The ductus arteriosus (DA) connects the pulmonary artery to the aorta to bypass the pulmonary circulation in utero. It normally closes within 24–72 hours after birth due to increased pulmonary resistance from an increase in oxygen partial pressure with the baby’s first breath. Medical treatment can help close the DA in certain situations where closure is delayed. However, in duct-dependent cardiac defects, the presence of the DA is crucial for survival and as such medical and surgical techniques have evolved to prevent closure. This review aims to outline the two main management options for keeping a ductus arteriosus patent. This includes stenting the PDA and shunting via a modified Blalock–Taussig shunt. Whilst both techniques exist, multicentre trials have found equal mortality end points but significantly reduced morbidity with stenting than shunting. This is also reflected by shorter recovery times, reduced requirement for extracorporeal membrane oxygenation (ECMO), and improved quality of life, although stent longevity remains a limiting factor.

Cardiovascular involvement in COVID-19 has different features. Here we report the ominous fate of a neglected adolescent with Williams syndrome that was infected by SARS-CoV-2 and ended by acute aortic dissection.

Introduction: Acute aortic syndrome (AAS) is a rare clinical syndrome with a high mortality encompassing acute aortic dissection, intramural hematoma and penetrating atherosclerotic ulcer. Up to 38% of cases are misdiagnosed on first presentation. There is a large variation in use of computed tomography to rule out AAS. The Canadian clinical practice guideline for the diagnosis of AAS was developed in order to reduce the frequency of misdiagnoses. As part of the guideline, a clinical decision aid was developed to facilitate clinician decision-making based on practice recommendations. Our objective was to validate the sensitivity of this clinical decision aid. Methods: Our validation cohort was recruited from a retrospective review of all cases of AAS diagnosed at three tertiary care emergency departments and one cardiac referral center from 2002-2019. Inclusion criteria: >18 years old, non-traumatic, symptoms <14 days and AAS confirmed on computed tomography, transesophageal echocardiography, intraoperatively or postmortem. The clinical decision aid assigns an overall score of 0-7 based on high risk pain features, risk factors, physical examination and clinical suspicion. Sensitivity with 95% confidence intervals are reported. Based on a national survey, a miss rate of <1% was predefined for the validation threshold. Results: Data was collected from 2002-2019 yielding 222 cases of AAS (mean age of 65 (SD 14.1) and 66.7% male). Kappa for data abstraction was 0.9. Of the 222 cases of AAS (type A = 125, type B = 95, IMH = 2), 35 (15.7%) were missed on initial assessment. Patients were risk stratified into low (score = 0, 2 (0.9%)) moderate (score = 1, 42 (18.9%)) and high risk (score ≥2,178 (80.2%)) groups. A score ≥1 had a sensitivity of 99.1% (95% CI 96.8-99.9%) in the detection of AAS. The clinical decision aid missed 0.9% (95% CI 0.3-3%) of cases. Conclusion: The Canadian clinical practice guideline's AAS clinical decision aid is a highly sensitive tool that uses readily available clinical information. Although the miss rate was <1%, the 95% confidence intervals crossed the predefined threshold. Further validation is needed in a larger population to ensure the miss rate is below an acceptable level.

Introduction: Acute aortic syndrome (AAS) is an uncommon, life-threatening emergency that is frequently misdiagnosed. The Canadian clinical practice guidelines for the diagnosis of AAS were developed in order to reduce the frequency of misdiagnoses and number of diagnostic tests. As part of the guidelines, a clinical decision aid was developed in order to facilitate clinician decision-making based on practice recommendations. The objective of this study was to identify barriers and facilitators among physicians to implementation of the decision aid. Methods: We conducted semi-structured interviews with emergency room physicians working at 5 sites distributed between urban academic and rural settings. We used purposive sampling, contacting ED physicians until data saturation was reached. Interview questions were designed to understand potential barriers and facilitators affecting the probability of decision aid uptake and accurate application of the tool. Two independent raters coded interview transcripts using an integrative approach to theme identification, combining an inductive approach to identification of themes within an organizing framework (Theoretical Domains Framework), discrepancies in coding were resolved through discussion until consensus was reached. Results: A majority of interviewees anticipated that the decision aid would support clinical decision making and risk stratification while reducing resource use and missed diagnoses. Facilitators identified included validation and publication of the guidelines as well as adoption by peers. Barriers to implementation and application of the tool included the fact that the use of D-dimer and knowledge of the rationale for its use in the investigation of AAS were not widespread. Furthermore, scoring components were, at times, out of alignment with clinician practices and understanding of risk factors. The complexity of the decision aid was also identified as a potential barrier to accurate use. Conclusion: Physicians were amenable to using the AAS decision aid to support clinical decision-making and to reduce resource use, particularly within rural contexts. Key barriers identified included the complexity of scoring and inclusion criteria, and the variable acceptance of D-dimer among clinicians. These barriers should be addressed prior to implementation of the decision aid during validation studies of the clinical practice guidelines.

Introduction: Type A aortic dissection(AD) is one of the most lethal diseases in medicine. Its mortality rate increases 1-2% per hour from the onset of symptoms to treatment. Timely diagnoses of ADs, therefore, are crucial to improve survival and decrease morbidity. There are various proposed clinical guidelines to help emergency physicians decide when a CTA is urgently needed with most widely quoted being the validated Aortic Dissection Detection Risk Score(ADD-RS) recommended by the American Heart Association. The addition of D-Dimer for further risk stratification has also been entertained. A recent article published in the American Journal of Emergency recommends using point of care ultrasound(POCUS) to expedite diagnosis. With the rising use of POCUS in the emergency department, it can be the missing link to timely AD diagnoses. This project aims to elucidate the prevalence of positive POCUS findings (pericardial effusion and dilated aortic root) in type A AD via a retrospective chart review. Methods: This study is a retrospective chart review of 200 patients with the diagnosis of AD treated at Southlake Regional Hospital. We included patients diagnosed with type A AD and excluded those diagnosed with type B AD. We collected data on their demographics, ADD-RS scores, investigation results, treatments, and clinical outcomes. The main focus of the chart review was on the presence of pericardial effusion or dilated aortic root on echocardiograms. Binomial statistical analysis was used to analyze the collected data. Results: We identified 126 patients with type A AD out of 200 charts reviewed. Thirteen (14% CI 8-23%, n = 93 p = 0.05) had wide mediastinum on their chest X-rays; twenty (95% CI 75-100%, n = 21 P = 0.05) had elevated D-dimer levels; and ninety-one (72% CI 64-80%, n = 126 p = 0.05) had positive ADD-RS. Only 88 out of 126 AD cases had documented echocardiograms. Sixty-eight (77% CI 67-86%, n = 88 p = 0.05) had either pericardial effusions or dilated aortic roots on their echocardiograms. Eighty-one (92% CI 84-95%, n = 88 p = 0.05) had either positive ADD-RS or positive echocardiogram findings, which is 20 (23% CI 14-33%, n = 88 P = 0.05) more cases than ADD-RS would have picked up alone. Conclusion: The absence of both pericardial effusion and dilated aortic root on echocardiogram in combination with a negative ADD-RS has a high sensitivity for ruling out type A AD. Our data support further research into the use of POCUS to expedite the diagnosis of type A AD in the emergency department.

Aortic dissection causes significant morbidity and mortality in adults and treatment guidelines are based on well-documented risk factors. Conversely, dissection after orthotopic heart transplantation is very rare, especially in the absence of infection, hypertension, or donor–recipient aortic size mismatch. Several forms of CHD are associated with aortic dilatation, but the incidence of aortic dissection and aneurysm in children is also low, which makes use of adult guidelines in children challenging. We present a 17-year-old Amish female with a homozygous gene mutation in the MYBPC3 gene known to cause lethal, infantile hypertrophic cardiomyopathy. She underwent orthotopic heart transplantation and then developed an asymptomatic aortic dissection despite no known risk factors.

A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life. As several chromosomal disorders are also associated with a bicuspid valve, there does not appear to be an apparent common trigger to the abnormal development of the aortic valve. The clinical care of the bicuspid aortic valve patient has been changed by a significant body of evidence that has improved the understanding of the natural history of the disease, including when to best intervene with valve replacement and when to provide prophylactic aortic root surgery. Moreover, as bicuspid valve disease is also part of various syndromes, we can identify high-risk patients in whom a bicuspid valve is much more unfavourable than in the normal population. This review provides an overview of all aspects of the bicuspid aortic valve condition and gives an updated perspective on issues from pathophysiology to clinical care of bicuspid aortic valve disease and associated aortic disease in asymptomatic, symptomatic, and pregnant patients, as well as our viewpoint on population screening.

Introduction: Acute aortic dissection (AAD) is a rare but fatal condition where over-investigation and missed diagnosis are common. Our objectives were to derive a highly sensitive clinical risk score for AAD and perform pilot validation. Methods: We started with two independent systemic reviews to firstly identify clinical variables associated with AAD and secondly to determine reasons for missed diagnosis. We searched Medline, Embase and the Cochrane database (1968-July 2016). Two reviewers screened articles and extracted data. Agreement was measured by Kappa and study quality by the QUADAS-2 tool. Bivariate random-effects meta-analyses (Revman 5 and SAS 9.3) were performed. Due to sampling bias found in the systematic reviews a matched case control study confirming the strength and direction of predictor variables was performed. The cases (2002-2014) included new emergency department (ED) or in-hospital diagnosis of non-traumatic AAD confirmed by computed tomography (CT). The controls (2010-2011) were a random age/sex matched sample of patients triaged with undifferentiated acute truncal pain (< 14 days). Finally, we used the beta coefficients derived from multivariate logistic regression of our case control study to assign a numerical strength of association to predictor variables. To mitigate the bias inherent in case control studies we adjusted the beta coefficient for each variable by the diagnostic odds ratio calculated from each systematic review. Pilot validation was performed on a retrospective sample of all those undergoing CTA to rule out AAD at two tertiary care ED over 12 months. Two abstractors were blinded to the final diagnosis. Results: We derived a two-step risk score based on the derivation sample which included 4960 patients(Clinical variables systematic review -9 studies, N=2400, low risk of bias, Kappa 0.9 & Reasons for missed diagnosis systematic review - 11 studies, N=800, low-moderate risk of bias, Kappa 0.89 & Case control study -194 AAD, 776 Controls). Step one is a RAPID assessment for AAD 1) Risk factors 2) Alternative diagnosis in the differential that mimics AAD- ACS, PE, Stroke 3) Physical exam- hypotension, pulse deficit 4) Impression- clinical suspicion of AAD and 5) Discomfort- migrating, tearing, pleuritic, thunderclap, severe pain. If any of the above factors are present proceed to step two. Step two stratifies patients based on history (low, moderate, high suspicion), physical exam (hypotension/pulse deficit) and risk factors. In the pilot validation (N=375,AAD=16) sensitivity was 100% (95%CI 79.4-100) and specificity 36.5% (95%CI 31.5-41.7%). Patients were successfully stratified into low (<2, 0% AAD), moderate (2, 2.2% AAD), high (>2, 19.6% AAD) and critical probability (>3, 62.5% AAD), with up to 36% reduction in imaging. Conclusion: We derived a highly sensitive new clinical risk score with the potential to reduce missed cases of AAD, reduce unnecessary imaging and expedite care.

Introduction: Le syndrome aortique aigu (SAA) est une condition rare et généralement mortelle qui demeure difficile à diagnostiquer. Le Score de détection de la dissection aortique (DDA) et le ratio de neutrophiles sur lymphocytes (NL) ont été proposés comme des éléments pouvant contribuer à exclure le diagnostic du SAA. Lobjectif primaire de cette étude est de déterminer la valeur diagnostique (sensibilité et spécificité et rapport de vraisemblance négatif [RV-]) de ces deux éléments de façon indépendante et combinée chez les patients suspectés de SAA au département durgence. Methods: Les patients ayant subi une angiographie par tomodensitométrie (angioCT) à la recherche dune dissection aortique entre 2008 et 2014 à lurgence dun hôpital tertiaire montréalais ont été inclus dans cette étude de cohorte rétrospective. Le score DDA a été établi à partir des dossiers médicaux et le ratio NL calculé à partir de la première formule sanguine prélevée chez ces patients. Pour le score DDA, un score de 1 ou plus et de 2 ou plus ont été évalués comme seuils de positivité. Pour le ratio NL, une valeur de plus de 4,6 a été choisie comme seuil puisquil sagissait du ratio proposé afin de différencier les patients atteints dun SAA de ceux souffrant dun anévrysme chronique. Pour lévaluation de la combinaison des deux tests, afin de maximiser la sensibilité, un score DAA de 1 ou plus ou un ratio NL de plus de 4,6 serait considéré comme positif. Le test de référence pour tous les patients était langiographie par tomodensitométrie. À partir de cela, la sensibilité, la spécificité et le rapport de vraisemblance négatif de chacun de ces tests/combinaison de tests et leurs intervalles de confiance (IC) ont été calculés. Results: Un total de 198 patients (99 hommes et 99 femmes) dun âge moyen de 63 ans (±16) ont été inclus dans létude, parmi lesquels 26 (13%) souffraient dun SAA. Un score DDA de 1 ou plus avait une sensibilité de 84,6% (IC 95% 65,1-95,6), une spécificité de 65,7% (IC 95% 58,1-72,8) et un VR- de 0,23 (IC 95% 0,09-0,58). Un score DDA de 2 ou plus avait une sensibilité de 23,1% (IC 95% 9,0-43,7), une spécificité de 95,3% (IC 95% 91,0-98,0) et un VR- de 0,81 (IC 95% 0,65-1,00). La sensibilité dun ratio NL de plus de 4,6 était de 42,3% (IC 95% 23,4-63,1), la spécificité de 58,7% (IC 95% 51,0-66,3) et le VR- de 0,98 (0,69-1,40). La combinaison du score DDA et du ratio NL avait une sensibilité de 88,5% (IC 95% 69,9-97,6), une spécificité de 38,4% (IC 95% 30,9-46,0) et un VR- de 0,30 (IC 95% 0,10-0,89). Conclusion: Avoir un score de DDA inférieur à 1 diminue significativement les chances davoir un SAA, nélimine pas cette possibilité et ne devrait pas être utilisé, sauf chez les patients ayant une probabilité pré-test déjà très faible. Le ratio NL en utilisant un seuil de 4,6 na aucune utilité diagnostique pour le SAA.

Cystinosis is a rare, autosomal-recessive genetic disorder. The kidneys are commonly involved, as there is cystinosin protein malfunction, and nephropathic cystinosis ensues. Although cardiac and vascular involvements are rare, we describe a unique case of aortic dissection in a 25-year-old female with cystinosis. We discuss the possible aetiologies of aortic dissection in this condition.

Sudden death from aortic dissection of an ascending aortic aneurysm is an uncommon but important finding in all series of sudden death in young, apparently healthy athletes. Individuals at risk include those having any of a variety of conditions in which structural weakness of the ascending aorta predisposes to pathological dilation under prolonged periods of increased wall stress. These conditions include Marfan syndrome, Loeys–Dietz syndrome, bicuspid aortic valve, and the vascular form of Ehlers–Danlos syndrome. Diagnostic criteria, surveillance strategies, medical management, and surgical indications are discussed. Finally, the current recommendations for sports participation are provided.

Introduction: Acute aortic dissection (AAD) is a life threatening condition making early diagnosis critical. Although 90% present with acute pain, the myriad of associated symptoms can make investigation and diagnosis a challenge. Our objectives were to assess emergency physician use of CT, yield of CT and ordering variation among physicians in patients presenting with pain for diagnosis of AAD. Methods: This historical cohort study of consecutive adult patients presenting to two tertiary academic care EDs over one calendar year included patients with a primary complaint of non-traumatic chest, back, abdominal or flank pain. Patients were excluded if clear diagnosis was made by basic investigations or exam. Primary outcome was rate of CT Thorax or Thorax/Abdomen ordered to rule out AAD as per clinical indication on diagnostic requisition. Secondary outcome was variation in CT ordering. Variation was measured with; Cochrane q test for homogeneity, proportion of positive CT’s (z-test) and mean CT’s (t test) ordered between high (>5CT/yr) and low (<5CT/yr) test users. Sample size of 6 per group was calculated based on an expected delta in mean CT ordered of 5 and a within group SD of 3. Results: 31,201 patients presented with chest, abdominal, back, flank pain during the study period. 8,472 were excluded based on a diagnosis made by clinical exam or basic investigations. 22,776 were included (Mean 47years SD 18.5yrs 56.2% Female). Most common diagnoses; Chest pain NYD(23.3%), Abdominal pain NYD(20.8%), Lower back pain NYD(10.5%), Renal Colic (5.3%), ACS (2.9%). CT was ordered to rule out AAD in 175 (0.7%) (Mean 62 years SD 16.5, 50.6% Female). Only 4(2.3%) were found to have an AAD. There was significant variation (range 0.6-12% Q test P<0.027) between proportion of CT's ordered by physicians. Between high (Mean 7.9 n=10 AAD=2) and low test users (Mean 2.3 n=41 AAD=2), there was significant difference in mean number of CT’s ordered (p<0.001) but no difference in number of AAD found (p<0.2). No AAD were missed. Conclusion: Current rate of imaging for aortic dissection is appropriately low but inefficient, with 98% of advanced imaging negative. There is significant variation in physician CT ordering (almost 20-fold) without an increase in diagnosis. These findings suggest great potential for more standardized and efficient use of CT for the diagnosis of AAD.

Introduction: Acute aortic dissection (AAD) is difficult to diagnose and if missed carries a significant mortality rate. Our aim was to assess the accuracy of history, physical exam and plain radiographs compared to advanced imaging in the diagnosis of AAD in adults presenting to the ED with a clinical suspicion of AAD. Methods: We conducted a librarian assisted systematic review. Databases searched: Pubmed, Medline, Embase and the Cochrane database from 1968 to January 2016. No restrictions for language were imposed. Studies were reviewed and data extracted by two independent reviewers. AAD was defined by CTA, MRI or TEE Prospective and retrospective studies of patients presenting with a clinical suspicion of AAD were included. Case series were excluded. Studies were combined if low clinical and statistical heterogeonity (I2<30%). Study quality was assessed using the QUADAS tool. Bivariate random effects meta analyses using Revman 5 and SAS 9.3 was performed. Results: We identified 792 records: 61 selected for full text review, 13 included and a further 7 from reference searches. 20 studies with 4721 participants were included (mean QUADAS score 12/14 SD 1.2, Kappa 0.8). Prevalence of AAD ranged from 9.6-76.1% (mean 39.1% SD 17.1%). Mean diagnosis in those without AAD varied between studies with ACS (30.3% SD 30.1%), Anuerysm(12.4% SD 9.8%), Chest wall pain(18.1% SD 13.3%) and PE(7.9% SD 7.85%) being the most common. The clinical findings most suggestive of AAD were, neurological deficit (specificity 94% LR 4.1 [95% CI, 3.1-5.2], I2 0%, n=9), hypotension(specificity 94% LR 2.6 [95% CI 1.6-4.2], I2 0%, n=8), pulse deficit (specificity 92% LR 3.4 [95% CI 1.8-6.4], I2 0%, n=9) and syncope (specificity 92% LR 1.4 [95% CI 1.1-1.8], I2 10%, n=6). The most useful for identifying patients less likely to have AAD were an absence of a widened mediastinum (sensitivity 80% LR 0.3 [95% CI 0.2-0.5], I2 20%, n=13) and an AHA Aortic dissection risk score <1 (n=2 sensitivity 91%,99% LR 0.02,0.22, [95% CI 0.003-0.128, 95%CI 0.2-0.3]). Conclusion: Suspicion for AAD should be raised with syncope, hypotension and pulse or neurological deficit in the appropriate clinical setting. Conversely the absence of a widened mediastinum and a low ADD score decreases likelihood. Clinical exam alone cannot rule out acute aortic dissection but it can help risk stratify for further testing.