scholarly journals Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

2020 ◽  
Vol 6 (2) ◽  
pp. 00218-2019
Author(s):  
Abdullah M. Aldalaan ◽  
Sarfraz A. Saleemi ◽  
Ihab Weheba ◽  
Abeer Abdelsayed ◽  
Pekka Hämmäinen ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Disease Severity ◽  
Tertiary Care ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
World Health ◽  
Cumulative Probability ◽  
Probability Of Survival ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.MethodsThis study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry. This registry enrolled patients with pulmonary hypertension, established through right heart catheterisation, under clinical management at a specialised tertiary care centre. Patients received standard care during the period of the registry.ResultsAt the time of this analysis, 64 CTEPH patients were enrolled in the registry. Mean age at diagnosis was 39.7 years and there was a female predominance (67.6%). At baseline, most patients were in World Health Organization functional classes III or IV (70.1%). At the last follow-up visit, most patients (63.2%) had undergone endarterectomy, showing significant improvement in disease severity from baseline. Patients who underwent endarterectomy showed numerically higher (p=0.126) probability of survival at 1 year (97.5%) versus those who did not undergo endarterectomy (94.4%).ConclusionPatients were diagnosed at relatively young age, but still showed high disease severity, suggesting delay in diagnosis. Patients who underwent surgical treatment showed substantial improvements in clinical and haemodynamic parameters, while the remaining patients tended to show disease progression. The 96.6% 1-year cumulative probability of survival was high compared to previous studies.

Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

2014 ◽  
Vol 112 (09) ◽  
pp. 598-605 ◽  
Author(s):  
Laurent Guérin ◽  
Francis Couturaud ◽  
Florence Parent ◽  
Marie-Pierre Revel ◽  
Florence Gillaizeau ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Systolic Pulmonary Artery Pressure ◽  
Prospective Multicentre Study ◽  
Right Heart Catheterisation ◽  
Venous Thromboembolic Events ◽  
Thromboembolic Pulmonary Hypertension ◽  
Acute Pe

SummaryChronic thromboembolic pulmonary hypertension (CTEPH) has been estimated to occur in 0.1–0.5% of patients who survive a pulmonary embolism (PE), but more recent prospective studies suggest that its incidence may be much higher. The absence of initial haemodynamic evaluation at the time of PE should explain this discrepancy. We performed a prospective multicentre study including patients with PE in order to assess the prevalence and to describe risk factors of CTEPH. Follow-up every year included an evaluation of dyspnea and echocardiography using a predefined algorithm. In case of suspected CTEPH, the diagnosis was confirmed using right heart catheterisation (RHC). Signs of CTEPH were searched on the multidetector computed tomography (CT) and echocardiography performed at the time of PE. Of the 146 patients analysed, eight patients (5.4%) had suspected CTEPH during a median follow-up of 26 months. CTEPH was confirmed using RHC in seven cases (4.8%; 95%CI, 2.3 – 9.6) and ruled-out in one. Patients with CTEPH were older, had more frequently previous venous thromboembolic events and more proximal PE than those without CTEPH. At the time of PE diagnosis, patients with CTEPH had a higher systolic pulmonary artery pressure and at least two signs of CTEPH on the initial CT. After acute PE, the prevalence of CTEPH appears high. However, initial echocardiography and CT data at the time of the index PE suggest that a majority of patients with CTEPH had previously unknown pulmonary hypertension, indicating that a first clinical presentation of CTEPH may mimic acute PE.

scholarly journals Risk assessment in medically treated chronic thromboembolic pulmonary hypertension patients

2018 ◽  
Vol 52 (5) ◽  
pp. 1800248 ◽  
Author(s):  
Marion Delcroix ◽  
Gerd Staehler ◽  
Henning Gall ◽  
Ekkehard Grünig ◽  
Matthias Held ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Hypertension ◽  
High Risk ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
European Respiratory Society ◽  
Thromboembolic Pulmonary Hypertension

Abbreviated versions of the risk stratification strategy of the European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension guidelines have been recently validated in patients with pulmonary arterial hypertension. We aimed to investigate their prognostic value in medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients from the COMPERA registry, which collects six variables of interest (World Health Organization Functional Class, 6-min walk distance, brain natriuretic peptide, right atrial pressure, cardiac index and mixed venous oxygen saturation).We included patients with at least one follow-up visit, no pulmonary endarterectomy and at least three of the six variables available, and classified the patients into low-, intermediate- and high-risk groups. As a secondary analysis, the number of noninvasive low-risk criteria was counted. The association between risk assessment and survival was evaluated.Data from inclusion and follow-up (median 7 months) visits were available for 561 and 231 patients, respectively. Baseline 1- and 5-year survival estimates were significantly different (p<0.0001) in the baseline low-risk (98.6% and 88.3%, respectively), intermediate-risk (94.9% and 61.8%, respectively) and high-risk (75.5% and 32.9%, respectively) cohorts. Follow-up data were even more discriminative, with 100%, 92% and 69% 1-year survival, respectively. The number of low-risk noninvasive criteria was also associated with survival.These analyses suggest that the ESC/ERS risk assessment may be applicable in patients with medically treated CTEPH.

scholarly journals Chronic thromboembolic pulmonary hypertension: a review

2021 ◽  
pp. 318-325
Author(s):  
CZ Chong ◽  
ELW Tay ◽  
CH Sia ◽  
KK Poh
Keyword(s):  
Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
World Health ◽  
Definitive Treatment ◽  
Pharmacological Agents ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension resulting from obstruction of the pulmonary artery by fibrotic thromboembolic material, usually initiated by recurrent or incomplete resolution of pulmonary embolism. This distinct form of pulmonary hypertension is classified under Group 4 of the World Health Organization classification. Further investigations are usually initiated, with transthoracic echocardiography followed by right heart catheterisation and pulmonary angiography as the gold standard. Definitive treatment is usually in the form of surgical pulmonary endarterectomy. Inoperable CTEPH is medically treated with pharmacological agents such as phosphodiesterase Type 5 inhibitors, endothelin receptor antagonists, soluble guanylate cyclase stimulators and prostacyclin. Recent developments have made balloon pulmonary angioplasty a viable option as well.

P2774Galectin-3, GDF-15, and ST2 in noninvasive assessment of myocardial remodelling in chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S D Kriechbaum ◽  
K Peters ◽  
R Ajnwojner ◽  
J S Wolter ◽  
M Haas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Disease Severity ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Control Group ◽  
Right Heart ◽  
Non Invasive ◽  
Galectin 3 ◽  
Myocardial Remodelling ◽  
Thromboembolic Pulmonary Hypertension

Abstract Background In chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary artery obstruction leads to impaired pulmonary hemodynamics and secondary right heart failure, which is highly predictive of outcome. Thus, the extent of myocardial -especially right heart- remodelling is an indicator of disease severity. Purpose The aim of the present study was to assess growth differentiation factor-15 (GDF-15), galectin-3, and suppression of tumorigenicity 2 (ST2) as non-invasive biomarkers of myocardial remodelling in patients suffering from CTEPH. Methods We analysed the serum levels of GDF-15, galectin-3 and ST2 in a cohort of 64 CTEPH patients and in a control group of 25 patients without cardiovascular disease. The biomarker levels were further correlated with clinical, laboratory, and hemodynamic data, including 6-minute walking distance (6-MWD), N-terminal pro-brain natriuretic peptide (NT-proBNP), mean pulmonary artery pressure (meanPAP), pulmonary vascular resistance (PVR), and right atrial pressure (RAP). Results The biomarker levels in the control group were: galectin-3: 3.5 ng/l (IQR 2.7–4.0), GDF-15: 92.6 pg/ml (IQR 78.5–129.1), and ST2: 48.65 ng/l (IQR 35.5–57.0). CTEPH patients had higher levels of GDF-15 (196.7 pg/ml; IQR 128.4–302.8; p<0.001) and ST2 (52.6 ng/l; IQR 44.5–71.9; p=0.05) but not galectin-3 (3.4 ng/l; IQR 2.7–4.3; p=0.84). In the CTEPH cohort, patients with a meanPAP >35 mmHg (GDF-15: p=0.01; ST2: p=0.04) and patients with a PVR >500 dyn sec cm–5 (GDF-15: p=0.004; ST2: p=0.002) had significantly increased biomarker levels. For the detection of a meanPAP >35mmHg, ROC analysis revealed an AUC of 0.71 for GDF-15 and 0.67 for ST2. The level of GDF-15 correlated with the level of NT-proBNP (rrs=0.69; p≤0.001) and the RAP (rrs=0.54; p≤0.001) and inversely with the 6-MWD (rrs=−0.47; p≤0.001). The level of ST2 correlated with the level of NT-proBNP (rrs=0.67; p≤0.001) and the RAP (rrs=0.54; p≤0.001) and inversely with the 6-MWD (rrs=-0.31; p=0.02). Conclusion Our results demonstrate that GDF-15 and ST2, non-invasive biomarkers of myocardial remodelling, are significantly elevated in patients suffering from CTEPH. The correlation of biomarker levels with established outcome predictors suggests a use as indicators of disease severity.

scholarly journals Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience

Open Heart ◽  
2020 ◽  
Vol 7 (1) ◽  
pp. e001144 ◽  
Author(s):  
Stephen P Hoole ◽  
John G Coghlan ◽  
John E Cannon ◽  
Dolores Taboada ◽  
Mark Toshner ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Functional Status ◽  
Minute Ventilation ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Carbon Dioxide Production ◽  
Functional Class ◽  
Right Heart Catheterisation ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Uk

ObjectiveInoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for the UK opened in October 2015 and we now describe the treatment of our initial patient cohort.MethodsThirty consecutive, inoperable, anatomically suitable, symptomatic patients on stable medical therapy for CTEPH were identified and offered BPA. They initially underwent baseline investigations including Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) quality of life (QoL) questionnaire, cardiopulmonary exercise test, 6 min walk distance (6MWD), transthoracic echocardiography, N-terminal probrain natriuretic peptide (NT pro-BNP) and right heart catheterisation. Serial BPA sessions were then performed and after completion, the treatment effect was gauged by comparing the same investigations at 3 months follow-up.ResultsA median of 3 (IQR 1–6) BPA sessions per patient resulted in a significant improvement in functional status (WHO functional class ≥3: 24 vs 4, p<0.0001) and QoL (CAMPHOR symptom score: 8.7±5.4 vs 5.6±6.1, p=0.0005) with reductions in pulmonary pressures (mean pulmonary artery pressure: 44.7±11.0 vs 34.4±8.3 mm Hg, p<0.0001) and resistance (pulmonary vascular resistance: 663±281 vs 436±196 dyn.s.cm-5, p<0.0001). Exercise capacity improved (minute ventilation/carbon dioxide production: 55.3±12.2 vs 45.0±7.8, p=0.03 and 6MWD: 366±107 vs 440±94 m, p<0.0001) and there was reduction in right ventricular (RV) stretch (NT pro-BNP: 442 (IQR 168–1607) vs 202 (IQR 105–447) pg/mL, p<0.0001) and dimensions (mid RV diameter: 4.4±1.0 vs 3.8±0.7 cm, p=0.002). There were no deaths or life-threatening complications and the mild-moderate per-procedure complication rate was 10.5%.ConclusionsBPA is safe and improves the functional status, QoL, pulmonary haemodynamics and RV dimensions of patients with inoperable CTEPH.

Long Term Results of Pulmonary Endarterectomy (PEA) in Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH). The Pavia Endarterectomy Program.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4006-4006
Author(s):  
Franco Piovella ◽  
Andrea M. D’Armini ◽  
Marisa Barone ◽  
Vincenzo Emmi ◽  
Chiara Beltrametti ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Nyha Class ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Class I ◽  
Pulmonary Endarterectomy ◽  
Artery Pressure ◽  
Thromboembolic Pulmonary Hypertension

Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which results from obstruction of the major pulmonary arteries by incompletely resolved or organized pulmonary emboli which have become incorporated into the pulmonary artery wall, eventually causing an increase in pulmonary vascular resistances. Pulmonary endarterectomy (PEA) is the treatment of choice. Careful pre- and post-operative management is essential for a successful outcome following PEA. In 1994, we started in Pavia a program in which members of a multidisciplinary team work in close interaction with the aim of increase experience in the challenging problems these patients present in the evaluative, surgical, and post-operative phases of their care. So far, 134 PEAs have been performed. Preoperatively, New York Heart Association (NYHA) class distribution was respectively 3-II, 56-III, and 75-IV; mean pulmonary artery pressure and pulmonary vascular resistances were 47 ± 13 mmHg and 1149 ± 535 dynes/sec/cm−5 respectively. The overall operative mortality has been 9.7% (in 2005 mortality rate was 4.5%). At present, 92% of the PEA patients are actively participating in the follow-up study. Follow-up visits are at 3 months after PEA, yearly for the following 5 years, and then at 7, 10, and 15 years postoperatively. Both early and late survivals were excellent. Survival rate at 3 months, 1 year, and 3 years were respectively of 89.5±2.7%, 87.3±3.0%, and 82.7±3.6%. Survival rates had not changed at 5, 7, and 10 years postoperative. Three months after PEA, 29 (58%) subjects were within NYHA class I, 18 (36%) in class II, and 3 (6%) in class III. At 1-year follow-up, 40 (80%) patients were within NYHA class I, 10 (20%) in class II. A statistically significant difference exists not only between the preoperative and the postoperative data (p <0.0001), but also between the functional status at 3 months and the other two postoperative controls (p <0.001). Table summarizes the results of hemodynamic tests collected at three months, one year and three years on the first 35 patients who completed the follow-up program. Hemodynamic data from 35 patients participating to the Pavia Pulmonary Endarterectomy Program with complete 3-year follow-up. CVP mPAP CO CI PVR PVRI CVP (mmHg) central venous pressure; mPAP (mmHg) mean pulmonary artery pressure; CO (L/min) cardiac output; CI (L/min/m2) cardiac index; PVR (dynes/sec/cm-5) pulmonary vascular resistances; PVRI (dynes/sec/cm-5/m2) pulmonary vascular resistances index; RV-EF (%) right ventricle ejection fraction. RV-EF A: Before-PEA 7±6 48±12 3.3±0.9 1.8±0.5 1125±412 2027±731 15±8 B:Before discharge 5±4 25±10 5.2±1.1 2.9±0.5 289±142 505±234 32±8 C: 3 months 2±2 24±11 5.1±1.4 2.8±0.6 231±198 542±271 32±7 D: 1 year 1±2 23±12 5.0±1.1 2.7±0.6 290±191 531±343 35±8 E: 3 years 2±2 24±12 4.9±1.1 2.6±0.5 317±226 579±393 34±8 p value A vs. B: nsA vs. C, D, and E: <0.0001B vs. C, D and E: <0.05 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E:

scholarly journals Comparison of 2-years follow-up of optimal medical therapy versus balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A R Pereira ◽  
R Cale ◽  
F Ferreira ◽  
S Alegria ◽  
S Sebaiti ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Functional Capacity ◽  
Oxygen Therapy ◽  
Elevated Serum ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Medium Term ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

Abstract Introduction Balloon pulmonary angioplasty (BPA) has emerged as a therapeutic option for chronic thromboembolic pulmonary hypertension (CTEPH) considered ineligible for pulmonary endarterectomy (PEA). The initial publications showed very good short-term outcomes for the technique, but there are limited data regarding medium-term outcomes and its comparison with optimal medical treatment (OMT). Objectives To evaluate and compare the medium-term outcomes of OMT versus (vs) BPA in inoperable CTEPH. Methods Retrospective study of consecutive patients (pts) with CTEPH followed in a referral centre for Pulmonary Hypertension. Selected those pts considered ineligible for PEA and with at least 2 years of follow-up. Comparison between two treatment strategies: OMT alone [maximum tolerated doses of pulmonary vasodilator drugs (PVD), as indicated] vs BPA (pts who completed the program with or without OMT). Endpoint was a composite of all-cause death and unplanned right heart failure admission at 2-year. Results From 62 pts, 19 pts were included (11 pts were excluded due to recent diagnosis; 32 were submitted to EAP): mean age 65.0±15.3 years, 89.5% female. At diagnosis, all pts had functional capacity limitation and elevated serum NTproBNP levels (median value 1255.0 pg/mL). Mean pulmonary arterial pressure (mPAP) was 46.2±9.3 mmHg and pulmonary vascular resistance (PVR) 15.3±8.3 Wood units (WU). Concerning treatment, 12 pts (63.2%) underwent OMT alone. These pts had higher NTproBNP levels (2670.0 vs 538.0 pg/mL, p&lt;0.01) and PVR values (19.7±7.6 vs 9.7±5.4 WU, p=0.01) and lower CI (1.6±0.3 vs 2.4±0.5 L/min/m2, p&lt;0.01), at baseline; the remaining basal features didn't differ among groups (Fig.A). At 2-year follow-up, pts submitted to BPA were under PVD in 71.4% of cases with a mean of 1±0.8 drugs per patient and no difference compared to OMT group (83.3%, 1.7±0.9 drugs per patient), although oxygen therapy was higher in medical group (50% vs 0%, p=0.04). A significant overall improvement was observed in BPA group (Table – A): all pts were in functional class I (p&lt;0.01), no one had right ventricular dysfunction (p&lt;0.01) and mPAP decreased to 25.1±6.7 mmHg (p=0.01) and RVP to 2.9±0.8 WU (p=0.01). Inversely, no change was observed in pts under OMT alone (p&gt;0.05 in all, Table – A). Endpoint rate was 31.6% with all adverse events occurring in the OMT group (50% vs 0%, p=0.04). After adjustment by Cox regression, no difference in baseline or follow-up features besides treatment influenced the outcome. Kaplan-Meier analysis (Graphic – B) confirmed significant benefit of BPA in 2-year outcome occurrence (long rank 4.6, p=0.03). Conclusions BPA strategy seems to improve medium-term functional capacity, right ventricular function and haemodynamics and decrease oxygen therapy dependence in inoperable CTEPH. Pts under OMT alone have a poor prognosis. These data encourage the development and implementation of the technique for inoperable CTEPH. FUNDunding Acknowledgement Type of funding sources: None.

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