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The Fontan operation is the final stage of single-ventricle palliation that effectively separates the pulmonary and systemic circulations. The procedure connects the inferior vena cava directly to the pulmonary arteries or to the previously created superior cavopulmonary anastomosis, which allows all of the deoxygenated systemic venous return to flow directly to the lungs. The single ventricle then pumps oxygenated pulmonary venous blood to the systemic circulation. Baseline higher central venous pressure drives the Fontan circulation. There is an increased incidence of severe spinal deformities in children with congenital heart disease that will require corrective surgery, including posterior spinal fusion. Anesthesia for posterior spinal fusion in Fontan patients presents significant challenges, especially as the patient is in the prone position, which further exacerbates hemodynamic instability. This chapter discusses the perioperative management of a Fontan patient for posterior spinal fusion.
To evaluate the circumstances in which recurrent laryngeal nerve palsy occurs after thyroid surgery.
This study assessed 1026 patients who underwent surgery for benign thyroid disease over a seven-year period in a retrospective, single-centre study.
With a total of 1835 recurrent laryngeal nerves at risk, there were 38 cases (2.07 per cent) of transient recurrent laryngeal nerve palsy and 8 (0.44 per cent) of permanent recurrent laryngeal nerve palsy. No explanation was found for 10 of the 46 cases of recurrent laryngeal nerve palsy. Among the 38 other cases, the probable causes included poor identification of the recurrent laryngeal nerve during surgery, involuntary resection of the nerve and several other factors.
Apart from accidental resection of the recurrent laryngeal nerve during thyroid surgery, the causes of post-operative recurrent laryngeal nerve palsy are often unclear and likely multifactorial. Poor identification of the recurrent laryngeal nerve during surgery is still the main cause of post-operative recurrent laryngeal nerve palsy, even when intra-operative neuromonitoring is used.
Myasthenia gravis (MG) is an autoimmune disorder that frequently affects young women of reproductive age. The multidirectional interplay between MG, pregnancy, and fetal health poses a complex scenario for pregnant women with MG and the healthcare team. Here, we reviewed our local experience with MG, pregnancy, and outcomes.
We performed a retrospective chart review of patients with MG attending the Prosserman Family Neuromuscular Clinic from 2001 to 2019 and who were referred to a high-risk pregnancy clinic. MG status was defined as stable, better, or worse. Information was collected on the delivery route, pregnancy, and neonatal complications.
We identified 20 women with MG for a total of 28 pregnancies. Worsening was observed in 50% of pregnancies: 18% during pregnancy, 25% following delivery, and 7% during both. 66.7% of patients with MG duration of 2 years or less had worsening during pregnancy. Three patients who stopped immunosuppressive treatment during pregnancy worsened and one had a crisis. C-section was done in 29% of pregnancies. The rate of delivery complications was 7% and of neonatal MG was 7%.
A high proportion of MG patients worsened during pregnancy, particularly those with disease duration less than 2 years, and those who discontinued immunosuppression during pregnancy. However, pregnancy was largely unaffected, rate of neonatal MG was low, frequencies of C-section, delivery complications, and premature births were similar to the general population. While the study has limitations due to the retrospective nature, these insights provide some guidance when counseling young myasthenic women about family planning.
A Fontan circulation requires a series of three-staged operations aimed to palliate patients with single-ventricle CHD. Currently, the most frequent technique is the extracardiac total cavopulmonary connection, an external conduit connecting the IVC and right pulmonary artery, bypassing the right side of the heart. Fontan candidates must meet strict criteria; they are assessed utilising both cardiac catheterisation and cardiac magnetic resonance. Postoperatively, treatment protocols prioritise antibiotic prophylaxis, diuretics, angiotensin-converting enzyme inhibitors, anticoagulation, and oxygen therapy with fluid restriction and a low-fat diet. These measures aim to reduce length of stay in the ICU and hospital by preventing acute complications such as infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury. Late complications of a Fontan procedure include circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease. The definitive management is cardiac transplantation, with promising innovations in selective embolisation of lymphatic vessels and Fontan-specific ventricular assist devices. Further research assessing current protocols in the perioperative management of Fontan patients would be beneficial for standardising current practice and improving outcomes.
Percutaneous closure of atrial septal defect is recognised as a safe and effective procedure, however, in some patients complications may occur. Although chest pain has been sporadically reported, its exact aetiology has been poorly studied. Herein, a 14-year-old female with an atypical and long-lasting chest pain after percutaneous atrial septal defect closure is described.
Although the term ‘tracheostomy’ simply means a hole entering the trachea, it can be usefully divided into tracheostomy (in which a hole is made between the anterior neck and the trachea, which remains otherwise intact) and laryngectomy (in which the larynx is removed and the trachea is joined to the neck as a blind-ending stoma). Both groups of patients have an increased risk of complications outside and within hospitals. Many of these are entirely avoidable by better knowledge and reliability of care. There is confusion about different types of tracheostomy and between management of complications of tracheostomy and laryngectomy stoma. There is a need for clear understanding of the differences and what represents good care of such patients and their airways. Complications include airway blockage and displacement. Harm may occur if airway emergencies are not managed promptly and in a structured manner. Standard operating procedures, algorithms and checklists have a role in improving reliability of care and approaches to emergencies. Recent evidence shows multidisciplinary teams using quality improvement principles can reduce complications, hasten decannulation and improve patient experience. The National Tracheostomy Safety Project and the Global Tracheostomy Collaborative have been established to improve education and safety of tracheostomy care.
Despite the training and skills of airway managers, airway management complications still occur and may cause patient harm or death. The causes are multifactorial and may include patient, environment and clinician factors. Airway complications likely contribute to a significant proportion of deaths due to anaesthesia and are certainly more common outside the operating theatre and especially in the critical care unit. Reported incidences of failure and harm during airway management vary depending on the population studied and definitions used. Numbers may be of less value than understanding themes that help us improve care and reduce harm. The chapter emphasises that conventional research (e.g. device evaluation studies and randomised controlled trials) may be of little use in identifying low frequency events and complications because of their restricted inclusion and exclusion criteria, the use of devices only by experts and in conventional settings and because of their focus on efficacy rather than safety. The chapter highlights the important and growing role of registries and databases. Several are described in detail including the 4th National Audit Project and the Dutch ‘mini-NAP’. The value and limitations of litigation databases are explored. Specific complications of note are described at the end of the chapter.
Cardiac strangulation is a rare and potentially deadly complication of epicardial pacemaker implantation. A young boy presenting with chest pain and tiredness almost 7 years after pacemaker implantation was found to have cardiac strangulation. Literature review revealed 22 cases reported to date with a worrying rise in the number of reports over the past 3 years. Strangulation is associated with implantation of leads at a young age and appears to be related to somatic growth. Serial assessment with chest X-ray and echocardiogram is recommended, at least until full adult growth is attained with further coronary artery imaging reserved for symptoms or suspicious echocardiographic findings. If cardiac strangulation is diagnosed prompt replacement of the offending system is needed.
The perioperative complications rate in paediatric cardiac surgery, as well as the failure-to-rescue impact, is less known in low- and middle-income countries.
To evaluate perioperative complications rate, mortality related to complications, different patients’ demographics, and procedural risk factors for perioperative complication and post-operative death.
Risk factors for perioperative complications and operative mortality were assessed in a retrospective single-centre study which included 296 consecutive children undergoing cardiac surgery.
Overall mortality was 5.7%. Seventy-three patients (24.7%) developed 145 perioperative complications and had 17 operative mortalities (23.3%). There was a strong association between the number of perioperative complications and mortality – 8.1% among patients with only 1 perioperative complication, 35.3% – with 2 perioperative complications, and 42.1% – with 3 or more perioperative complications (p = 0.007). Risk factors of perioperative complications were younger age (odds ratio 0.76; (95% confidence interval 0.61, 0.93), previous cardiac surgery (odds ratio 3.5; confidence interval 1.33, 9.20), extracardiac structural anomalies (odds ratio 3.03; confidence interval 1.27, 7.26), concomitant diseases (odds ratio 3.23; confidence interval 1.34, 7.72), and cardiopulmonary bypass (odds ratio 6.33; confidence interval 2.45, 16.4), whereas the total number of perioperative complications per patient was the only predictor of operative death (odds ratio 1.89; confidence interval 1.06, 3.37).
In a program with limited systemic resources, failure-to-rescue is a major contributor to operative mortality in paediatric cardiac surgery. Despite the comparable crude mortality, the operative mortality among patients with perioperative complications in our series was significantly higher than in the developed world. A number of initiatives are needed in order to improve failure-to-rescue rates in low- and middle-income countries.
The major complication of end jejunostomy is excessive fluid and electrolyte loss through the stoma, leading to hypovolaemia and dyselectrolytaemia within days and malnutrition within weeks. The aim was to compare the results of two nutritional approaches: unrestricted and restricted oral intake in patients with end jejunostomy commencing home parenteral nutrition (HPN) in terms of liver and renal biochemical markers and time to reconstructive bowel surgery with correlation to stoma output. Twenty patients with stabilised high output end-jejunostomy were divided into two groups. Group A consisted of ten patients with oral intake restricted to keep stomal output under 1000 ml. Group B consisted of ten patients with unrestricted oral intake. The following parameters were evaluated over 6 months: stomal output, self-estimation of general condition, body weight gain, plasma bilirubin and creatinine, number of hospitalisations prior to reconstructive surgery, the frequency of ostomy bag emptying, feelings of hunger and thirst in the daytime, and the time to reconstructive surgery. Stoma losses were compensated by parenteral supply. In group B, lower quality of life was observed, reflected by weakness, permanent feelings of hunger and thirst and the need for night-time emptying of the stoma bag. Patients in group B developed more complications and required more time to prepare for surgery. One death occurred in group B due to renal insufficiency followed by septic complications. Restricted oral intake seems to be more effective for prevention of HPN-related complications and shortening of time to surgery. Unrestricted oral intake appears to provoke uncontrolled losses of energy and protein, inhibiting weight gain.
Inflammatory bowel disease (IBD) typically involves the large bowel, small bowel (ileum / jejunum), or both. Involvement of the upper GI tract is less common, although its frequency is uncertain because common causes of inflammation such as gastro-oesophageal reflux and Helicobacter pylori infection also require exclusion. In the setting of known IBD, upper GI inflammation generally favours Crohn’s disease over ulcerative colitis (UC), while granulomas strongly suggest involvement by IBD and indicate Crohn’s disease rather than UC. New upper GI inflammation may raise the possibility of IBD, while unexplained new upper GI granulomas require exclusion of Crohn’s disease. Unfortunately, few histological patterns apart from granulomas are specific or discriminant. Lymphocytic oesophagitis is a poorly defined entity associated with IBD and/or Crohn’s disease in some studies. Focally enhanced gastritis (FEG), though initially regarded as typical of Crohn’s disease, probably has limited value apart from perhaps predicting IBD in children. Rarely, UC patients develop a duodenitis resembling the colorectal changes histologically. UC may also be associated, infrequently, with characteristic patterns of gastritis. Compared with adults, children with IBD are more likely to have upper GI involvement, to develop GI granulomas, and to undergo investigation for upper GI disease. Overall, upper GI granulomas assist with the diagnosis and classification of IBD but few other upper GI features are discriminatory between IBD and other causes or between UC and Crohn’s disease.
Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.
Methods and results:
Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.
A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.
Selective Serotonin Reuptake Inhibitors (SSRIs) have been accused of causing bleeding problems as a side effect. Theories about the mechanism are still being discussed. We report a case, presenting bleeding problems, during sertraline treatment. The SSRIs are widely used to treat depression and many other psychiatric disorders. Their lower severity of side effects and being markedly safer in overdose are some of the reasons of their preference as primary choice in most of the cases. Besides their common side effects like, agitation, headache, insomnia, weight gain or loss, and sexual dysfunction, SSRIs also have been suspected of increasing the risk of bleeding. A population-based cohort study supported the hypothesis of an increased risk of upper gastrointestinal bleeding during the use of SSRIs, and they also indicated that this effect is potentiated with concurrent use of NSAIDs or low-dose aspirin. We would like to report our recent experience with one patient who was on sertraline, 50mg/day.
We analysed the impact of the TaqI A1 allele of the D2 dopamine receptor gene on the risk for alcoholism, trying to depict three explanations frequently proposed to explain discrepancies in association and linkage studies: that the A1 allele may act as a marker rather than as a vulnerability factor, that stratification biases and unevaluated controls may explain positive results, and that the A1 allele is modifying the phenotype rather than increasing the risk for alcoholism. We thus tested another (dinucleotide STRP) marker within the DRD2 gene, selected a new homogenous sample of 113 alcoholic patients and 49 unaffected controls strictly matched for ethnic origins, and systematically assessed both samples with a semi-structured interview to detect (in both samples) alcohol dependence, but also such related traits as specificities of complications.
The frequency of the A1 allele was not significantly different between alcoholics and controls but when comparing different subgroups of alcoholics, the A1 allele was significantly more frequent in alcoholic patients with somatic complications (OR = 3.00, CI[1.37-6.62]), social and professional complications (OR = 2.72, CI[1.25-5.90]), or with co-morbid dependence (OR = 2.88, 95% IC [1.16-7.15]). The association for co-morbid dependence and somatic complications was also positive when taking into consideration both STRP and TaqIA polymorphisms.
The A1 allele does not increase the risk for alcoholism per se in our sample, but may be involved in a related trait which is partially dependent on the diagnosis of alcoholism, through a disequilibrium with another close mutation.
Stenting of ostial pulmonary artery stenosis presents several unique challenges. These include difficulty in defining anatomy and need for precise stent placement in order to avoid missing the ostial stenosis or jailing either the contralateral branch pulmonary artery or the ipsilateral upper lobe branch.
A retrospective review of outcomes was conducted in 1.5 or 2-ventricle patients who underwent stent placement for ostial branch pulmonary artery stenosis. Specific catheterisation lab techniques were reviewed.
Forty-seven branch pulmonary arteries underwent stent placement for ostial stenosis in 43 patients. The median age and weight were 3.7 (0.3–18.1) years and 14.2 (5.6–70.0) kg, respectively. Three (2–8) angiographic projections were needed to profile the ostial stenosis. Open-cell stents were used in 23 and stents were modified in 5 cases. Following stent implantation, the minimum diameter improved from 3.6 (0.8–10.5) to 8.1 (4.2–16.5) mm (p < 0.001). The gradient improved from 21 (0–66) to 4 (0–27) mmHg (p < 0.001). Stent malposition occurred in eight (17%) of the stents placed. Five migrated distally causing suboptimal ostial coverage necessitating placement of a second stent in four. Three migrated proximally and partially jailed the contralateral pulmonary artery. Intentional jailing of the upper lobe branch occurred in four additional cases. At a follow-up of 2.4 (0.3–4.9) years, 15 stents underwent further dilation and 1 had a second stent placed within the exiting stent.
Ostial branch pulmonary artery stenosis may require additional angiography to accurately define the ostial stenosis. Treatment with stents is effective but carries high rates of stent malposition.
There has been great concern with the use of radiofrequency ablation in infants since radiofrequency ablation lesions were shown to have a progressing nature in immature myocardium of animals. In this report, we present a 2-month-old infant with life-threatening medically refractory supraventricular tachycardia. Radiofrequency ablation successfully cured arrhythmia; however, late effects of radiofrequency ablation lesions resulted in a progressive mitral valve perforation requiring surgical repair.
Atrial septal defect is the third most common CHD. A hemodynamically significant atrial septal defect causes volume overload of the right side of the heart. Preterm children may suffer from both pulmonary and cardiac comorbidities, including altered myocardial function. The aim of this study was to compare the rate of adverse events following atrial septal defect closure in preterm- and term-born children.
We performed a retrospective cohort study including children born in Sweden, who had a surgical or percutaneous atrial septal defect closure at the children’s hospitals in Lund and Stockholm, between 2000 and 2014, assessing time to the first event within 1 month or 1 year. We analysed differences in the number of and the time to events between the preterm and term cohort using the Kaplan–Meier survival curve, a generalised model applying zero-inflated Poisson distribution and Gary-Anderson’s method.
Overall, 413 children were included in the study. Of these, 93 (22.5%) were born prematurely. The total number of adverse events was 178 (110 minor and 68 major). There was no difference between the cohorts in the number of events, whether within 1 month or within a year, between major (p = 0.69) and minor (p = 0.84) events or frequencies of multiple events (p = 0.92).
Despite earlier procedural age, larger atrial septal defects, and higher comorbidity than term children, preterm children appear to have comparable risk for complications during the first year after surgical or percutaneous closure.
Introduction: Complications in early pregnancy are common and have many physical and emotional consequences. Locally, there is no early pregnancy loss clinic or standardized guide in the emergency department (ED) for referral and follow-up decisions, and both initial management of patients and follow up can be inconsistent. This study aimed to obtain consensus on the best approach to initial work-up, management, and follow up for patients who present to the ED with early pregnancy complications, with the goal of using this consensus to produce a standardized guide for emergency provider use. Methods: A literature review was completed to produce evidence-based recommendations which were used to initiate a modified Delphi consensus process. A survey was distributed, with three rounds completed. Participants included emergency providers, obstetrician-gynecologists, a radiologist, a sample of family medicine physicians including some involved in primary care obstetrics, and nurse practitioners. An obstetric specialist from outside the local region was also involved. Results: Consensus was reached on several key recommendations, however some areas remained without clear accepted best practice. There was consensus that physical components of early pregnancy complications are addressed well, but that we could improve on patient flow and more consistent follow up. Important investigations to be done for patients were identified. The timing of formal ultrasound, necessity and timing of obstetrician consultation, and safety of discharge was addressed for various patient scenarios including stable and unstable patients, with and without adnexal pain, with intrauterine pregnancy of uncertain viability, and with pregnancy of unknown location. Management of confirmed early pregnancy loss in the ED and family medicine clinics was addressed. Barriers to an early pregnancy loss clinic included lack of funding, space, and staffing as well as lack of resources and uncertain patient volumes. A feasible alternative to an early pregnancy loss clinic was for willing providers to keep appointment times available to facilitate confirmation of follow-up prior to discharge. Other suggested alternatives included an early pregnancy loss clinic, a nurse educator, and having a standardized guideline in the ED. Conclusion: Through a consensus approach, several recommendations were agreed upon for improving care for patients presenting to the ED with early pregnancy complications.
Patients with Parkinson’s disease psychosis (PDP) are often treated with an atypical antipsychotic, especially quetiapine or clozapine, but side effects, lack of sufficient efficacy, or both may motivate a switch to pimavanserin, the first medication approved for management of PDP. How best to implement a switch to pimavanserin has not been clear, as there are no controlled trials or case series in the literature to provide guidance. An abrupt switch may interrupt partially effective treatment or potentially trigger rebound effects from antipsychotic withdrawal, whereas cross-taper involves potential drug interactions. A panel of experts drew from published data, their experience treating PDP, lessons from switching antipsychotic drugs in other populations, and the pharmacology of the relevant drugs, to establish consensus recommendations. The panel concluded that patients with PDP can be safely and effectively switched from atypical antipsychotics used off label in PDP to the recently approved pimavanserin by considering each agent’s pharmacokinetics and pharmacodynamics, receptor interactions, and the clinical reason for switching (efficacy or adverse events). Final recommendations are that such a switch should aim to maintain adequate 5-HT2A antagonism during the switch, thus providing a stable transition so that efficacy is maintained. Specifically, the consensus recommendation is to add pimavanserin at the full recommended daily dose (34 mg) for 2–6 weeks in most patients before beginning to taper and discontinue quetiapine or clozapine over several days to weeks. Further details are provided for this recommendation, as well as for special clinical circumstances where switching may need to proceed more rapidly.