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Background: Despite increasing numbers of reports, biliary tract intraductal papillary mucinous neoplasm (BT-IPMN) is not yet recognized as a unique neoplasm. The aim of the present study was to define the presence of BT-IPMN in a large series of resected biliary neoplasms., Methods: From May 1994 to December 2006, BT-IPMN cases were identified by reviewing pathology specimens of all resected cholangiocarcinomas and other biliary neoplasms when cystic, papillary or mucinous features were cited in pathology reports., Results: BT-IPMN was identified in 23 out of 253 (9%) specimens using the strict histopathological criteria of IPMN. The most common presenting symptom was abdominal discomfort which was present in 15 patients (65%). Only one of the original operative pathology reports used the term IPMN; 16 (70%) used the terms cystic, mucinous and/or papillary. BT-IPMN was isolated to non-hilar extra-hepatic ducts in 12 (52%), intra-hepatic ducts in 6 (26%) and hilar extra-hepatic ducts in 5 patients (22%). Carcinoma was found in association with BT-IPMN in 19 patients (83%); 5-year survival was 38% after resection., Conclusion: BT-IPMN occurs throughout the intra- and extra-hepatic biliary system and can be identified readily as a unique neoplasm. Broader acceptance of BT-IPMN as a unique neoplasm may lead to a better understanding of the pathogenesis of biliary malignancies.

Background: Performing a Ross operation in patients with severe left ventricular dysfunction is controversial. The objective in this retrospective study was to determine the outcome of 15 patients with aortic valve disease (11 had aortic insufficiency and 4 had aortic insufficiency and aortic stenosis) associated with reduced left ventricular function (ejection fraction < 40%) treated with a pulmonary autograft., Methods: We identified 15 patients with severe left ventricular dysfunction from 226 consecutive pulmonary autograft procedures done between age 18 and 50 years from 1986 to 2001. Patients had documented preoperative ejection fraction less than 40% and were in New York Heart Association class III or IV. Preoperative ejection fraction ranged from 18% to 37% (mean, 31% +/- 6.5%). Transthoracic echocardiograms obtained preoperatively and at 1-week, 6-month, and 1- and 2-year intervals were reviewed. Records were evaluated for survival, clinical status, left ventricular function, and valve function., Results: There were no operative deaths, late deaths, or reoperations. All patients had follow-up examinations within the past year and are clinically well (67% > 2 years follow-up). Ten of 15 patients (67%) had substantially improved ventricular function (> 20% increase). The average ejection fraction increased from 31% +/- 7% preoperatively to 51% +/- 11% at 2 years, and the increase is significant from 1 week on (p < 0.02). Average left ventricular mass index decreased by 41% at 6 months (n = 10; p = 0.009) and by 44% at 2 years (n = 9; p = 0.02). Mean Z values for left ventricular mass decreased from 7.6 to 3.6 after more than 2 years (p = 0.007)., Conclusions: The Ross operation is an appropriate option in adults age 50 or younger in the presence of decreased left ventricular ejection fraction. Neither operative mortality nor postoperative sequelae were identified in our subset of patients. Excellent survival and ventricular recovery are predicted.