The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke, Robert; Diller, Gerhard‐Paul; Baumgartner, Helmut

doi:10.1586/14779072.2013.811966

Cited by 11 publications

(10 citation statements)

References 96 publications

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“…Adults with congenital heart disease (CHD) have a lower life expectancy than their healthy counterparts [ 1 ], especially when pulmonary arterial hypertension (PAH) develops [ 2 – 7 ]. PAH-specific therapy has become the cornerstone in the management of these patients because of its beneficial effects on disease symptoms, haemodynamics and quality of life (QoL) [ 3 , 8 – 10 ].…”

Section: Introductionmentioning

confidence: 99%

Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease

et al. 2015

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BackgroundDecrease in quality of life (QoL) in left-sided heart failure precedes poor survival, which can be reversed with exercise training. We investigated whether QoL is associated with mortality in pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) patients.MethodsIn this observational study, PAH-CHD adults referred for PAH-specific therapy were included. QoL surveys (SF36) were recorded during 2 years of therapy. Based on shift in SF36 scores during this period, patients had either decreased or non-decreased QoL. Subsequently, the patients were followed for mortality.ResultsThirty-nine PAH-CHD patients (mean age 42, 44 % male, 49 % Down’s syndrome) were analysed. Following PAH-specific therapy, SF36 physical component summary (PCS) decreased in 13 (35–31 points, p = 0.001) and showed no decrease in 26 patients (34–43 points, mean values, p < 0.001). Post-initiation phase, median follow-up was 4.5 years, during which 12 deaths occurred (31 %), 10 (56 %) in the decreased and 2 (10 %) in the non-decreased group (p = 0.002). Cox regression showed a decrease in SF36 PCS predicted mortality (HR 3.4, 95 % CI 1.03–11, p = 0.045).ConclusionsIn PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.Electronic supplementary materialThe online version of this article (doi:10.1007/s12471-015-0666-9) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease

et al. 2015

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“…The positive impact of disease targeting therapies in patients with CHD-PAH is well recognised and has been confirmed by numerous previous studies 2. Currently, the most frequently used therapies are ERAs or PDE-5 inhibitors.…”

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confidence: 67%

“…This progress has been largely driven by the availability of new therapeutic agents that have been gradually introduced into clinical practice and have been demonstrated to improve exercise capacity, quality of life and possibly survival in selected patients subgroups 1 2. Unlike in patients with idiopathic PAH, recognised to have a median life expectancy <3 years if left untreated,3 many physicians have not felt the urgency of treating patients with CHD-PAH equally aggressively.…”

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confidence: 99%

Riociguat for pulmonary hypertension in congenital heart disease: opportunities and challenges

Lammers

Diller

2015

Heart

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“…Retrospective observational studies also point to a potential improvement in the prognosis of patients with ES when treated with targeted drugs [67,69,70]. Overall, there is evidence of symptomatic improvement with drug therapy for PAH in patients with ES categorized as WHO-FC III, both in the short term based on RCT data and in the mid-to long-term under "real-world" conditions [71]. Formally, there is no evidence for patients in WHO-FC II, although in clinical practice a precise determination of WHO-FC is problematic because of frequently fluctuating symptoms.…”

Section: Eisenmenger Syndromementioning

confidence: 97%

Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018

Kaemmerer

Apitz

Brockmeier

et al. 2018

International Journal of Cardiology

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In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article.

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The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Cited by 11 publications

References 96 publications

Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease

Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease

Riociguat for pulmonary hypertension in congenital heart disease: opportunities and challenges

Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018

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