Pulmonary arterial hypertension (PAH) secondary to congenital heart disease (CHD) is the third most common cause of PAH, and it is becoming increasingly common as improvements in the management of CHD have led to increased life expectancy for these patients. The medical management of PAH due to CHD (PAH-CHD) is largely the same as what has been used for the treatment of idiopathic PAH, though the body of literature supporting this management decision is very small. There are currently few studies available which specifically focus on the treatment of PAH-CHD. The purpose of this literature review is to compare the results of those studies that assessed the response to medical therapy among adults with PAH-CHD; studies were excluded if they focused on paediatric patients, did not include an assessment of 6 min walking distance or specifically assessed combination therapies. This review found that riociguat, bosentan, epoprostenol and sildenafil were all capable of improving functional capacity and haemodynamic parameters in patients with PAH-CHD, but whether this corresponds to an increase in mortality remains to be seen. Limitations of this review include the small sample size and variable duration of the included studies, which makes drawing direct comparisons between studies and the study drugs difficult. The lack of large, randomised double-blind clinical trials comparing different drugs head to head highlights an area that is ripe for ongoing medical research, the results of which may help shape future treatment algorithms tailored specifically for adults with PAH-CHD.
Refractory angina is a life-disabling disease, even with the discovery of antianginal drugs and the advances in revascularization surgically or percutaneously to improve symptoms. Over the last decade a renewal of interest in an old surgical modality of narrowing the coronary sinus has evolved. Although the surgical procedure idea was born in 1940 it was overshadowed by the development of coronary artery bypass graft and percutaneous interventions. Recently, a percutaneous approach of reducing the coronary sinus size has been developed and several clinical studies have been reported in refractory angina patients. We review the history of coronary sinus intervention, and explore coronary sinus stent possible mechanism of action, device design, and the clinical data supporting its use.
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