pulmonology dr w.k. herbst dr j. nel 9/04/2010. history mrs.m 32 yr female from kroonstad referred...
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PULMONOLOGYPULMONOLOGY
Dr W.K. HerbstDr W.K. Herbst
Dr J. NelDr J. Nel
9/04/20109/04/2010
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HISTORYHISTORY
Mrs.MMrs.M
32 yr female from Kroonstad 32 yr female from Kroonstad
Referred from Hematology withReferred from Hematology with
PolycythaemiaPolycythaemia
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RESPIRATORY HISTORYRESPIRATORY HISTORY
Progressive DYSPNEA since June 2009Progressive DYSPNEA since June 2009
PlatypneaPlatypnea
No symptoms suggestive of infectionNo symptoms suggestive of infection
No embolic phenomenaNo embolic phenomena
No hyperviscosity symptomsNo hyperviscosity symptoms
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RESPIRATORY HISTORYRESPIRATORY HISTORY
PlatypneaPlatypnea Increase in dyspnea induced by the upright position Increase in dyspnea induced by the upright position
and relieved by recumbencyand relieved by recumbency Associated with orthodeoxia Associated with orthodeoxia Decrease in blood flow through PAVM in the Decrease in blood flow through PAVM in the
dependent portions of the lungs upon assuming the dependent portions of the lungs upon assuming the supine position supine position
Other causesOther causesHepatopulmonary syndrome Hepatopulmonary syndrome Atrial septal defects (including patent foramen ovale)Atrial septal defects (including patent foramen ovale)
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EXAMINATIONEXAMINATION
ClubbedClubbed
CyanoticCyanotic
CVSCVS No signs of pulmonary hypertensionNo signs of pulmonary hypertension
Respiratory: normalRespiratory: normalAbdominal: normal Abdominal: normal Neuro: normalNeuro: normal
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LAB RESULTSLAB RESULTSFULL BLOOD COUNT & PLATELETS FULL BLOOD COUNT & PLATELETS Flags Ref RangesFlags Ref Ranges White Cell Count ............. White Cell Count ............. 4.02 4.02 x 10^9/l x 10^9/l 4.00 - 10.00 4.00 - 10.00 Red Cell Count ............... Red Cell Count ............... 8.25 8.25 x 10^12/l x 10^12/l H H 4.13 - 5.67 4.13 - 5.67 Haemoglobin .................. Haemoglobin .................. 24.0 24.0 g/dl g/dl H H 12.1 - 16.3 12.1 - 16.3 Haematocrit .................. Haematocrit .................. 0.701 0.701 l/l l/l H H 0.370 - 0.490 0.370 - 0.490 MCV .......................... MCV .......................... 85.0 85.0 fl fl 79.1 - 98.9 79.1 - 98.9 MCH .......................... MCH .......................... 29.1 29.1 pg pg 27.0 - 32.0 27.0 - 32.0 MCHC ......................... MCHC ......................... 34.2 34.2 g/dL g/dL 32.0 - 36.0 32.0 - 36.0 Platelets .................... Platelets .................... 189 189 x 10^9/l x 10^9/l 178 - 400 178 - 400
MORPHOLOGY AND COMMENT MORPHOLOGY AND COMMENT RED CELL MORPHOLOGY .......... RED CELL MORPHOLOGY .......... PolycythaemiaPolycythaemia
RETICULOCYTE STUDIES RETICULOCYTE STUDIES Flags Ref RangesFlags Ref Ranges Reticulocyte Count ........... Reticulocyte Count ........... 2.38 2.38 % % H H 0.50 - 2.00 0.50 - 2.00 Absolute Reticulocyte Count .. Absolute Reticulocyte Count .. 0.196 0.196 10^12/l 10^12/l H H 0.050 - 0.100 0.050 - 0.100 Haematocrit .................. Haematocrit .................. 0.701 0.701 l/l l/l H H 0.370 - 0.490 0.370 - 0.490 Reticulocyte Production Index Reticulocyte Production Index 3.7 3.7
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BLOOD GASBLOOD GAS
BLOOD GAS ANALYSIS BLOOD GAS ANALYSIS pH 7.40 7.37 - 7.43pH 7.40 7.37 - 7.43 pCO2 ......................... 32.00 mmHg L 35.00 - 45.00pCO2 ......................... 32.00 mmHg L 35.00 - 45.00 pCO2 ......................... 4.26 kPa L 4.65 - 5.98pCO2 ......................... 4.26 kPa L 4.65 - 5.98 pO2 .......................... 41.5 mmHg L 69.0 - 84.0pO2 .......................... 41.5 mmHg L 69.0 - 84.0 pO2 .......................... 5.52 kPa L 9.05 - 11.17pO2 .......................... 5.52 kPa L 9.05 - 11.17 Standard Bicarbonate (SBC) ...20.6 mmol/l 20.0 - 25.0Standard Bicarbonate (SBC) ...20.6 mmol/l 20.0 - 25.0 Base Excess .................. -3.8 mmol/l L -2.0 - 3.0Base Excess .................. -3.8 mmol/l L -2.0 - 3.0 O2 Saturation ................ 78 % L 95 – 98O2 Saturation ................ 78 % L 95 – 98
NO IMPROVEMENT ON OXYGENNO IMPROVEMENT ON OXYGEN
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PROBLEMPROBLEM
1. Hypoxia Not improving on Oxygen therapy
2. Polycythaemia
3. Clubbing
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HYPOXEMIAHYPOXEMIA
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CXRCXR
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VENTILATION PERFUSIONVENTILATION PERFUSION
Left lung base shows perfusion and ventilation defectLeft lung base shows perfusion and ventilation defect Structural lung diseaseStructural lung disease
Kidneys clearly visible in perfusion studyKidneys clearly visible in perfusion studySUGGESTIVE OF UNDERLYING SHUNTSUGGESTIVE OF UNDERLYING SHUNT
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HRCT CHESTHRCT CHEST
Loss of volume left Loss of volume left hemithoraxhemithorax
Tortious ectatic Tortious ectatic tubular structures tubular structures left lung baseleft lung base
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CT PULMONARY ANGIOGRAMCT PULMONARY ANGIOGRAM
Multiple dilated tortious Multiple dilated tortious vessels of LLLvessels of LLL
Clear feeding artery from Clear feeding artery from left pulmonary arteryleft pulmonary artery
Clear draining vein to left Clear draining vein to left pulmonary veinpulmonary vein
Large left Large left pulmonary pulmonary AV fistulaAV fistula
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3-D RECONSTRUCTION
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Pulmonary arteriovenous Pulmonary arteriovenous malformations (PAVMs)malformations (PAVMs)
First described 1897First described 1897
Abnormal communications between Abnormal communications between pulmonary arteries and veins pulmonary arteries and veins
Differential diagnosis ofDifferential diagnosis of HypoxemiaHypoxemia Pulmonary nodulesPulmonary nodules HemoptysisHemoptysis
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PAVMsPAVMs
Fairly uncommonFairly uncommon Annual incidence of 4.3 cases/year at a large medical centerAnnual incidence of 4.3 cases/year at a large medical center
Twice as often women as in men Twice as often women as in men
Incidence increases in fifth and sixth decadesIncidence increases in fifth and sixth decades
70% associated with hereditary hemorrhagic 70% associated with hereditary hemorrhagic telangiectasia (HHT/ Osler-Weber-Rendu syndrome) telangiectasia (HHT/ Osler-Weber-Rendu syndrome)
30 percent of patients with HHT have PAVM30 percent of patients with HHT have PAVM
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CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS
SYMPTOMSSYMPTOMS
EpistaxisEpistaxis
DyspneaDyspnea
HemoptysisHemoptysis
PlatypneaPlatypnea
SIGNS
HHTTelangiectasis
Bruit
Clubbing
Cyanosis
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Diagnostic modalities
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WHY TREAT ???
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COMPLICATIONSCOMPLICATIONS
Stroke/ TIA/ Brain abscessStroke/ TIA/ Brain abscess Paradoxic embolizationParadoxic embolization
Migraine headacheMigraine headache
HypoxemiaHypoxemia
Polycythemia Polycythemia
Anemia Anemia
Exercise intoleranceExercise intolerance
Hemoptysis and hemothoraxHemoptysis and hemothorax
PHTPHT RareRare
Congestive heart failureCongestive heart failure
Infective endocarditisInfective endocarditis
If HHTIf HHT EpistaxisEpistaxis GIT bleedingGIT bleeding Skin bleedsSkin bleeds
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TREATMENTTREATMENT
Indications includeIndications include
Progressive enlargement of lesionsProgressive enlargement of lesions Paradoxic embolizationParadoxic embolization Symptomatic hypoxemiaSymptomatic hypoxemia
Treating all PAVMs that can be technically Treating all PAVMs that can be technically embolizedembolized
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TREATMENTTREATMENT
Surgery Local excision and Local excision and
lobectomylobectomy Pneumonectomy Pneumonectomy Vascular ligationVascular ligation
Embolization
Angiographic Angiographic occlusion of feeding occlusion of feeding arteries to a PAVMarteries to a PAVM
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OUR PATIENTMicroscopy results
Vascular lesion Consisting of abnormally shaped arteries and
venules many with very large diameter
Abnormal distribution of the bronchiolis and bronchi
Suggesting a congenital etiology
Lesion originates from a single artery and draining vein
Features suggests
Arteriovenous Arteriovenous malformationmalformation
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CHEST X RAY POST SURGERYCHEST X RAY POST SURGERY
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OUR PATIENT
Follow up in 1 month at clinic
Hypoxia Polycythaemia
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REFERENCESUptodate.com
Harrison’s Online Copyright 2004-2005
Chowdhury UK, Kothari SS, Bishnoi AK, Gupta R, Mittal CM, Reddy S (February 2008). "Successful Lobectomy for Pulmonary Arteriovenous Malformation Causing Recurrent Massive Haemoptysis". Heart Lung Circ.
Bernstein D. Other Congenital Heart and Vascular Malformations. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 432
Cottin V, Chinet T, Lavolé A, et al. Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore). Jan 2007