Rationale and design of GISSI OUTLIERS VAR Study in bicuspid aortic valve patients: prospective longitudinal, multicenter study to investigate correlation between surgical, echo distinctive features, histologic and genetic findings in phenotypically homogeneous outlier cases.

BACKGROUND/OBJECTIVES: Bicuspid aortic valve (BAV) is the most common congenital heart disorder, affecting up to 2% of the population. Involvement of aortic root and ascending aorta (aneurysm or, eventually, dissection) is frequent in patients with pathologic or normal functioning BAV. Unfortunately, there are no well-known correlations between valvular and vascular diseases. In VAR protocol, with a new strategy of research, we analysemultiple aspects of BAV disease through correlation between surgical, echo, histologic and genetic findings in phenotypically homogeneous outlier cases. METHODS: VAR protocol is a prospective, longitudinal, multicenter study. It observes 4 homogeneous small groups of BAV surgical patients (15 patients each): isolated aortic regurgitation, isolated ascending aortic aneurysm, aortic regurgitation associated with aortic aneurysm, isolated aortic stenosis in older patients (>60years). Echo analysis is extended to first-degree relatives and, in case of BAV, genetic test is performed. Patients and relatives are enrolled in 10 cardiac surgery/cardiologic centers throughout Italy. CONCLUSIONS: The aim of the study is to identify predictors of favorable or unfavorable evolution of BAV in terms of valvular dysfunction and/or aortic aneurysm. Correlations between different features could help in identification of various BAV risk groups, rationalizing follow-up and treatment.

Serial echocardiographic assessment of neo-aortic regurgitation and root dimensions after the modified Ross procedure.

BACKGROUND AND AIM OF THE STUDY: Concern exists regarding progressive root dilatation after the modified Ross procedure. The present prospective echocardiographic study aimed to provide further insight into neo-aortic regurgitation (nAR) and neoaortic root dimensions over time in adult Rotterdam Ross root patients, and to study potential risk factors for nAR and dilatation. METHODS: All Rotterdam Ross patients aged > or = 16 years at surgery were subjected to a prospective biennial standardized echocardiographic protocol. Analysis over time of nAR according to the jet length and jet diameter method, autograft annulus and sinotubular junction (STJ) diameters was carried out using a multilevel linear model in 90 patients who had two or more echocardiographic measurements (mean 5; range 2-9; total 458) up to 14 years (mean 7 years) after surgery. RESULTS: The mean (+/- SE) initial postoperative jet length nAR was grade 0.9 +/- 0.09, and the annual increase 0.1 +/- 0.02 (p < 0.001). Initial annulus and STJ diameters were 25 +/- 0.5 mm and 36 +/- 0.6 mm, while annual increases were 0.4 +/- 0.07 mm and 0.5 +/- 0.09 mm, respectively (p < 0.001). Patients who eventually underwent an autograft reoperation (n = 10) had significantly greater initial nAR and greater progression of nAR, and a greater initial annulus diameter. The annual annulus and STJ diameter increase was greater in patients who underwent autograft reoperation. Compared to freestanding root replacement, patients with inclusion cylinder aortic root replacement had smaller initial annulus and STJ diameters that did not increase over time. Female gender was associated with a greater initial jet length and jet diameter nAR and a greater increase over time in jet diameter nAR. Preoperative aortic regurgitation or combined aortic stenosis and regurgitation were associated with greater initial annulus and STJ diameters. Neither bicuspid valve disease, patient age, preoperative ascending aorta aneurysm, prior aortic valve surgery nor hypertension had an effect on initial or progression of nAR, annulus, and STJ diameter. CONCLUSION: The annual increase in nAR and root dimensions is small, but persistent, after autograft aortic root replacement in adults, and further reoperations should be anticipated. Use of the inclusion cylinder root replacement technique seems to prevent neo-aortic dilatation.

Assessment of the impact of the removal of cerebrospinal fluid on cerebral tissue volumes by advanced volumetric 3D-MRI in posthaemorrhagic hydrocephalus in a premature infant.

Current clinical practice in the premature infant with posthaemorrhagic ventricular dilatation (PHVD) includes drainage of cerebrospinal fluid (CSF). This case study used advanced volumetric three dimensional magnetic resonance imaging to document the impact of CSF removal on the volume of regional brain tissues in a premature infant with PHVD. The removal of a large volume of CSF was associated with an identical reduction in CSF volume, but more dramatically with a significant increase in the regional volumes of cortical grey matter and myelinated white matter. The alterations in cerebral cortical grey matter and myelinated white matter volumes may provide insight into the established association of PHVD with deficits in cognitive and motor functions.

Mini-sternotomy for aortic valve surgery.

In the recent years more and more efforts have been made widely to introduce new techniques in the minimally invasive cardiac surgery. At our Department and the Linköping University Hospital, Cardiothoracic Surgery Department, from August 1996 to January 1997, aortic valve surgery was performed in 23 adult patients (9 female, 14 male), age 28-86 years (mean age 62.5 years). Twenty-two patients had aortic valve replacement, among these, in 3 cases concomitant aortic annulus dilatation was made and in one case reduction-plasty of the dilated ascending aorta. In another one case resection of a sub-aortic membrane was performed. The operations and postoperative period were free of complications in all patients. Following an average 36 hours intensive care all patients were discharged after an average of 11.2 day hospital stay. The authors introduce the new surgical technique and present its advantages and disadvantages. Mini-sternotomy has less detrimental structural and functional effects on the thorax. Moreover, due to its minimal surgical trauma, this less invasive technique reduces patient morbidity, hospital stay and cost of care. Since mini-sternotomy is a safe and advantageous technique, the authors recommend applying this new technique in most of aortic valve operations.