Anticoagulants ; therapeutic use ; Child, Preschool ; Fontan Procedure ; adverse effects ; Heparin ; therapeutic use ; Humans ; Male ; Protein-Losing Enteropathies ; drug therapy ; etiology

BACKGROUNDThe fenestration function is by allowing a right-to-left shunt resulting in an increased cardiac index, associated with mild arterial oxygen desaturation. Subsequent transcatheter fenestration closure can be performed after haemodynamic assessment. The purpose of this study was to compare the outcomes of extracardiac connection (EC) with or without fenestration.

METHODSNinety-five consecutive patients diagnosed with univentricular heart disease underwent EC using Gore-Tax conduits at the Department of Children's Heart Center, Justus-Liebig-University Giessen Germany from June 1996 to July 2007. According to EC with or without fenestration, the patients were assigned to two groups (group A with fenestration and group B without fenestration). Mortality, effusions, postoperative mean pulmonary artery pressure, postoperative oxygen saturation, postoperative thrombosis, postoperative neurological problems, and the postoperative loss of sinus rhythm were compared. In group A, 23 patients had fenestration closed interventionally after a mean time of 20-22 months.

RESULTSMortality and postoperative mean pulmonary artery pressure in group B (3 and (15.1 +/- 3.4) mmHg, respectively) were significantly higher than group A (0 and (13.2 +/- 2.8) mmHg, respectively). Postoperative oxygen saturation, postoperative thrombosis, postoperative neurological problems, and the postoperative loss of sinus rhythm did not differ between cohorts.

CONCLUSIONSFenestrating an extracardiac tunnel seems to improve acute postoperative mortality by rising cardiac output. The induced right-to-left shunt shows no morbidity postoperatively. If a stabilized chronic hemodynamic situation is achieved, an interventional closure of the fenestration can be performed to advance the arterial saturation and improve the exercise tolerance of the patients.

Adolescent ; Child ; Child, Preschool ; Fontan Procedure ; adverse effects ; methods ; mortality ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Infant ; Pulmonary Artery ; physiopathology

Fontan-associated liver disease (FALD) is one of the main complications after the Fontan procedure, manifesting mostly as liver fibrosis and even cirrhosis, with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis. The specific cause is unknown, although it is considered to be associated with long-term elevated central venous pressure, impaired hepatic artery blood flow, and other relevant factors. The absence of association between laboratory tests, imaging data, and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult. A liver biopsy is the gold standard for diagnosing and staging liver fibrosis. The most important risk factor for FALD is time following the Fontan procedure; therefore, it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma. Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.
Humans ; Liver Diseases/pathology* ; Liver Cirrhosis/pathology* ; Liver/pathology* ; Carcinoma, Hepatocellular/pathology* ; Liver Transplantation/adverse effects* ; Fontan Procedure/adverse effects* ; Postoperative Complications/pathology* ; Liver Neoplasms/pathology*

OBJECTIVETo investigate the association between preoperative, operative, and postoperative factors and persistent pleural effusion after the extracardiac Fontan procedure.

METHODSNinety-five consecutive patients diagnosed with univentricular heart underwent extracardiac connection using Gore-Tax conduits at the Department of Children's Heart Centre, Justus-Liebig-University Giessen in Germany from June 1996 to July 2007. The outcome measures were duration and volume of chest tube drainage after surgical intervention. The investigated factors included age and weight at the time of operation, anatomical diagnosis, preoperative oxygen saturation, mean pulmonary artery pressure, ventricular end-diastolic pressure, fenestration, cardiopulmonary bypass time, conduit size, postoperative pulmonary artery pressure, administration of angiotensin-converting enzyme inhibitors, and postoperative infection. Associations between these factors and persistent pleural effusion after the extracardiac Fontan procedure were analyzed.

RESULTSEvery patient suffered postoperative effusion. The median duration of postoperative chest tube drainage was 9 days (range, 3-69 days), and the median volume was 12 mL kg(-1) x d(-1) (range, 2.0-37.5 mL x kg(-1) d x (-1)). Thirty-seven (38.9%) patients had pleural drainage for more than 15 days, and the volume in 35 (36.8%) patients exceeded 25 mL x kg(-1) x d(-1). Nineteen (20%) patients required placement of additional chest tubes for re-accumulation of pleural effusion after removal of previous chest tubes. Fifteen (17.8%) patients were hospitalized again due to pleural effusion after discharge. The median length of hospital stay after the operation was 14 days (range, 4-78 days). Multivariate analysis results showed that non-fenestration, low preoperative oxygen saturation, and postoperative infections were independent risk factors for prolonged duration of pleural drainage (P < 0.05). Long cardiopulmonary bypass time, non-fenestration, small conduit size, and low preoperative oxygen saturation were independent risk factors for excessive volume of pleural drainage (P < 0.05).

CONCLUSIONSFor reduing postoperative duration and volume of pleural drainage following Fontan procedure, it seems to be important to improve the preoperative oxygen saturation, use large size of conduit, shorten cardiopulmonary bypass time, and make fenestration during the operation, as well as avoid postoperative infections.

Adolescent ; Child ; Child, Preschool ; Female ; Fontan Procedure ; adverse effects ; Humans ; Infant ; Male ; Pleural Effusion ; etiology ; physiopathology ; Postoperative Complications ; etiology ; Risk Factors ; Treatment Outcome