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CASE PRESENTATION

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CASE PRESENTATION Prof. Dr. Birsen MUTLU I.U. Cerrahpasa Medical Faculty Department of Pulmonology ATRIAL SEPTAL DEFECT (ASD) FREQUENCY Congenital heart disease is ... – PowerPoint PPT presentation

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Title: CASE PRESENTATION


1
CASE PRESENTATION
Prof. Dr. Birsen MUTLU I.U. Cerrahpasa Medical
Faculty Department of Pulmonology
2
H. B.
  • 56 years-old
  • Female
  • Born in Trabzon, lives in Istanbul
  • Housewife
  • Married

3
COMPLAINTS
  • Dyspnea
  • Dry cough
  • Weakness
  • Swelling legs
  • Dry mouth
  • Dry eyes

4
HISTORY
  • Experiencing dyspnea for ten years on effort
    (climbing up stairs)
  • For the last one year, complaining about dyspnea
    and weakness even during her daily activities
  • The patient, who started to complain about
    dyspnea while resting 2-3 months ago and the
    legs of whom started to swell, was hospitalized
    for further examination and treatment.

5
  • HISTORY Hypertension (15 years)
  • Diabetes mellitus Type 2 (5 yrs)
  • FAMILIAL HISTORY Nothing special
  • Addiction None
  • Allergy Undefined

6
  • REGULARLY USED MEDICINE
  • Trimetazidine Hcl (Vastarel ) 20 mg tb 3x1
  • Ramipril (Delix ) 2,5 mg tb 1x1
  • Rosiglitazone maleate (Avandia ) 4 mg tb 1x1
  • Glimepride (Glirid ) 1 mg tb 2x1

7
PHYSICAL EXAM - 1
  • Conscious, cooperative
  • Dyspnea, orthopnea ()
  • Body temp37 ºC
  • Obese
  • Cyanosis (-)
  • Palpable lymphadenomegaly (-)
  • Clubbing (-)
  • Raynauds phenomenon (-)

8
PHYSICAL EXAM - 2
  • RESPIRATORY SYSTEM
  • Respiration per minute 20
  • Two hemithoraces equally contribute to
    respiration
  • Respiratory sounds decreased bilaterally, in
    both lower zones, Late inspiratory crackles heard

9
PHYSICAL EXAM - 3
  • CARDIO - VASCULAR SYSTEM
  • Apex beat 80/mins, rhythmic
  • TA 110/70 mmHg
  • Heart sounds S1-S2 (), grade 2/6 systolic
    murmur at tricuspid and pulmonary area
  • ABDOMEN
  • No organomegali
  • No painful region
  • No mass and fluid

10
PRE - DIAGNOSIS ???
  1. Pulmonary Hypertension
  2. Left Heart Failure
  3. Interstitial Lung Disease
  4. CTELD
  5. Cor Pulmonale

11
CHEST X-RAY
H.B. 04. 06. 2007
12
PRE - DIAGNOSIS ???
  1. Pulmonary Hypertension
  2. Left Heart Failure
  3. Interstitial Lung Disease
  4. CTELD
  5. Cor Pulmonale

13
LABORATORY FINDINGS
14
HAEMOGRAM
  • WBC 9200/mm³
  • Hb 12,2 gr/dL
  • Hct 36,5
  • PLT 259000/mm³

15
  • Sedimentation 38 mm/h
  • CRP 21,5 mg/L
  • BCG Scar 2 scars
  • PPD 11 mm enduration
  • Blood Biochemistry Normal
  • Comp Urine Count Normal

16
WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???
  1. Computerized Tomography
  2. Lung Function Tests
  3. TTD Ecocardiography
  4. ECG
  5. Serological Examination for Vasculitis

17
COMPUTERIZED TOMOGRAPHY
k
l
18
COMPUTERIZED TOMOGRAPHY
rv
ra
19
COMPUTERIZED TOMOGRAPHY
rv
ra
20
COMPUTERIZED TOMOGRAPHY
21
HRCT
22
HRCT
23
ECG
24
TT DOPPLER ECHOCARDIOGRAPHY
  • Right cardiac spaces are extended
  • Left ventricle EF 60
  • Moderate tricucpid insufficiency
  • Pulmonary arterial peak systolic pressure 85
    mmHg

25
RIGHT HEARTH CATHETERIZATION
  • PULMONARY ARTERIAL PRESSURE
  • Before adenosin 75/38 mmHg,
  • mean 50 mmHg
  • After adenosin 60/38 mmHg,
  • mean 45
    mmHg

Vasoreactivity negative
26
PRE - DIAGNOSIS ???
  1. Pulmonary Hypertension
  2. Left Heart Failure
  3. Interstitial Lung Disease
  4. CTELD
  5. Cor Pulmonale

27
SPIROMETRE
28
SPIROMETRE
29
LUNG VOLUMES
30
DIFFUSION CAPACITY
31
ARTERIAL BLOOD GASES
  • PO2 7O,8 mmHg
  • PCO2 40,5 mmHg
  • SaO2 94,2
  • PH 7,46
  • HCO3 28,9 mmol/L
  • ? Room condution

32
PULMONARY HYPERTENSION
  • Hemodynamic definition (Right Heart
    Cateterization)
  • Average of PAP gt 25 mmHg while resting
  • Average of PAP gt 30 mmHg in exercise
  • Pulmonary wedge pressure lt 15 mmHg

ACCP Evidence-based clinical practice guidelines
Chest 2004 WHO, 2003 Galie N, Torbicki A, Barst
R, et al.Guidelines on diagnosis and treatment of
pulmonary arterial hypertension. Eur Heart J
2004 25 2243-2278
33
CLASSIFICATION OF PULMONARY HYPERTENSION(Simonnea
u et al. J Am Coll Cardiol 2004)
34
6DYT FUNCTIONAL CLASS
  • 6DYT
  • Distance 240 m
  • SaO2 93 - 87
  • Borg scala dyspnea 3-9
  • NYHA IV

35
ROMATOLOGICAL MARKERS
  • p ANCA
  • c ANCA
  • MPO ANCA
  • PR3 ANCA
  • Anti SSA
  • Anti SSB
  • Anti SCL 70
  • ANTI JO1
  • RF 48,6 (0-15)
  • ANA slightly brindled ()
  • Anti ds DNA
  • Anti SM
  • Anti SM/RNP
  • C3C, C4

negative
negative
36
SCHIRMERS TEST
  • Right eye 20 mm
  • Left eye 17 mm
  • Normal

37
WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???
  • A. BAL
  • B. Transbronchial Biopsy
  • C. Salivary Gland Biopsy
  • D. Open Lung Biopsy
  • E. Transthoracic Biopsy

38
LUNG BIOPSY
  • Lung biopsy is not recommended as a routine due
    to the risks involved
  • It might be exceptionally performed with the
    condition that a specific problem is answered
    through tissue diagnosis

Quality of evidence Experts view Benefit Good
Suggestion power E/A
Badesch DB, Abman SH, Simonneau G, Rubin LJ,
McLaughlin VV. Medical Therapy for Pulmonary
Arterial Hypertension. Updated ACCP
Evidence-Based Clinical Practice Guidelines.
Chest 2007 13119171928
39
WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???
  • A. BAL
  • B. Transbronchial Biopsy
  • C. Salivary Gland Biopsy
  • D. Open Lung Biopsy
  • E. Transthoracic Biopsy

40
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41
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42
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43
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44
DIAGNOSIS
SJÖGRENS SYNDROME
45
SJÖGRENS SYNDROME
  • It is a slowly-proceeding, chronic, systemic and
    auto-immune disease that is characterized by the
    lymphocitic infiltration of exocrine glands.
  • It proceeds mainly by dryness in the mouth and
    eyes ? Sicca syndrome
  • Prevalance 0,5-5 (mostly asymptomatic)
  • ?/? 9/1
  • Age 40-60

Kelleys textbook of rheumatology. Philadelphia
Saunders, 20051105-24
46
SJÖGRENS SYNDROME
  • 50-60 is composed of secondary Sjögrens
    syndrome
  • Rheumatoid arthritis
  • Systemic lupus erythematous (SLE)
  • Scleroderma
  • Mixed connective-tissue disease
  • Primary biliary cirrhosis
  • Myositis
  • Thyroiditis
  • Vasculitis
  • Chronic active hepatitis
  • Mixed cryoglobulinemia

Clinical Manifestations and Early Diagnosis of
Sjögren Syndrome Arch Intern Med.
20041641275-1284
47
CLINICAL FINDINGS
  • Exocrine gland disease
  • Nonexocrine organ disease

  • Arthralgia/Arthritis 60

  • Raynauds phenomenon 37

  • Lymphadenomegaly 14

  • Lung involvement 14

  • Vasculitis
    11
  • Kidney involvement 9

  • Liver involvement 6

  • Lymhoma 6

  • Splenomegaly 3

  • Peripheral neuropathy 2

  • Myositis
    1

? Radiological involvement
30
Harrisons principles of internal medicine. 16th
ed. 2005. 1990-1993
48
LUNG INVOLVEMENT
  • Interstitial lung disease
  • Nonspesific interstitial pneumonia
  • Lymphocitic interstitial pneumonia
  • Bronchiolitis obliterans organized pneumonia
  • Usual interstitial pneumonia
  • Desiccation of tracheobronchial tree
  • Diffuse or focal nodular infiltrates
    (pseudolymphoma, malignant lymphoma, amiloidosis
  • Pulmonary vasculitis and primary pulmonary
    hypertension (rare)

  • Interstitial lung diseases
  • Marvin I. Schwarz Talmadge E. King Mosby year
    Book 2003568-572

49
CRITERIA FOR DIAGNOSIS
  • Symptoms for dryness in eyes
  • Findings for dryness in eyes (Schirmers test
    5 mm/min)
  • Symptoms for dryness in mouth
  • Findings for dryness in mouth (unstimulated
    saliva secretion 1,5ml/15min

  • Parotid sialography , Scintigraphy )
  • Minor salivary gland biopsy
  • Autoantibodies (Anti-Ro / SS-A, Anti-La / SS-B)

4 criteria should be positive (one biopsy, one
autoantibody)
Classification criteria for Sjogren's syndrome a
revised version of the European criteria proposed
by the
American-European Consensus Group.Ann Rheum Dis.
200261(6)554-8.
50
CLINICAL OBSERVATION
  • Quensyl tb (Hidroxyclorkin) (2x1)
  • Deltacortil tb (2x1)
  • In the second month, the patient stopped quensyl
    by her decision

In the fourth month, she applied again due to
increase in her complaints
51
CLINICAL OBSERVATION
  • Dyspnea has become more severe
  • Coughing has increased
  • Bruises in hands and feet in the last 20 days
  • Medical exam
  • Dyspnea, orthopnea ()
  • Cyanosis ()
  • Pretibial edema /
  • Respiratory sounds decrased in both lungs,
    prolonged expirium , wheezing ()
  • Heart sounds 2/6 systolic murmur () at
    tricuspid and pulmonary area

52
CHEST X-RAY
H.B. 11.01.2008
53
PA GRAFILER
11.01.2008
04.06.2007
54
SPIROMETRY
04.06.2007
11.01.2008
55
DIFFUSION CAPACITY
04.06.2007
11.01.2008
  • DLCO 9,04 (48) mL/mmHg/min
  • DLCO/VA 3,35 (67) mL/mmHg/min/L
  • 9,02 (47)
  • 4,97 (99)

56
ARTERIAL BLOOD GASES
04.06.2007
11.01.2008
  • PO2 7O,8 mmHg
  • PCO2 40,5 mmHg
  • SaO2 94,2
  • PH 7,46
  • HCO3 28,9 mmol/L
  • PO2 48 mmHg
  • PCO2 46 mmHg
  • SaO2 84,8
  • PH 7,45
  • HCO3 30 mmol/L

57
WHAT WOULD YOU THINK AT THIS POINT ???
  1. The patient should be followed up
  2. Immunsuppresive dose should be increased
  3. Treatment for PH should be started
  4. Another factor should be looked for
  5. The disease is progressing

58
2. COMPUTERIZED TOMOGRAPHY
59
2. COMPUTERIZED TOMOGRAPHY
60
2. HRCT
61
WHAT WOULD YOU THINK AT THIS POINT ???
  1. The patient should be continued to be followed
  2. Immunsuppresive dose should be increased
  3. Treatment for PH should be started
  4. Another factor should be looked for
  5. The disease is in progress

62
2. TT DOPPLER ECHOCARDIOGRAPHY
  • Right cardiac spaces are extended
  • Left ventricle EF 60
  • Slight tricuspid failure
  • Pulmonary artery peak systolic pressure
    75mmHg
  • (6
    months ago 85 mmHg)

63
2. RIGHT HEART CATHETERISATION
  • Pulmonary arterial pressure 77/35 mmHg
    (average 50)
  • Atrial septal defect with shift from left to
    right!!!
  • Transesophageal Echocardiogram proposed

64
TRANSESOPHAGEAL ECOCARDIOGRAPHY
  • An atrial septal defect with a 2,4 cm diameter
    was found in the interatrial septum
  • It was discussed in the council of cardiology
    Cardiovascular surgery
  • It was decided to make an operation to close ASD

65
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66
ATRIAL SEPTAL DEFECT (ASD)
  • FREQUENCY
  • Congenital heart disease is seen in 0,5-1,2 of
    infants
  • ASD composes 7-11 of all congenital heart
    diseases and 30 of congenital heart diseases in
    adults
  • It is the most frequently found new shant lesion
    in adolescence
  • It can emerge at any age

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
67
ATRIAL SEPTAL DEFECT (ASD)
  • SIZE
  • Small 2 cm
  • Large gt2 cm
  • (Diameter is usually 2-4 cm)

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
68
ATRIAL SEPTAL DEFECT (ASD)
  • TYPES
  • Ostium Secundum (most frequent) ? 65- 75 is
    women
  • Ostium primum
  • Sinus venosus
  • Coronary sinus defect

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
69
ATRIAL SEPTAL DEFECT (ASD)
  • While secundum type adolescents and young adults
    are asymptomatic, more than half of them develop
    dyspnea, rapid fatigue, atrial flutter or
    fibrilation, right heart failure or pulmonary
    hypertension with the age of 40

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
70
ATRIAL SEPTAL DEFECT (ASD)
  • In 5-10 of untreated ASD especially women
    a pulmonary vascular disease develops
  • Their 10-year survival rate is found to be 95
    after suregery and 84 with medical treatment

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
71
PH TYPES WHEN DIAGNOSED
Portal hypertension
  • 2 risk factors 4

Humbert et al. Am J Respir Crit Care Med 2006
72
Taken from the photo archive of Prof. Dr.
Müzeyyen Erk (2007)
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