Heart transplantation in children after mechanical circulatory support: comparison of heart transplantation with ventricular assist devices and elective heart transplantation.

Heart transplantation (HTx) is an ultimate treatment for children with end-stage heart failure or inoperable congenital heart disease. The supply of hearts is inadequate; therefore, different mechanical support systems must be used as bridge to HTx in pediatric patients with postoperative low output. The use of ventricular assist devices (VADs) as bridge to HTx in children is limited because of size differences. The purpose of this study was to evaluate the overall long-term outcome of pediatric circulatory support before pediatric HTx. From 1989 through 2004, 91 pediatric patients underwent isolated HTx. Seven of them required mechanical support before transplantation. We reviewed retrospectively the course of 91 children (mean age 14.7 years) who underwent HTx. Group A consisted of elective HTx patients who were treated as outpatients before HTx, whereas group B was the VAD-HTx bridging group (n=7; mean age 12.31 +/- 2.8 years). Mean duration of VAD support was 108 +/- 98 days (minimum 1 day, maximum 258 days). Overall survival rate after HTx was 80% at 1 year without significant differences between groups. Five of seven patients survived and could be discharged after successful HTx, for a survival rate of 77%. The mean follow-up period was 16.76 +/- 10.6 months. No differences in posttransplantation long-term survival and rejection episodes occurred between patients transplanted with or without VAD. VAD therapy can keep pediatric patients with end-stage heart failure alive until successful HTx, and bridge to HTx is a safe procedure in pediatric patients. After HTx, survival rates of these children are similar to those of patients awaiting elective HTx.

Cesarean section in a patient with non-compaction cardiomyopathy managed with ECMO.

Isolated ventricular non-compaction is a rare cardiomyopathy associated with left heart failure, severe arrhythmias and thromboembolism. We report about our interdisciplinary strategy in a patient with severe isolated ventricular non-compaction cardiomyopathy scheduled for caesarean section in general anaesthesia. Monitoring included placement of an arterial line, a central venous catheter and a pulmonary artery catheter with pacing option. Small introducer gates were placed in the femoral artery and vein to facilitate quick percutaneous institution of extracorporeal life support via extracorporeal membrane oxygenation in case of acute cardiac failure refractory to medical treatment. Inotropic pharmacological therapy with 3 µg/kg/min dobutamine and 0.25 mg/kg/min milrinone was started before surgery. Induction of general anesthesia and rapid sequence intubation was performed with an analgesic dose of 0.5 mg/kg S ketamine, 0.25 mg/kg etomidate and 5 mg rocoronium followed by 1.5 mg/kg succinylcholine. This regimen provided completely stable hemodynamics in this critical period until delivery of the child and continuation of anaesthesia with continuous infusion of propofol and remifentanyl. The current strategies, particularly the preparation for femoro-femoral extracorporeal membrane oxygenation, may be considered in similar cases with a high risk of acute cardiac decompensation which may be refractory to medical treatment. Anaesthesiologist involved in performing caesarean section in women with complex cardiac disease, should encompass extracorporeal membrane oxygenation standby in management of the perioperative period.

Two cases of Marfan syndrome.

The early identification of the Marfan Syndrome should be essential for the prevention of the aortic dilatation and dissection, but the wide phenotypic expression of the disorder makes the clinical diagnosis very difficult. The aim of this study is to emphasise the necessity of a widely applicable method of morpho-clinical diagnosis. The diagnosis was confirmed on surgical biopsies from ascending aortic wall and aortic cusps, using routine morphological techniques. The macroscopical examination revealed a thin ascending aortic wall, presenting a transversal intimal tear, without secondary dissecting hematoma, and an aortic dilated annulus, producing aortic regurgitation. Microscopically, the diagnosis was supported by the identification of cystic medical necrosis, a specific degenerative lesion in the Marfan Syndrome, in patients with or without family history of aortic aneurysms. This study defines the morpho-clinical changes of the disease, emphasising the necessity of a permanent monitorization of the patient after surgery, due to the risk of late complications.

Morphological evaluation of the surgically removed aortic and mitral valves.

In 2001, of the 114 valves surgically excised at the Iasi Center of Cardiology from 59 men and 54 women (mean age 51 years; age range: 25 to 78 years), half were aortic and the other half mitral valves. The 57 aortic valves, from 20 women and 37 men (mean age 51.1; age range 25 to 78 years), had been surgically replaced. Functionally, 57.63 percent (30) were stenotic, 21.05 percent (12) were incompetent, and 26.31 percent (15) were both stenotic and incompetent (aortic disease). Pure stenosis was related to calcification of degenerative (73.3 percent), bicuspid (3.33 percent), post-inflammatory (20 percent), and undetermined (3.33 percent) causes. Pure regurgitation was not related to calcification and causes included infective endocarditis (50 percent), bicuspid valve (16.66 percent), postinflammatory (16.66 percent), aortic root dilatation (8.33 percent), and undetermined (8.33 percent). Aortic disease was secondary to postinflammatory etiologies (40 percent), degenerative calcification (33.33 percent), bicuspid and undetermined (13.33 percent each) causes. The reminder of 57 mitral valves, from 35 women and 24 men (mean age 45.5 years; age range 28 to 71 years), were surgically replaced. Functionally, 40.35 percent (23) were purely stenotic, 19.29 percent (11) were purely regurgitant, and 40.35 percent (23) both stenotic and regurgitant (mitral disease). The causes of pure stenosis were postinflammatory (presumably rheumatic) disease in 91.3 percent (21 cases) and degenerative disease in 8.7 percent (3 cases). Pure regurgitation etiology involved floppy valves in 45.45 percent (5 cases), degenerative disease in 27.27 percent (3 cases), postinflammatory disease in 18.18 percent (2 cases) and infective endocarditis 9.1 percent (1 case).

[Morphologic and clinical correlations in medullar thymoma]. / Corelatii morfoclinice în timomul medular.

The medullar thymoma is a rare and distinctive epithelial thymoma, a thymic tumor characterized histologically by a mixture of spindle epithelial cells and lymphoid cells. We are presenting this tumor to a 68 years old man, admitted at CCI, for a mediastinal tumor, treated by tumorectomy, for revealing the cytological, histological and immunohistochemical characteristic features. The surgical biopsy was prepared by using usual histological techniques and haematoxilin eosin and Van Gieson stainings. We are discussing the relation between the thymoma clinicopathological and prognostic features, resulting a clear correlation between histological type and clinical study. We also pointed the Muller-Hermelink thymoma histological subtypes and their correspondence with OMS histological types, reflecting realistically the thymoma clinical behavior.