Rheumatic Heart Disease: JACC Focus Seminar 2/4.

It is a sad reality that although eminently preventable, and despite possessing such knowledge for >70 years, rheumatic heart disease (RHD) remains the most common cause of cardiovascular morbidity and early mortality in young people worldwide. A disease of the poor, RHD is one of the most neglected diseases. Several challenges are unique to the acute rheumatic fever/RHD continuum and contribute to its persistence, including its sequestration among the poorest, its protracted natural history, the erratic availability of penicillin, and the lack of a concerted effort in endemic regions. However, there is cause for optimism following a resurgence in scientific interest over the last 15 years. This review presents the latest advancements in epidemiology, diagnosis, and management. It also discusses pressing research questions on disease pathophysiology, the barriers to implementation of effective management strategies, and pragmatic policy solutions required for translation of current knowledge into meaningful action.

Novel handheld ultrasound technology to enhance non-expert screening for rheumatic heart disease in the Republic of Palau: A descriptive study.

AIM: Non-expert training in rheumatic heart disease (RHD) detection is a valuable strategy in resource-limited settings. Here we present an innovative handheld ultrasound application featuring views of correct probe position, imaging protocol and echocardiographic loops of RHD pathology versus normal, accessible during real-time scanning. METHODS: This prospective study was implemented into a pre-existing school health screening programme at an elementary school in Koror, Palau. Six learners with no prior ultrasound experience were taught a simplified screening protocol in which a mitral regurgitation jet ≥1.5 cm and/or presence of aortic insufficiency were considered a positive screen. All consented children underwent echocardiographic screening by experts with standard portable echocardiography. All positive cases and 25% of negative cases were referred for handheld scans by learners. RESULTS: A total of 26 (4.1%) children were diagnosed with borderline or definite RHD. Mean sensitivity and specificity compared to expert RHD diagnosis over all learners was 71% (standard deviation (SD) 11.3) and 92% (SD 4.9), respectively. For the three highest scanning learners, mean sensitivity was 88% (SD 4.9) and mean specificity was 90% (SD 5.7). For all definite RHD cases, sensitivity was 100% for all but one learner, who achieved sensitivity of 60%. The novel application was used in 229 of 624 (36%) of all handheld scans and 50 of 112 (45%) of expert-diagnosed positive scans, with protocol and reference features most frequently used. Utilisation of the novel application overall decreased per day of scanning per learner. CONCLUSION: Adjunctive handheld ultrasound technology may help ease non-experts into RHD screening.

Prevention and control of rheumatic heart disease: Overcoming core challenges in resource-poor environments.

Rheumatic heart disease (RHD) has long receded as a significant threat to public health in high-income countries. In low-resource settings, however, the specter of RHD remains unabated, as exemplified by recent data from the Global Burden of Diseases Study. There are many complex reasons for this ongoing global disparity, including inadequate data on disease burden, challenges in effective advocacy, ongoing poverty and inequality, and weak health systems, most of which predominantly affect developing nations. In this review, we discuss how each of these acts as a core challenge in RHD prevention and control. We then examine key lessons learnt from successful control programs in the past and highlight resources that have been developed to help create strong national RHD control programs.

Keep an eye out: A rare case of acute-onset Charles Bonnet Syndrome after Stanford type A aortic dissection repair surgery.

Charles Bonnet Syndrome describes the triad of complex visual hallucinations secondary to ophthalmic pathology in psychologically normal people. We present a rare case of acute-onset Charles Bonnet Syndrome following cardiac surgery that resulted in profound loss of visual acuity in both eyes with characteristic visual hallucinations that were initially mistaken for delirium. Computed tomography of the brain revealed bilateral occipital infarcts, providing the substrate for Charles Bonnet Syndrome. A high index of suspicion should be maintained in cognitively intact patients with visual loss who are also experiencing visual hallucinations to ensure prompt diagnosis and management of this often overlooked condition.

Extra-corporeal membrane oxygenation for refractory cardiogenic shock after adult cardiac surgery: a systematic review and meta-analysis.

BACKGROUND: Postcardiotomy cardiogenic shock (PCCS) refractory to inotropic support and intra-aortic balloon pump (IABP) occurs rarely but is almost universally fatal without mechanical circulatory support. In this systematic review and meta-analysis we looked at the evidence behind the use of veno-arterial extra-corporeal membrane oxygenation (VA ECMO) in refractory PCCS from a patient survival rate and determinants of outcome viewpoint. METHODS: A systematic review was performed in January 2017 using PubMed (with no defined time period) using the keywords "postcardiotomy", "cardiogenic shock", "extracorporeal membrane oxygenation" and "cardiac surgery". We excluded papers pertaining to ECMO following paediatric cardiac surgery, medical causes of cardiogenic shock, as well as case reports, review articles, expert opinions, and letters to the editor. Once the studies were collated, a meta-analysis was performed on the proportion of survivors in those papers that met the inclusion criteria. Meta-regression was performed for the most commonly reported adverse prognostic indicators (API). RESULTS: We identified 24 studies and a cumulative pool of 1926 patients from 1992 to 2016. We tabulated the demographic data, including the strengths and weaknesses for each of the studies, outcomes of VA ECMO for refractory PCCS, complications, and APIs. All the studies were retrospective cohort studies. Meta-analysis of the moderately heterogeneous data (95% CI 0.29 to 0.34, p < 0.01, I 2  = 60%) revealed overall survival rate to hospital discharge of 30.8%. Some of the commonly reported APIs were advanced age (>70 years, 95% CI -0.057 to 0.001, P = 0.058), and long ECMO support (95% CI -0.068 to 0.166, P = 0.412). Postoperative renal failure, high EuroSCORE (>20%), diabetes mellitus, obesity, rising lactate whilst on ECMO, gastrointestinal complications had also been reported. CONCLUSION: Haemodynamic support with VA ECMO provides a survival benefit with reasonable intermediate and long-term outcomes. Many studies had reported advanced age, renal failure and prolonged VA ECMO support as the most likely APIs for VA ECMO in PCCS. EuroSCORE can be utilized to anticipate the need for prophylactic perioperative VA ECMO in the high-risk category. APIs can be used to aid decision-making regarding both the institution and weaning of ECMO for refractory PCCS.

Sudden Death in a Diver: A Diagnostic Conundrum.

We discuss the case of an experienced diver who ran out of air during his final ascent while scuba diving. He lost consciousness rapidly after surfacing and despite immediate cardiopulmonary resuscitation, could not be revived. On arrival at the emergency department he was noted to have copious amounts of blood in his upper airway and had developed extensive subcutaneous emphysema. Large amounts of air were observed in the central circulation following a postmortem computerized tomography scan as well as pneumomediastinum, a small right-sided hemothorax, and extensive subcutaneous emphysema. We discuss several potential pathophysiological mechanisms that might explain these findings. Finally, we end with a recommendation for an expedient whole-body postmortem computerized tomography scan and autopsy by a suitably qualified pathologist in the investigation of all dive-related fatalities, where possible.

Rheumatic heart disease screening: Current concepts and challenges.

Rheumatic heart disease (RHD) is a disease of poverty, is almost entirely preventable, and is the most common cardiovascular disease worldwide in those under 25 years. RHD is caused by acute rheumatic fever (ARF) which typically results in cumulative valvular lesions that may present clinically after a number of years of subclinical disease. Therapeutic interventions, therefore, typically focus on preventing subsequent ARF episodes (with penicillin prophylaxis). However, not all patients with ARF develop symptoms and not all symptomatic cases present to a physician or are correctly diagnosed. Therefore, if we hope to control ARF and RHD at the population level, we need a more reliable discriminator of subclinical disease. Recent studies have examined the utility of echocardiographic screening, which is far superior to auscultation at detecting RHD. However, there are many concerns surrounding this approach. Despite the introduction of the World Heart Federation diagnostic criteria in 2012, we still do not really know what constitutes the most subtle changes of RHD by echocardiography. This poses serious problems regarding whom to treat and what to do with the rest, both important decisions with widespread implications for already stretched health-care systems. In addition, issues ranging from improving the uptake of penicillin prophylaxis in ARF/RHD-positive patients, improving portable echocardiographic equipment, understanding the natural history of subclinical RHD and how it might respond to penicillin, and developing simplified diagnostic criteria that can be applied by nonexperts, all need to be effectively tackled before routine widespread screening for RHD can be endorsed.

Extracorporeal Life Support for Refractory Cardiac Arrest from Accidental Hypothermia: A 10-Year Experience in Edinburgh.

BACKGROUND: Cardiac arrest caused by accidental hypothermia is a rare phenomenon with a significant mortality rate if untreated. The consensus is that these patients should be rewarmed with extracorporeal life support (ECLS) with the potential for excellent survival and neurologic outcomes. However, given the lack of robust data and clinical trials, the optimal management of such patients remains elusive. OBJECTIVE: In this single-center study, we looked at the outcomes of all adult patients undergoing salvage ECLS for cardiac arrest caused by accidental hypothermia over a 10-year period from June 2006 to June 2016. METHODS: These data were obtained from the Royal Infirmary of Edinburgh cardiothoracic surgery database. The patients' hard copy case notes, TrakCare (InterSystems Corp, Cambridge, MA), picture archiving and communications system (PACS), and WardWatcher databases were used to cross-check the accuracy of the acquired data. RESULTS: Eleven patients met the inclusion criteria. The etiology of hypothermia was exposure to cold air (64%) and cold water immersion (36%). Two (18%) were treated with extracorporeal membrane oxygenation and the rest with cardiopulmonary bypass. The mean age was 51 years (range 32-73), and the mean core body temperature on admission was 20.6°C (range <18-24°C). The overall survival rate to hospital discharge was 72%, with 75% of survivors having no chronic neurologic impairment. CONCLUSION: Our case series shows the remarkable salvageability of patients suffering prolonged cardiac arrest caused by accidental hypothermia, particularly in the absence of asphyxia, trauma, or severe hyperkalemia. ECLS is a safe and effective rewarming treatment and should be used to aggressively manage this patient group.

A 20-year multicentre outcome analysis of salvage mechanical circulatory support for refractory cardiogenic shock after cardiac surgery.

BACKGROUND: Refractory post-cardiotomy cardiogenic shock (PCCS) is a relatively rare phenomenon that can lead to rapid multi-organ dysfunction syndrome and is almost invariably fatal without advanced mechanical circulatory support (AMCS), namely extra-corporeal membrane oxygenation (ECMO) or ventricular assist devices (VAD). In this multicentre observational study we retrospectively analyzed the outcomes of salvage venoarterial ECMO (VA ECMO) and VAD for refractory PCCS in the 3 adult cardiothoracic surgery centres in Scotland over a 20-year period. METHODS: The data was obtained through the Edinburgh, Glasgow and Aberdeen cardiac surgery databases. Our inclusion criteria included any adult patient from April 1995 to April 2015 who had received salvage VA ECMO or VAD for PCCS refractory to intra-aortic balloon pump (IABP) and maximal inotropic support following adult cardiac surgery. RESULTS: A total of 27 patients met the inclusion criteria. Age range was 34-83 years (median 51 years). There was a large male predominance (n = 23, 85 %). Overall 23 patients (85 %) received VA ECMO of which 14 (61 %) had central ECMO and 9 (39 %) had peripheral ECMO. Four patients (15 %) were treated with short-term VAD (BiVAD = 1, RVAD = 1 and LVAD = 2). The most common procedure-related complication was major haemorrhage (n = 10). Renal failure requiring renal replacement therapy (n = 7), fatal stroke (n = 5), septic shock (n = 2), and a pseudo-aneurysm at the femoral artery cannulation site (n = 1) were also observed. Overall survival to hospital discharge was 40.7 %. All survivors were NYHA class I-II at 12 months' follow-up. CONCLUSION: AMCS for refractory PCCS carries a survival benefit and achieves acceptable functional recovery despite a significant complication rate.

Interventional radiology in acute pancreatitis: friend or foe?

CONTEXT: Peripancreatic fluid collections are known complications of pancreatitis. The majority of fluid collections can be drained percutaneously under radiological guidance. Although radiological percutaneous drainage is regarded as safe, here it resulted in catastrophic haemorrhage from the colon due to an iatrogenic injury. CASE REPORT: We present a case of a 70-year-old man who presented with acute alcohol-related severe necrotizing pancreatitis and an associated massive peripancreatic fluid collection. The drainage of this collection was attempted under computed tomography (CT) scan guidance. During the procedure the splenic artery and the splenic flexure of the colon were inadvertently damaged leading to life threatening per rectal bleeding requiring emergency angiographic embolisation of the splenic artery. CONCLUSION: Radiological drainage of peripancreatic fluid collections is generally regarded as having lower rates of complications compared to surgical necrosectomy. However, in this case it leads to a life threatening per rectal bleed requiring emergency splenic artery embolisation.