Heart transplantation in children and adults with Down syndrome: A single centre experience.

BACKGROUND: In recent years, rapid advances in cardiac surgery and changes in attitude towards patients with cognitive disability have led to these patients receiving cardiac transplantation. METHOD: This is a retrospective report describing the experience of four patients with Down Syndrome who received heart transplantation in a single institution. RESULTS: Anthracycline-induced cardiomyopathy was the most common cause of heart failure in this group (3/4). Two patients were bridged to transplantation, one by using a combination of extra-corporeal membrane oxygenation and biventricular assist device and the other by using a durable implantable left ventricular assist device. All the four patients are alive with the longest surviving patient 17 years after transplantation. Against strong hypothetical predictions, we observed no propensity for the development of post-transplant infections or lymphoproliferative disorders. CONCLUSION: Down Syndrome should not be the sole contraindication to heart transplantation. The decision for transplantation should be on a case-by-case basis provided adequate social support is in place.

Diagnosis and predicted outcomes of patients with cystic fibrosis related liver disease considered for lung transplantation.

INTRODUCTION: There is no gold standard criterion for the diagnosis of cystic fibrosis-related liver disease (CFRLD) and there is uncertainty over its impact on the outcome of lung transplantation. METHOD: Lung recipients (n = 238) were divided into two groups-CFRLD and non-CFRLD based on a modified aspartate aminotransferase-to-platelet ratio index (APRI) score (mAPRI) to diagnose CFRLD and predict severity of liver disease. Groups were compared to assess validity of the diagnosis and survival outcomes. RESULT: The new diagnostic criterion was effective at differentiating CFRLD from non-CFRLD. There was no significant difference in the survival between two groups at short, medium, or long term demonstrated by the Kaplan-Meier plot with survival of 85%, 73%, 47%, 18.6%, and 4.7% at 1, 2, 5, 10, and 15 years respectively. A mAPRI score of greater than .2 had a sensitivity of 43.0% but a specificity of 82.5 % for diagnosis of CFRLD and 46.5% sensitivity but 100% specificity in predicting an ultrasound/biopsy proven hepatic abnormality associated with CFRLD. CONCLUSION: A mAPRI sore is a highly specific non-invasive tool for diagnosis of CFRLD. Recipients with CFRLD but grossly preserved hepatocellular function have a similar outcome to patients without CFRLD.

Is there a renoprotective value to leukodepletion during heart valve surgery? A randomized controlled trial (ROLO).

BACKGROUND: Acute Kidney Injury (AKI) adversely affects outcomes after cardiac surgery. A major mediator of AKI is the activation of leukocytes through exposure to the cardiopulmonary bypass circuit. We evaluate the use of leukodepletion filters throughout bypass to protect against post-operative AKI by removing activated leukocytes during cardiac surgery. METHODS: This is a single-centre, double-blind, randomized controlled trial comparing the use of leukodepletion versus a standard arterial filter throughout bypass. Elective adult patients undergoing heart valve surgery with or without concomitant procedures were investigated. The primary clinical outcome measured was the development of AKI according to the KDIGO criteria. Secondary measures included biomarkers of renal tubular damage (urinary Retinol Binding Protein and Kidney Injury Molecule-1), glomerular kidney injury (urinary Micro Albumin and serum Cystatin C) and urinary Neutrophil Gelatinase Associated Lipocalin, as well as the length of hospital stay and quality of life measures through EQ-5D-5L questionnaires. RESULTS: The ROLO trial randomized 64 participants with a rate of recruitment higher than anticipated (57% achieved, 40% anticipated). The incidence of AKI was greater in the leukodepletion filter group (44% versus 23%, risk difference 21, 95% CI - 2 to 44%). This clinical finding was supported by biomarker levels especially by a tendency toward glomerular insult at 48 h, demonstrated by a raised serum Cystatin C (mean difference 0.11, 95% CI 0.00 to 0.23, p = 0.068) in the leukodepleted group. There was however no clear association between the incidence or severity of AKI and length of hospital stay. On average, health related quality of life returned to pre-operative levels in both groups within 3 months of surgery. CONCLUSIONS: Leukocyte depletion during cardiopulmonary bypass does not significantly reduce the incidence of AKI after valvular heart surgery. Other methods to ameliorate renal dysfunction after cardiac surgery need to be investigated. TRIAL REGISTRATION: The trial was registered by the International Standard Randomized Controlled Trial Number Registry ISRCTN42121335 . Registered on the 18 February 2014. The trial was run by the Bristol Clinical Trials and Evaluation Unit. This trial was financially supported by the National Institute of Health Research (Research for Patient Benefit), award ID: PB-PG-0711-25,090.

Pre-transplant ventricular assist device explant.

Explantation of a left ventricular assist device (LVAD) may be challenging even in the most experienced hands. We aim to describe the technique for explantation of an LVAD together with the heart as applicable to all contemporary implantable mechanical assist devices. In order to ensure safe explantation, particular care must be taken at three distinct stages: at the time of LVAD implantation, at pre-transplant assessment and at the time of heart transplantation. The preparation for a safe explantation at LVAD implantation includes positioning the driveline and the outflow graft away from the back of the sternum to ensure protection from injury during re-entry into the chest. At transplant assessment, essential investigations include computed tomography (CT) of the chest and ultrasound imaging of femoral vessels. At the time of heart transplantation, the site of peripheral access should be prepared and vessels exposed in case of a need for emergency bypass. We advise careful dissection starting from the lower aspect of the under surface of the sternum, moving as proximally as possible before attempting to use the oscillating saw. Much of the dissection of the heart is done off-pump. Cardiopulmonary bypass may be established either through peripheral vessels or the outflow graft in an emergency. Central direct cannulation is then established. After the heart and major vessels are isolated, explantation of the heart may begin either en-bloc or after splitting the ventricles in a sagittal plane. The basal regions of both ventricles and both atria are removed, leaving generous cuffs for anastomosis of the left atrium, pulmonary artery, aorta, inferior and superior vena cava (SVC). The apex of the heart is then removed with the device taking care not to injure the phrenic nerve.

The midterm outcome and MACE of robotically enhanced grafting of left anterior descending artery with left internal mammary artery.

BACKGROUND: We assessed the midterm outcome and the incidence of major adverse cardiovascular events in UK's largest Da Vinci assisted robotic coronary revascularisation cohort. This study was set up at the Imperial College NHS Trust, St. Mary's Hospital, London, United Kingdom. METHOD: Benchmarking approach through retrospective audit of the regional outcomes against standards in the published literature. Data was collected from the patient's records, communication with the primary care physicians and the national strategic tracing service. The results were compared with the published literature. Patients who underwent robotic assisted coronary revascularisation were included. Other robotic procedures or minimally invasive revascularisation without the use of the Da Vinci robot were excluded. The main outcome measure was the midterm survival up to five years and the incidence of major adverse cardiovascular events (MACE) up to three years. RESULTS: Since April 2002, one hundred consecutive patients underwent either off pump robotic assisted single vessel small thoracotomy (SVST, n=88), or off pump total endoscopic coronary artery bypass grafting (TCAB, n=12). All patients were operated on by the same primary surgeon but different assisting surgeons. All patients received a left internal mammary arterial (LIMA) graft as planned. The primary outcome of total one month and three years MACE and up to five year survival was 0, 9 and 96% respectively. CONCLUSIONS: The procedural success rates in terms of morbidity and mortality up to five years are compatible to the outcomes observed outside the United Kingdom. These results are not inferior to that of conventional off pump single vessel coronary surgery or percutaneous coronary intervention to the LAD.

Staged hybrid treatment for contained rupture of a large right coronary artery aneurysm.

We describe a staged approach to the management of a rare acute condition--contained rupture of a large right coronary artery aneurysm. A covered stent was deployed percutaneously to isolate the aneurysm at presentation followed by planned coronary bypass grafting. Treatment interval was complicated by new-onset pulmonary tuberculosis and subacute thrombosis of the covered stent leading to nonfatal inferior myocardial infarction. Coronary surgery was performed after complete antitubercular treatment and resolution of the acute pericarditis/thrombosis as a consequence of the contained rupture. The advantages of this staged approach included the following: (a) The covered stent prevented both acute myocardial infarction and progressive pseudoaneurysm expansion in the acute phase. (b) Deferred surgery was rendered technically less hazardous while avoiding the undesirable option of having to exclude an extremely calcified dominant right coronary artery. The patient made an excellent postoperative recovery with complete resolution of her symptoms at 6 weeks' follow-up.

Leukodepletion for patients undergoing heart valve surgery.

BACKGROUND: There is some evidence for the benefits of leukodepletion in patients undergoing coronary artery surgery. Its effectiveness in higher risk patients, such as those undergoing heart valve surgery, particularly in terms of overall clinical outcomes, is currently unclear. OBJECTIVES: To assess the beneficial and harmful effects of leukodepletion on clinical, patient-reported and economic outcomes in patients undergoing heart valve surgery. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (2013, Issue 3 of 12) in The Cochrane Library, the NHS Economic Evaluations Database (1960 to April 2013), MEDLINE Ovid (1946 to April week 2 2013), EMBASE Ovid (1947 to Week 15 2013), CINAHL (1982 to April 2013) and Web of Science (1970 to 17 April 2013) on 19 April 2013. We also searched the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP), the US National Institutes of Health (NIH) clinical trials database and the International Standard Randomised Controlled Trial Number Register (ISRCTN) in April 2013 for ongoing studies. No language or time period restrictions were applied. We examined the reference lists of all included randomised controlled trials and contacted authors of identified trials. We searched the 'grey' literature at OpenGrey and handsearched relevant conference proceedings. SELECTION CRITERIA: Randomised controlled trials comparing a leukocyte-depleting arterial line filter with a standard arterial line filter, on the arterial outflow of the heart-lung bypass circuit, in elective patients undergoing heart valve surgery. DATA COLLECTION AND ANALYSIS: Data were collected on the study characteristics, three primary outcomes (1. post-operative in-hospital all-cause mortality within three months, 2. post-operative all-cause mortality excluding inpatient mortality < 30 days, 3. length of stay in hospital, 4. adverse events and serious adverse events) and seven secondary outcomes (1. tubular or glomerular kidney injury, 2. validated health-related quality of life scales, 3. validated renal injury scales, 4. use of continuous veno-venous haemo-filtration, 5. length of stay in intensive care, 6. costs of care). Data were extracted by one author and verified by a second author. Insufficient data were available to perform a meta-analysis or sensitivity analysis. MAIN RESULTS: Eight studies were eligible for inclusion in the review but data on prespecified review outcomes were available from only one, modestly powered (24 participants) study (Hurst 1997). There were no differences between a leuko-depleting versus standard filter in length of stay in the intensive care unit (ICU) (mean difference (MD) 0.80 days; 95% confidence interval (CI) -0.24 to 1.84) or length of hospital stay (MD 0.20 days; 95% CI -1.78 to 2.18). AUTHORS' CONCLUSIONS: There are currently insufficient good quality trials with valve surgery patients to inform recommendations for changes in clinical practice. A future National Institute for Health Research (NIHR)-funded feasibility study (recruiting mid-year 2013) comparing leukodepletion with a standard arterial line filter in patients undergoing elective heart valve surgery (the ROLO trial) will be the largest study to date and will make a significant contribution to future updates of this review.

The mechanism of endocardial lead-induced tricuspid regurgitation.

Using this case report we attempt to define the mechanism of endocardial lead-induced tricuspid regurgitation (TR) in particular the direct effect of endocardial pacing leads on the competence of the tricuspid valve. We recommend a high index of suspicion and an early diagnostic strategy in order to reduce long-term morbidity which is associated with this condition and the need for a potentially avoidable surgery.

A surgical strategy for cor triatriatum atrial septal defect--A1 lam subclass.

Atrial fibrillation and a heart murmur were diagnosed in a 68-year-old woman during a routine medical examination. She presented 2 years later with pulmonary edema. A transthoracic echocardiography examination revealed a tunneled atrial septal defect (ASD) and severe tricuspid regurgitation. Transesophageal echocardiography and 3-dimensional computed tomography evaluations revealed multiple intracardiac defects, including abnormal atrial septation suggestive of a typical cor triatriatum sinistrum (A1 Lam subclass), a rare congenital defect in adults. The patient underwent tricuspid valve repair with concomitant closure of the ASD by using the cor triatriatum curtain to form an autologous transposition flap. The intraoperative transesophageal and predischarge imaging evaluations confirmed an excellent repair. The patient made a swift recovery and demonstrated improvement in her symptoms at follow-up. This previously undescribed technique eliminates the need for a prosthetic implant and is applicable in >80% of cor triatriatum cases in which an ASD exists.

Use of contrast-enhanced computed tomography for management of a late-onset spontaneous massive chest wall hematoma.

This is a rare case of a 68-year-old woman who was rehospitalized after uneventful redo double-valve surgery. An 8.3 × 12.9 × 16.4 cm tense right chest wall hematoma was diagnosed. This was precipitated by a single cough. Contrast-enhanced computed tomography revealed a bleeding source. Hematoma evacuation and hemostasis following emergency warfarin reversal produced an excellent outcome.

An audit of pain control pathways following video-assisted thoracoscopic surgery.

OBJECTIVE: To compare different modes of pain management following video-assisted thoracoscopic surgery (VATS) to our national standard. METHODS: This is an audit based on patient's experiences. One hundred consecutive patients who underwent VATS with or without pleurodesis were managed by one of the following pain relief pathways: (A) thoracic paravertebral block + morphine patient-controlled analgesia (PCA), (B) percutaneous thoracic paravertebral catheter +/- morphine PCA, (C) thoracic epidural +/- morphine PCA, (D) morphine PCA alone, and (E) intravenous or subcutaneous morphine as required. Pain score was documented up to four times per day for each patient. The incidence of severe pain was defined as visual analog scale ≥ 7. The results were compared with the standard set by the audit commission for postoperative pain relief in the UK. The mean daily pain scores were calculated retrospectively for all patients. RESULTS: There were no statistically significant differences in mean daily pain scores irrespective of having a pleurodesis. The percentage of patients experiencing severe pain was 34% [mean visual analog scale = 8 (standard deviation = 1.0)]. This was almost seven times the standard. Among these pathways, B had the least percentage incidence of severe pain (16.7%) followed by A (25.0%) D (33.3%), C (35.7%), and E (52.4%). CONCLUSIONS: We are not compliant with the standards set by the audit commission. Pain management in theater recovery needs to be targeted. In the light of these results, we recommend the use of percutaneous thoracic paravertebral catheter +/- morphine PCA for postoperative VATS pain relief.

Robotically assisted atraumatic coronary artery bypass: a feasible option for off-pump coronary surgery.

This retrospective study of the largest single center experience (100 patients) with off-pump robotically assisted coronary procedures in the United Kingdom (April 2002-June 2008) aimed to rationalize patient selection, describe the technique, and determine the learning curve, technical feasibility and operative outcome of robotically assisted Atraumatic Coronary Artery Bypass (ACAB). Selected patients underwent either a robotic Totally Endoscopic Coronary Artery Bypass (12) or robotically assisted ACAB (88) using a standard Da Vinci robot with three arms. A fifth of all cases had percutaneous interventions as part of a hybrid strategy. The majority of patients were overweight men. After one hundred robotic coronary procedures, this operation is now performed as part of a routine theatre list. The mean operative and total procedure times for robotically assisted atraumatic procedures were 157 and 238 min, respectively. These measurements were significantly less in the atraumatic than the totally endoscopic group with a 34.3 and 20.6% reduction, respectively (P < 0.001; equal variance not assumed). The procedural learning curve was short and independent from internal thoracic artery harvesting. We have proven conclusively that robotically assisted ACAB is feasible, more so than the totally endoscopic procedure in this particular setting. Even in the absence of an ideal stabilizer device, this procedure causes minimal disruption to the daily operating room schedule. We have also proven that body mass index is a weak predictor of the ease of robotic internal thoracic artery harvesting and should not affect patient selection.

Mechanisms underlying erythrocyte and endothelial nitrite reduction to nitric oxide in hypoxia: role for xanthine oxidoreductase and endothelial nitric oxide synthase.

Reduction of nitrite (NO(2)(-)) provides a major source of nitric oxide (NO) in the circulation, especially in hypoxemic conditions. Our previous studies suggest that xanthine oxidoreductase (XOR) is an important nitrite reductase in the heart and kidney. Herein, we have demonstrated that conversion of nitrite to NO by blood vessels and RBCs was enhanced in the presence of the XOR substrate xanthine (10 micromol/L) and attenuated by the XOR inhibitor allopurinol (100 micromol/L) in acidic and hypoxic conditions only. Whereas endothelial nitric oxide synthase (eNOS) inhibition had no effect on vascular nitrite reductase activity, in RBCs L-NAME, L-NMMA, and L-arginine inhibited nitrite-derived NO production by >50% (P<0.01) at pH 7.4 and 6.8 under hypoxic conditions. Western blot and immunohistochemical analysis of RBC membranes confirmed the presence of eNOS and abundant XOR on whole RBCs. Thus, XOR and eNOS are ideally situated on the membranes of RBCs and blood vessels to generate intravascular vasodilator NO from nitrite during ischemic episodes. In addition to the proposed role of deoxyhemoglobin, our findings suggest that the nitrite reductase activity within the circulation, under hypoxic conditions (at physiological pH), is mediated by eNOS; however, as acidosis develops, a substantial role for XOR becomes evident.

A comparison of radiographic signs of pulmonary inflammation during ECMO between silicon and poly-methyl pentene oxygenators.

INTRODUCTION: The inflammatory response caused by extracorporeal membrane oxygenation (ECMO) is clearly visible within the first 24 h of cannulation. The inflammatory process affects all areas of the lung, even areas previously spared by the primary disease. OBJECTIVE: To compare the change in the radiographic signs of inflammatory response to ECMO between poly-methyl pentene and silicon oxygenators. STUDY DESIGN: Retrospective review of neonates and adults pre- and post-replacement of silicon oxygenators with poly-methyl pentene devices. Data were collected from Extracorporeal Life Support Organisation (ELSO) registry forms and patient records. Results were analysed by quantitative and semi-quantitative methods. RESULTS: There was a significant reduction in the radiographic signs of inflammatory response to ECMO, and a reduction in the time taken to revert to pre-ECMO state in the neonatal poly-methyl pentene group compared to silicon. However, there was no significant reduction in the duration of ECMO runs and the percentage survival between these groups in the neonates. In adults, there was no difference in severity of radiographic signs between groups. However, the inflammatory changes were relatively delayed in the adult poly-methyl pentene group. CONCLUSION: Polymethyl pentene (Medos) oxygenators have reduced the host's response phenomenon 'white out' in neonates, and caused a delayed response in adults. This is most likely a consequence of smaller blood contact surface area combined with the effect of heparin coating of the oxygenator membrane. However, recovery was not a function of the type of gas exchange device used.

Successful use of venovenous extracorporeal membrane oxygenation in accidental hypothermic cardiac arrest.

Cardiopulmonary bypass is usually used for rewarming and for providing cardiac support in patients with severe hypothermia and cardiovascular instability. We report the first case of accidental severe hypothermia associated with prolonged cardiac arrest that was successfully managed by venovenous extracorporeal membrane oxygenation.

Performance of polymethyl pentene oxygenators for neonatal extracorporeal membrane oxygenation: a comparison with silicone membrane oxygenators.

OBJECTIVE: To review the performance of polymethyl pentene versus silicone oxygenators in terms of efficiency in priming and oxygenation, oxygenator resistance, requirements for coagulation proteins and consumption of blood products, for neonatal extracorporeal membrane oxygenation (ECMO) patients. STUDY DESIGN: Forty consecutive neonates were selected retrospectively pre- and post-introduction of the new polymethyl pentene (PMP) oxygenators. They formed two equal groups. After calculation of the sample size, data were collected from ELSO registry forms and patient records. Results were analysed using parametric and non-parametric tests. RESULTS: Neonatal PMP (N-PMP) oxygenators were smaller, faster and easier to prime. They were less efficient than silicone oxygenators, especially in carbon dioxide elimination, and, therefore, required higher sweeps. The preservation of coagulation proteins was significantly better, but there was no reduction in the consumption of blood products, despite having less than half the surface area and significantly lower blood path resistance. CONCLUSION: Small PMP oxygenators (Medos Hilite 800 LT) provide adequate gas exchange and offer technical advantages in terms of more efficient priming, reduced haemodynamic resistance and better control and preservation of coagulation proteins than silicone oxygenators.

A potential propensity for failure secondary to clot embolism in neonatal ECMO.

OBJECTIVE: To report a single case of oxygenator failure caused by clot embolism originating from the bladder; and to discuss some preventative options. CASE REPORT: A 2.5 kg neonate with a diagnosis of influenza A received veno-arterial (V-A) extracorporeal membrane oxygenation (ECMO) for cardiorespiratory support. Halfway through treatment, she underwent an elective circuit change for numerous clots in her circuit. The patient continued to consume vast quantities of platelets and developed a fatal oxygenator failure after 18 days. DISCUSSION: Amongst the factors influencing the outcome in events of a sudden unexpected oxygenator failure are the severity of patient illness, the size of the clot relative to the size of the oxygenator, the availability of a previously primed circuit and the ease and speed of priming a new oxygenator. CONCLUSION: There is a need for improvement in the design of small oxygenators and ECMO circuits. Adjustment of the coagulation parameters and lowering the tolerance towards clots in the circuit by electively changing them may reduce the incidence of sudden unexpected oxygenator failure. However, using a slightly larger Medos oxygenator may gain valuable time needed to arrange an oxygenator/circuit change.