Genetic and prenatal findings in two Japanese patients with Schinzel-Giedion syndrome.

We report two Japanese patients with Schinzel-Giedion syndrome. When polyhydramnios is observed, additional fetal findings such as overlapping fingers, hydrocephalus, hydronephrosis, and very characteristic facial appearance comprising high, prominent forehead, hypertelorism, and depressed nasal root may suggest Schinzel-Giedion syndrome.

B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload.

BACKGROUND: Recent studies showed that children with univentricular heart have elevated plasma B-type natriuretic peptide (BNP) levels prior to bidirectional cavopulmonary anastomosis (BDCPA). However, it remains to be established whether BNP levels reflect the degree of hemodynamic overload and relate to long-term outcome in univentricular circulation. METHODS: Fifty one consecutive children with functionally univentricular heart prior to BDCPA were studied. All patients underwent cardiac catheterization and BNP measurement. Ventricular end-systolic wall stress (ESWS) and end-diastolic wall stress (EDWS) were calculated from cardiac catheterization data. RESULTS: Median age was 1.1 years and 34% were female. Median BNP concentration was 90.4 pg/ml. Patients with high BNP (≥ 100 pg/ml) had higher pulmonary to systemic flow ratio (p = 0.014), a greater end-diastolic volume (p=0.009), more severe atrioventricular valve regurgitation (p= 0.02) and lower ventricular mass to end-diastolic volume ratio (p=0.006). BNP levels strongly related to EDWS (r = 0.75, p< 0.0001) and ESWS (r = 0.63, p < 0.0001). During median follow-up period of 3.2 years, 15 patients died and one underwent heart transplantation for refractory heart failure. On multivariate Cox regression analysis, high BNP concentration was an independent predictor of death or transplantation (HR 3.05, CI: 1.06-8.83, p = 0.04). CONCLUSIONS: High BNP concentration at the first palliative stage towards Fontan circulation reflects high wall stress due to high volume load and insufficient ventricular hypertrophy. Moreover, high BNP levels at this stage were independently related to long-term outcome. BNP may be used as a guide to identify patients with high workload due to inadequate adaptation to hemodynamic load, who are at high risk.

Surgical anatomy of arteries running transversely in the pancreas, with special reference to the superior transverse pancreatic artery.

BACKGROUND/AIMS: In pancreatic surgery, the pancreas is usually divided over the portal vein. Knowledge of transversely oriented arterial systems within the pancreas that would need to be ligated in most forms of pancreatic surgery is clinically important. We studied the anatomy of arteries running transversely in the pancreas. METHODOLOGY: Thirty-eight cadavers donated for education and research were examined. The parenchyma of the pancreas was removed gradually from both the ventral and dorsal sides to reveal these arteries. Arteries at least 0.5 mm in diameter were sketched and counted. RESULTS: The mean number of divided arteries over the portal vein at least 0.5 mm in diameter per specimen was 2.5 +/- 1.1 (range 1 to 5). The divided arteries were mainly transverse pancreatic (TP), superior TP, and dorsal pancreatic (DP) arteries. The superior TP artery was observed in 24/38 specimens (63.2%) and ran along the superior ventral side of the head of the pancreas in 79.2% of specimens. The superior TP artery was formed between the gastroduodenal (GD) and DP arteries in 15/38 specimens (39.5%). In pancreatic surgery, surgeons need to be aware of the anatomy of the superior TP artery. The TP artery usually originated from the GD artery and its branches. The TP artery joined with the DP artery in 61.3% of specimens and ran along the inferior surface of the body and tail of the pancreas. The TP artery frequently anastomosed with the great pancreatic artery. Arterial catheters for continuous delivery of protease inhibitors in acute necrotizing pancreatitis should be placed into the GD artery, because this will achieve perfusion of the entire pancreatic parenchyma. CONCLUSIONS: We termed the artery originating from the GD or DP arteries, located at the cranial side of the head of the pancreas, the superior TP artery. Surgeons should pay close attention to the anatomy of the superior TP artery during pancreatic surgery.