Prof Paul A Corris Newcastle University Newcastle Upon Tyne Pulmonary Hypertension Which of the following statements are true A breathless patient with normal LV systolic function and whose RHC results show a mean PAP of 30 mmHg and a PCW of 23 mmHg has pulmonary arterial hypertension ID: 931957
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Slide1
Pulmonary Hypertension Transplantation
Prof Paul A CorrisNewcastle UniversityNewcastle Upon Tyne
Slide2Pulmonary Hypertension
Which of the following statements are trueA breathless patient with normal LV systolic function and whose RHC results show a mean PAP of 30 mmHg and a PCW of 23 mmHg has pulmonary arterial hypertensioniPAH is associated with thyroid disease
Heritable PAH is associated with mutations in the gene EIF2AK4Porto pulmonary hypertension is characterised by severe hypoxia due to intra pulmonary shuntingThrombocytopaenia is a recognised feature of iPAH
Slide3Pulmonary Hypertension
A 65 year old female with LcSscl presents with progressive breathlessness. Her thoracic CT shows a dilated RV and PA and no lung fibrosis.RHC(mmHg) shows mean PA 50 , PCW 7, RA 17CO 2.8 L/minWhich of following are true?
She has PAHShe has RHC features supportive of HFpEFShe should receive diureticsShe should receive upfront oral combination therapy with an ERA and a PDE5iShe should be anticoagulated
Slide4Pulmonary Hypertension
Which of the following statements are trueThe V/Q scan is the test with the highest sensitivity for excluding CTEPHThe prognosis of PAH in association with CTD is better than for iPAH
The prognosis of PAH associated with CHD is better than for iPAHPH associated with sarcoid should be treated with targeted PH therapy as in patients with iPAHStudies have shown an increase in mortality if patients with IPF and PH are treated with endothelin receptor antagonists
Slide5Pulmonary Hypertension
Which of the following are true statements regarding CTEPHIncreased risk associated with blood group OOccurs in 20% of patients after an acute PEShould be treated initially by ThrombolysisThe diagnosis mandates placement of an IVC filter
There is an increased risk of developing CTEPH associated with CSF shunt placement
Slide6Pulmonary Hypertension
The following are recognised causes of pulmonary hypertension.SchistosomiasisSickle cell diseaseMalaria
CytomegalovirusMitral stenosis
Slide7Pulmonary Hypertension
A 25 year old female presents with a short 3 month history of progressive breathlessness and exertional chest pain with syncope. The cardiologist immediately arranges a cardiac catheter. Left sided pressures are normal RHC pressures are as follows (mmHg)Mean PA 60 RA 12 PCW 8 and CO is 2.8 L/minWhich of the following statements are trueThe diagnosis is iPAH and no other investigations are required
There is no clinical role for genetic testingHeart Lung Transplantation should be consideredA trial of high dose Nifedipine should be considered Her history of developing high altitude sickness when climbing Mt Kilimanjaro during a gap year is irrelevant
Slide8Transplantation
A 40 year old man is admitted with severe abdominal pain nine months after bilateral lung transplantation for IPF. His CXR the week before organized by his GP for malaise is shown in figure A
His admission erect abdominal film is shown.
The most likely underlying diagnosis isA Disseminated
nocardiasisB Disseminated aspergillosisC Small cell lung cancer of donor lung origin
D Post transplant lymphoproliferative disease
E Unknown primary carcinoma
Slide9PTLD
Usually B cell originEarly and late presentation (organ transplanted)Clonality and grade determines therapyAssociated with EBV commonlyReduce Immunosuppression Rituximab CHOP
etc
Slide10Transplantation
What is single most appropriate advice to give the patient ?
A Mycobacterium abscessus is an absolute contraindication to lung transplantationB The presence of this organism confers no added risks for transplantation
C The organism must be eradicated prior to transplant referralD Appropriate multidrug antimicrobial therapy should be startedE There is a need to undertake BAL to confirm the pathogenic presence of this organism
29 year old man with advanced CF is being prepared for transplant referral. Sputum cultures are positive for Mycobacterium abscessus on 3 occasions
Slide11NTM
All CF patients should be screened pre referralPresence according to guidelinesMolecular typingAbscessus species problematicTreat pre referral and continue after transplant ? indefinitelyMulti drug regimen marked drug toxicityOther NTM much less problematic
Slide12Transplantation
What is the most likely diagnosis ?
A Bronchiolitis obliteransB Acute rejectionC Recurrence of his emphysema
D Lymphocytic bronchiolitisE Stricture of anastomosis in main bronchusHis PEF isA 300 L/min B 180 L/min
His MMEF isA > 100 L/min B < 60 L/min
A 54 year old man who underwent single lung transplantation for emphysema and alpha 1 antitrypsin deficiency six months previously presents with increasing breathlessness. A flow volume curve shows the appearances above
Slide13Transplantation
A 60 year old obese woman with advanced IPF whose BMI is 35 is referred for transplantation.Which one answer below is true ?
A She has an increased risk of primary graft dysfunction if accepted and undergoes transplantationB She has no added risks of an adverse outcome providing there are no other relative contraindicationsC She has 50% less chance of surviving 5 years compared
with a 35 year old with a BMI of 24D She can only be considered for bilateral lung transplantationE She can only be considered for single lung transplantation
Slide14Slide15Slide16Slide17TransplantationPotential role of diastolic dysfunction in LV in obese patients mimicking PGD
Slide18Transplantation
A 25 year old woman with c
ystic fibrosis who has stable lung function and no evidence of BOS 5 years following lung transplantation tells you she is 3 months pregnant. She has a GFR of 50 mls/min. Which of the following statements are correctA She should be advised that continuing her pregnancy is too dangerous for her and her babies health to support on medical grounds
B She should be advised that the risk of her delivering a child without serious birth defect is only 20%C She should be advised to stop her immunosuppression immediatelyD She should be advised that she is at high risk of developing renal failure if her pregnancy continuesE She should be advised she has a greater than 50% chance of developing pre eclampsia before she reaches 40 weeks
Slide19The Journal of Heart and Lung Transplantation
Volume 33, Issue 6, June 2014, Pages 593–598 Original Clinical SciencePregnancy after lung and heart-lung transplantationMitesh V. Thakrar, MDa, b, , , Katie Morley, RNa, James L. Lordan, MDa,
Gerard Meachery, MDa, Andrew J. Fisher, MDa, Gareth Parry, MDa, Paul A. Corris, MDa
Slide20Transplantation
Which of the following statements is/are true of all surviving recipients 5 years post Lung Transplantation?A 40% have developed hypertensionB 25% have evidence of renal dysfunction
C 40% have diabetesD 20% have dyslipidaemiaE 70% have Bronchiolitis Obliterans Syndrome (BOS)
Slide21Slide22Slide23Transplantation
A 29 year old man with CF who is negative for CMV serologically received lungs from a donor who was CMV positive. Which of the following statements is/are true ?A Transplantation should not have been carried out because all patients have to be CMV matchedB Post operative blood transfusion should have utilised irradiated blood to ensure viral transfer does not occur
C The patient should be given valganciclovir as soon as symptoms of CMV infection occur typically at 14 daysD CMV hyperimmune globulin should be given twice weekly for 3 monthsE Prophylactic valganciclovir
should be given immediately for at least 3 months
Slide24Delay of CMV infection in high-risk CMV mismatch lung transplant recipients due to prophylaxis with oral
ganciclovir
Volume 18, Issue 2, pages 179–185, April 2004Scott M Palmer1, Dan C Grinnan1, B Diane Reams2, Mark P Steele1, Robert H Messier3 andR Duane Davis3Article first published online: 8 MAR 2004DOI: 10.1046/j.1399-0012.2003.00152.
Slide25Guidelines on the use of irradiated blood components Prepared by the BCSH Blood Transfusion Task Force Address for correspondence: BCSH Secretary
British Society for Haematology 100 White Lion Street London N1 9PF e-mail bcsh@b-s-h.org.uk Writing Group: Jennie Treleaven1; Andrew Gennery
2; Judith Marsh3; Derek Norfolk4; Lizanne Page5; Anne Parker6
; Frank Saran7; Jim Thurston8; David Webb9.
Slide26Transplantation
50 year old patient who received bilateral lung transplantation for iPAH and was very well presents at 12 months with acute onset fever cough and breathlessness . She is hypoxic and has CXR above. Her creatinine is 150.
Which of the following is/are TrueA Acute rejection is most likely diagnosisB Patient should have a CTPA since PE most likelyC Pneumocystis infection is most likely D Therapy should be commenced empirically using high doses of both a penicillin and macrolideE Therapy should be commenced empirically with a penicillin and a
quinalone
Slide27Acute rejection episodes per patient
per month during first 12 months
0
0.1
0.2
0.3
0.4
0-1
2-3
4-6
7-12
Months from randomisation
Rate of rejection
episodes per month
Aza
MMF
Slide28Transplantation (Exam)
Which of the statements below is/are true ?Donor lungs have to be both ABO compatible and tissue type matched for the recipient to receive donor lungs .Patients who are HIV positive have an absolute contraindication to lung transplantation.Long term results of bilateral lung transplantation are better than single lung transplantation in patients with COPD
A potential recipient should have class 1 and class 2 HLA antibody status measured pre listing.The GFR of a potential recipient should be greater than 50 mls/min before accepting a patient for lung transplantation.
Slide29Transplantation (Exam)
A 27 year old man with CF presents with acute abdominal pain, malaise, breathlessness and fever 18 weeks after lung transplantation. His routine 12 week biopsy showed no evidence of rejection. He has new onset leucopenia and looks unwell. CT scan is as above.
He was a CMV negative recipient of an organ from a CMV positive donor and is on prophylactic valganciclovir.Which statements is/ are trueHe most likely has acute vascular rejection and should have iv steroids empirically
He most likely has CMV disease and should initially have empirical treatment dose valganciclvir and PCR of blood to quantify CMV viral load.He should be scheduled for urgent bronchoscopy with biopsy and lavage CMV pneumonitis is best diagnosed by PCR of BAL fluid.He most likely has pseudomonal
pneumonia
Slide30Transplantation (Exam)
A 58 year old patient with IPF and UIP on lung biopsy and traction bronchiectasis reported on CT with a mean pulmonary artery pressure of 30 mmHg is referred for transplant assessment. Which statements is/are true?He has a better survival outlook in UK after listing if he is offered single lung transplantation.He is only suitable for consideration of bilateral lung transplantation.
He should receive treatment with targeted PH therapy if placed on waiting listHe should be routinely listed to have cardiopulmonary bypass if accepted for transplant surgery.He is not a suitable candidate for transplant surgery.
Slide31Transplantation (Exam)
A 49 year old woman otherwise fit with severe PiZZ associated emphysema and an asymptomatic 20% stenosis of her LAD coronary artery and no pressure change across the lesion is referred for transplantation. Which statement below is true ?She should be offered heart lung transplantation .She should be offered single lung transplantation after coronary stenting.
She should be offered bilateral lung transplantation after coronary stenting.She should be offered bilateral lung transplantation without need for stentingShe is not suitable for further consideration of transplantation.
Slide32Pulmonary Physiology T or F
A 34 year old male presents with breathlessness and has widespread alveolar shadowing. His arterial PaO2 is 5.5 Kpa and PaCO2 3.2 Kpa breathing air. VC is 60% predicted and TLCO is 150% predicted
The blood gases are more likely to be venousHis A-a gradient is >10 KpaMost likely he has cardiogenic pulmonary oedemaHe should be managed on an HDUHe should
initially be given 24% oxygen via venturi mask
Slide33Pulmonary Physiology
The Total lung capacity as measured by body plethysmography is determined by inspiratory muscle strength and recoil pressure of the lungLung fibrosis results in a reduced lung compliance and increased recoil pressure
The hypoxia seen at rest in lung fibrosis is principally due to impaired gas exchange due to the thickened alveolar wallsFlow is constant throughout expiration during a maximal effortPeak flow shows diurnal variation in normal subjects
Slide34Pulmonary Physiology
A 44 year old chronically breathless patient has an FEV1 of 30%p an FVC 0f 60% p a TLC of 250%p and a TLCO of 25% pHe has mixed obs. restrictive diseaseHe most likely has asthmaHis VC (relaxed) is most likely to be greater
than his FVCHis RV is most likely to be normalThe physiology is typical of advancedLangerhans Cell Granulomatosis
Slide35Pulmonary Physiology
Cardiopulmonary exercise test in a 37 yr old female shows a VO2P of 65% pred. HR max was 70% pred. And VE max was 50% pred. The RER at the end of exercise was 0.9 and lactate was 1.2The test was limited by the onset of premature anaerobic respiration
The test was limited by respiratory diseaseThe result suggests a mitochondrial myopathyThe test indicated a cardiac limitation to exerciseThe test results are in keeping with a suboptimal effort
Slide36Pulmonary Physiology
In normal human subjectsTidal volume increases before respiratory rate on exerciseThe increase in ventilation is linear until nearing maximumExpiratory flow is principally governed by lung recoil pressure during tidal breathing
The intercostal muscles principally determine inspiratory volumeI am as confident of success in the exam as the England cricket team has of success in next test at Lords