Inflammatory myofibroblastic tumor of heart in a 4-month-old infant.

Primary tumors of heart are rare in infants. We report a case of inflammatory myofibroblastic tumor in a 4-month-old baby girl who presented with a massive pericardial effusion. She underwent complete surgical excision of the tumor. This is the first reported case from the Middle Eastern region. The clinical behavior and management of these rare infantile cardiac tumors are discussed briefly in this report.

Impaired systemic ventricular relaxation affects postoperative short-term outcome in Fontan patients.

OBJECTIVES: Systemic ventricular end-diastolic pressure has been used as a predictor of outcome in patients undergoing the Fontan operation. However, this index only evaluates late diastolic function and does not assess active ventricular relaxation during the phase of early diastole, a key component of systemic venous pathway flow. This study sought to examine whether impaired preoperative systemic ventricular relaxation, expressed as the time constant of isovolumic relaxation (tau), affects short-term postoperative outcome in Fontan patients. METHODS: All patients who underwent Fontan operation between May 1998 and November 2001 were enrolled. Tau was calculated from digitized preoperative systemic ventricular pressure tracings. Standard preoperative invasive indices were also recorded and analyzed. These independent variables were then entered into a multiple stepwise regression model, with length of intensive care unit stay, length of hospital stay, and prolonged pleural effusion as outcome variables. RESULTS: Twenty-seven patients fulfilled inclusion criteria. Systemic left ventricle predominated, and all patients had undergone prior staged palliation. Extracardiac Fontan was the commonest operative technique. Of the independent variables examined, tau was the only statistically significant predictor of length of intensive care unit stay (P <.001) and length of hospital stay (P =.002). None of the independent variables predicted pleural effusion greater than 10 days. CONCLUSIONS: Tau was the only significant preoperative invasive predictor of short-term outcome in the Fontan patients. This illustrates the importance of systemic ventricular relaxation and highlights the need for a more comprehensive assessment of diastolic function before the Fontan operation.

Pancreatitis in Fontan patients is related to impaired ventricular relaxation.

BACKGROUND: Pancreatitis following cardiopulmonary bypass is a well-known complication in adults undergoing cardiac surgery. However, the occurrence of pancreatitis in pediatric patients undergoing repair of congenital heart disease is under-appreciated. Post-Fontan patients are particularly prone to postoperative pancreatitis. In an effort to identify specific perioperative factors predictive of postoperative pancreatitis, we retrospectively reviewed a group of Fontan patients. METHODS: From June 1996 to June 2001, 40 patients underwent a modified Fontan operation. Four patients developed acute pancreatitis postoperatively. The preoperative, intraoperative, and postoperative hemodynamics and ventricular function parameters were retrospectively analyzed and compared to 10 randomly selected Fontan patients who did not have pancreatitis. Preoperative echocardiographic and angiographic data, including digitized ventricular pressure tracings, were reviewed to obtain ventricular relaxation time constant (tau), pulmonary vascular resistance (PVR), ventricular end diastolic pressure (VEDP), positive dp/dt and negative dp/dt values. RESULTS: Patients developing acute pancreatitis had very high mortality (50%) compared to no mortality in the control group. The mean preoperative tau was significantly prolonged (41 ms vs 26 ms in control group, p < 0.001), and pre operative systemic output (Qs) lower in the pancreatitis group (mean 2.75 L/min/m2) compared with controls (Qs of 5.09 L/min/m2, p < 0.03). CONCLUSIONS: Impaired ventricular relaxation and decreased preoperative cardiac output are predictive of increased risk of postoperative pancreatitis in Fontan patients. Evaluation of preoperative diastolic function in these patients may provide additional insights in to clinical outcome following the Fontan procedure.