Laparoscopic Heller myotomy with Toupet fundoplication: revisiting GERD in treated achalasia.

INTRODUCTION: With the advent of the laparoscopic era in the 1990s, laparoscopic Heller myotomy replaced pneumatic dilation as the first-line treatment for achalasia. An advantage of this approach was the addition of a fundoplication to reduce gastroesophageal reflux disease (GERD). More recently, Peroral Endoscopic Myotomy has competed for first-line therapy, but the postoperative GERD may be a weakness. This study leverages our experience to characterize GERD following LHM with Toupet fundoplication (LHM+T ) so that other treatments can be appropriately compared. METHODS: A single-institution retrospective review of adult patients with achalasia who underwent LHM+T from January 2012 to April 2022 was performed. We obtained routine 6-month postoperative pH studies and patient symptom questionnaires. Differences in questionnaires and reflux symptoms in relation to pH study were explored via Kruskal-Wallis test or chi-square tests. RESULTS: Of 170 patients who underwent LHM+T , 51 (30%) had postoperative pH testing and clinical symptoms evaluation. Eleven (22%) had an abnormal pH study; however, upon manual review, 5 of these (45.5%) demonstrated low-frequency, long-duration reflux events, suggesting poor esophageal clearance of gastric refluxate and 6/11 (54.5%) had typical reflux episodes. Of the cohort, 7 (15.6%) patients reported GERD symptoms. The median [IQR] severity was 1/10 [0, 3] and median [IQR] frequency was 0.5/4 [0, 1]. Patients with abnormal pH reported more GERD symptoms than patients with a normal pH study (3/6, 50% vs 5/39, 12.8%, p = 0.033). Those with a poor esophageal clearance pattern (n = 5) reported no concurrent GERD symptoms. CONCLUSION: The incidence of GERD burden after LHM+T is relatively low; however, the nuances relevant to accurate diagnosis in treated achalasia patients must be considered. Symptom correlation to abnormal pH study is unreliable making objective postoperative testing important. Furthermore, manual review of abnormal pH studies is necessary to distinguish GERD from poor esophageal clearance.

Preoperative esophageal testing predicts postoperative reflux status in sleeve gastrectomy patients.

BACKGROUND: Patients who undergo vertical sleeve gastrectomy (VSG) are at risk of postoperative GERD. The reasons are multifactorial, but half of conversions to Roux-en Y gastric bypass are for intractable GERD. Our institution routinely performs preoperative pH and high-resolution manometry studies to aid in operative decision making. We hypothesize that abnormal pH studies in concert with ineffective esophageal motility would lead to higher rates of postoperative reflux after VSG. METHODS: A single institution retrospective review was conducted of adult patients who underwent preoperative pH and manometry testing and VSG between 2015 and 2021. Patients filled out a symptom questionnaire at the time of testing. Postoperative reflux was defined by patient-reported symptoms at 1-year follow-up. Univariate logistic regression was used to examine the relationship between esophageal tests and postoperative reflux. The Lui method was used to determine the cutpoint for pH and manometric variables maximizing sensitivity and specificity for postoperative reflux. RESULTS: Of 291 patients who underwent VSG, 66 (22.7%) had a named motility disorder and 67 (23%) had an abnormal DeMeester score. Preoperatively, reflux was reported by 122 patients (41.9%), of those, 69 (56.6%) had resolution. Preoperative pH and manometric abnormalities, and BMI reduction did not predict postoperative reflux status (p = ns). In a subgroup analysis of patients with an abnormal preoperative pH study, the Lui cutpoint to predict postoperative reflux was a DeMeester greater than 24.8. Postoperative reflux symptoms rates above and below this point were 41.9% versus 17.1%, respectively (p = 0.03). CONCLUSION: While manometry abnormalities did not predict postoperative reflux symptoms, GERD burden did. Patients with a mildly elevated DeMeester score had a low risk of postoperative reflux compared to patients with a more abnormal DeMeester score. A preoperative pH study may help guide operative decision-making and lead to better counseling of patients of their risk for reflux after VSG.

Type II hiatal hernias: do they exist or are they actually parahiatal hernias?

BACKGROUND: Type II hiatal hernias (HH) are characterized by a portion of the gastric fundus located above the esophageal hiatus adjacent to the esophagus while the gastroesophageal junction (GEJ) remains fixed below the esophageal hiatus. This type of HH has been called the "true" paraesophageal hernia (PEH) because the fundus appears to the side of the esophagus. In our experience, Type II HHs are occasionally identified on radiographic testing, however they are rarely, if ever, confirmed intraoperatively. This led to our question: Does Type II HH exist? METHODS: We searched for evidence of type II HH in three locations: 1. Retrospective review of all first-time PEH repairs (excluding Type I HHs and re-operative cases) performed at the University of Washington Medical Center from 1994 to 2021; 2. Operative videos available on YouTube and WebSurg websites; and 3. Abstracts from the SAGES annual meetings from 2005 to 2021. RESULTS: We found no evidence of Type II HH in any of our three searches. We performed 846 PEH repairs: 760 Type III, 75 Type IV, and 11 parahiatal. Upon website video review, we found only one possible type II hernia, though it too was likely a para-hiatal hernia. No video or case presentations of a type II HH were identified within SAGES annual meeting abstracts. CONCLUSION: Type II HHs do not exist as they are currently defined. Although uncommon, parahiatal hernia can easily be misinterpreted as Type II HH. We should consider changing the hiatal hernia classification system to prevent ongoing clinical confusion.

Successful retrieval of embolised Occlutech atrial septal defect occluder from descending aorta in a 4-year-old child using a "mother and child" technique: a case report and literature review.

A 9 mm Occlutech septal occluder Flex II device was retrieved in a 4-year-old 22 kg child; A 6 Fr Cook® Flexor sheath (child) was inserted into a 9 Fr Occlutech® ASD Delivery Set (mother). Once the tip of the smaller sheath was close to the device, a 4 Fr right Judkins catheter was introduced with a snare. The right atrial hub was captured and withdrawn to the level of the 6 Fr sheath which was then withdrawn into the 9 Fr sheath before being removed completely. The "mother and child" technique offers a greater likelihood of slenderising and retrieving embolised devices.

Successful transition for adolescent survivors of CHD: the Cambridge cardiac transition pathway.

PURPOSE: National standards to ensure effective transition and smooth transfer of adolescents from paediatric to adult services are available but data on successful transition in CHD are limited. The aim of this study is to assess the effectiveness of our transition pathway. METHODS: Adolescents with CHD, aged 15-19 years, who attended the joint cardiac transition clinic between 2009 and 2018 were identified from the Patient Administration Systems. Patient attendance at their first adult CHD service appointment at Royal Papworth Hospital was recorded. RESULTS: 179 adolescents were seen in the joint cardiac transition clinic in the 9-year study period. The median age of the patients when seen was 16 (range 15-19) years. 145 patients were initially planned for transfer to the Royal Papworth Hospital adult CHD service. Three patients were subsequently excluded and the success of the transfer of care in 142 patients were analysed. 112 (78%) attended their first follow-up in the adult CHD clinic as planned, 28 (20%) attended after reminders were sent out with 5/28 requiring multiple reminders, and only 2 (1.4%) failed to attend. Overall, transfer of care was achieved in 140 (98.6%) patients. CONCLUSION: A dedicated joint cardiac transition clinic involving multi-professional medical and nursing teams from paediatric and adult cardiology services appears to achieve high engagement rates with the adult services. This approach allows a 'face' to be put on a named clinician delivering the adult service and should be encouraged.

Fragmented health systems in COVID-19: rectifying the misalignment between global health security and universal health coverage.

The COVID-19 pandemic has placed enormous strain on countries around the world, exposing long-standing gaps in public health and exacerbating chronic inequities. Although research and analyses have attempted to draw important lessons on how to strengthen pandemic preparedness and response, few have examined the effect that fragmented governance for health has had on effectively mitigating the crisis. By assessing the ability of health systems to manage COVID-19 from the perspective of two key approaches to global health policy-global health security and universal health coverage-important lessons can be drawn for how to align varied priorities and objectives in strengthening health systems. This Health Policy paper compares three types of health systems (ie, with stronger investments in global health security, stronger investments in universal health coverage, and integrated investments in global health security and universal health coverage) in their response to the ongoing COVID-19 pandemic and synthesises four essential recommendations (ie, integration, financing, resilience, and equity) to reimagine governance, policies, and investments for better health towards a more sustainable future.

Characteristics and outcomes of patients undergoing paraesophageal hernia repair with selective use of biologic mesh.

BACKGROUND: The use of biologic mesh in paraesophageal hernia repair (PEHR) has been associated with decreased short-term recurrence but no statistically significant difference in long-term recurrence. Because of this, we transitioned from routine to selective use of mesh for PEHR. The aim of this study was to examine our indications for selective mesh use and to evaluate patient outcomes in this population. METHODS: We queried a prospectively maintained database for patients who underwent laparoscopic PEHR with biologic mesh from October 2015 to October 2018, then performed a retrospective chart review. The decision to use mesh was made intraoperatively by the surgeon. Recurrence was defined as the presence of > 2 cm intrathoracic stomach on postoperative upper gastrointestinal (UGI) series. RESULTS: Mesh was used in 61/169 (36%) of first-time PEHRs, and in 47/82 (57%) of redo PEHRs. Among first-time PEHRs, the indications for mesh included hiatal tension (85%), poor crural tissue quality (11%), or both (5%). Radiographic recurrence occurred in 15% of first-time patients (symptomatic N = 2, asymptomatic N = 3). There were no reoperations for recurrence. Among redo PEHRs, the indication for mesh was most commonly the redo nature of the repair itself (55%), but also hiatal tension (51%), poor crural tissue quality (13%), or both (4%). Radiographic recurrence occurred in 21% of patients (symptomatic N = 4, asymptomatic N = 1). There was 1 reoperation for recurrence in the redo-repair group. CONCLUSIONS: We selectively use biologic mesh in a third of our first-time repair patients and in over half of our redo-repair patients when there is a perceived high risk of recurrence based on hiatal tension, poor tissue quality, or prior recurrence. Despite the high risk for radiologic recurrence, there was only 1 reoperation for recurrence in the entire cohort.

Managing CHD in Tertiary NICU in Collaboration with a Cardiothoracic Center.

Increasingly non-cardiac tertiary neonatal intensive care units (NCTNs) manage newborns with CHD prior to planned transfer to specialist cardiac surgical centres (SCSC). It improves patient flow in SCSCs, enables families to be nearer home, and improves psychological well-being Parker et al. (Evaluating models of care closer to home for children and young people who are ill: a systematic review, 2011). This practice has gradually increased as the number of SCSCs has decreased. This study examines the effectiveness of this expanding practice. The management provided, length of stay in the NCTN and outcomes are described for one UK NCTN situated at a significant distance from its SCSC. A retrospective observational study of cardiac-related admissions to a NCTN between January 2010 and December 2019 was conducted. 190 neonates were identified: 41 had critical CHD; 64 had major CHD. The cohort includes babies with a wide range of cardiac conditions and additional complexities. 23.7% (n = 45) required transfer to a specialist center after a period of stabilization and growth ranging from several hours to 132 days. 68% (n = 130) were discharged home or repatriated to a local NICU. Of the remaining 15 babies, 13 were transferred to other specialties including the hospice. Two died on NICU. The mortality was consistent with the medical complexity of the group Best and Rankin (J Am Heart Assoc 5:e002846, 2016), Laas et al. (BMC Pediatr 17:124, 2017). 8.9% (n = 17) died before age 2. Nine babies had care redirected due to an inoperable cardiac condition or life-limiting comorbidities. Our study demonstrates a complex neonatal cohort with CHD can be managed effectively in a NCTN, supporting the current model of care. The NCTN studied was well supported by pediatricians with expertise in cardiology alongside visiting pediatric cardiologists.

DNAAF1 links heart laterality with the AAA+ ATPase RUVBL1 and ciliary intraflagellar transport.

DNAAF1 (LRRC50) is a cytoplasmic protein required for dynein heavy chain assembly and cilia motility, and DNAAF1 mutations cause primary ciliary dyskinesia (PCD; MIM 613193). We describe four families with DNAAF1 mutations and complex congenital heart disease (CHD). In three families, all affected individuals have typical PCD phenotypes. However, an additional family demonstrates isolated CHD (heterotaxy) in two affected siblings, but no clinical evidence of PCD. We identified a homozygous DNAAF1 missense mutation, p.Leu191Phe, as causative for heterotaxy in this family. Genetic complementation in dnaaf1-null zebrafish embryos demonstrated the rescue of normal heart looping with wild-type human DNAAF1, but not the p.Leu191Phe variant, supporting the conserved pathogenicity of this DNAAF1 missense mutation. This observation points to a phenotypic continuum between CHD and PCD, providing new insights into the pathogenesis of isolated CHD. In further investigations of the function of DNAAF1 in dynein arm assembly, we identified interactions with members of a putative dynein arm assembly complex. These include the ciliary intraflagellar transport protein IFT88 and the AAA+ (ATPases Associated with various cellular Activities) family proteins RUVBL1 (Pontin) and RUVBL2 (Reptin). Co-localization studies support these findings, with the loss of RUVBL1 perturbing the co-localization of DNAAF1 with IFT88. We show that RUVBL1 orthologues have an asymmetric left-sided distribution at both the mouse embryonic node and the Kupffer's vesicle in zebrafish embryos, with the latter asymmetry dependent on DNAAF1. These results suggest that DNAAF1-RUVBL1 biochemical and genetic interactions have a novel functional role in symmetry breaking and cardiac development.

Building the case for embedding global health security into universal health coverage: a proposal for a unified health system that includes public health.

In the wake of the recent west African Ebola epidemic, there is global consensus on the need for strong health systems; however, agreement is less apparent on effective mechanisms for establishing and maintaining these systems, particularly in resource-constrained settings and in the presence of multiple and sustained stresses (eg, conflict, famine, climate change, and globalisation). The construction of the International Health Regulations (2005) guidelines and the WHO health systems framework, has resulted in the separation of public health functions and health-care services, which are interdependent in actuality and must be integrated to ensure a continuous, unbroken national health system. By analysing efforts to strengthen health systems towards attaining universal health coverage and investments to improve global health security, we examine areas of overlap and offer recommendations for construction of a unified national health system that includes public health. One way towards achieving universal health coverage is to broaden the definition of a health system.

Politics and Universal Health Coverage--The Post-2015 Global Health Agenda.

What political, social, and economic factors allow a movement toward universal health coverage to take hold in some low- and middle-income countries? Can we use that knowledge to help other such countries achieve health care for all?

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.

Accurate diagnosis of CHD by Paediatricians with Expertise in Cardiology.

Introduction Paediatricians with Expertise in Cardiology assess children with a full history, examination, and often perform an echocardiogram. A minority are then referred to an outreach clinic run jointly with a visiting paediatric cardiologist. The accuracy of the echocardiography diagnosis made by the Paediatrician with Expertise in Cardiology is unknown. Materials and methods We conducted a retrospective review of clinic letters for children seen in the outreach clinic for the first time between March, 2004 and March, 2011. Children with CHD diagnosed antenatally or elsewhere were excluded. We recorded the echocardiography diagnosis made by the paediatric cardiologist and previously by the Paediatrician with Expertise in Cardiology. RESULTS: The Paediatrician with Expertise in Cardiology referred 317/3145 (10%) children seen in the local cardiac clinics to the outreach clinic over this period, and among them 296 were eligible for inclusion. Their median age was 1.5 years (range 1 month-15.1 years). For 244 (82%) children, there was complete diagnostic agreement between the Paediatrician with Expertise in Cardiology and the paediatric cardiologist. For 29 (10%) children, the main diagnosis was identical with additional findings made by the paediatric cardiologist. The abnormality had resolved in 17 (6%) cases by the time of clinic attendance. In six (2%) patients, the paediatric cardiologist made a different diagnosis. In total, 138 (47%) patients underwent a surgical or catheter intervention. Discussion Paediatricians with Expertise in Cardiology can make accurate diagnoses of CHD in children referred to their clinics. This can allow effective triage of children attending the outreach clinic, making best use of limited specialist resources.

The use of crural relaxing incisions with biologic mesh reinforcement during laparoscopic repair of complex hiatal hernias.

INTRODUCTION: Laparoscopic hiatal hernia repair has a better chance of success if the hiatus is closed without tension. This study attempts to answer the following questions: (1) What is the rate of hiatal hernia recurrence in patients who undergo hiatal closure with diaphragmatic relaxing incisions? (2) Can biologic mesh be safely substituted for synthetic mesh as coverage of the relaxing incisions? METHODS: We identified all patients who underwent laparoscopic hiatal hernia repair at our institution between 2007 and 2013 and reviewed their clinical records. Radiologic recurrence was identified by an experienced radiologist and defined as the presence of any abdominal contents located above the diaphragm on esophagram. Clinical recurrence was defined as little or no improvement in symptoms, the development of a new symptom, or the need for medical, endoscopic, or surgical treatment of postoperative symptoms. RESULTS: A minimum of 6 months of radiologic and clinical follow-up was available for 146 (40 %) patients, including 16 with relaxing incisions. There were 66 (45 %) recurrent hernias detected on esophagram. There was no difference in the rate of recurrent hiatal hernia among the three groups: Primary closure of the hiatus (21/36 [58 %]), primary closure with biologic mesh reinforcement (36/94 [38 %]), and relaxing incision with biologic mesh reinforcement (9/16 [56 %]; p = 0.428). Two reoperations were performed on patients who underwent left relaxing incisions and developed symptomatic diaphragmatic hernias through the left relaxing incisions. There were no complications associated with use of biologic mesh at the hiatus. CONCLUSIONS: Rate of recurrent hiatal hernia is similar between patients who undergo diaphragmatic relaxing incisions and patients who undergo primary hiatal closure. Relaxing incisions can be safely performed on either crus; however, biologic mesh should not be used to patch a left-sided relaxing incision due to the risk of developing a diaphragmatic hernia.

The comparative role of echocardiography and MRI for identifying critical lesions in patients with single-ventricle physiology, before bidirectional cavopulmonary connection.

BACKGROUND: In the era of multi-modality imaging, this study compared contemporary, pre-operative echocardiography and cardiac MRI in predicting the need for intervention on additional lesions before surgical bidirectional cavopulmonary connection. METHODS: A total of 72 patients undergoing bidirectional cavopulmonary connection for single-ventricle palliation between 2007 and 2012, who underwent pre-operative assessment using both echocardiography and MRI, were included. The pre-determined outcome measure was any additional surgical or catheter-based intervention within 6 months of bidirectional cavopulmonary connection. Indices assessed were as follows: indexed dimensions of right and left pulmonary arteries, coarctation of the aorta, adequacy of interatrial communication, and degree of atrioventricular valve regurgitation. RESULTS: Median age at bidirectional cavopulmonary connection was 160 days (interquartile range 121-284). The following MRI parameters predicted intervention: Z score for right pulmonary artery (odds ratio 1.77 (95% confidence interval 1.12-2.79, p=0.014)) and left pulmonary artery dimensions (odds ratio 1.45 (1.04-2.00, p=0.027)) and left pulmonary artery report conclusion (odds ratio 1.57 (1.06-2.33)). The magnetic resonance report predicted aortic arch intervention (odds ratio 11.5 (3.5-37.7, p=0.00006)). The need for atrioventricular valve repair was associated only with magnetic resonance regurgitation fraction score (odds ratio 22.4 (1.7-295.1, p=0.018)). Echocardiography assessment was superior to MRI for predicting intervention on interatrial septum (odds ratio 27.7 (6.3-121.6, p=0.00001)). CONCLUSION: For branch pulmonary arteries, aortic arch, and atrioventricular valve regurgitation, MRI parameters more reliably predict the need for intervention; however, echocardiography more accurately identified the adequacy of interatrial communication. Approaching bidirectional cavopulmonary connection, the diagnostic strengths of MRI and echocardiography should be acknowledged when considering intervention.

Zellweger syndrome and secondary mitochondrial myopathy.

Defects in peroxisomes such as those associated with Zellweger syndrome (ZS) can influence diverse intracellular metabolic pathways, including mitochondrial functioning. We report on an 8-month-old female infant and a 6-month-old female infant with typical clinical, radiological and laboratory features of Zellweger syndrome; light microscopic and ultrastructural evidence of mitochondrial pathology in their muscle biopsies; and homozygous pathogenic mutations of the PEX16 gene (c.460 + 5G > A) and the PEX 12 gene (c.888_889 del p.Leu297Thrfs*12), respectively. Additionally, mitochondrial respiratory chain enzymology analysis in the first girl showed a mildly low activity in complexes II-III and IV. We also review five children previously reported in the literature with a presumptive diagnosis of ZS and additional mitochondrial findings in their muscle biopsies. In conclusion, this is the first study of patients with a molecularly confirmed peroxisomal disorder with features of a concomitant mitochondrial myopathy and underscores the role of secondary mitochondrial dysfunction in Zellweger syndrome, potentially contributing to the clinical phenotype.

Radar observations of individual rain drops in the free atmosphere.

Atmospheric remote sensing has played a pivotal role in the increasingly sophisticated representation of clouds in the numerical models used to assess global and regional climate change. This has been accomplished because the underlying bulk cloud properties can be derived from a statistical analysis of the returned microwave signals scattered by a diverse ensemble comprised of numerous cloud hydrometeors. A new Doppler radar, previously used to track small debris particles shed from the NASA space shuttle during launch, is shown to also have the capacity to detect individual cloud hydrometeors in the free atmosphere. Similar to the traces left behind on film by subatomic particles, larger cloud particles were observed to leave a well-defined radar signature (or streak), which could be analyzed to infer the underlying particle properties. We examine the unique radar and environmental conditions leading to the formation of the radar streaks and develop a theoretical framework which reveals the regulating role of the background radar reflectivity on their observed characteristics. This main expectation from theory is examined through an analysis of the drop properties inferred from radar and in situ aircraft measurements obtained in two contrasting regions of an observed multicellular storm system. The observations are placed in context of the parent storm circulation through the use of the radar's unique high-resolution waveforms, which allow the bulk and individual hydrometeor properties to be inferred at the same time.