Hemoadsorption Contribution in Failing Fontan Pediatric Heart Transplantation.

INTRODUCTION: A systemic inflammatory response is triggered in patients undergoing cardiothoracic surgery with cardiopulmonary bypass (CPB). This response is particularly evident in pediatric patients, especially those of low weight and after undergoing long CPB, and can severely impair the surgical result. Adsorptive blood purification techniques have been proposed to limit this systemic inflammatory response. To test its efficacy, we added the hemoadsorption filter Jafron HA 380 to CPB in a much compromised pediatric patient who underwent heart transplantation. METHODS: A 10-year-old single ventricle patient previously treated with Fontan operation was listed for heart transplantation due to the evidence of failing Fontan condition. He experienced many episodes of cardiac arrest and underwent heart transplantation in much compromised general and hemodynamic conditions. The hemoadsorption filter Jafron HA 380 was used for all the duration of CPB, and the inflammatory biomarker interleukin 6 (IL-6) was assayed. RESULTS: Postoperative outcome was uneventful and comparable to that of elective pediatric heart transplantation. IL-6 levels showed an impressive postoperative reduction, and after 2 days, the IL-6 level was comparable with a typical uneventful post-transplant course. CONCLUSIONS: The use of hemoadsorption filter can contribute to improve the pediatric transplant results, especially in very high-risk patients.

Atypical microdeletion 22q11.2 in a patient with tetralogy of Fallot.

The 22q11.2 microdeletion syndrome (22q11.2 DGS) is characterized by an extreme intrafamilial and interfamilial variability. The main clinical features are congenital heart defects, palatal abnormalities, learning disability, facial dysmorphisms and immune deficiency. In 85-90% of cases, the 22q11.2 DGS is caused by a heterozygous ~3-Mb deletion, including the TBX1 gene, considered one of the major genes responsible for heart defects. Individuals with atypical deletions with at least one breakpoint outside low copy repeats have been reported. Our patient is a child presenting tetralogy of Fallot (TOF) with an atypical 22q11.2 deletion proximal to the critical DiGeorge region. The rearrangement was inherited from the healthy mother and spanned ~642-970 kb, encompassing DGCR6 and PRODH, two novel possible candidate genes for conotruncal heart defects.

Malnutrition and chyle leakage: A life-threatening duo in heart transplantation post-Fontan procedure.

Protein-losing enteropathy and chyle leakage may lead to severe malnutrition in heart transplantation for failing Fontan. Nutritional management may be challenging from defining nutrient needs to diagnosis of malnutrition enteropathy, and expertise is necessary. Body composition and hematological nutritional indices may help define malnutrition severity and guide nutritional strategy.

Are probiotics safe? Bifidobacterium bacteremia in a child with severe heart failure.

Although few cases of bacteremia or sepsis caused by probiotics have been reported, it is important to consider their pathogenic potential, especially in some categories of patients. We report a case of Bifidobacterium spp bacteremia in a child with heart disease, undergoing probiotic supplementation to prevent antibiotic-associated diarrhea.

Liver stiffness modifications shortly after total cavopulmonary connection.

OBJECTIVES: Hepatic damage in Fontan circulation setting is well known. Mainly represented by fibrosis, it is still poorly understood. Transient hepatic elastography (Fibro-Scan) is a useful tool to test liver stiffness and is commonly used in the evaluation of liver fibrosis. Unfortunately, the increase of inferior vena cava pressure consequent to total cavopulmonary connection can probably interfere with Fibro-Scan evaluation altering the result evaluation with the conventional scale. To verify this hypothesis and to quantify the impact of venous pressure on Fibro-Scan results, we checked perioperative liver stiffness changes in patients undergoing total cavopulmonary connection. METHODS: A prospective observational study was carried out on 9 patients undergoing extracardiac total cavopulmonary connection. Mean age at operation was 4.3 ± 0.6 years, and mean weight 14 ± 2.4 kg. Hepatic stiffness was analysed with Fibro-Scan evaluation immediately before and 4 months after surgery. Results were matched with several perioperative data: pulmonary pressures, conduit size, fenestration, hepatic enzymes and coagulation setting. RESULTS: Preoperative hepatic stiffness was 6.2 ± 1.5 kPa, with a significant increase to 11.2 ± 4 kPa at a mean follow-up of 4 months (P < 0.01). Mean alanine aminotransferase was 16.9 ± 8.3 mg/dl preoperatively and 16.3 ± 8.7 mg/dl at discharge. Both values showed a significant correlation with liver stiffness raise, their Pearson Correlation Indexes being 0.8 and 0.7, respectively (P < 0.01). Mean aspartate aminotransferase/alanine aminotransferase ratio at discharge was 1.7 ± 0.5 and showed a significant negative correlation with liver stiffness raise, its Pearson Correlation Index being -0.7 (P < 0.01). CONCLUSIONS: Liver stiffness increases rapidly after total cavopulmonary connection and perioperative variations in some liver enzymes appear to correlate with this change. Since a true anatomical damage is known to develop gradually with Fontan circulation, early liver stiffness raise is likely due to parenchymal congestion only. Fibro-Scan can easily recognize and assess the entity of such a change. For these reasons, this diagnostic tool must be considered useful only to monitor liver stiffness changes and evolution with time, but a conventional evaluation of results, like in other acquired hepatic fibrosis, can be misleading.

Penetrating aortic ulcers after absorption of intramural hematoma.

We report a case of a type B aortic intramural hematoma, which rapidly expanded, with ulcer-like projections, after complete absorption of the hematoma. One month after the initial presentation, a new computed tomographic scan showed the appearance of a thin ulcer-like projection in the aortic wall. Three months later, after the patient reported a new episode of chest pain, a computed tomographic scan revealed 2 penetrating ulcers and rapid aortic dilation; the aortic hematoma had been completely absorbed. Urgent thoracic aortic replacement was undertaken. Three years postoperatively, the patient was asymptomatic, with no lesion or dilation of the aorta upon computed tomography. After an acute hematoma, the strength and structure of the aortic wall can alter rapidly. Damage and weakening of the aortic wall are caused mainly by infiltration of inflammatory cells, which have pronounced proteolytic and elastolytic activity. Due to the unstable nature of the lesion, optimal management remains controversial. In our patient, the inflammatory process led to the development of 2 aortic ulcers and aortic dilation within 3 months of the acute lesion, requiring urgent surgical intervention.

Pulmonary valve replacement with mechanical prosthesis: long-term results in 4 patients.

Pulmonary valve replacement is rarely performed and is usually done with biological prostheses or grafts. The use of mechanical prostheses is still a debated issue. We analyzed the outcome of 4 patients at 11 years after pulmonary valve replacement with a mechanical prosthesis. During follow-up, all patients remained asymptomatic, with no arrhythmias and good anticoagulation. An echocardiogram showed preserved ventricular contraction and normal function of the prostheses, with low pressure gradients. No infective endocarditis occurred. Mechanical prostheses appear to be a good choice for pulmonary valve replacement. Surgical and clinical results were satisfactory with no complications, acceptable pressure gradients, and good ventricular function after more than 10 years. Lifelong anticoagulation is mandatory, but this is usually well tolerated.

Aortic coarctation: an overview.

In severe aortic coarctation in the neonatal period, surgical repair is required soon after clinical stabilization. Elective repair of isolated aortic coarctation is nowadays indicated at 3-6 months of life or at the time of diagnosis. At present, no single operation appears to have a clear superiority. However, during the first months of life, an extended end-to-end anastomosis is considered the best option by most authors, even though weight at operation and anatomy of the aortic arch are also significant determinants of late recoarctation. In cases of aortic arch hypoplasia, which occurs in up to 70% of neonatal and infant coarctations, especially when associated anomalies are present, surgery seems the treatment of choice. After 3 months of age and in the adult population, balloon angioplasty and stent placement are considered a suitable option. Recently, we adopted a median sternotomy approach without the use of extracorporeal circulation for the treatment of aortic coarctation with a hypoplastic aortic arch. We treated 11 patients with satisfactory results at an average follow-up of 40 months.

Securing atrial lines with Silastic ligatures.

We present a safe and easy-to-apply method of securing monitoring atrial cannulae after pediatric cardiac operations. We used Silastic ligatures together with purse strings on the tip of the atrial appendages in more than 350 cases, in a 5-year period. We never reported malfunctioning during their use, and no bleeding or rupture complication was detected by echocardiographic control after withdrawal.

Partial hepatic vein diversion in pulmonary arteriovenous malformations in congenital heart disease.

The late appearance of pulmonary arteriovenous malformations after operations for complex congenital cardiac conditions may be explained in many ways. The necessity of hepatic blood flow to the lungs is now generally accepted for carrying some labile substance that is secreted by the liver and that has not yet been identified. Rerouting of hepatic blood to the lungs in cardiac patients can be accomplished with different methods, most of which are predisposed to thrombosis because of the slow flow in the tunnel. My colleagues and I describe a case of partial diversion of hepatic blood into the pulmonary circulation and suggest a strategy to decrease the likelihood of conduit thrombosis and to treat pulmonary arteriovenous malformations.