Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal

Santos, Manuel Filipe; Gomes, A.; Cruz, Célia; Rocha, Jaime; Ricardo, Miguel; Gonçalves, Fabienne; Carvalho, L.; Vicente, Margarida; Melo, Alzira; Reis, Abílio

doi:10.1016/j.repce.2018.02.019

Cited by 2 publications

(9 citation statements)

References 25 publications

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“…The group of CTEPH patients was well characterized in a large prospective international registry which enrolled patients between February 2007 and January 2009. 7 As the phenotype of CTEPH patients may be population dependent, 8 several national reports as summarized in Table 1 7,9–29 were of added value. Most of them, however, recruited patients before the marketing authorization of riociguat by the European Medicines Agency through the European Union (27/03/2014), and before the first experience with balloon pulmonary angioplasty (BPA) outside of Japan.…”

Section: Introductionmentioning

confidence: 99%

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Kopeć

Dzikowska-Diduch

Mroczek

et al. 2021

Therapeutic Advances in Chronic Disease

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Background: Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients’ management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies. Methods: We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry. Results: We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants ( n = 301; 58.3%) were preferred over vitamin K antagonists ( n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment. Conclusions: The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH. Clinical Trial Registration: clinicaltrials.gov/ct2/show/NCT03959748

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Section: Introductionmentioning

confidence: 99%

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Kopeć

Dzikowska-Diduch

Mroczek

et al. 2021

Therapeutic Advances in Chronic Disease

View full text Add to dashboard Cite

show abstract

“…CTEPH is a distinct form of pulmonary vascular disease categorised under group 4 of the WHO clinical classification of PH 1–6. It arises as a consequence of mechanical obstruction caused by thromboembolic material adhering to the PA branches, leading to increased PVR and subsequent PH 1 4.…”

Section: Discussionmentioning

confidence: 99%

“…However, early identification and accurate diagnosis of CTEPH pose challenges, often leading to a delay of approximately 14 months from symptom onset to diagnosis in expert centres. This delay is due to the variable nature of the disease, including a honeymoon period where patients may be asymptomatic, as well as non-specific clinical signs and symptoms related to progressive right heart failure 1–5…”

Section: Discussionmentioning

confidence: 99%

“…Undiagnosed or untreated CTEPH poses a high risk of mortality due to right heart failure 1–5. The haemodynamic result after PEA is drastic and immediately evident in the operating room.…”

Section: Outcome and Follow-upmentioning

confidence: 99%

“…After 1 year, there is a reverse remodelling of the right ventricle, with the disappearance of ventricular hypertrophy and the recovery of a normal ventricular wall motion. The functional improvement of patients is linked to the patient’s age or to the duration of the disease 1–5. During the 10-year follow-up period (table 1), the patient remained asymptomatic, without any evidence of chronic complications or documented PH.…”

Section: Outcome and Follow-upmentioning

confidence: 99%

See 2 more Smart Citations

10-year success story in CTEPH treatment: breaking the myth of rareness

Moura de Azevedo,

Furtado,

Carvalho

et al. 2024

BMJ Case Rep

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Unlike acute pulmonary embolism (PE), the resolution of thrombi is ineffective in chronic thromboembolic pulmonary hypertension (CTEPH), leading to reorganisation and fibrotic changes within the pulmonary arteries. The authors report the case of a man in his 60s with polycythemia vera, under warfarin, following an acute PE. He was admitted a year later with right heart failure and haemodynamic instability. Acute over chronic PE caused this severe presentation, confirmed by right heart catheterisation and pulmonary scintigraphy. The challenging diagnosis and management involved transfer to a centre specialised in pulmonary vascular disease. Normalisation of functional and haemodynamic parameters, sustained in 10-year follow-up, was achieved with anticoagulation and triple therapy with prostanoids as a bridge to pulmonary thromboendarterectomy. Targeted medical therapy, not standard at that time, was crucial to recovering conditions for transfer. An individualised approach, integrating multidisciplinary pulmonary hypertension expertise, provides the basis for the best care for CTEPH.

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Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal

Cited by 2 publications

References 25 publications

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

10-year success story in CTEPH treatment: breaking the myth of rareness

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