3 Likewise, 99 out of 316 patients died in the Leuven database during follow-up, of whom 18 suddenly. In the American National Institute of Health registry, 106 deaths were reported in a cohort of 194 patients with idiopathic PAH, of which 26% were sudden. Sudden cardiac death is now encountered more often in PAH patients. 2 Moreover, new imaging modalities enable us to recognize major complications, previously missed. While the survival is better, pulmonary hypertension continues to cause substantial morbidity and mortality, as progression of the pulmonary vasculopathy leads to progressive RV failure. In the current era, 8 drugs from 3 pharmacologic classes (endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids), administered per os, via inhalation, subcutaneously, or intravenously, have been approved for PAH patients, improving survival. Two decades ago, the median survival rate from diagnosis, despite the available supportive treatment, 1 was less than 3 years. Pulmonary arterial hypertension (PAH) is a devastating disease, leading to right ventricular (RV) heart failure and death. We review the complications leading to sudden death in PAH. PAH referral centers provide multidisciplinary emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients. The advent of PAH-targeted drug therapy has reduced referral for lung transplantation however, severe complications require rapid diagnosis, decision making, and possible registration on a lung transplantation waiting list. Left main compression syndrome, pulmonary artery dissection, pulmonary artery rupture, and severe hemoptysis are reported as complications leading to sudden cardiac death, an event encountered more often in PAH patients. While the survival is better, progression of pulmonary vasculopathy contributes to pulmonary artery dilatation. PAH-specific drug therapy has improved clinical outcomes and survival. Progressive increase in pulmonary vascular resistance ultimately leads to right ventricular heart failure and death. After the incision is made, a thin, flexible tube is inserted into an artery to guide the artificial heart valve up to your heart to replace the diseased valve.Pulmonary arterial hypertension (PAH) is a disease of small pulmonary arteries, characterized by vascular proliferation and remodeling. Your doctor will make a small incision on your body. TAVR is less invasive than open-heart surgery. Transcatheter aortic valve replacement (TAVR) Learn more about surgical heart valve repair. Patients usually need to stay in the hospital for a week or more, before beginning a longer period of recovery. Open-heart surgery is done to remove the damaged valve and replace it with an artificial valve. Your heart team (a specialized care team that includes interventional cardiologists, cardiac surgeons, imaging specialists, anesthesiologists, and other doctors as needed) will decide which of the following treatment options is best for you: MedicationĬertain medications may ease some of your symptoms.Ī tiny balloon is inflated in the aortic valve to try and improve blood flow, but this treatment typically provides only temporary relief.
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