Idiopathic ventricular fibrillation - Long term prognosis in relation to clinical findings and ECG patterns in a Swedish cohort.

BACKGROUND: Idiopathic ventricular fibrillation (IVF) is a rare cause of sudden cardiac arrest which may pose therapeutic and prognostic challenges. To date, the only effective treatment for survivors of cardiac arrest is the insertion of an implantable cardioverter-defibrillator (ICD). We sought to review the long-term outcome of a Swedish cohort with IVF. METHODS AND RESULTS: Fifty patients with IVF diagnosis between 1988 and 2016 (mean age at index 34.3, 56% male), were followed for a median 13.8 years in this retrospective multicenter observational study. No cardiac mortality was reported. 32% (n = 16) of patients had recurrence of ventricular fibrillation or sustained ventricular tachycardia, requiring ICD therapy, at a median time of 1.9 years (range 0.1-20.3) from the index event. Annual incidence rate of ventricular tachyarrhythmia was 3.1%. Abnormal ECG at baseline did not predict appropriate ICD therapy (p = 0.56). During the follow-up period, 14% (n = 7) patients received a cardiac diagnosis. Follow-up genetic testing was low (26%), however did confirm pathogenic mutations in three cases. CONCLUSION: Idiopathic VF is a rare diagnosis with a relatively good prognosis provided ICD therapy is initiated. Routine clinical follow-up is recommended due to potential late emerging cardiac pathology. ECG changes are common, but have no prognostic value in determining the risk of ventricular arrhythmias recurrence. Screening for genetic diseases has previously been low, and this calls for improvement, especially since cheaper and more comprehensive genetic panels are now readily available.

Fecal fat, bile acid, and sterol excretion abd biliary lipid changes in jejunoileostomy patients.

Fecal fat, bile acid, and neutral sterol excretion and biliary bile acid, phospholipid, and cholesterol were studied in 36 patients 6 to 12 months after jejunoileostomy for obesity. No relationship was observed between the degree of steatorrhea and weight loss, although fecal fat rose sharply in all except 2 patients. Mean neutral sterol excretion in feces was unchanged after operation except in cholecystectomized patients. No relationship could be demonstrated between serum cholesterol decrease and fecal biel acid, which rose 3-fold, or between cholesterol and the sum of fecal fat and neutral sterol. Fecal excretion of cholic and deoxycholic acid together increased more than the total excretion of cxcretion patterns were demonstrable: moderate fecal fat (24 g or less on 65-g intake) was associated with a predominance of secondary bile acids in feces and in bile; high fecal fat (above 24 g/day) was associated with predominantly primary bile acids. A high incidence of gallstones or previous cholecystectomy (36%) was found preoperatively and of 25 patients with normal cholecystograms, four developed stones and five had nonvisualization of gallbladder 6 to 12 months postoperatively. Bile saturation ratio and lithogenic index were not consistently changed postoperatively. No clear cut increase in lithogenic potential or in bile acid (lithocholic acid) hepatotoxic potential after jejunoileostomy was demonstrable.

Bile salt metabolism in tropical sprue.

Mean and peak jejunal bile salt concentrations during digestion of a standard fat meal were found to be significantly lower in six Puerto Rican patients with untreated tropical sprue, all of whom had steatorrhoea, than in six asymptomatic subjects who had normal fat absorption. Bile salt pool size and turnover time did not differ significantly in the two groups.It is suggested that bile salt concentrations may be reduced in the proximal small intestine of patients with tropical sprue as a result of excessive dilution by intestinal fluid. The finding of low bile salt concentrations in two asymptomatic subjects indicates that bile salt lack alone may not be sufficient to produce steatorrhoea.