A rare case of primary ovarian carcinoid.

We present a rare case in an elderly Caucasian lady of primary ovarian carcinoid tumor, causing symptoms of the carcinoid syndrome and severe carcinoid heart disease. Carcinoid tumors are the commonest neuroendocrine tumors, the majority of which are found in the gastrointestinal tract and lungs. Ovarian carcinoids represent only 0.1% of all ovarian neoplasms and 1% of all carcinoid tumors. Carcinoid syndrome can present with flushing, diffuse watery diarrhea, wheezing and breathlessness and in advanced cases lead to carcinoid heart disease. This involves damage to the right heart structures and pulmonary hypertension, which can cause significant breathlessness and peripheral edema, and is associated with a worse prognosis. Diagnosis involves multi-modality radiological imaging and biochemical analysis of neuroendocrine tumor markers, and the first line treatment should always be resection of the tumor where possible. Prognosis is generally favorable, except in some cases with metastasis.

A surgical strategy for cor triatriatum atrial septal defect--A1 lam subclass.

Atrial fibrillation and a heart murmur were diagnosed in a 68-year-old woman during a routine medical examination. She presented 2 years later with pulmonary edema. A transthoracic echocardiography examination revealed a tunneled atrial septal defect (ASD) and severe tricuspid regurgitation. Transesophageal echocardiography and 3-dimensional computed tomography evaluations revealed multiple intracardiac defects, including abnormal atrial septation suggestive of a typical cor triatriatum sinistrum (A1 Lam subclass), a rare congenital defect in adults. The patient underwent tricuspid valve repair with concomitant closure of the ASD by using the cor triatriatum curtain to form an autologous transposition flap. The intraoperative transesophageal and predischarge imaging evaluations confirmed an excellent repair. The patient made a swift recovery and demonstrated improvement in her symptoms at follow-up. This previously undescribed technique eliminates the need for a prosthetic implant and is applicable in >80% of cor triatriatum cases in which an ASD exists.

Sudden infant death syndrome and cardiac arrhythmias.

There is a considerable body of evidence that common bacterial toxins, absorbed from the mucosal surface or delivered as part of a transient bacteremia, have a pathogenic role in sudden infant death syndrome (SIDS). The candidate organisms are Staphylococcus aureus and Escherichia coli. Death in SIDS is rapid, with infants progressing from well, or only mildly unwell, to death in less than 20 min. This mode of death is not typical of infection but it is consistent with toxin action on cardiovascular or respiratory control. Both S. aureus and E. coli secrete toxins (cytolysins and colicins) that create channels in cell membranes and disturb ion currents. Recent evidence indicates that between 5 and 15% of SIDS cases carry potentially lethal loss-of-function mutations in cardiac channelopathy genes. However, only a minority of individuals with these mutations die of SIDS and the hypothesis proposed is that toxin-gene interaction could explain the deaths. Furthermore, channelopathy mutations predispose to sudden death at all ages and since episodes of transient bacteremia occur throughout life the idea of toxin-gene interaction could have wider applicability. These ideas can be investigated and answered in the near future using the new science of proteomics.

Congenital pulmonary vein stenosis beyond childhood.

We report a case of anomalous connection of pulmonary veins with insertional stenoses causing undiagnosed severe pulmonary hypertension in a 37-year-old woman. We report the use of non-invasive diagnostic investigations in making the diagnosis, and the apparent cure of this rare condition with resolution of right heart failure following surgical treatment.