pulmonary hypertension case presentationstatic.livemedia.gr/hcs2/documents/al19480_us41... ·...
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Pulmonary Hypertension
Case Presentation
Maria Drakopoulou
Clinical Fellow in ACHD & Centre for PH,
Royal Brompton Hospital (London)
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Background
DoB:
• 20th August 1990 (27y F)
Cardiac Diagnoses:
• Situs solitus, levocardia, AV and VA concordance
• Perimembranous VSD, PFO
Presented at RBH at the age of 12 years old
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Calculating PVR in patients with CHD
K. Dimopoulos, S.J. Wort, M.A. Gatzoulis, European Heart Journal (2014) 35, 691–700
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Diagnostic cardiac catheterization (12/7/2002)
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I
PVR
Bidirectional/R-L shuntL-R
R-L
IV-VII III
Mechanical stress
Endothelial DysfunctionVascular Remodelling
Endothelium Mild SevereModerate
Pulmonary Hypertension
associated with L-R
shunts
<4 UW/m2 >8 UW/m24-8
Eisenmenger Syndrome
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Left to right shunt
Pulmonary hypertension?
Clinical signs suggesting inoperability
RHC
PVRi < 4WUxm2 PVRi > 8WUxm2PVRi 4-8WUxm2
Vasodilator test Occlusion test
Surgery Medical therapy
+
-
+ -
PVR reduct > 20%
PVRi < 4-6 WU
PVR:SVR < 0.33
PAPm reduct > 25%
PAPd/PASd reduc > 50%
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Diagnostic cardiac catheterization (12/7/2002)
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VSD closure (23/10/2003)
14mm Amplatzer perimembranous VSD occluder
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Diagnostic cardiac
catheterization (31/5/2005)
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Clinical Follow-up
Clinical Status:
Deteriorated in her exercise capacity
Walks for approximately 5 minutes on the flat
WHO class III
Clinical Examination:
Height: 166cm, Weight: 60kg
BP 110/63, HR 73bpm, SpO2 98% on air
JVP not raised
Heart Sounds:
S1 normal, increased S2
Normal lung sounds
No hepatomegaly, no peripheral oedema
June 2011
Started Ambrisentan 5mg
Clinical Status:
Improved exercise capacity,
WHO Class II
Manages 10mins on the flat and ~ 2 flights of stairs
December 2014
2005
Wants to attempt pregnancy
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NNNNNHeart rate increases
Circulatory volumes increase
Cardiac output increases
Increased preload
Systemic vasodilatation
Endothelial dysfunction
Reduced colloid osmotic pressure
Coagulopathy
Increased oxygen consumption
Prolonged Valsalva
IVC decompression volume
Bleeding & autotransfusion
Pain & distress
Increased shunting
IATROGENIC !
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Why such an issue in PAH?
• Increase in HR & pro-arrhythmic
• Increase in 02 consumption (20%)
• Increased tidal volume & minute ventilation
• Elevated diaphragm - 4cm
• Hypercoagulability
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Pregnancy Assessment
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TTEPre- Pregnancy Assessment
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TTEPre- Pregnancy Assessment
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TTEPre- Pregnancy Assessment
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TTEPre- Pregnancy Assessment
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CMR Pre- Pregnancy Assessment
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Cardiopulmonary Exercise Test with MVO2(June 2014)
Pre- Pregnancy Assessment
Date 01/04/2014 3/12/2014
Age 23.6 24.3
RER 1.15 1.02
Max HR 179 160
PVO2 15.2 15.1
PVO2 % predicted 42% 42%
VE/VCO2 63.0 57.8
Time 6m, 42s 9m, 25s
Rhythm SR+RBBB
Resting Pulse 64 Resting BP 90 62
Peak Exercise
Pulse
160 Peak Exercise BP 98 62
50
40
30
20
10
0
10 30 50 70P
VO
2
Age
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Right Heart CatherizationPre- Pregnancy Assessment
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Pregnancy Risk
Estimation
ESC 2011 Guidelines, European Heart Journal (2011) 32, 3147–3197
Pre- Pregnancy Assessment
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MDT Meeting (December 2014)
• Preconception counseling:
– Patient advised that there is a high mortality risk associated with pregnancy
• Plan:
– To be followed at the antenatal clinic
– Ambrisentan was stopped and Sildenafil 20mg td was commenced
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Clinical Follow-up
Clinical Status:
Deteriorated in her exercise capacity
Walks for approximately 5 minutes on the
flat
WHO class III
Clinical Examination:
Height Weight
BP 110/63, HR 73bpm, SpO2 98% on air
JVP not raised
Heart Sounds:
S1 normal, increased S2
Normal lung sounds
No hepatomegaly, no peripheral oedema
June 2011
Started Ambrisentan 5mg
December 2014
2005
Wants to attempt
Pregnancy
January 2016
MiscarriageClinical Status:
Improved exercise capacity,
WHO Class II
Manages 5-10mins on the flat and
~ 2 flights of stairs
Pregnancy
May 2016
Optimization PAH therapy
Sildenafil 50mg td
Start anticoagulation
Clexane 60mg bd
Multidisciplinary approach with monthly visits
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Kiely DG et al, BJOG 2009
4 w
kly
un
til 28
wk
s; 2 w
kly
un
til 32
wk
sth
en w
eekly
Minimising Risk?
Sildenafil then Iloprost
Echo, 6MWT, BNP
Often use GA
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Admission
20.11.2016
November
2016
Medication 19/11/2016
Sildenafil 50mg td √
Clexane 40mg od √
-Small volume hemoptysis potentially related
to an upper respiratory infection
-Increasing SOB and presyncope on effort
which represented a change in symptoms from
her last review in clinic 3 weeks earlier.
23+6 weeks
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ECG (November 2016)Pregnancy
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Chest X-Ray (20/11/2016)
Pregnancy
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TTE (21/11/2016)
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During Admission
ECG
Chest X-Ray
Echocardiogram
20.11.2016
November
2016
MDT (20/11/2016)
-Start inhaled iloprost therapy
(5mcg 3 hourly)
Medication 19/11/2016 20/11/2016
Sildenafil 50mg td √ √
Clexane 40mg od √ √
Inhaled Iloprost 5mcg 3 hourly √
23+6 weeks
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Admission
ECG
Chest X-Ray
Echocardiogram
21.11.2016
MDT (20/11/2016)
-Start inhaled iloprost therapy
(5mcg 3 hourly)
MDT (25/11/2016)
-Insertion of a PICC line
-IV Epoprostenol and escalation of therapy by 1-2ng/day according to tolerance
-Transfer to HDU
25.11.2016
Medication 19/11/2016 20/11/2016 25/11/2016
Sildenafil 50mg td √ √ √
Clexane 40mg od √ √ √
Inhaled Iloprost 5mcg 3 hourly √ √
IV Epoprostenol √
November
2016
24+4 weeks
26 weeks
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Admission
ECG
Chest X-Ray
Echocardiogram
21.11.2016
MDT (20/11/2016)
-Start inhaled iloprost therapy
(5mcg 3 hourly)
MDT (25/11/2016)
-Insertion of a PICC line
-IV Epoprostenol and escalation of therapy by 1-2ng/day according to tolerance
-Transfer to HDU
25.11.2016
November
2016
24+4 weeks
26 weeks
Medication 30/11/2016 2/12/2016 5/12/2016
Sildenafil 50mg td √(50mg td)
√(50mg td)
√(25mg bd,
40od)
Clexane 40mg od √ √ √
Inhaled Iloprost 5mcg 3 hourly √
IV Epoprostenol √ (5ng.kg/min)
√(6ng.kg/min)
√(6.5ng.kg/min)
Dexamethasone √
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HDU Admission (06/12/2016)
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TTE (16.12.2016)
Pregnancy27+5 weeks
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Admission
Echocardiogram
16th December 2016
-Up-titrate epoprostenol by 0.5ng/kg/min 12 hourly up
to 15ng/kg/min or maximum tolerated dose (not less
than 10ng/kg/min)
-CPAP is required intermittently
MDT meeting
27 January 2017
Elective C-Section
November
2016
27+5 weeks
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Elective C-Section at 32+4 weeks (31/1/2016)
• Under TOE investigation
Elective C-Section
-Estimated 700mls blood loss intra-operatively
-Managed on continued epoprostenol infusion
and continued sildenafil 25mg tds
-Baby transferred to Chelsea and Westminster
neonatal unit for monitoring
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Admission31 January 2017
Elective C-Section
Post-Op C-Section
-Continued epoprostenol infusion and continued sildenafil
25mg TDS
-On oxytocin infusion due to PV bleeding
Plan:
Physiotherapy
Negative fluid balance
May
2016
32+2 weeks
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TTE Pre-Discharge (9/2/2017)
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• Repeat echocardiogram in 4 weeks and revision of epoprostenol infusion
• Continue Epoprostenol for 3-6 months and arrange a right heart catheterization
• Follow Hb level (given Ferinject 500mg prior to discharge)
• Long-term contraception
– Started a progesterone only mini-pill, until she can be reviewed for Mirena Coil insertion in 3
months time.
Follow-up Plan
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Alonso-Gonzalez R, et al. Int J Cardiol 2015;184:717–723
Postoperative PAH survival
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Bedard E et al, EHJ 2009
idiopath
ic
congenital
other
congenital
Is the risk changing? Systematic review of
maternal outcome
73 PAH pregnancies
(excluded termination)
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Bedard E et al, EHJ 2009
congenital
Advanced therapies
72% idiopathic
52% congenital
27% other
Is the risk changing? Systematic review of
maternal outcome
73 PAH pregnancies
(excluded termination)
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Conclusions
• PAH can develop at any stage of CHD patients’ life and has an impact on quality of life, exercise
capacity, morbidity and mortality.
• Cautious approach for defects closure : ‘I can close it’ does not mean ‘I should close it’.
• Pregnant women with PAH should be referred to PAH specialist centre with close MDT
collaboration between pulmonary hypertension specialists, obstetricians, critical care specialists
and neonatologists.
• Pregnancy is still associated with a significant risk of maternal mortality and rapid deterioration
may occur requiring escalation of therapy and semi-urgent delivery.
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Professor Michael A Gatzoulis (Academic Lead)
Dr Lorna Swan (Clinical Lead)
Dr Kostas Dimopoulos (ACHD PAH)
Dr Anselm Uebing (Intervention)
Dr Rafa Alonso (Inpatient Care, HF, Transplantation)
Dr John Wort (PAH)
Dr Laura Price (PAH)
Dr Sonya Babu-Narayan (Academic MRI)
Dr Philip Kilner (Consultant in cardiac ACHD-MRI)
Dr Wei Li (Consultant in Echocardiography)
Professor Darryl F Shore (Cardiac Surgical Lead)
Mr B Sethia (Consultant Cardiac Surgeon)
Mr Olivier Ghez(Consultant Cardiac Surgeon)
Mr Michielon Guideon (Consultant Cardiac Surgeon)
Ms R Patel (Obstetrician Chelsea and Westminster Hospital)