Pulmonary Arterial Hypertension Complicating Congenital Heart Disease: Advances in Therapy

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Congenital heart disease (CHD) is the most common inborn defect, affecting approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to dramatic improvements in patients' quality of life and long-term survival. Nevertheless, despite these innovations many patients require life-long follow-up and are at risk of numerous complications, namely, residual or progressive hemodynamic lesions, arrhythmia, sudden cardiac death, and the development of heart failure and pulmonary arterial hypertension (PAH), despite timely surgical intervention. Ill advisedly, some patients are also lost to follow-up, assuming that their early life surgical or catheter-based intervention was curative, hence missing out on invaluable lifetime screening and timely interventions that may be necessary. Additionally, there are many patients with undiagnosed or unoperated CHD in the developing world presenting later in life with irreversible complications. Even in this modern era of diagnosis and screening, PAH-CHD remains a burden in patients with CHD, with approximations of up to 10% in this heterogeneous group and is associated with an adverse impact on quality of life and survival.

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