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Biomedical subjects

J V de Giovanni

Publications and source records attributed to J V de Giovanni.

9 recordsLinked to original sources

Management of end-stage central venous access in children referred for possible small bowel transplantation.

UNLABELLED: The 3-year survival after small bowel transplantation (SBTx) has improved to between 73% and 88%. Impaired venous access for parenteral nutrition can be an indication for SBTx in children with chronic intestinal failure. AIM: To report our experience in management of children with extreme end-stage venous access. SUBJECTS: The study consisted of 6 children (all boys), median age of assessment 27 months (range, 13-52 months), diagnosed with total intestinal aganglionosis (1), protracted diarrhea (1), and short bowel syndrome (4), of which gastroschisis (2) and malrotation with midgut volvulus (2) were the causes. All had a documented history of more than 10 central venous catheter insertions previously. All had venograms, and 1 child additionally had a magnetic resonance angiogram to evaluate venous access. Five of 6 presented with thrombosis of the superior vena cava (SVC) and/or inferior vena cava. METHODS: Venous access was reestablished as follows: transhepatic venous catheters (5), direct intra-atrial catheter via midline sternotomy (4), azygous venous catheters (2), dilatation of left subclavian vein after passage of a guide wire and then placing a catheter to reach the right atrium (1), radiological recanalization of the SVC and placement of a central venous catheter in situ (1), and direct puncture of SVC stump(1). Complications included serous pleural effusion after direct intra-atrial line insertion, which resolved after chest drain insertion (1), displacement of transhepatic catheter needing repositioning (2), and SVC stent narrowing requiring repeated balloon dilatation. OUTCOME: Four children with permanent intestinal failure on assessment were offered SBTx, 3 of which were transplanted and were established on full enteral nutrition; the family of 1 child declined the procedure. In the remaining 2 children in whom bowel adaptation was still a possibility, attempts were made to provide adequate central venous access as feeds and drug manipulations were undertaken. One of them received liver and SBTx nearly 3 years after presenting with end-stage central venous access, because attempts to achieve independence from parenteral nutrition had failed. The other child died immediately after a transhepatic venous catheter placement, possibly from a nutritional depletion syndrome as no physical cause of death was found. Direct intra-atrial catheters in transplanted children proved to be adequate for the management of uncomplicated transplantation, although the usual infusion protocol had to be modified considerably, and the lack of access would have been critical if massive blood transfusion had been required during the transplant procedure. CONCLUSION: It was possible to reestablish central venous access in all cases. However, this was time consuming and difficult to assemble a skilled team consisting of one of more: surgeon, cardiologist, interventional radiologist, and transplant anesthetist. Small bowel transplantation is easier and safer with adequate central venous access, and we advocate liaison with an SBTx center at an early stage.

Catheterization, Central Venous↗

Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience.

OBJECTIVE: To describe a 12 year experience with staged surgical management of the hypoplastic left heart syndrome (HLHS) and to identify the factors that influenced outcome. METHODS: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure (median age 4 days, range 0-217 days). Subsequently 203 patients underwent a bidirectional Glenn procedure (stage II) and 81 patients underwent a modified Fontan procedure (stage III). Follow up was complete (median interval 3.7 years, range 32 days to 11.3 years). RESULTS: Early mortality after the Norwood procedure was 29% (n = 95); this decreased from 46% (first year) to 16% (last year; p < 0.05). Between stages, 49 patients died, 27 before stage II and 22 between stages II and III. There were one early and three late deaths after stage III. Actuarial survival (SEM) was 58% (3%) at one year and 50% (3%) at five and 10 years. On multivariable analysis, five factors influenced early mortality after the Norwood procedure (p < 0.05). Pulmonary blood flow supplied by a right ventricle to pulmonary artery (RV-PA) conduit, arch reconstruction with pulmonary homograft patch, and increased operative weight improved early mortality. Increased periods of cardiopulmonary bypass and deep hypothermic circulatory arrest increased early mortality. Similar factors also influenced actuarial survival after the Norwood procedure. CONCLUSION: This study identified an improvement in outcome after staged surgical management of HLHS, which was primarily attributable to changes in surgical technique. The RV-PA conduit, in particular, was associated with a notable and independent improvement in early and actuarial survival.

Cardiopulmonary Bypass↗

The use of Amplatzer devices to occlude vascular fistulae.

The Amplatzer occluders are Nitinol-based devices that are preshaped for specific indications, primarily to close atrial septal defects, patent foramen ovale/fenestrations, or to occlude patent arterial ducts or one designed specifically for closure of ventricular septal defects. The flexible quality of the Nitinol together with the different designs of Amplatzer occluders make it possible for these plug devices to be used for conditions other than what they are designed for. This is particularly the case for fistulae where no specific devices are available and hence improvisation may be required. The established use of the Amplatzer occluder devices primarily for atrial septal defects and for patent arterial ducts provide the confidence to use them for alternative conditions where few or no alternatives are available or if surgery is the only option. This article describes the use of Amplatzer occluder devices for two unusual conditions, namely to occlude a pulmonary arteriovenous malformation and to close an aorto-left ventricular tunnel.

Adolescent↗

Covered stents in the treatment of aortic coarctation.

Coarctation of the aorta can be managed in different ways depending upon the age of presentation, anatomical details of the coarctation, the aortic arch anatomy, and whether the coarctation is native or a recurrence following surgery. In infants, surgery is the preferred treatment of choice, whereas in older children and adults, percutaneous procedures, such as balloon angioplasty or stent implantation, are becoming increasingly popular methods for treating coarctation. Various types of stents have been used and this paper specifically addresses the use of covered or graft Jomed stents in a small group of patients with coarctation.

Adolescent↗

Total anomalous pulmonary venous connection: outcome of surgical correction and management of recurrent venous obstruction.

OBJECTIVE: Total anomalous pulmonary venous connection (TAPVC) can be corrected with low mortality and good outcome. If complicated by pulmonary vein stenosis (PVS), either at presentation or secondary to the repair, the long-term outcome is compromised. We have evaluated an institutional experience with TAPVC, with particular regard to the evolving management of PVS. METHODS: Retrospective analysis of 85 consecutive patients with non-isomeric TAPVC undergoing surgical correction over a 10-year period (1988-1997). In addition, three patients were referred to us with secondary PVS, having had their primary procedure elsewhere. Attention was focused on incidence of PVS, and strategies for management. RESULTS: Median age at first operation was 33 days (range 1-533). Site of drainage was supracardiac (43/88), infracardiac (20/88), cardiac (17/88), and mixed (8/88). On presentation, 35% of patients were ventilated. Early mortality was 7% (6/85), with one late non-cardiac death. 82% of the original patients (70/85) are currently well at a median follow-up of 64 months (range 6-119). The incidence of PVS requiring intervention was 11% (9/85). Median time to PVS was 41 days. In these patients, 18 balloon angioplasties, four endovascular stent placements (in two patients), and a further 23 surgical procedures were performed. Of the nine patients undergoing re-intervention after initial surgery at our institution, five (56%) survived. Two of these have no residual obstruction and right ventricular pressure (RVP) < 50% systemic, two have unilateral obstruction and RVP < 50% systemic, and one has bilateral obstruction and RVP 80% systemic. Of the three patients referred to us with secondary PVS, two are alive and well, and one died early after the first re-operation. CONCLUSIONS: Intrinsic obstruction (endocardial sclerosis or thickening) is associated with worse prognosis and earlier re-intervention than extrinsic (anatomical) obstruction. We advocate an early, aggressive approach to the management of patients with TAPVC, especially in the presence of PVS. This complication is most appropriately managed by a combination of re-operation and repeated balloon dilation.

Angioplasty, Balloon↗

Endocarditis with multiple intracardiac shunts: identification and repair.

An 8-year-old boy who suffered from Hirschsprung's disease had development of tricuspid valve endocarditis that progressed to aortic root abscess formation, development of a fistulous communication between aorta and right atrium, atrial and ventricular septal defects, and a left ventricle to right atrium defect. Several surgical procedures were required. Operation consisted initially of closure of the septal defects and aortic valve repair. This was followed by homograft replacement of the aortic valve for persistent infection, and further closure of a left ventricle to right atrium fistula.

Abscess↗

Improving survival in bacterial endocarditis.

Twenty six cases of bacterial endocarditis in infancy and childhood were treated with a mortality of less than 8%. Twenty two had recognised congenital heart disease and four had previously normal hearts. Thirteen out of the 24 cases with positive blood cultures had staphylococcal infection. Appropriate antibiotics were identified by measurement of the minimum bactericidal concentration as an indicator of antibiotic sensitivity. The bactericidal activity of the patient's serum during treatment was monitored by back titration against the causative organism to assess the optimal antibiotic combination and dosage. Treatment was continued for six weeks in most cases, comprising intravenous treatment for at least the first three weeks, but then being substituted with oral treatment in some. Twenty one cases were successfully treated medically. Five developed complications requiring surgery, two of whom died.

Administration, Oral↗

Evaluation of oral and low dose intravenous prostaglandin E2 in management of ductus dependent congenital heart disease.

Prostaglandin E2 was given orally to 59 infants with ductus dependent congenital heart disease, and intravenous infusions were substituted for varying periods in 27 of them. An additional three neonates received intravenous treatment alone. Mean oral maintenance dose was 27 micrograms/kg per hour and the mean intravenous dose was 0.005 micrograms/kg per minute. Mean duration of treatment was 49 days (range 16 hours to 272 days). Oral treatment was almost always effective and was especially suitable for long term use. Low dose intravenous treatment was readily substituted when indicated. Complications were usually 'minor'. Growth of the infants and of their pulmonary arteries facilitated later surgical management.

Administration, Oral↗

Diagnosis of right ventricular outflow obstruction in infants by cross sectional echocardiography.

Cross sectional echocardiographic studies were assessed prospectively in 58 infants in whom right ventricular outflow obstruction was subsequently shown angiographically. A subcostal cut was used to display simultaneously the short axis of the aortic root and the long axis of the right ventricular outflow tract. This facilitated the differentiation of the common right ventricular outflow tract obstructive lesions. Tetralogy of Fallot was diagnosed correctly in 22 of 26 infants; pulmonary atresia with intact septum in all of 14 neonates; isolated severe pulmonary valve stenosis in all of nine infants; and pulmonary atresia with ventricular septal defect in eight of nine infants. The subcostal approach is the technique of choice for evaluating right ventricular outflow tract obstruction as it is more reliable than the left parasternal approach.

Coronary Circulation↗