A short story of the short QT syndrome.

Short QT syndrome is a recently recognized cause of cardiac rhythm disorders, including sudden cardiac death. Although the syndrome is rare, its potential lethality justifies routinely screening the electrocardiograms of patients with syncope or unexplained atrial or ventricular arrhythmias to look for this diagnosis. This review discusses recent advances in the understanding of the pathogenesis of this syndrome and outlines some of the challenges in establishing the diagnosis.

Atrial arrhythmias after lung and heart-lung transplant: effects on short-term mortality and the influence of amiodarone.

BACKGROUND: The incidence and effect of atrial fibrillation or flutter (AF) after lung transplant are variable. An effect of pharmacologic treatment on outcomes is undetermined. METHODS: One hundred thirty-seven consecutive lung or heart-lung transplant patients were reviewed retrospectively. Uni- and multivariate analyses were performed to determine statistically significant risk factors for AF and short-term mortality. RESULTS: AF occurred in 45.0% of patients within 26 days. By univariate analysis, male gender was predictive of AF (hazard ratio [HR] = 2.25, 95% confidence interval [CI] 1.21 to 4.20, p = 0.011). Mortality within 200 days occurred in 36 of 137 (22.6%) patients. Those with AF had higher mortality than those without AF (27 of 62 [43.5%] vs 9 of 75 [12%]; p < 0.0001). Twenty-four of the 38 (63.1%) patients treated with amiodarone died, whereas 3 of 26 (11.5%) patients treated without amiodarone died (p < 0.0001). Mortality was similar among patients without AF, and those with AF not receiving amiodarone (9 of 75 [12%] vs 3 of 26 [11.5%], p = 1.00). By multivariate analysis, chronic obstructive pulmonary disease (HR = 0.395, CI 0.175 to 0.892, p = 0.025), primary pulmonary hypertension (HR = 7.245, CI 1.89 to 27.84, p = 0.0039), and use of amiodarone (HR = 2.967, CI 1.187 to 7.415, p = 0.020) were associated with death. Amiodarone was shown to be a significant statistical moderator (p < 0.0001) and mediator (p < 0.001) of mortality in AF patients. CONCLUSIONS: There was a high incidence of AF after lung or heart-lung transplant, and a significant increase in mortality in AF patients treated with amiodarone. In patients with severe lung pathology, amiodarone pulmonary toxicity may be more common than previously known, and may be a significant contributor to mortality. Amiodarone use should be restricted in the lung transplant patient population.