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      Pulmonary Arterial Hypertension Medical Management of the Adult Patient with Congenital Heart Disease

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          Congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation. CHD-associated PAH has an estimated prevalence of 5–10% in adult patients, with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension (PAH)-targeted pharmacotherapy. Although limited data exist, targeted PAH pharmacotherapy has proven to be beneficial in patients with CHD-associated PAH, with observed improvement in functional class, increase in exercise capacity, and improvement in quality of life and cardiopulmonary hemodynamics. Additionally, there has been increasing interest in the “treat-to-close” strategy. PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’ operability in repairing the cardiac defect. Although there have been significant advances in the management of this disease state in the past 2 decades, mortality remains high, and ongoing clinical trials are needed to better understand the treat-to-close strategy.

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          Most cited references 38

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          Congenital heart disease in the general population: changing prevalence and age distribution.

          Empirical data on the changing epidemiology of congenital heart disease (CHD) are scant. We determined the prevalence, age distribution, and proportion of adults and children with severe and other forms of CHD in the general population from 1985 to 2000. Where healthcare access is universal, we used administrative databases that systematically recorded all diagnoses and claims. Diagnostic codes conformed to the International Classification of Disease, ninth revision. Severe CHD was defined as tetralogy of Fallot, truncus arteriosus, transposition complexes, endocardial cushion defects, and univentricular heart. Prevalence of severe and other CHD lesions was determined in 1985, 1990, 1995, and 2000 using population numbers in Quebec. Children were subjects <18 years of age. The prevalence was 4.09 per 1000 adults in the year 2000 for all CHD and 0.38 per 1000 (9%) for those with severe lesions. Female subjects accounted for 57% of the adult CHD population. The median age of all patients with severe CHD was 11 years (interquartile range, 4 to 22 years) in 1985 and 17 years (interquartile range, 10 to 28 years) in 2000 (P<0.0001). The prevalence of severe CHD increased from 1985 to 2000, but the increase in adults was significantly higher than that observed in children. In the year 2000, 49% of those alive with severe CHD were adults. The prevalence in adults and median age of patients with severe CHD increased in the general population from 1985 to 2000. In 2000, there were nearly equal numbers of adults and children with severe CHD.
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            Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

            Data on the effect of initial combination therapy with ambrisentan and tadalafil on long-term outcomes in patients with pulmonary arterial hypertension are scarce.
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              Selexipag for the Treatment of Pulmonary Arterial Hypertension.

              In a phase 2 trial, selexipag, an oral selective IP prostacyclin-receptor agonist, was shown to be beneficial in the treatment of pulmonary arterial hypertension.

                Author and article information

                Cardiovascular Innovations and Applications
                Compuscript (Ireland )
                May 2018
                May 2018
                : 3
                : 1
                : 1-8
                1University of Florida, Division of Pulmonary, Critical Care and Sleep Medicine, 1600 SW Archer Rd, M452, PO Box 100225, Gainesville, FL, USA
                2University of Florida, Department of Pharmacy, Gainesville, FL, USA
                Author notes
                Correspondence: Hassan Alnuaimat, MD, University of Florida, Division of Pulmonary, Critical Care and Sleep Medicine, 1600 SW Archer Rd, M452, PO Box 100225, Gainesville, FL 32610, USA, E-mail: hassan.alnuaimat@
                Copyright © 2018 Cardiovascular Innovations and Applications

                This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 Unported License (CC BY-NC 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See



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