PULMONOLOGYPULMONOLOGY

Dr W.K. HerbstDr W.K. Herbst

Dr J. NelDr J. Nel

9/04/20109/04/2010

HISTORYHISTORY

Mrs.MMrs.M

32 yr female from Kroonstad 32 yr female from Kroonstad

Referred from Hematology withReferred from Hematology with

PolycythaemiaPolycythaemia

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

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)

EXAMINATIONEXAMINATION

ClubbedClubbed

CyanoticCyanotic

CVSCVS No signs of pulmonary hypertensionNo signs of pulmonary hypertension

Respiratory: normalRespiratory: normalAbdominal: normal Abdominal: normal Neuro: normalNeuro: normal

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

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

PROBLEMPROBLEM

1. Hypoxia Not improving on Oxygen therapy

2. Polycythaemia

3. Clubbing

HYPOXEMIAHYPOXEMIA

CXRCXR

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

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

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

3-D RECONSTRUCTION

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

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

CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS

SYMPTOMSSYMPTOMS

EpistaxisEpistaxis

DyspneaDyspnea

HemoptysisHemoptysis

PlatypneaPlatypnea

SIGNS

HHTTelangiectasis

Bruit

Clubbing

Cyanosis

Diagnostic modalities

WHY TREAT ???

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

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

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

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

CHEST X RAY POST SURGERYCHEST X RAY POST SURGERY

OUR PATIENT

Follow up in 1 month at clinic

Hypoxia Polycythaemia

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