Disease targeting therapies in patients with Eisenmenger syndrome: Response to treatment and long-term efficiency

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

“…Subsequent observational studies confirmed the beneficial effect on symptoms and exercise capacity and, with a few exceptions [57,58], provided evidence that this symptomatic improvement is maintained mid-term. More recently, two studies have addressed the long-term impact of DTT in ES patients [59,60], confirming a sustained positive impact on WHO class and 6MWD in the long term. Improvement in quality of life has also been demonstrated [61].…”

“…However, these were stable ES patients and, therefore, the study does not adequately reflect routine clinical practice of initiating combination therapy in patients deteriorating on oral monotherapy. Two observational studies [60,66] in contrast have demonstrated that initiation of dual or combination therapy in ES patients with a clinical indication improves 6MWD and hemodynamics. Therefore, in accordance to current guidelines, combination therapy may be considered in WHO class III ES patients.…”

Pulmonary arterial hypertension associated with congenital heart disease

“…3). Long-term PAH-specific therapy in patients with Eisenmenger's syndrome has been shown to improve both objective exercise capacity and subjective symptoms, although escalation of therapy over time may be required if symptoms deteriorate during treatment [61]. There are also data to show that PAH-specific therapies increase QoL scores in patients with PAH-CHD [49].…”

The spectrum of pulmonary arterial hypertension in adults with congenital heart disease: management from a physician and nurse specialist perspective

“…Longitudinal studies have now demonstrated a long-term benefit from the use of PAH-therapies in PAH-CHD, in terms of exercise capacity and functional state, despite the progressive nature of the disease [5]. Moreover, evidence of an improvement in survival with PAH-therapies is now available from a retrospective propensity score adjusted single centre study on a large number of ES patients [4].…”

“…Despite significant similarities in pulmonary pathology, PAH-CHD differs from other types on PAH and expertise in both CHD and PAH is required for optimal management. While conventional measures and avoidance of pitfalls remain extremely important, the recent availability of PAH therapies has revolutionized the treatment of PAH-CHD [4,5]. We provide an overview of the modern management of the entire spectrum of PAH-CHD in adults, both from a physician and clinical nurse specialist perspective.…”