Martin J. Elliott

Patient handover from surgery to intensive care: using Formula 1 pit‐stop and aviation models to improve safety and quality

Background:  We aimed to improve the quality and safety of handover of patients from surgery to intensive care using the analogy of a Formula 1 pit stop and expertise from aviation.

Stem-cell-based, tissue engineered tracheal replacement in a child: A 2-year follow-up study

BACKGROUND Stem-cell-based, tissue engineered transplants might offer new therapeutic options for patients, including children, with failing organs. The reported replacement of an adult airway using stem cells on a biological scaffold with good results at 6 months supports this view. We describe the case of a child who received a stem-cell-based tracheal replacement and report findings after 2 years of follow-up. METHODS A 12-year-old boy was born with long-segment congenital tracheal stenosis and pulmonary sling. His airway had been maintained by metal stents, but, after failure, a cadaveric donor tracheal scaffold was decellularised. After a short course of granulocyte colony stimulating factor, bone marrow mesenchymal stem cells were retrieved preoperatively and seeded onto the scaffold, with patches of autologous epithelium. Topical human recombinant erythropoietin was applied to encourage angiogenesis, and transforming growth factor β to support chondrogenesis. Intravenous human recombinant erythropoietin was continued postoperatively. Outcomes were survival, morbidity, endoscopic appearance, cytology and proteomics of brushings, and peripheral blood counts. FINDINGS The graft revascularised within 1 week after surgery. A strong neutrophil response was noted locally for the first 8 weeks after surgery, which generated luminal DNA neutrophil extracellular traps. Cytological evidence of restoration of the epithelium was not evident until 1 year. The graft did not have biomechanical strength focally until 18 months, but the patient has not needed any medical intervention since then. 18 months after surgery, he had a normal chest CT scan and ventilation-perfusion scan and had grown 11 cm in height since the operation. At 2 years follow-up, he had a functional airway and had returned to school. INTERPRETATION Follow-up of the first paediatric, stem-cell-based, tissue-engineered transplant shows potential for this technology but also highlights the need for further research. FUNDING Great Ormond Street Hospital NHS Trust, The Royal Free Hampstead NHS Trust, University College Hospital NHS Foundation Trust, and Region of Tuscany.

Ultrafiltration and modified ultrafiltration in pediatric open heart operations

The capillary leak associated with cardiopulmonary bypass results in an increase in content of water in the tissues measurable by an increase in total body water after cardiac operation. Following work by Magilligan in the 1970s, ultrafiltration was introduced during bypass as a means of hemoconcentrating patients and potentially removing water from the tissues. Conventional methods proved inconsistent; thus, we modified the technique to ultrafilter the patients immediately after cessation of bypass. Modified ultrafiltration takes 10 minutes and results in an elevation of the on-bypass hematocrit to about 35% or 40%. In pilot studies comparing bypass in absence of ultrafiltration with conventional ultrafiltration and modified ultrafiltration, only the modified technique was seen reliably to reduce the elevation in total body water to only 4%, within a narrow range. Subsequent prospective studies confirmed the reduction in accumulation of total body water and also demonstrated a reduction in blood loss and in requirements for blood transfusion. Systolic blood pressure was observed to increase uniformly in all patients undergoing modified ultrafiltration. This effect was investigated and found to be associated with a marked increase in cardiac index, no change in systemic vascular resistance, a decrease in heart rate, and a marked decrease in pulmonary vascular resistance. Recently, we have demonstrated an increase in contractility and a decrease in myocardial wall volume. The modified technique of ultrafiltration has been employed successfully in more than 400 patients in our institution, and represents an excellent option for perioperative management of both accumulation of fluid in the tissues and hemodynamics after hypothermic bypass.

The nomenclature, definition and classification of hypoplastic left heart syndrome

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair. The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Lung transplantation and life extension in children with cystic fibrosis.

BACKGROUND Lung transplantation has been available as therapy for end-stage lung disease since the early 1980s, but survival after transplantation remains poor, with continued controversy as to the survival benefit from the procedure. We examined the effect of lung or heart-lung transplantation on the survival of a cohort of children with cystic fibrosis and severe lung disease. METHODS Between May, 1988, and May, 1998, 124 children with cystic fibrosis were accepted for lung transplantation. 47 received transplants, 68 died while they awaited organs, and nine remained on the active waiting list. We constructed a proportional-hazards model that used variables of prognostic significance in this population. By including transplant status as a time-dependent covariate, we were able to calculate a hazard ratio for transplantation. Date of entry into the study was the date when children were added to the list for transplantation, and measurements were taken at this time. Children were accepted for transplantation if they had a life expectancy of 2 years or less, a poor quality of life, and no contraindications to transplantation. FINDINGS After 1 year, 35 (74%) children were still alive; after 5 years 12 (33%) children were alive. The univariate hazard ratio for transplantation was 0.41 (95% CI 0.23-0.74; p=0.003). Transplantation remained significantly associated with survival after correction for differences in age, sex, height-corrected forced expiratory volume in 1 s, minimum oxygen saturation during a 12 min walk, haemoglobin concentration, albumin concentration, and age-corrected resting heart rate (hazard ratio 0.31 [0.13-0.72]; p=0.007). INTERPRETATION If centres follow our criteria for accepting patients for transplantation, and achieve similar survival after transplantation, they could expect a survival benefit for their patients in line with our results.

Tracheal allograft reconstruction: the total North American and worldwide pediatric experiences

BACKGROUND We reviewed both the North American and the total worldwide pediatric experience with tracheal allograft reconstruction as treatment for patients with long segment and recurrent tracheal stenosis. METHODS The stenosed tracheal segment is opened to widely patent segments. The anterior cartilage is resected and the posterior trachealis muscle or tracheal wall remains. A temporary silastic intraluminal stent is placed and absorbable sutures secure the chemically preserved cadaveric trachea. After initial success with this technique in Europe, several North American centers have now performed the procedure. The cumulative North American experience includes 6 patients (3 adults and 3 children). Worldwide, more than 100 adults and 31 children, aged 5 months to 18 years, with severe long segment tracheal stenosis have undergone tracheal allograft reconstruction. RESULTS In North America, 5 of 6 patients have survived, with one early death due to bleeding from a tracheal-innominate artery fistula in a previously irradiated neck. Worldwide, 26 children survived (26 of 31 = 84%) with follow-up from 5 months to 14 years. Only 1 of 26 pediatric survivors (1 of 26 = 3.8%) had a tracheostomy. CONCLUSIONS Tracheal allograft reconstruction demonstrates encouraging short- to medium-term results for patients with complex tracheal stenosis. Allograft luminal epithelialization supports the expectation of good long-term results.

The role of airway stents in the management of pediatric tracheal, carinal, and bronchial disease

OBJECTIVE A variety of stents are available to aid in the management of complex tracheal, carinal and bronchial stenoses. We reviewed our multi-institutional experience with airway stents in children. METHODS Thirty-three children (age, 13 days-18 years) from four institutions have had a total of 40 stents placed to aid in the management of complex airway stenoses. Three stent types were utilized: 29 silastic stents, five expandable metal stents and six customized carinal stents (four patients had two stents and one patient had four stents). Thirty children had tracheal stents, six children had bronchial stents, and two infants had carinal stents (three children had stenting of more than one area and two had stenting of all three locations). Twenty-eight patients (age, 5 months-18 years; mean, 8.06 years; SEM, 1.13 years) had stents placed after a variety of airway reconstructive procedures. Four underwent stenting in a non-operative setting and one as preoperative stabilization. RESULTS Twenty-seven patients survived. One patient died early due to bleeding. Five patients died late: two due to bleeding, one from mediastinitis, and two patients with functional airways died late from unrelated problems. Complications are related to stent type and location. Carinal stents can migrate; several techniques are available to help manage this problem. Wire stents are essentially non-removable requiring periodic dilation. Silastic stents stimulate granulation tissue formation requiring periodic bronchoscopic removal. CONCLUSION Tracheal stenting can aid in the management of pediatric airway problems. Complications are common, but can be managed with appropriate intervention.

Management of pulmonary venous obstruction after correction of TAPVC: risk factors for adverse outcome

OBJECTIVE Recurrent pulmonary venous obstruction (PVO) occurs in 0-18% of infants undergoing correction of total anomalous pulmonary venous connection (TAPVC). Limited published data suggest that PVO usually develops within 6 months of primary repair, and that outcomes of reoperations are poor. This study aimed to review our experience of reoperations for PVO post-TAPVC repair and to identify risk factors for adverse outcome. METHODS Twenty patients underwent reoperation for PVO between 1982 and 2002. Clinical data were reviewed. TAPVC was mostly infracardiac (11 patients). TAPVC was obstructed in nine patients. PVO developed early (<6 months) in seven patients, and late in 13 (>6 months). Time of presentation was unrelated to type of PVO (anastomotic vs. ostial). Repair was accomplished using various techniques (anastomotic enlargement with native atrial tissue, enlargement with pericardium, free or in situ, or other prosthetic material). Follow-up ranged from 1 month to 15 years (average 44 months). RESULTS Thirteen patients received one reoperation, while seven had multiple reoperations. In 13 patients, PVO was defined as new onset (no obstruction post-TAPVC repair), and in seven patients as residual (minimal obstructive changes post-TAPVC repair that progressed to PVO). Ten patients presented with anastomotic PVO, six with anastomotic and ostial PVO (involving the PVs), three with ostial PVO, and one with coronary sinus-left atrial junction stenosis. Mortality was 25% (5/20). Six of the ten patients with anastomotic PVO underwent one reoperation (2/6 died); the other four developed ostial PVO after reoperation, requiring multiple procedures (2/4 died). Mode of presentation (new onset vs. residual), site of obstruction (anastomotic vs. ostial), preoperative RV pressure (<0.8 vs. >0.8 systemic), number of reoperations (single vs. multiple), residual obstruction (presence or absence), and operative approach (Gore-tex or not) did not seem to affect outcomes. Risk factors for death were early presentation (<6 months) and persistence of pulmonary hypertension after reoperation; early presentation was also a risk factor for multiple reoperations. CONCLUSIONS Our findings support the conclusion that early presentation and postoperative pulmonary hypertension have the greatest adverse impact on outcome. Of these, failure to achieve a low-pressure pulmonary vascular system seems to be the variable that most strongly prevents survival. In our series, neither ostial PVO nor multiple re-interventions significantly increased surgical risk. The negative impact of postoperative residual obstruction on outcome was not striking. However, an aggressive surgical approach to this disease is still warranted. Although the role of each technique in obtaining long-lasting relief of PVO remains to be established, the use of artificial material seems unwise.

Early age at repair prevents restrictive right ventricular (RV) physiology after surgery for tetralogy of Fallot (TOF): Diastolic RV function after TOF repair in infancy

OBJECTIVES To assess diastolic right ventricular (RV) physiology after tetralogy of Fallot repair in infancy. BACKGROUND Restrictive RV physiology after tetralogy of Fallot repair is related to type of repair, pulmonary regurgitation, and late arrhythmias. METHODS Forty-seven patients were investigated, 27 and 20 patients in Lund and London, respectively. Median age at repair was 0.78 years (0.08-0.99) and median follow-up was 3.0 years (0.08-10.4). Restrictive RV physiology was assessed by Doppler echocardiography. RESULTS Thirteen patients (28%) had restrictive RV physiology at follow-up, three of 19 patients (16%) with transatrial repair and 10 of 28 patients (32%) with transventricular repair, respectively (p=0.1). Ten percent of the patients repaired before 6 months of age were restrictive at follow-up, increasing to 38% with repair after 9 months. Transannular patch (TAP) repair was performed in 55% of the patients, including eight of 10 patients (80%) with repair before 6 months of age. Thirty-one percent of the patients with TAP repair were restrictive. These restrictive patients had more severe preoperative pulmonary stenosis (p < 0.05), were older at repair (p < 0.05), and had shorter duration of pulmonary regurgitation (p < 0.001) at follow-up. CONCLUSIONS Restrictive RV physiology is inversely related to age at repair and independent of type of outflow tract repair. Since TAP repair is more common in early repair, and restriction seems to be less frequent, long-term follow-up to assess adverse effects of pulmonary regurgitation is mandatory.

Coarctation of the Aorta with Arch Hypoplasia: Improvements on a New Technique

The association between coarctation and arch hypoplasia can be difficult to manage surgically. Several complex techniques have been designed for its management (e.g., resection of coarctation with either retrograde subclavian flap or carotid subclavian angioplasty). However, residual gradients are frequent. Recently, a new technique of extensive end-to-end anastomosis was described that has the advantages of leaving the left subclavian artery intact and resulting in a wide anastomosis. The present report details a modification of this technique, which more extensively dissects the arch branches and clamps the aortic arch almost 50% of the way across the innominate branch. The descending aorta is mobilized to the diaphragm. Anastomosis is completed with 7-0 PDS or polypropylene sutures. This type of repair has been performed in 7 patients, with a maximum residual gradient at the end of the procedure of 5 mm Hg. These modifications represent an excellent option to the surgeon in the management of this difficult clinical entity.