In our population with a high proportion of complex CHD, CTI-related IART was the most frequent mechanism, although non-CTI-related IART was present in 49% of patients (alone or with concomitant CTI-related IART). A cut-off voltage of 0.5 mV could identify 95.4% of the substrates in non-CTI-related IART.
In our series of 94 CHD patients with a high proportion of moderately to highly complex cardiac defects, severe consequences of IART were frequent. Transposition of the great arteries with systemic right ventricle and severe dilation of VsA were independently associated to severity. Early invasive procedures should be considered for these high-risk patients.
A 47-year-old woman was admitted to the hospital with a history of sudden headache, vomiting and slight diplopia on left lateral gaze, without alteration in the level of consciousness. The patient had been diagnosed as suffering from stage II breast cancer, 10 months earlier, after detection of a 2-cm mass in the left breast. A total mastectomy and axillary dissection was performed. Pathological examination revealed an invasive ductal carcinoma without axillary involvement. Tamoxifen and adjuvant chemotherapy with cyclophosphamide, 5-fluorouracil and methotrexate was administered.On physical examination the patient appeared well. Blood pressure was 90/60 mmHg. No lymphadenopathy was found. Examination of the gastrointestinal, respiratory and cardiovascular system was unremarkable. Neurological examination showed bitemporal hemianopsia and a complete third cranial nerve palsy on the left side.Findings on haematology, urine analysis, serum chemistry, electrocardiography and chest Xray were normal. Hypothalamic and pituitary function tests show deficiency of adrenocorticotropin, thyroid-stimulating hormone, growth hormone, luteinising hormone and prolactin. Cranial magnetic resonance imaging (MRI) was performed (figure). Staging work-up including bone scan and adbominal ultrasound did not disclose metastatic lesions. '46
A 39-year-old man, with no history of alcohol intake, who had had an esophago-ileo-colo-gastroplasty with ileotransversostomy, developed diplopia, seizures, metabolic acidosis, and cardiac failure and finally refractory hyperdynamic shock. He died 20 h after admission to our intensive care unit from cardiocirculatory collapse. Postmortem results revealed low erythrocyte transketolase activity, which was increased by 22% by in vitro addition of thiamine diphosphate (TDP effect). Cerebral pathology showed the alterations of Wernicke's encephalopathy. We discuss the possible mechanisms of fatal cardiovascular collapse and the unusual presentation of a case without a history of alcoholic intake or clinical malnutrition.
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