Although the incidence of descending necrotizing mediastinitis(DNM) is low, this is a serious disease because it"s mortality have been reported to be as high as 40~50%. Currently, many authors have emphasized aggressive surgical approaches rather than medical treatment alone. We report good results in 2 DNM patients treated by less invasive approach with video-assisted thoracoscopic surgery. Less invasive methods with video-assisted thoracoscopic surgery can reduce hospital stay and morbidity if effective drainage can be achieved in selected DNM patients.
Drainage ; Funnel Chest ; Humans ; Incidence ; Length of Stay ; Marfan Syndrome ; Mediastinitis* ; Mortality ; Thoracic Surgery, Video-Assisted*

Marfan syndrome is infrequently diagnosed early in infancy. The morphologic characteristics and prognosis in infantile Marfan syndrome may be quite different from those in older patients. Characteristic cardiac findings in early life include mitral valve prolapse, valvular regurgitation, and aortic root dilation. Morbidity and mortality may be high for infants diagnosed with Marfan syndrome. Cardiac surgery for cardiovascular complication in infants often resulted in mortality due to the patient's young age and small size of valve. But recently, some reports say that operations for cardiovascular complications of Marfan syndrome can be performed in children with low operative morbidity and mortality. Echocardiographic findings of this patient were mitral valve prolapse, severe mitral regurgitation and aortic root dilatation. She underwent mitral valve replacement due to severe mitral valve prolapse and mitral regurgitation, and will have an aortic root and valve replacement in the near future. We experienced a case of infantile Marfan syndrome diagnosed by echocardiographic findings, and reported the case with associated literature.
Child ; Dilatation ; Echocardiography ; Humans ; Infant ; Marfan Syndrome* ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Mortality ; Prognosis ; Thoracic Surgery

PURPOSE: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy. MATERIALS AND METHODS: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed beta-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available. RESULTS: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05). CONCLUSION: In MFS patients who underwent ARR, the addition of RAAS blockade to beta-blocker was associated with reduction of aortic dilatation and clinical events.
Adrenergic beta-Antagonists/pharmacology ; Aged ; Aneurysm, Dissecting/complications/mortality/surgery ; *Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors/*therapeutic use ; Aorta/pathology/*surgery ; Aortic Aneurysm/complications/mortality/surgery ; Aortic Valve ; Female ; Humans ; Male ; Marfan Syndrome/mortality/*surgery ; Middle Aged ; Renin-Angiotensin System/*drug effects

BACKGROUND: This study was undertaken to analyze the outcome of composite valve graft replacement(CVGR) for the treatment of aneurysms of the ascending aorta involving the aortic root. MATERIAL AND METHOD: Between April 1995 and June 2001, 56 patients had replacement of the ascending aorta and aortic root with a composite graft valve and were reviewed retrospectively. Aortic regurgitation was present in 50 patients(89%), Marfan's syndrome in 18 patients(32%), and bicuspid aortic valve in 7(12.5%). The indications for operation were annuloaortic ectasia(AAE) in 30 patients(53.6%), aortic dissection in 13(23.2%), aneurysms of the ascending aorta involving aortic root in 11(19.6%), and aortitis in 2(3.6%). Cardiogenic shock due to the aortic rupture was present in 2 patients. Nine patients(16%) had previous operations on the ascending aorta or open heart surgery. The operative techniques used for CVGR were the aortic button technique in 51 patients(91%), the modified Cabrol technique in 4, and the classic Bentall technique in 1. The concomitant procedures were aortic arch replacement in 24 patients(43%), coronary artery bypass graft in 8(14.3%), mitral valve repair in 2, redo mitral valve replacement in 1, and the others in 7. The mean time of circulatory arrest, total bypass, and aortic crossclamp were 21+/-14 minutes, 186+/-68 minutes, and 132+/-42 minutes, respectively. RESULT: Early mortality was 1.8%(1/56). The postoperative complications were left ventricular dysfunction in 16 patients(28.6%), reoperation for bleeding in 7(12.5%), pericardial effusion in 2, and the others in 7. Fifty-three patients out of 55 hospital survivors were followed up for a mean of 23.2+/-18.7 months(1-75 months). There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage, and CVGR-related late mortality was 1.9%. The 1- and 6-year actuarial survival was 98.1+/-1.9% and 93.2+/-5.1%, respectively. Two patients required reoperation for complication of CVGR(3.8%) and two other patients required subsequent operations for dissection of the remaining thoracoabdominal aorta. The 1- and 6-year actuarial freedom from reoperation was 97.8+/-2.0% and 65.3+/-26.7%, respectively. CONCLUSION: This study suggests that aortic root replacement with a composite valve graft for a variety of aneurysms of the ascending aorta involving the aortic root is a safe and effective therapy, with good early and intermediate results. Careful follow-up of all patients following composite graft root replacement is important to long-term survival.
Aneurysm ; Aorta ; Aorta, Thoracic ; Aortic Rupture ; Aortic Valve ; Aortic Valve Insufficiency ; Aortitis ; Bicuspid ; Cerebral Hemorrhage, Traumatic ; Coronary Artery Bypass ; Follow-Up Studies ; Freedom ; Hemorrhage ; Humans ; Marfan Syndrome ; Mitral Valve ; Mortality ; Pericardial Effusion ; Postoperative Complications ; Reoperation ; Retrospective Studies ; Shock, Cardiogenic ; Survivors ; Thoracic Surgery ; Transplants* ; Ventricular Dysfunction, Left