Right ventricular cardiomyopathy: a rare and underdiagnosed cause of sudden death in young adults

We present an example of right ventricular cardiomyopathy, a very uncommon condition which caused sudden death in an adolescent boy. This case report is used to highlight the pathological features of this rare and poorly understood condition, which is often undiagnosed (AU)

The rapunzel syndrome: report of a case and review of the literature

The Rapunzel Syndrome, a rare manifestation of trichobezoar, occurs when bolus gastrointestinal obstruction is produced by an unusual trichobezoar with a long tail that extends to or beyond the ileocaecal valve. A five-year-old Jamaican girl presented with this abnormality and was found at laparotomy also to have an ileo-ileal intussuception. For the Rapunzel Syndrome, we recommended bezoar extraction at laparotomy via multiple enterotomies. In addition, psychiatric evaluation and therapy is essential due to the commonly associated finding of underlying emotional stress. (AU)

Permanent pacemaker implantation and follow-up programme: the Barbados and Eastern Caribbean experience - abstract

Fifty-four years after the first pacemaker implant was carried out on humans, the first successful implantation of a permanent pacing system was performed in Barbados in 1986, through the collaborative efforts of the Pacemaker Industry, Rotary International and Rotary Barbados, The Watson Clinic Foundation of Florida and The Queen Elizabeth Hospital, Barbados. In a programme in which pacemakers are supplied and implanted, free of cost to indigent individuals, 26 pacemaker systems have been implanted in 16 males and 10 females, average age 64 years, and age range 27-86 years. There were 17 Barbadians, 4 Antiguans, 2 St. Lucians and one each from Dominica, St. Vincent and Montserrat. The major indication for implantation was symptomatic third degree atrio-ventricular block, which occured in 11 (42.3 percent) of patients. Recurrent dizziness and/or loss of consciousness was the most common symptom - in 21 (80 percent) of patients, and the most common underlying cardiac conditions were myocardial ischaemia in 10 patients, and fibrosis in 7 patients. Cephalic vein cut down and subclavian vein puncture were performed on fourteen (14) and twelve (12) patients respectively. All pacemakers implanted were of the "ventricular pacing and sensing type (VVI)", of which 13 were programmable. Seventeen of the systems were provided by The Intermedics Pacemaker Company and nine by American Pacemakers. Twenty-three (88.5 percent) patients had an entirely successful outcome post implantation with complete resolution of symptoms. One patient died 5 weeks after implantation as a direct result of the procedure, another died within hours of emergengy implantation which had been carried out following several episodes of cardiac arrest, the pacing system was functioning normally at the time of death. A third suffered a cerebro-vascular accident with a progressively poorly functioning left ventricle some months after implantation, again the pacing system was functioning normally. Finally, one patient with previously diagnosed ischaemic heart disease suffered an acute myocardial infarction two months post implant from which he made an uneventful recovery with the pacing system intact. Two patients contracted insignificant wound infections. Pacemaker follow-up programmes aim by continued long-term follow-up to reduce the incidence of sudden and unpredicted pacemaker system failure and detect sub-standard performance of some models. Such a computer-assisted follow-up programme was started at the Queen Elizabeth Hospital, Barbados, on April 3, 1985, with more recently an associated programme at Holberton Hospital, Antigua. Sixty-five patients are registered in the clinic, 33 of whom are males and 32 females, average age 60 years, range 6-88 years. Forty (61.5 percent) patients in the pacemaker follow-up clinic have pacemaker systems in situ with 18 systems being from Intermedics Company, 9 American, 6 Medtronic, 4 Teletronics and one each from Vitatron and Cordis. During the follow-up period two patients had premonitory symptoms and electro-stimulogram derived data to suggest impending system failure, which duly occurred in both patients, one of whom had the successful implantation of a new pacing system while the other had a change of the battery system. Of the 25 patients registered and considered potential candidates for pacemaker implantation, one with asymptomatic complete atrio-ventricular block died. The successful implementation of a permanent pacemaker implantation and follow-up programme for Barbados and the Eastern Caribbean has been established. The major indication for implantation has been symptomatic acquired complete atrio-ventricular block. The main challenge of the computer-assisted pacemaker follow-up programme has been the need to monitor, be familiar with, and have programming equipment appropriate for pacemakers manufactured by several companies. The clinic has been particularly successful in the early detection of pacemaker system failure and the meticulous development of statistical patient data (AU)

Jamaican cardiomyopathy

Eight cases of cardiomyopathy found in Jamaica are described. The ages ranged from 19 to 60 years. There were six men and two women. All the hearts were enlarged, the weights ranging from 480-370 g, except for one patient in whom the heart weighed 275 g. The principal pathological finding was fibrous thickning of the endocardium confined to the left side in seven patients and most evident in the ventricles. Four hearts showed mural thrombi which in two instances had given rise to systemic emboli. Valvular involvement -slight-was seen in only three patients. The endocardial thickenings contained deposits of fibrin and originated from them. This cardiomyopathy resembles most closely "heart muscle" disease found in Nigeria (Summary)

Observations on the pathogenesis of fibrosis in rats on a protein deficient diet

White, male, Sprague-Dawley rats on a protien deficient diet developed fatty change in parenchymal cells of the liver, followed by fibrosis. Under these circumstances, however, the fibrosis seemed to occur as a result of a continous gradual necrosis of liver cells rather than through formation of fatty cysts. (Summary)