112 AJTCCM VOL. 29 NO. 3 2023
ORIGINAL RESEARCH: ARTICLE
Background. Pulmonary endarterectomy (PEA) is the only denitive and potentially curative therapy for chronic thromboembolic
pulmonary hypertension (CTEPH), associated with impressive improvements in symptoms and haemodynamics. However, it is only
oered at a few centres in South Africa. e characteristics and outcomes of patients undergoing PEA in Cape Town have not been
reported previously.
Objectives. To assess the dierence in World Health Organization functional class (WHO-FC) before and at least 6 weeks aer surgery.
Methods. We interrogated the adult cardiothoracic surgery database at the University of Cape Town between December 2005 and April2021
for patients undergoing PEA at Groote Schuur Hospital and a private hospital.
Results. A total of 32 patients underwent PEA, of whom 8 were excluded from the nal analysis owing to incomplete data or a histological
diagnosis other than CTEPH. e work-up of these patients for surgery was variable: all had a computed tomography pulmonary
angiogram, 7 (29%) had a ventilation/perfusion scan, 5 (21%) underwent right heart catheterisation, and none had a pulmonary
angiogram. e perioperative mortality was 4/24 (17%): 1 patient (4%) had a cardiac arrest on induction of anaesthesia, 2 patients (8%)
died of postoperative pulmonary haemorrhage, and 1 patient (4%) died of septic complications in the intensive care unit. Among the
survivors, the median (interquartile range) improvement in WHO-FC was 2 (1 - 3) classes (p=0.0004); 10/16 patients (63%) returned
to a normal baseline (WHO-FC I).
Conclusion. Even in a low-volume centre, PEA is associated with signicant improvements in WHO-FC and a return to a normal baseline
in survivors.
Keywords. Chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, pulmonary embolism, pulmonary hypertension.
Afr J Thoracic Crit Care Med 2023;29(3):e294. https://doi.org/10.7196/AJTCCM.2023.v29i3.294
e natural history of acute pulmonary embolism (PE) in most
patients is complete fibrinolysis with near-total resolution of
vascular obstruction, a reduction in pulmonary vascular resistance
(PVR), and restoration of normal haemodynamics. However, a
small minority of patients will have persistent elevations in mean
pulmonary arterial pressure (mPAP) and PVR for several months
after a precipitating event despite effective anticoagulation,
termed chronic thromboembolic pulmonary hypertension
(CTEPH).[1,2] CTEPH is not only found in patients after acute
PE; ~25%ofpatientswithCTEPH do not have any history of a
precipitating PE or deep-vein thrombosis.[3] Staphylococcal infection,
endothelial dysfunction, defective brinolysis and dysfunctional
angiogenesis have been proposed as pathophysiological
mechanisms for the failure of clot resolution.[4] Clinical risk
Pulmonary endarterectomy for chronic thromboembolic
pulmonary hypertension in Cape Town, South Africa
S A Davies-van Es,1 MB ChB, FCP (SA); T C Pennel,2 MB ChB, FC Cardio (SA), PhD; J Brink,2 MB ChB, FC Cardio (SA);
G J Symons,1,3 MB ChB, FCP (SA), Cert Pulmonology (SA);
G L Calligaro,3,4 BSc Hons, MB BCh, FCP (SA), MMed (Int Med), Cert Pulmonology (SA)
1 Division of Acute General Medicine, Department of Medicine, Faculty of Health Sciences, University of Cape Town and Groote Schuur Hospital, Cape Town,
South Africa
2 Chris Barnard Division of Cardiothoracic Surgery, Department of Surgery, Faculty of Health Sciences, University of Cape Town, South Africa
3 Division of Pulmonology, Department of Medicine, Groote Schuur Hospital, Cape Town, South Africa
4 Division of Pulmonology, Department of Medicine, Groote Schuur Hospital and University of Cape Town Lung Institute, Cape Town, South Africa
Corresponding author: S A Davies-van Es (sophiedve@gmail.com)
Study synopsis
What the study adds. South African patients undergoing pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary
hypertension (CTEPH) have a marked improvement in functional status, with many returning to a normal functional baseline. However,
the small number of patients included in this study indicates that PEA is probably underutilised. Pre- and postoperative assessment is
inconsistent, despite availability of established guidelines.
Implications of the ndings. More patients should be referred to specialist centres for assessment for this potentially curative procedure.
Use of guidelines to standardise investigations and monitoring of patients with CTEPH may improve patient selection for surgery.
AJTCCM VOL. 29 NO. 3 2023 113
ORIGINAL RESEARCH: ARTICLE
factors for CTEPH include permanent intravascular devices,
inflammatory bowel disease, polycythaemia vera, splenectomy,
antiphospholipidsyndrome, high-dose thyroidreplacement therapy
and malignancy.[5,6]
e pathological basis for the elevation in mPAP and PVR is
unresolved thrombus that has not undergone fibrinolysis but
instead has been transformed into hard, hyalinised material. is
organised clot becomes incorporated into the pulmonary arterial
wall and causes chronic obstruction of the major pulmonary
arteries, either completely via total occlusion of blood flow or
incompletely by the formation of bands and webs (irregular areas
of adherent thromboembolic material).[7] e increase in PVR leads
to chronic right ventricular strain and ultimately, over a period of
months to years, to right heart failure.[2,7] CTEPH has been assigned
its own grouping (group IV) in the World Health Organization
(WHO) classication of pulmonary hypertension, owing to unique
considerations around its diagnosis and treatment.[8]
Pulmonary endarterectomy (PEA) is the treatment of choice for
patients with CTEPH,[1,4] provided there are no contraindications,
and is potentially curative. The procedure involves bilateral
complete endarterectomies down to the subsegmental branches
of the pulmonary vasculature, performed during periods of deep
hypothermic circulatory arrest (DHCA), via a median sternotomy,
and on cardiopulmonary bypass.[9,10] PEA has been shown in multiple
previous studies to significantly improve PVR and pulmonary
arterial pressure to normal or near normal, as well as improving
6-minute walk test (6MWT) distance and WHO functional class
(WHO-FC).[10-15]
Cardiothoracic expertise in performing PEA for CTEPH is not
widely available in South Africa (SA) in either the public or the
private sector. Little is known about outcomes in SA patients who
have undergone this potentially life-saving procedure. We aimed
to describe the preoperative characteristics of patients who have
undergone PEA in Cape Town, SA, as well as to report the in-hospital
mortality and functional and haemodynamic outcomes of these
patients aer surgery.
Methods
Study design and oversight
We conducted a retrospective study of patients who had undergone
PEA at Groote Schuur Hospital and a private hospital in Cape Town.
Informed consent was waived, as only routine clinical data were used.
Ethical approval was granted by the University of Cape Towns Human
Research Ethics Committee (HREC) (ref. no. 568/2019). e study
is reported in accordance with the Strengthening the Reporting of
Observational Studies in Epidemiology (STROBE) statement for
observational trials.[16]
Study population
Patients undergoing surgery between December 2005 and April 2021
were enrolled. Determination of candidacy was based on conventional
physiological and radiological assessments of operability as determined
by a multidisciplinary team (pulmonologists, cardiac surgeons and
radiologists). Selection criteria and preoperative work-up were not
protocolised, but factors considered were the surgical accessibility of
the disease, comorbidities, and adherence to anticoagulation. Patients
who were referred for consideration for surgery but not deemed
eligible were not captured in the database.
e PEA procedure was performed as outlined above, with the
initial strategy used for DHCA being to cool patients to 25oC with
10-minute arrest intervals. Later, with the development of experience
necessary to operate on more distal disease, patients were cooled to
20oC for 20-minute arrest intervals, and this became the standard.
All patients received standard intensive care unit (ICU) care at both
institutions, and postoperative follow-up and investigations were
driven by clinician request and patient preference.
Study procedures
Sociodemographic and clinical data on our patients were extracted
from the Chris Barnard Division of Cardiothoracic Surgery database
(HREC ref. no. R045/2016). Where possible, missing data were
obtained by folder review. Demographic and clinical data, data from
special investigations, and information regarding the pre-, intra- and
postoperative course were captured into a data collection sheet and
from there into a password-protected Excel database, version 16.66.1
(Microso, USA), accessible only to the specied investigators. e full
list of variables can be found in the data collection sheet (Appendix1,
available online at https://www.samedical.org/le/2053).
Outcomes
e primary outcome was the dierence in WHO-FC before and
at least 6 weeks aer PEA (when the patient was deemed to have
recovered from the effects of surgery). Anticipated secondary
outcomes were changes in 6MWT distance, right ventricular systolic
pressure (RVSP, measured by echocardiography), and postoperative
haemodynamics (mPAP, PVR, cardiac index and stroke volume index,
measured by right heart catheterisation (RHC)).
Statistical analysis
Continuous variables were presented as means with standard
deviations (SDs) (for normally distributed data) and medians with
interquartile ranges (IQRs) (for non-normally distributed data),
and categorical data as frequencies and percentages. Assumption of
normality was determined by the Shapiro-Wilk test; normally and
non-normally distributed data were compared for pre- and post-
surgical values (when available) using Student’s t-test or the Wilcoxon
rank-sum test, respectively. Statistical analyses were performed using
Stata version 12.1 (StataCorp, USA).
Results
Study population
Between December 2005 and April 2021, 32 patients underwent PEA
and were enrolled in the registry. e median (IQR) number of cases
per year was 2 (1 - 3). Eight patients were subsequently excluded from
the nal analysis: 3 were found to have pulmonary artery sarcoma and
not CTEPH on histological examination, 1 was incorrectly captured as
a PEA but underwent acute thrombectomy for fresh PE, and patient
notes could not be found for the nal 4 patients (Fig.1).
Twenty-four patients with conrmed CTEPH and with available
data were included in the final analysis. Their demographic and
clinical details are shown in Table1. Almost 80% were in WHO-
FC III or IV, and 11/24 (46%) were in clinical right heart failure. All
114 AJTCCM VOL. 29 NO. 3 2023
ORIGINAL RESEARCH: ARTICLE
were anticoagulated. Only two-thirds (n=16/24; 67%) had a history
of previous documented venous thromboembolism (VTE). The
median (IQR) time from the diagnosis of CTEPH to surgery was 123
(21-287) days.
Preoperative work-up and PEA procedure
e preoperative work-up (Table2) was highly variable. All patients
had a preoperative computed tomography pulmonary angiogram
showing proximal obstructive burden, while only 7/24 (29%)
had a ventilation/perfusion scan. Only 5/24 patients (21%) had
preoperative RHC, and none had a pulmonary angiogram. Relevant
haemodynamic parameters from the 5 RHCs are presented in
Table3. All patients had a preoperative echocardiogram: in 2 cases
there was no tricuspid regurgitation, and the RVSP could not be
measured; in the remainder, the median (IQR) RVSP was 82 (64 -
89) mmHg. Only 13/24 patients managed a preoperative 6MWT,
for which the mean (SD) distance was 322 (140) m. Details of the
median cardiopulmonary bypass time, aortic cross-clamp time and
circulatory arrest time during deep hypothermia are given in Table4.
ree patients (13%) had concomitant procedures performed during
the operation; 2patients had a tricuspid valve annuloplasty, and 1 had
a mitral valve annuloplasty.
Treatment outcomes
In-hospital mortality was 4/24 (17%): 1 patient (4%) had a cardiac
arrest on induction of anaesthesia, and was placed on bypass with
the surgery performed as a salvage procedure but could not be
weaned o bypass; 2 patients (8%) died of postoperative pulmonary
haemorrhage; and 1 patient (4%) died in the ICU of septic
complications 9 days aer surgery. e median (IQR) length of stay
in the ICU for survivors was 4 (2 - 5) days, with a median (IQR) length
of mechanical ventilation of 1 (1 - 1) day. e median (IQR) length
of hospital stay was 9 (8 - 20) days. Of the 24 PEAs, 17 (71%) were
performed aer the extracorporeal membrane oxygenation (ECMO)
programme was established, with 1 patient (4%) requiring ECMO
in the postoperative period. Sixteen patients (67%) experienced one
or more intra- or postoperative complications: bleeding requiring
transfusion (n=5/24; 21%), arrhythmias (n=3/24; 13%), sternal wound
sepsis (n=3/24; 13%), anaesthetic complications (n=2/24; 8%), need
for re-look surgery (n=2/24; 8%), acute renal failure (n=3/24; 13%),
pericardial eusion/cardiac tamponade (n=2/24; 8%), pleural eusion
requiring repeat draining (n=1/24; 1%), haemothorax (n=1/24; 1%)
and pneumonia with septic shock and multiorgan failure (1/24; 1%).
Postoperative outcomes
Of the 20 patients (83%) who survived to hospital discharge, 16 were
seen for clinical follow-up between 6 weeks and 11 months aer
Patients in
database,
N=32
Excluded as no patient notes found,
n=4
Excluded as non-CTEPH, N=3
• Pulmonary artery sarcomas, n=3
Thrombectomy for acute PE, n=1
Patients without outcome data, n=8
• Died in postoperative period, n=4
• Lost to follow-up, n=4
n=28
Patients with CTEPH
who had PEA,
n=24
Final outcome
analysis,
n=16
Fig.1. CONSORT ow diagram. (CTEPH = chronic thromboembolic
pulmonary hypertension; PE = pulmonary embolism; PEA = pulmonary
endarterectomy.
Table1. Patient characteristics (N=24)
n (%)*
Age (years), mean (SD) 41 (10)
Female 18 (75)
Medical comorbidities
Hypertension 7 (29)
HIV 6 (25)
Asthma/COPD 3 (13)
CKD 3 (13)
Obesity 2 (8)
Valvular heart disease 2 (8)
rombophilia 2 (8)
Diabetes mellitus 1 (4)
History of previous VTE (all) 16 (67)
Pulmonary embolism 13 (54)
Deep-vein thrombosis 3 (13)
Days from diagnosis to surgery, median (IQR) 123 (21 - 302)
WHO-FC
I 0
II 5 (21)
III 14 (58)
IV 5 (21)
Signs of right heart failure 11 (46)
Type of anticoagulation
Warfarin 19 (79)
DOAC 4 (17)
LMWH 1 (4)
Diuretic therapy 17 (71)
PH-specic therapy (sildenal) 2 (8)
Preoperative IVC lter 9 (38)
Preoperative mechanical ventilation 0
Preoperative inotropes 0
SD = standard deviation; COPD = chronic obstructive pulmonary disease;
CKD = chronic kidney disease; VTE = venous thromboembolism; IQR = interquartile range;
WHO-FC = World Health Organization functional class; DOAC = direct oral anticoagulant;
LMWH = low-molecular-weight heparin; PH = pulmonary hypertension;
IVC = inferior vena cava.
*Except where otherwise indicated.
AJTCCM VOL. 29 NO. 3 2023 115
ORIGINAL RESEARCH: ARTICLE
surgery (median (IQR) follow-up period 4 (2 - 5) months). eir
postoperative outcomes are shown in Table5. Of the 4 patients lost
to follow-up, 3 were still alive at 4 months aer surgery (based on
National Health Laboratory Service data), making the 4-month
mortality 4/23 (17%). e median (IQR) improvement in WHO-FC
was 2 (1- 3) classes (p=0.0004); 10/16 patients (63%) returned to a
normal baseline (WHO-FC I). A 6MWT was performed for 9 patients,
with a mean (SD) distance of 445 (108) m attained. No patients
underwent postoperative RHC; those who had a postoperative
echocardiogram with measurable RVSP (n=6 patients) had a median
(IQR) RVSP of 33 (30 - 52) mmHg.
Discussion
is study, which is to our knowledge the only report of outcomes of
CTEPH surgery in SA, has four main ndings: (i) that PEA results in
signicant improvement in functional class for patients with CTEPH;
(ii) that PEA is underutilised in our setting for the treatment of CTEPH;
(iii) that our post-surgical outcomes are worse than those reported in
other large international cohorts; and (iv) that diagnostic approaches,
preoperative work-up and postoperative follow-up of CTEPH at our
two hospitals are not standardised and require strengthening.
CTEPH is a potentially surgically curative form of pulmonary
hypertension, with PEA resulting in signicant overall improvements
in exercise capacity (median decrease in severity of 2 functional
classes) and a return to a normal baseline (WHO-FC I) for almost
two-thirds of survivors. Median time at assessment in our study was
4 months; improvement in functional class and exercise capacity may
take 3 - 12 months while the right heart undergoes remodelling,[4] so
this improvement might have been even greater if assessed at a later
time point.
e small number of patients operated on over a 15-year period
shows that PEA is severely underutilised in our setting, in both the
public and private sectors. A recent prospective observational study
(the FOCUS study) that followed up patients aer acute PE showed
a cumulative incidence of 2.3% for CTEPH at 2 years;[17] estimates
from other smaller studies range between 0.1% and 9.1%.[4] Studies
from two secondary-level hospitals in our drainage area(which
includes three secondary-level hospitals as well as the tertiary
referral centre) showed the number of patients with conrmed PE at
each institution over a 2-year period to be 41[18] and 43.[19] Although
we cannot say for certain what the total annual incidence of PE in
our drainage area (which includes the public institutions as noted,
as well as numerous private facilities) would be, we can assume it to
be a signicant number of cases. erefore, even using conservative
estimates for CTEPH aer PE for our population (which would
already underestimate the true incidence, as there is not always a
history of prior VTE), a diagnostic and treatment gap is apparent.
Potential factors accounting for the low number of CTEPH diagnoses
and PEAs performed aect every part of the referral and treatment
pathway. Under-recognition and underdiagnosis (including access
to diagnostic imaging) and the paucity of specialist pulmonary
hypertension services are important obstacles in resource-limited
countries.[20] In addition, lack of awareness of the surgical options
for management, resource constraints on cardiothoracic and ICU
capacity (which limit centre volume), competing priorities, and
a conservative institutional approach to addressing pulmonary
arterial obstruction beyond level 1 (involving one of the main
pulmonary arteries) and level 2 (starting at the level of the lobar
branchesorpastthe origin of the upper lobe artery)[21] disease may
play a role.
The post-surgical outcomes reported in this study are
considerably worse than in previous reports of PEA from other
settings and eras. PEA outcomes in high-volume centres approach
those of routine cardiac surgery owing to improved management
of the cardiac and pulmonary complications of PEA and the well-
established use of ECMO. The latest in-hospital mortality rate
reported by the centre with the greatest experience globally with
PEA (University of California San Diego) is 2.2%,[10] with mortality
in the international CTEPH registry (which includes centres in
Canada and Europe) reported as 4.7%.[12] e inverse association
between centre volume and outcome that has been described for
other complex cardiothoracic procedures, including heart and lung
transplantation,[22-25] undoubtedly also applies to our low-volume
Table2. Preoperative investigations (N=24 unless otherwise
shown)
n (%)*
CTPA performed 24 (100)
V/Q scan performed 7 (29)
Echocardiogram performed 24 (100)
RVSP (mmHg), median (IQR) (n=22) 82 (64 - 89)
6MWT performed 13 (54)
6MWT distance (m), mean (SD) (n=13) 322 (140)
RHC performed 5 (21)
mPAP (mmHg,) mean (SD) (n=5) 49 (3.5)
PVR (Wood units), median (IQR) (n=5) 7.9 (6.3 - 13.7)
Cardiac output (L/min), mean (SD) (n=5) 4.0 (0.7)
CTPA = computed tomography pulmonary angiogram; V/Q = ventilation/perfusion;
RVSP = right ventricular systolic pressure; IQR = interquartile range;
6MWT = 6-minute walk test; SD = standard deviation; RHC = right heart catheterisation;
mPAP = mean pulmonary artery pressure; PVR = pulmonary vascular resistance.
*Except where otherwise indicated.
Unable to assess RVSP for 2 patients as no tricuspid regurgitation.
Table3. Right heart catheterisation (N=5)
Patient Age (years) Mean PAP (mmHg) PVR (Wood units) Average CO (L/min)
1 33 52 13.5 3.4
2 59 53 13.7 3.7
3 42 45 6.3 5.1
4 48 49 7.9 3.5
5 64 46 6 4.4
PAP = pulmonary artery pressure; PVR = pulmonary vascular resistance; CO = cardiac output.
116 AJTCCM VOL. 29 NO. 3 2023
ORIGINAL RESEARCH: ARTICLE
centre. In addition, we have evolving institutional experience
with extracorporeal support during the study period, having only
established a nascent programme towards the end of 2015. However,
despite most of the cases being performed aer the service was
established, the use of rescue ECMO in our study was rare, similar to
the ~5% incidence reported in other large surgical series.[4] It is more
likely that the numerically higher mortality is a function of small
patient numbers that include some high-risk cases, with over a h
of patients (21%) in WHO-FC IV prior to surgery. In comparison,
only 10% of operated patients from the University of California San
Diego series[10] and 13% of operated patients from the international
CTEPH registry[12] were in WHO-FC IV prior to surgery.
Finally, our study demonstrates that diagnostic approaches,
preoperative work-up and postoperative follow-up of CTEPH at
our institution are not standardised and are probably suboptimal.
Preoperative RHC was performed sparingly despite being
mandated in all pulmonary hypertension guidelines to conrm the
diagnosis, as well as to establish the severity of the haemodynamic
impairment. Pulmonary angiography, the gold standard for
depicting the pulmonary vasculature and which can be performed
at the same time as the RHC, was not done on any patient, probably
because there has been a lack of institutional expertise in both the
performance and interpretation of this modality. e nding on
histological examination of pulmonary artery sarcomas (a rare
and aggressive malignant tumour, often mistaken for PE based
on similar clinical and radiological features[26]) in almost 10% of
patients reinforces the importance of thorough preoperative imaging
and evaluation. Postoperative non-invasive follow-up was also
inconsistent, with even echocardiographic assessments generally
lacking. Guidelines for the diagnosis of CTEPH and for follow-up
aer PEA have been outlined in a recent consensus statement from
the International Society for Heart and Lung Transplantation[4] and
should be a reference document for centres going forward. At our
institution, we have subsequently taken measures to aim towards a
standardised practice falling in line with these recommendations,
includingmandating ventilation/perfusion scans and pulmonary
angiograms.
Several limitations of this study deserve emphasis. First, while the
number of patients undergoing PEA was already small, a quarter had
to be excluded from the initial analysis and there were ultimately only
outcome data for half of the operated patients. ese exclusions may
aect the conclusions that can be drawn from these data, and the small
numbers prevented analyses of risk factors for mortality. Second, we
only included patients who actually underwent PEA; the number of
patients referred with CTEPH but not considered operable could not
be established owing to the retrospective nature of the study. is is
important, as up to a quarter of patients deemed inoperable are in fact
candidates aer additional imaging such as pulmonary angiography,
which as mentioned was not performed in this study. Up to 90% of
patients are surgical candidates in expert centres,[27,28] and it would
have been interesting if we could have reported our denominator
of referrals for PEA. Lastly, the preoperative assessment and
postoperative follow-up were not standardised, and there was a dearth
of data on haemodynamics or even echocardiographic measures of
right ventricular function postoperatively. However, functional class
remains an easy-to-record and patient-centred outcome measure for
this group.
Table4. PEA procedure (N=24 unless otherwise shown)
n (%)*
Cardiopulmonary bypass time (minutes),
median (IQR) (n=23)
155 (137 - 174)
Cross-clamp time (minutes),
median(IQR)(n=23)
80 (46 - 91)
Circulatory arrest time (minutes),
median(IQR)
25 (14 - 35)
Lowest core temperature (oC), median (IQR) 24 (22 - 25)
Concomitant procedures performed (all) 3 (13)
Tricuspid valve annuloplasty 2 (8)
Mitral valve annuloplasty 1 (4)
ECMO 1 (4)
Days in ICU, median (IQR) 4 (2 - 5)
Days in hospital, median (IQR) 9 (8 - 20)
Days ventilated, median (IQR) 1 (1 - 1)
Patients with complications (all) 16 (67)
Bleeding (requiring transfusion) 5 (21)
Sternal wound sepsis 3 (13)
Acute renal failure 3 (13)
Arrhythmia 3 (13)
Need for re-look surgery 2 (8)
Anaesthetic complication 2 (8)
Pleural eusion 1 (4)
Haemothorax 1 (4)
Pericardial eusion/cardiac tamponade 2 (8)
Pneumonia 1 (4)
In-hospital mortality 4 (17)
PEA = pulmonary endarterectomy; IQR = interquartile range;
ECMO = extracorporeal membrane oxygenation; ICU = intensive care unit.
*Except where otherwise indicated.
Table5. Postoperative outcomes (N=16 unless otherwise
shown)
n (%)*
4-month mortality (n=23) 4 (17)
Time to follow-up post surgery (months),
median(IQR)
4 (2 - 5)
WHO-FC at follow-up
I 10 (63)
II 5 (31)
III 1 (6)
IV 0
Signs of right heart failure 2 (13)
Postoperative echocardiogram performed 8 (50)
RVSP (mmHg), median (IQR) (n=6) 33 (30 - 58)
Postoperative 6MWT performed 9 (56)
Postoperative 6MWT distance (m),
mean(SD)(n=9)
445 (108)
Postoperative RHC performed 0
IQR = interquartile range; WHO-FC = World Health Organization functional class;
RVSP = right ventricular systolic pressure; 6MWT = 6-minute walk test; SD = standard
deviation; RHC = right heart catheterisation.
*Except where otherwise indicated.
Unable to assess RVSP for 2 patients as no tricuspid regurgitation.
AJTCCM VOL. 29 NO. 3 2023 117
ORIGINAL RESEARCH: ARTICLE
Conclusion
In summary, this study gives insight into current practices and
outcomes at our institution regarding PEA for CTEPH. Surviving
patients experienced signicant functional improvement, although
haemodynamic outcomes could not be assessed. Prolonged ICU stay
was not required, and the need for ECMO was minimal. Preoperative
work-up was variable, and this may have inuenced patient selection
for surgery. In addition, consideration should be given to longer-
term specialist follow-up for those presenting with acute PE, as the
diagnosis of CTEPH in our setting is almost certainly oen missed or
signicantly delayed.
Declaration. e research for this study was done in partial fullment
of the requirements for SADvEs MMed (Internal Medicine) degree at
the University of Cape Town. TCP an GC are members of the AJTCCM
editorial board. Another editor was given responsibility for overseeing the
peer review of this submission.
Acknowledgements. We would like to acknowledge the contribution of all
the patients included in this study as well as that of all the doctors, nurses
and other hospital sta involved in their care.
Author contributions. SADvE, TCP, JB, GJS and GLC were involved
in conceptualising and designing the study. JB and TCP assisted with
access to the database, data collection and review of the surgical aspects
of the paper. SADvE did all other data collection. SADvE, GLC and
GJS interpreted the data and wrote the rst dra. All authors read and
commented on the nal manuscript.
Funding.None.
Conicts of interest.None.
1. Galiè N, Humbert M, Vachiery JL, etal. 2015 ESC/ERS Guidelines for the diagnosis
and treatment of pulmonary hypertension: e Joint Task Force for the Diagnosis
and Treatment of Pulmonary Hypertension of the European Society of Cardiology
(ESC) and the European Respiratory Society (ERS): Endorsed by: Association for
European Paediatric and Congenital Cardiology (AEPC), International Society for
Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37(1):67-119. https://doi.
org/10.1093/eurheartj/ehv317
2. Wilkens H, Konstantinides S, Lang IM, etal. Chronic thromboembolic pulmonary
hypertension (CTEPH): Updated recommendations from the Cologne Consensus
Conference 2018. Int J Cardiol 2018;272S:69-78. https://doi.org/10.1016/j.
ijcard.2018.08.079
3. Pepke-Zaba J, Delcroix M, Lang I, etal. Chronic thromboembolic pulmonary
hypertension (CTEPH): Results from an international prospective registry. Circulation
2011;124(18):1973-1981. https://doi.org/10.1161/circulationaha.110.015008
4. De Perrot M, Gopalan D, Jenkins D, etal. Evaluation and management of patients
with chronic thromboembolic pulmonary hypertension – consensus statement from
the ISHLT. J Heart Lung Transplant 2021;40(11):1301-1326. https://doi.org/10.1016/j.
healun.2021.07.020
5. Delcroix M, Kerr K, Fedullo P. Chronic thromboembolic pulmonary hypertension:
Epidemiology and risk factors. Ann Am orac Soc 2016;13(Suppl 3):S201-S206.
https://doi.org/10.1513/AnnalsATS.201509-621AS
6. Kim NH, Lang IM. Risk factors for chronic thromboembolic pulmonary hypertension.
Eur Respir Rev 2012;21(123):27-31. https://doi.org/10.1183/09059180.00009111
7. Lang IM, Dorfmüller P, Vonk Noordegraaf A. The pathobiology of chronic
thromboembolic pulmonary hypertension. Ann Am Thorac Soc 2016;13(Suppl
3):S215-S221. https://doi.org/10.1513/AnnalsATS.201509-620AS
8. Humbert M, Kovacs G, Hoeper MM, etal. 2022 ESC/ERS guidelines for the diagnosis
and treatment of pulmonary hypertension. Eur Heart J 2022;43(38):3618-731. https://
doi.org/10.55788/c838601b
9. Jamieson SW, Kapelanski DP, Sakakibara N, etal. Pulmonary endarterectomy:
Experience and lessons learned in 1,500 cases. Ann orac Surg 2003;76(5):1457-
1462. https://doi.org/10.1016/s0003-4975(03)00828-2
10. Madani MM, Auger WR, Pretorius V, etal. Pulmonary endarterectomy: Recent
changes in a single institutions experience of more than 2,700 patients. Ann orac
Surg 2012;94(1):97-103. https://doi.org/10.1016/j.athoracsur.2012.04.004
11. Condliffe R, Kiely DG, Gibbs JS, etal. Improved outcomes in medically and
surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir
Crit Care Med 2008;177(10):1122-1127. https://doi.org/10.1164/rccm.200712-
1841OC
12. Mayer E, Jenkins D, Lindner J, etal. Surgical management and outcome of
patients with chronic thromboembolic pulmonary hypertension: Results from an
international prospective registry. J orac Cardiovasc Surg 2011;141(3):702-710.
https://doi.org/10.1016/j.jtcvs.2010.11.024
13. istlethwaite PA, Kaneko K, Madani MM, Jamieson SW. Technique and outcomes
of pulmonary endarterectomy surgery. Ann orac Cardiovasc Surg 2008;14(5):274-
282.
14. Matsuda H, Ogino H, Minatoya K, etal. Long-term recovery of exercise ability
after pulmonary endarterectomy for chronic thromboembolic pulmonary
hypertension. Ann orac Surg 2006;82(4):1338-1343. https://doi.org/10.1016/j.
athoracsur.2006.03.105
15. Skoro-Sajer N, Marta G, Gerges C, etal. Surgical specimens, haemodynamics and
long-term outcomes aer pulmonary endarterectomy. orax 2014;69(2):116-122.
https://doi.org/10.1136/thoraxjnl-2013-203746
16. Von Elm E, Altman DG, Egger M, Pocock SJ, Gøtzsche PC, Vandenbroucke JP.
The Strengthening the Reporting of Observational Studies in Epidemiology
(STROBE) statement: Guidelines for reporting observational studies. Lancet
2007;370(9596):1453-1457. https://doi.org/10.1016/s0140-6736(07)61602-x
17. Valerio L, Mavromanoli AC, Barco S, etal. Chronic thromboembolic pulmonary
hypertension and impairment aer pulmonary embolism: e FOCUS study. Eur
Heart J 2022;43(36):3387-3398. https://doi.org/10.1093/eurheartj/ehac206
18. Bulajic B, Welzel T, Vallabh K. Clinical presentation and diagnostic work up of
suspected pulmonary embolism in a district hospital emergency centre serving a
high HIV/TB burden population. Afr J Emerg Med 2019;9(3):134-139. https://doi.
org/10.1016/j.aem.2019.05.003
19. Ramlakhan R, Andronikou S, Rajkumar A. e prevalence and radiological ndings
of pulmonary embolism in HIV-positive patients referred for computed tomography
pulmonary angiography in the Western Cape of South Africa. Cardiovasc J Afr
2017;28(4):221-228. https://doi.org/10.5830/cvja-2016-083
20. Davies-van Es S, Calligaro G, Manning K, Williams H, Dheda K, Symons G.
e aetiology, clinical presentation and treatment of patients with pulmonary
hypertension in Cape Town: A preliminary report from the Groote Schuur Hospital
Pulmonary Hypertension Registry. Afr J orac Crit Care Med 2018;24(4):133-138.
https://doi.org/10.7196/ajtccm.2018.v24i4.218
21. Madani MM. Surgical treatment of chronic thromboembolic pulmonary
hypertension: Pulmonary thromboendarterectomy. Methodist DeBakey Cardiovasc
J 2016;12(4):213-218. https://doi.org/10.14797/mdcj-12-4-213
22. Shudo Y, Wang H, Lingala B, et al. Evaluation of risk factors for heart-lung
transplant recipient outcome: An analysis of the United Network for Organ
Sharing Database. Circulation 2019;140(15):1261-1272. https://doi.org/10.1161/
circulationaha.119.040682
23. ourani VH, Brennan JM, Edelman JJ, etal. Association of volume and outcomes
in 234 556 patients undergoing surgical aortic valve replacement. Ann orac Surg
2022;114(4):1299-1306. https://doi.org/10.1016/j.athoracsur.2021.06.095
24. Russo MJ, Iribarne A, Easterwood R, etal. Post-heart transplant survival is inferior
at low-volume centers across all risk strata. Circulation 2010;122(11 Suppl):S85-S91.
https://doi.org/10.1161/circulationaha.109.926659
25. Tie HT, Shi R, Zhou Q, Wang K, Zheng XQ, Wu QC. Annual case volume on
mortality aer coronary artery bypass graing: A dose-response meta-analysis.
Interact Cardiovasc orac Surg 2019;29(4):568-575. https://doi.org/10.1093/icvts/
ivz151
26. Cervilla-Munoz E, Galeano-Valle F, Del-Toro-Cervera J, Calleja-Carton E, Demelo-
Rodriguez P. Dierential diagnosis and treatment approach to pulmonary artery
sarcoma: A case report and literature review. ERJ Open Res 2020;6(3):00124-2020.
https://doi.org/10.1183/23120541.00124-2020
27. DArmini AM, Morsolini M, Mattiucci G, etal. Pulmonary endarterectomy for distal
chronic thromboembolic pulmonary hypertension. J orac Cardiovascular Surg
2014;148(3):1005-1011. https://doi.org/10.1016/j.jtcvs.2014.06.05224
28. Jenkins DP, Biederman A, D’Armini AM, etal. Operability assessment in CTEPH:
Lessons from the CHEST-1 study. J oracic Cardiovasc Surg 2016;152(3):669-674.
e3. https://doi.org/10.1016/j.jtcvs.2016.02.062
Submitted 31 october 2022. Accepted 28 May 2023. Published 19 September 2023.