The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups

Gall, Henning; Felix, Janine F.; Schneck, Franziska K.; Milger, Katrin; Sommer, Natascha; Voswinckel, Robert; Franco, Oscar H.; Hofman, Albert; Schermuly, Ralph Theo; Weißmann, Norbert; Grimminger, Friedrich; Seeger, Werner; Ghofrani, Hossein Ardeschir

doi:10.1016/j.healun.2017.02.016

Cited by 247 publications

(232 citation statements)

References 49 publications

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“…In terms of survival, the findings are similar to those of the PAPUCO study that reported a high rate of mortality at 6 months follow-up [5]. Nonetheless, the overall 3-year survival in this group of patients is 5 to 10% lower than those reported in studies outside Africa [6,[22][23]. This may be attributed to the exclusion of patients with mild PH in this study, as well as the barriers to early diagnosis and treatment of PH in this setting [5].…”

Section: Discussionsupporting

confidence: 84%

“…Age was one of the prognostic factors for PH mortality. Older age has been shown to place PH patients at an increased risk of mortality [6,22]. The Giessen Pulmonary Hypertension registry demonstrated that age <50 years predicted survival in all PH groups [22].…”

Section: Discussionmentioning

confidence: 99%

“…Older age has been shown to place PH patients at an increased risk of mortality [6,22]. The Giessen Pulmonary Hypertension registry demonstrated that age <50 years predicted survival in all PH groups [22]. A separate study showed that individuals aged ≥65 yrs have high mortality rates [24].…”

Section: Discussionmentioning

confidence: 99%

“…Several clinical variables were associated with mortality similar to other studies. These included poor functional class (WHO functional class III/IV), diabetes mellitus and atrial fibrillation [6,22,24,25].…”

Section: Discussionmentioning

confidence: 99%

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Long-Term Outcomes and Factors Associated with Mortality in Patients with Moderate to Severe Pulmonary Hypertension in Kenya

Ngunga

Abeid

Jeilan

et al. 2020

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Background: Pulmonary hypertension is poorly studied in Africa. The long-term survival rates and prognostic factors associated with mortality in patients with moderate to severe pulmonary hypertension (PH) in Africa are not well described. Objectives:To determine the causes of moderate to severe PH in patients seen in contemporary hospital settings, determine the patients' one-year survival and the factors associated with mortality following standard care. Methods: A retrospective review of patients diagnosed with moderate to severe PH at Aga Khan University Hospital (AKUHN) from August 2014 to July 2017 was carried out. Clinical and outcome data were collected from medical records and the hospital mortality database. Telephone interviews were conducted for patients who died outside the hospital. Survival analysis was done using Kaplan-Meier, and log-rank tests were used to assess differences between subgroups. Cox regression modelling with multivariable adjustment was used to identify factors associated with all-cause mortality. Results: A total of 659 patients with moderate to severe PH were enrolled. Median follow-up time was 626 days. The survival rates of the patients at 1 and 2 years were 73.8% and 65.9%, respectively. The following variables were significantly associated with mortality: diabetes mellitus [adjusted HR 1.52, 95% CI (1.14-2.01)], WHO functional class III/IV [adjusted HR 3.49, 95% CI (2.46-4.95)], atrial fibrillation [adjusted HR 1.53, 95% CI (1.08-2.17)], severe PH [adjusted HR 1.72, 95% CI (1.30-2.27)], right ventricular dysfunction [adjusted HR 2.42, 95% CI (1.76-3.32)] and left ventricular dysfunction [adjusted HR 1.91, 95% CI (1.36-2.69)].Obesity [adjusted HR 0.68, 95% CI (0.50-0.93)] was associated with improved survival. Conclusion: Pulmonary hypertension is associated with poor long-term outcomes in African patients. Identification of prognostic factors associated with high-risk patients will assist in patient management and potentially improved outcomes.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Long-Term Outcomes and Factors Associated with Mortality in Patients with Moderate to Severe Pulmonary Hypertension in Kenya

Ngunga

Abeid

Jeilan

et al. 2020

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“…Our data confirm and expand on previous findings by KYLHAMMAR et al [5] and HOEPER et al [4]. Our study broadens the application of this risk assessment tool to SSc-PAH, which typically carries a dismal prognosis compared to other groups [11].…”

supporting

confidence: 92%

Risk assessment in scleroderma patients with newly diagnosed pulmonary arterial hypertension: application of the ESC/ERS risk prediction model

et al. 2018

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The evolving role of interventional cardiology in the treatment of pulmonary hypertension

Gurevich

Prins

2020

Cathet Cardio Intervent

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Pulmonary hypertension (PH) is a heterogeneous group of diseases defined by a mean pulmonary arterial pressure greater than 20 mmHg. Clinically, PH is classified into five groups and the group of PH generally defines the cause of PH and the therapeutic options. Currently, medical therapies that target the prostacyclin, endothelin, and nitric oxide pathways are used in pulmonary arterial hypertension and chronic thromboembolic PH (CTEPH) patients. Moreover, surgery can improve outcomes in PH as pulmonary thromboendarterectomy can be curative for CTEPH and lung transplantation is used for end‐stage PH. Despite these diverse treatment options, PH patients continue to have high symptom burden and poor long‐term outcomes. However, advances in percutaneous technology are opening new avenues for the management of PH. In this review, we discuss the available data supporting the use of four interventional procedures: balloon atrial septostomy, transcatheter Potts shunt, balloon pulmonary angioplasty, and pulmonary artery denervation for the treatment of PH. These procedures provide hemodynamic and functional improvements in PH patients, but they come with their own unique risk profiles. Hopefully, these procedures will continue to be refined and thereby provide a venue for interventional cardiology to safely and effectively improve outcomes for PH moving forward.

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The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups

Abstract: This is the largest single-center PH cohort described so far. Some parameters used in clinical practice do not independently predict survival. Age, gender and 6MWD outperformed NYHA functional class in predicting survival across all etiologic groups.

Cited by 247 publications

References 49 publications

Long-Term Outcomes and Factors Associated with Mortality in Patients with Moderate to Severe Pulmonary Hypertension in Kenya

Long-Term Outcomes and Factors Associated with Mortality in Patients with Moderate to Severe Pulmonary Hypertension in Kenya

Risk assessment in scleroderma patients with newly diagnosed pulmonary arterial hypertension: application of the ESC/ERS risk prediction model

The evolving role of interventional cardiology in the treatment of pulmonary hypertension

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