Congenital SVA is usually single sinus
valsalva
 Disease associated SVA often affect >1

73. presentation
 If unruptured, this type aneurysm may be
asymptomatic, undetected or detected via
medical imaging performed for other
reasons.
May be see even in patients older than 60
years.
 If ruptured aneurysm typically leads to an
aortocardiac shunt (10%) and
progressively worsening heart failure(60 %
– 90%)
Most commonly occur btn puberty and

74. presentation..
A Ruptured SVA progresses in 3 stages*:
1. Acute chest or RUQ pain
2. Subacute dyspnea on exertion or at rest (heart
failure syndrome) with progressive or acute
onset
3. Progressive cough, dyspnea, edema, and
oliguria

75. Physical examination
 Unruptured are asymptomatic
 Features of left-right shunt are seen if rupture
 A loud, superficial, "machine-type" continuous
murmur is accentuated in diastole
 A palpable thrill along the right or left lower
parasternal border
 Bounding pulses
 40 - 44% associated aortic regurgitation*
* Moustafa S, et al. Sinus of Valsalva aneurysms--47 years of a single center experience and systematic overview of published reports. Am
J Cardiol. Apr 2007

76. INVESTIGATION
 ECG- may show biventricular hypertrophy, or it may be
normal
 CXR - This may demonstrate generalized
cardiomegaly and
usually heart failure.
 ECHO- 2D and pulsed Doppler echo, may detect the
walls
of the aneurysm and disturbed flow within the
aneurysm
or at the site of perforation
TEE may provide more precise information than the
TTE approach

77. complication
1. Ruptured SVA
◦ major cause of death if associated infection or HF
before the age of 20years (most congenital aneurysm
rupture during 3rd or 4th decade)
◦ may form fistula ie: intracardiac, aortocardiac or
extracardiac fistula
◦ may rupture to pericadial space – cardiac tamponade
2. myocardial infarction
3.Heart block

78. Treatment
1. Medical management usually involves
stabilization (eg, Rx HF, arrhythmia,
Endocarditis if present) and perioperative
assessment
2. Surgery is definitive treatment for both
* Transcatheter closure using amplatzer
devices

79. Prognosis
 Poor with rupture unless urgent surgical
repair
 Most unruptured SVA progressively dilate
and eventually rupture
 Actuarial survival rate for patients with
congenital SVA is 95% at 20 years, since
most SVAs do not rupture prior to age 20
years

80. Wish list
 TEE
 MRI heart
 Surgery to repair

81. Additional discussion
 Precautions take for patient during giving contrast
◦ risk vs benefit should be weight before giving contrast
◦ use of antidotes incase suspecting hospital induced AKI
◦ the choice of contrast should be non-iodinated
◦ The dose of contrast to be adjusted for GFR
 Most likely cause of cardiomegaly in this patient is
the mass near the aortic sinus that is occuping the
space hence the heart has to use extra effort to
pump blood
 myxoma would be the unlikely dx as its not the
right age and its unlikely to be in that location
 Surgery in a patient with CMP and EF20% needs