Initial experience with hybrid surgery in congenital heart disease in a single center : P171

Introduction: A hybrid intervention is a joint procedure involving the interventional cardiologist and the cardiac surgeon. At our institution we have opted for this type of approach in congenital heart disease since 2005. We report here our initial experience. Cases: 1. A 3 year old boy with double aortic arch and multiple muscular ventricular septal defects (VSD),was readdressed for pulmonary band (PAB) removal and residual VSD closure after previous palliation. After surgical removal of the PAB, the surgeon provided a minimal transventricular access for placement of a 6mm Amplatzer® muscular VSD occluder by the cardiologist under transoesophageal guidance. The patient was extubated the same day and discharged after 5 days. 2. An 8 year old girl with Williams syndrome was followed for two large VSDs and severe peripheral pulmonary arteries (PA) stenosis. The membranous VSD was closed surgically, the muscular VSD during the same operation by direct placement of a 12 mm Amplatzer® muscular VSD occluder. During rewarming, balloon angioplasty of peripheral PA stenosis was achieved under fluoroscopy. Patient was extubated the following day and discharged after 8 days. 3. A 9 year old boy post tetralogy of Fallot repair had severe distal stenosis of the right ventricular to PA conduit.With patient on partial cardiopulmonary bypass, an incision was made on the conduit and a CP 8 Zig 16 stent placed on the stenosis. The child passed on full bypass and the definitive placement of the stent achieved. The child was extubated at the end of the intervention and discharged after 6 days. 4. A newborn presented at 2 days life with complex aortic arch anatomy: left aortic arch and right descending thoracic aorta perfused directly from a right arterial duct and left PA atresia. The arterial duct was stented with a Genesis XD stent dilated at 7mm. Two days later the cardiac surgeon made banded the right PA. The child was extubated after the operation and discharged a week later. Conclusion: Hybrid approach opens new ways of correction or palliation in congenital heart disease with encouraging results and less morbidity.

Micromeasurement of total and regional CO2 productions in the one-day-old chick embryo.

A conductometric micromethod combined with image analysis system has been developed allowing to determine the CO2 production within 'two-dimensional' tissues, i.e., flat and thin cell layers or epithelial sheets. The preparation was mounted into an airtight chamber separated in two compartments by a thin silicone membrane permeable to gases. The lower compartment contained the nutritive medium and the preparation. The upper compartment and a conductivity measuring capillary connected in series were perfused with a solution of Ba(OH)2. The CO2 produced by the tissue precipitated as BaCO3 and the resulting decrease of electrical conductivity was linearly related to the total CO2 production. In addition, the pattern of CO2 production was directly observable as the BaCO3 crystals formed upon the silicone membrane over the regions which produced CO2. The spatial distribution of the crystals was quantified by video image processing and the regional CO2 production evaluated with a spatial resolution of 100 microns. This new microtechnique was originally developed to study the CO2 production in the early chick blastoderm which is a disc 1-5 cells thick. At the stage of young neurula the CO2 production was found to be 235 +/- 37 nmol.h-1 (mean +/- SD; n = 10) per blastoderm and large variations of local CO2 production were detected from one region to another (from 0.6 to 6.5 nmol.h-1.mm-2). These results indicate a high metabolic and functional differentiation of cells within the blastoderm. The possible applications and improvements of such a microtechnique are discussed.

Phosphorylation of phosphatidylinositol-3-kinase-protein kinase B and extracellular signal-regulated kinases 1/2 mediate reoxygenation-induced cardioprotection during hypoxia.

In vivo exposure to chronic hypoxia (CH) depresses myocardial performance and tolerance to ischemia, but daily reoxyenation during CH (CHR) confers cardioprotection. To elucidate the underlying mechanism, we tested the role of phosphatidylinositol-3-kinase-protein kinase B (Akt) and p42/p44 extracellular signal-regulated kinases (ERK1/2), which are known to be associated with protection against ischemia/reperfusion (I/R). Male Sprague-Dawley rats were maintained for two weeks under CH (10% O(2)) or CHR (as CH but with one-hour daily exposure to room air). Then, hearts were either frozen for biochemical analyses or Langendorff-perfused to determine performance (intraventricular balloon) and tolerance to 30-min global ischemia and 45-min reperfusion, assessed as recovery of performance after I/R and infarct size (tetrazolium staining). Additional hearts were perfused in the presence of 15 micromol/L LY-294002 (inhibitor of Akt), 10 micromol/L UO-126 (inhibitor of ERK1/2) or 10 micromol/L PD-98059 (less-specific inhibitor of ERK1/2) given 15 min before ischemia and throughout the first 20 min of reperfusion. Whereas total Akt and ERK1/2 were unaffected by CH and CHR in vivo, in CHR hearts the phosphorylation of both proteins was higher than in CH hearts. This was accompanied by better performance after I/R (heart rate x developed pressure), lower end-diastolic pressure and reduced infarct size. Whereas the treatment with LY-294002 decreased the phosphorylation of Akt only, the treatment with UO-126 decreased ERK1/2, and that with PD-98059 decreased both Akt and ERK1/2. In all cases, the cardioprotective effect led by CHR was lost. In conclusion, in vivo daily reoxygenation during CH enhances Akt and ERK1/2 signaling. This response was accompanied by a complex phenotype consisting in improved resistance to stress, better myocardial performance and lower infarct size after I/R. Selective inhibition of Akt and ERK1/2 phosphorylation abolishes the beneficial effects of the reoxygenation. Therefore, Akt and ERK1/2 have an important role to mediate cardioprotection by reoxygenation during CH in vivo.

Krikothyreotomie mit dem "Quicktrach"-Koniotomiebesteck [Cricothyreotomy using the Quicktrach coniotomy instrument set].

Percutaneous cricothyroidotomy may be a lifesaving procedure for airway obstruction, which cannot be relieved by endotracheal intubation and can be performed with specially designed instruments. A new device, the "Quicktrach", was evaluated by an anatomical preparation, flow and resistance measurements, and puncture of the cricothyroid membrane in 55 corpses. The size of the parts of the instrument (needle, plastic cannula, depth gauge) in relation to the size of the larynx is adequate, thus there is little likelihood of perforation of the posterior wall of the larynx. Resistance of the plastic cannula is sufficiently low to allow for adequate ventilation. The duration of time until the cannula is positioned properly in the trachea is significantly shorter, when an incision prior to the puncture is done (83 +/- 88 seconds without incision versus 35 +/- 41 seconds with incision; mean +/- SD). The "Quicktrach" is easy to apply even by inexperienced persons. The incidence of damage to the larynx (lesions including fractures of the thyroid, cricoid and 1. tracheal cartilage in 18%; soft tissue injury in 9%) is relatively high, however considering the live saving character of the procedure these numbers appear to be acceptable. Technical problems which occur with the use of the device are discussed and suggestions for improvement are made.

Endoscopic laryngotracheal cleft repair without tracheotomy or intubation.

OBJECTIVES: The objectives of this study are to present the technique and results of endoscopic repair of laryngotracheoesophageal clefts (LTEC) extending caudally to the cricoid plate into the cervical trachea and to revisit the classification of LTEC. METHODS: The authors conducted a retrospective case analysis consisting of four infants with complete laryngeal clefts (extending through the cricoid plate in three cases and down into the cervical trachea in one case) treated endoscopically by CO2 laser incision of the mucosa and two-layer endoscopic closure of the cleft without postoperative intubation or tracheotomy. RESULTS: All four infants resumed spontaneous respiration without support after a mean postoperative period of 3 days with continuous positive airway pressure (CPAP). They accepted oral feeding within 5 postoperative days (range, 3-11 days). No breakdown of endoscopic repair was encountered. After a mean follow up of 48 months (range, 3 mos to 7 y), all children have a good voice, have no sign of residual aspiration, but experience a slight exertional dyspnea. CONCLUSION: This limited experience on the endoscopic repair of extrathoracic LTEC shows that a minimally invasive approach sparing the need for postoperative intubation or tracheotomy is feasible and safe if modern technology (ultrapulse CO2 laser, endoscopic suturing, and postoperative use of CPAP in the intensive care unit) is available.

The rare cancer network: ongoing studies and future strategy.

The Rare Cancer Network (RCN) was formed in the early 1990's to create a global network that could pool knowledge and resources in the studies of rare malignancies whose infrequency prevented both their study with prospective clinical trials. To date, the RCN has initiated 74 studies resulting in 46 peer reviewed publications. The First International Symposium of the Rare Cancer Network took place in Nice in March of 2014. Status updates and proposals for new studies were heard for fifteen topics. Ongoing studies continue for cardiac sarcomas, thyroid cancers, glomus tumors, and adult medulloblastomas. New proposals were presented at the symposium for primary hepatic lymphoma, solitary fibrous tumors, Rosai-Dorfman disease, tumors of the ampulla of Vater, salivary gland tumors, anorectal melanoma, midline nuclear protein in testes carcinoma, pulmonary lymphoepithelioma-like carcinoma, adenoid cystic carcinoma of the trachea, osteosarcomas of the mandible, and extra-cranial hemangiopericytoma. This manuscript presents the abstracts of those proposals and updates on ongoing studies, as well a brief summary of the vision and future of the RCN.

Congenital tracheal anomalies.

Congenital tracheal lesions are rare, but important, causes of morbidity in infants and children. Consequently, experience in their management is limited and dispersed. Given its small diameter, the juvenile trachea is obstructed easily by various natural causes, or following a surgical intervention. The diagnosis of a congenital, tracheal, obstructive anomaly is based on a high degree of suspicion in infants and children with respiratory distress accompanied by retraction. In this article, the authors discuss the various causes of these conditions, their diagnostic features, and the treatment possibilities.