Complications leading to sudden cardiac death in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a disease of small pulmonary arteries, characterized by vascular proliferation and remodeling. Progressive increase in pulmonary vascular resistance ultimately leads to right ventricular heart failure and death. PAH-specific drug therapy has improved clinical outcomes and survival. While the survival is better, progression of pulmonary vasculopathy contributes to pulmonary artery dilatation. 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. 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. PAH referral centers provide multidisciplinary emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients. We review the complications leading to sudden death in PAH.

Successful epoprostenol withdrawal in pulmonary arterial hypertension: case report and literature review.

Pulmonary arterial hypertension is a rare and devastating disease characterized by vascular proliferation and remodeling. Epoprostenol, the drug counterpart of the eicosanoid prostacyclin, produced by the vascular endothelial cells, is the drug of choice for this disease. Its capacity to act rapidly and to significantly improve survival prospects in severe pulmonary hypertension patients has been supported by a wealth of evidence. Intravenous epoprostenol was believed to require therapy of indefinite duration. Since 2001, oral drugs have been approved for specific treatment. The availability of newer and less invasive drug therapies for pulmonary arterial hypertension led physicians to withdraw epoprostenol in carefully selected patients. We report a case of successful intravenous epoprostenol interruption in a patient with idiopathic disease. A literature review on epoprostenol withdrawal in pulmonary hypertension in adult patients is also provided.

Cardiac resynchronization therapy decreases the mitral coaptation point displacement in heart failure patients.

BACKGROUND: In patients with left ventricular (LV) dysfunction the mitral leaflet coaptation point (CPMA) is displaced towards the LV apex. The aim of our study was to estimate the value of CPMA measurement as a simple index regarding the acute effects of cardiac resynchronization therapy (CRT), which is coming to be an established method of treatment for congestive heart failure (CHF). METHODS: We studied 20 patients with CHF (NYHA III-IV) and LV ejection fraction (LVEF) 22 +/- 4%. All patients received CRT and an echocardiogram was performed within 24-48 hours. The echocardiographic indices LV end-diastolic diameter (LVEDD) and end-systolic diameter (LVESD), LVEF, mitral annulus diameter (MAD), and the degree of intraventricular desynchronization, were measured at CRT off and CRT on. The CPMA, the distance between the coaptation point of the mitral leaflets and the mitral annulus, was measured from the apical 4-chamber view in end-systole at both CRT on and CRT off. RESULTS: CRT improved both the contractility and dimensional indices in CHF patients. CPMA decreased from 11.3 +/- 2 mm at CRT off to 9.1 +/- 1.8 mm after CRT on (p < 0.001) and MAD from 38.9 +/- 3.9 mm at CRT off to 37.5 +/- 3.7 mm at CRT on (p < 0.002). LVEF improved from 24.5 +/- 5.7% at CRT off to 29.5 +/- 5.1% at CRT on (p < 0.001). There was an improvement in LV synchronization from 88 +/- 7 ms at CRT off to 48 +/- 3 ms at CRT on (p < .001). CPMA was correlated with MAD (r = 0.52, p < 0.05 and r = 0.59, p < 0.05 at CRT off and CRT on, respectively). Moreover, the absolute change in CPMA was correlated with LVESD (r = 0.68) and LVEDD (r = 0.65), both p < 0.05, with the time difference of the basal segments of the septal and lateral wall at CRT on (r = 0.68, p < 0.01), and inversely correlated with LVEF (r = -0.55, p < 0.05). CONCLUSION: In patients with severe LV systolic dysfunction and dilatation CRT was associated with an improvement in both CPMA and MAD.

Resolution of Thrombi in Left Atrial Appendage Aneurysm.

Atrial appendage aneurysm is a rare cardiac disease and may be complicated by embolic events and rhythm disturbances. We describe a case of a congenital left atrial aneurysm presenting with a cerebrovascular accident in a 57-year-old female. The diagnosis was made by transesophageal echocardiography, which revealed the presence of intraaneurysmal thrombi. The patient initially received anticoagulant therapy and the thrombi were resolved. Subsequently, she was successfully operated on and the aneurysm was removed. This is the first report of thrombi resolution in a left atrial appendage aneurysm.