Mid-aortic syndrome is a rare condition characterised by segmental narrowing of the thoracoabdominal aorta. Here, we demonstrate a case of mid-aortic syndrome in a 30-month-old female who was diagnosed via transesophageal echocardiography after presenting with dilated cardiomyopathy and severe heart failure requiring placement of a left ventricular assist device.

Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the potential pathophysiology involved with their symptoms.

Introduction: Evidence is accumulating that a CT plus a CT angiogram (CTA) of the head and neck may be adequate to rule out subarachnoid haemorrhage (SAH) in patients with a thunderclap headache, thus potentially negating the need for lumbar puncture. One of the most widely cited objections to this strategy is the fear of detecting “incidental asymptomatic aneurysms,” lesions seen on angiography that are not in fact the cause of the patient's symptoms. Currently existing data on the background rate of aneurysms are based on cadaveric studies, invasive angiography, or MRI, and thus does not reflect the true rate of incidental aneurysms that would be detected using a CT plus CTA strategy. This study characterizes the rate of incidental aneurysms identified on CTA in an emergency department population. Methods: In this multicentre retrospective cohort study we analyzed the electronic medical records of all emergency department patients ≥ 18 years of age who underwent CTA of the head and neck over a two month period across four urban tertiary care emergency departments. Two independent reviewers evaluated the final radiology reports and extracted relevant data. The primary outcome of interest was the presence of incidental intracranial aneurysm, defined as a newly diagnosed aneurysm not associated with evidence of acute hemorrhage. Secondary outcomes included aneurysm location and size. Results: Of 739 charts meeting inclusion criteria, incidental intracranial aneurysms were detected in 21 cases or 2.85% (95% confidence interval, 1.77 - 4.32). An additional 20 aneurysms were identified but excluded from the analysis as they were previously known (n = 9) or were associated with evidence of acute hemorrhage (n = 11) and thus were not considered incidental. Of 21 patients with identified incidental aneurysms, 7 had multiple aneurysms. The most common aneurysm sites were internal carotid artery (n = 13), middle cerebral artery (n = 6) and anterior cerebral artery (n = 4). The average size of incidental aneurysm was 4.1 mm. Conclusion: The rate of incidental intracranial aneurysm among emergency department patients undergoing CTA of the head and neck is lower than many previously described estimates obtained through invasive angiography and MRI studies. To our knowledge, this is the first study on the prevalence of incidental intracranial aneurysms in an emergency department specific population and may therefore help guide clinicians when considering using a CT plus CTA rule out strategy for patients presenting with acute headache suspicious for SAH.

Introduction: Out of hospital cardiac arrest (OHCA) continues to carry a very high mortality rate, with approximately 10% surviving to hospital discharge. In 2015, the American Heart Association release updated guidelines dictating best practices in post-return of spontaneous circulation (ROSC) care, advocating for more liberal utilization of emergent coronary angiography. We sought to determine if the post-ROSC care at our centre during our study period adhered to the previously published (2010) guidelines. Methods: We performed a retrospective analysis (Sept. 2011 - June 2015) of the Resuscitation Outcomes Consortium (ROC) database, which contains pre-hospital, hospital and outcomes data on adult, EMS-treated, non-traumatic OHCA. Patients under 18 years, with missing age data or with obvious non-cardiac causes of arrest were excluded. Key variables included rates of post-ROSC emergent angiography, survival to hospital discharge and survival to hospital discharge with favourable neurologic outcome (modified Rankin score 2). Results: During the study period, there were a total of 997 OHCA; 86 met exclusion criteria. Of the 911 remaining patients, 557 (61.1%) were transported to a local ED. Of those transported to the ED, 262 (47.0%) achieved sustained ROSC, defined as survival to ED discharge. Of those who achieved sustained ROSC, median age was 65 years (IQR=21.75), 66.8% were male. ECG interpretation data was available on 214 patients, of whom 56 had definite STEMI, and 135 had definite absence of STEMI. 37/56 (66.1%) definite STEMI patients received coronary angiography within 24 hours of presentation, as per AHA guidelines. 58/262 (22.1%) post-ROSC patients overall received coronary angiography within 24 hours of presentation to the ED. Of those 58 patients who received emergent angiography, 38 (65.5%) underwent percutaneous coronary intervention (PCI). No patients received fibrinolysis. Of post-ROSC patients who received emergent coronary angiography, 40/58 (69.0%) survived to hospital discharge and 37/58 (63.8%) survived with good neurologic outcome. In comparison, 55/204 (27.0%) who did not receive emergent angiography survived to hospital discharge and 18.8% survived with good neurologic outcome. Conclusion: Only 22.1% of patients with OHCA, and only 66.1% with ECG-proven STEMI underwent emergent coronary angiography post-ROSC. Further investigation into causes for delay or the withholding of emergent angiography is necessary.

Takayasu arteritis is an idiopathic chronic granulomatous panarteritis predominantly affecting the aorta and its main branches. Although idiopathic, genetic contribution to disease susceptibility is being increasingly recognised. Rare in children, Takayasu arteritis is a worldwide disease with significant morbidity and mortality. Its diagnosis is a challenge and requires awareness of the condition as clinical features at presentation are non-specific and assessing disease activity is difficult. In the inflammatory stage, treatment is essential to prevent the insidious course and vascular damage: stenotic, occlusive lesions, aneurysms, and aortic regurgitation. New imaging modalities, such as CT scan, MRI, and 18F-fluorodeoxyglucose positron emission tomography, have expanded the possibilities for non-invasive diagnosis and monitoring; however, digital subtraction arteriography remains the gold standard for the diagnosis of Takayasu arteritis. Steroids are the first-line medical treatment. The combined use of methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, and biological agents is common. Revascularisation therapy should be considered in uncontrolled hypertension secondary to renal artery stenosis, symptomatic coronary ischaemia, cerebrovascular disease, severe aortic regurgitation, limb ischaemia, and aneurysms at risk of rupture, using surgical or endovascular procedures and taking into consideration that complications, especially restenosis, are frequent. Disease activity increases the likelihood of complications after revascularisation. Surgical intervention has shown better long-term outcomes, although the endovascular approach is evolving. The aim of this review was to describe key points of the diagnosis, treatment, and follow-up of Takayasu arteritis in childhood.

In this study, we describe a very rare case of anomalous left coronary artery arising from the right pulmonary artery in association with coarctation of the aorta. A 3-month-old boy presented with refractory congestive heart failure since 20 days after birth. The initial echocardiography suggested the diagnosis of left coronary artery-to-right pulmonary artery fistula associated with coarctation; however, selective coronary angiography demonstrated the rare anomaly of the left coronary artery arising from the right pulmonary artery. Subsequently, he underwent successful transcatheter balloon angioplasty for aortic coarctation and surgical repair of left coronary artery re-implantation.