Successful surgical repair for Emery–Dreifuss muscular dystrophy valvular disease with long-term follow-up

Abstract An aorto-esophageal fistula (AEF) is a rare yet life-threatening cause of upper gastrointestinal bleeding. We report our experience with open surgical management of two cases of AEF. Both cases presented with almost identical presentations: hematemesis and hemodynamic instability. The aorta in the first patient was normal; the defect was small and was repaired with a Dacron patch. The second patient had an aneurysmal aorta, which was replaced with a Dacron graft. Both cases were performed under partial bypass. The esophageal rent in both patients was debrided, primarily closed and buttressed with a vascularized intercostal pedicle. Nonavailability of endovascular personnel and equipment along with hemodynamic instability of the patient influenced our surgical strategy. Long-term follow-up of these patients is necessary to analyze the outcomes of our surgical repair.

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1158 Autograft degeneration, clinical outcomes and predictors after 21 years follow up in Ross procedure. A single center experience

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.662 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

C Pericet Rodriguez ◽

L Pardo Gonzalez ◽

A Fernandez Ruiz ◽

R Gonzalez Manzanares ◽

M Ruiz Ortiz ◽

...

Keyword(s):

Learning Curve ◽

Valvular Disease ◽

Ross Procedure ◽

Aortic Lesion ◽

Probability Of Survival ◽

Single Center Experience ◽

Echocardiographic Evaluation ◽

Long Term Follow Up

Abstract Background Autograft regurgitation and the need of autograft reintervention are possible complications of Ross procedure. Purpose Our Aim was to identify rates of autograft degeneration, reintervention and predictive factors valvular disease in a prospective series of a reference cardiovascular surgery hospital. Methods Since November 1997 to July 2009, a total of 107 patients diagnosed of aortic valvular disease requiring surgical treatment underwent Ross surgery (mean age 30 ± 11 years, 69% male, 21 patients <18 years). In all of them, a comprehensive clinical and echocardiographic evaluation was performed before the intervention and at discharge, at 6, 12 months and annually after surgery. Results At the end of follow-up (21 years, median: 17 years, interquartile rank 12-19 years), echocardiographic and clinical data were available in 95 (89%) and 105 (98%) patients, respectively. 30 patients (32%) developed at least moderate aortic regurgitation and 18 of them (17%) required autograft reintervention. Probability of survival free from at least moderate autograft regurgitation and reintervention at the end of follow up was 71% and 83% respectively. Two patients died because of reintervention related complications. A larger native pulmonary annulus size was the only factor, associated to autograft reintervention (HR 1.24 95% [CI] 1.04-1.48, p = 0.01) and at least moderate autograft regurgitation (HR 1.19 95% [CI] 1.03-1.37, p = 0.02). Autograft reintervention was also associated to intervention in the learning curve period (first 12 cases, HR 3.78, [CI] 95% 1.42-10.08, p = 0.008). We found no significant association of these outcomes with previous cardiac surgery, age, sex, aetiology of aortic lesion or native aortic annulus diameter. Conclusion At long term follow-up after Ross procedure, 32% of patients developed at least moderate autograft regurgitation and 17% required autograft reintervention. A larger size of the native pulmonary annulus and intervention in the learning curve period were associated with the need of autograft surgery in the long term follow up.

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