Propranolol Therapy in Tetralogy of Fallot: Treating the Echocardiogram or Treating the Patient.

Hypercyanotic spells are one of the defining clinical features of Tetralogy of Fallot (TOF). Limited data exist on peak Doppler right ventricular outflow tract (RVOT) gradient as a risk factor for the development of hypercyanotic spells, frequency of prophylactic use of propranolol based on peak RVOT gradient, and its impact on preventing the occurrence of hypercyanotic spells. We aimed to quantify peak RVOT gradients as measured on transthoracic echocardiography in infants with unrepaired TOF and assess for correlation with clinical symptoms of hypercyanotic spells. We also assessed the frequency of pre-operative use of propranolol, indication for medication initiation, and occurrence of hypercyanotic spells with or without propranolol use. Retrospective analysis was performed on patients at our institution who were born between February 1, 2011 and May 31, 2023. Patients were excluded if they were maintained on prostaglandin infusion or underwent palliative shunt placement or balloon valvuloplasty prior to complete surgical repair. Demographics, occurrence of hypercyanotic spells, propranolol use, peripheral oxygen saturation, age at surgical repair, and peak RVOT gradient at the time of propranolol initiation were collected from the electronic medical record. If no propranolol use was recorded, the single highest maximum RVOT gradient prior to surgery was collected. 203 patients were identified, of which 92 patients were included in analysis. Thirty-six (39%) patients received propranolol and 19% of patients developed hypercyanotic spells prior to surgery. Patients with higher peak RVOT gradients were more likely to be started on propranolol even in the absence of overt symptoms, and they also demonstrated more systemic desaturation. Additionally, peak RVOT gradient was found to be a poor predictor for the development of hypercyanotic spells. Wide clinical variation exists in the prophylactic use of propranolol for prevention of hypercyanotic spells. Peak RVOT gradient is not a reliable tool for prophylactic propranolol initiation to prevent hypercyanotic spells.

Simultaneous bilateral application of the Scepter mini balloon microcatheter for occlusion of ethmoidal dural arteriovenous fistulas.

Ethmoidal dural arteriovenous fistulas are a rare entity accounting for 10 % of all dAVFs.3-6 Haemorrhage occurs in up to 91 % of cases, which is a particularly high risk and warrants therapeutic intervention.8-9 Endovascular treatment for these fistulas using the conventional detachable microcatheter technique is associated with certain limitations and risks; 8.3 % rate of incomplete obliteration and an 8.3 % rate of complications. Complications include reflux of liquid embolic agent, posterior ischaemic optic neuropathy, acute visual loss, and small subdural haematoma secondary to a micro-perforation.8,10-12 We present our recent experience with the Scepter Mini Balloon Microcatheter for the endovascular treatment of ethmoidal dural arteriovenous fistulas in 3 patients, involving bilateral simultaneous inflation of the balloon. It demonstrates a novel application of this technology with good outcomes. It supports the use of this microcatheter in treating ethmoidal dural arteriovenous fistulas endovascularly, either as a first-line option or as an adjunct to surgery.

Primary endovascular embolisation of intracranial arteriovenous malformations (AVM)-UK single centre experience.

PURPOSE: Intracranial arteriovenous malformations (AVMs) treated at our institution with modern techniques of endovascular intervention were analysed for the rate of complete occlusion, associated morbidity, and mortality. To our knowledge, this is the first series from the UK evaluating the effectiveness of endovascular embolisation as a primary treatment for selected cases. METHODS: All newly referred AVMs between January 2017 and June 2022 were reviewed and those treated with primary endovascular intervention were identified. Details of the endovascular procedures were retrospectively reviewed. RESULTS: In 5½ years, 41.1% of AVMs referred to our institution have been triaged for primary endovascular intervention. Sixty-eight AVMs were embolised and followed-up: 44 ruptured and 24 unruptured. Spetzler-Martin grading varied from I to III, and a single AVM was grade IV. The approach was arterial in 73.5%, solely venous in 7.4%, and combined in 19.1%. The mean follow-up was 18 months for imaging and 26 months for clinical assessment. Complete obliteration was achieved in 95.6%. Ruptured AVM cohort: The rate of functional deterioration was 13.6%. Unruptured AVM cohort: The rate of functional deterioration secondary to complications from embolisation was 4.2%. CONCLUSIONS: Endovascular embolisation may be a favourable option for primary AVM treatment in carefully selected patients. However, selection criteria need to be better delineated for more specialists to consider this as a primary therapy.

Multi-modality treatment approach for paediatric AVMs with quality-of-life outcome measures.

PURPOSE: Despite the potentially devastating and permanently disabling effects of paediatric arteriovenous malformations (pAVMs), there is a paucity of studies reporting long-term quality-of-life (QoL) outcomes in AVM patients. We aim to evaluate the management strategies for paediatric intracranial pAVMs in the UK and long-term QoL outcomes using a validated paediatric quality-of-life outcome measure. METHODS: In this single-centre case-series, we retrospectively reviewed a prospectively maintained database of all paediatric patients (i.e. 0-18 years old) with intracranial AVMs, who were managed at Alder Hey Children's Hospital from July 2007 to December 2021. We also collected the PedsQL 4.0 score for these patients as a measure of QoL. RESULTS: Fifty-two AVMs were included in our analysis. Forty (80%) were ruptured, 8 (16%) required emergency intervention, 17 (35%) required elective surgery, 15 (30%) underwent endovascular embolisation, and 15 (30%) patients underwent stereotactic radiosurgery. There was an 88% overall obliteration rate. Two (4%) pAVMs rebled, and there were no mortalities. Overall, the mean time from diagnosis to definitive treatment was 144 days (median 119; range 0-586). QoL outcomes were collected for 26 (51%) patients. Ruptured pAVM presentation was associated with worse QoL (p = 0.0008). Location impacted psychosocial scores significantly (71.4, 56.9, and 46.6 for right supratentorial, left supratentorial, and infratentorial, respectively; p = 0.04). CONCLUSION: This study shows a staged multi-modality treatment approach to pAVMs is safe and effective, with superior obliteration rates with surgery alone. QoL scores are impacted by AVM presentation and location regardless of treatment modality.

The Angiographic and Clinical Follow-up Outcomes of the Wide-Necked and Complex Intracranial Aneurysms Treated With LVIS EVO-Assisted Coiling.

BACKGROUND: The Low Profile Visible Intraluminal Support EVO (LVIS EVO) is a self-expandable braided stent, which was recently introduced for the treatment of intracranial aneurysms. Full visibility of the stent and a relatively high metal coverage ratio are the unique features of the LVIS EVO. OBJECTIVE: To assess the safety, efficacy, and midterm durability of LVIS EVO stent-assisted coiling for the treatment of wide-necked intracranial aneurysms. METHODS: The endovascular databases were reviewed to identify patients treated with LVIS EVO-assisted coiling. The technical success and immediate clinical/angiographic outcomes were assessed. Periprocedural and delayed complications were evaluated. The follow-up angiographic/clinical outcomes were investigated. The preprocedural/follow-up neurological statuses were assessed with the modified Rankin Scale. RESULTS: One hundred three aneurysms in 103 patients (63 females) with a mean age of 54.9 ± 11.3 years were included. The mean maximum sac diameter was 6.2 ± 2.9 mm. The procedural technical success rate was 100%. Immediate postprocedural angiography showed complete occlusion in 77.7%. The mean duration of the angiographic follow-up was 8.8 ± 3.6 months. Follow-up angiography showed complete aneurysm occlusion in 89% of the 82 patients with angiographic follow-up. Recanalization was observed in 7.3% of 82 patients. Two patients (2.4%) required retreatment. In addition, 8.7% of the patients had at least 1 complication, and 2.9% of the patients developed a permanent morbidity. All patients had mRS scores ≤2. CONCLUSION: The results of this study demonstrate that SAC with LVIS EVO is a relatively safe, efficient, and durable treatment for wide-necked and complex intracranial aneurysms.

Case Report: Multimodality evaluation and clinical management of a single coronary artery.

A 14-year-old male with no significant medical history presented with intermittent palpitations for 2-3 months that occurred at rest and were associated with light-headedness. Electrocardiogram in clinic showed sinus arrhythmia with early repolarization and no ischemic changes. The echocardiogram showed normal cardiac structure and function, however, there was a concern for possible anomalous origin of the left coronary artery. Contrast-enhanced CT coronary artery angiogram confirmed a single coronary origin from the right coronary sinus. The single main coronary artery gave rise to the right coronary artery (RCA) and the left coronary artery (LCA). The LCA demonstrated a trans-septal course before it gave rise to the left anterior descending and left circumflex artery. There were intraarterial and intramural portions of the LCA, and the sinoatrial node artery arose from the LCA. The RCA demonstrated a normal course to the right atrioventricular groove, and the posterior descending artery arose from the RCA. Treadmill exercise stress test showed excellent functional capacity without exercise-induced chest pain or ischemic ECG changes. Invasive coronary angiography ruled out luminal narrowing or dynamic compression. Given the absence of physiologic or anatomic evidence of coronary flow restriction, no intervention was pursued and the palpitations were deemed to be likely unrelated to the coronary anomaly and eventually subsided spontaneously on 6 month follow-up.

Management of WEB device migration and mal-position in endovascular treatment of cerebral aneurysms.

With increasing advances in technology, the breadth of aneurysms that are treatable via an endovascular approach has increased. Wide necked aneurysms remain difficult to treat but the emergence of dedicated intrasaccular flow disruption devices such as the Woven EndoBridge (WEB, Micorvention) has increasingly seen previously ruptured and unruptured wide necked aneurysms successfully embolised and secured from the circulation using a single device.We are reporting two cases of WEB device treatment from the earliest experience with this device at our institution. These were complicated by partial extrusion in one case and remote migration of the WEB device in another case. Our initial cases highlight the importance of case selection and the need for accurate WEB sizing which are paramount to ensure complete occlusion of the aneurysm without complications of dislocation or extrusion into the parent vessel. Since then, we have performed over 170 cases with the WEB device. We also present a comprehensive review of the limited literature available on the management of mal-positioned and dislocated WEB devices. This allows us to reflect on how to avoid these complications and the different management options at the disposal of the neuro-interventionalist once such a complication has already occured. Rescue devices and manoeuvres that we reflect on include microcatheter manipulation, alligator retrieval device, stent retrievers, microsnares, aspiration, and stenting. Ultimately, each case needs to be individually evaluated and the best strategy selected depends on the context and specific circumstances.

Lodewyk H.S. van Mierop (March 31, 1927-October 17, 2021): a true giant.

We honour a great man and a true giant. Lodewyk H.S. van Mierop (March 31, 1927 - October 17, 2021), known as Bob, was not only a Paediatric Cardiologist but also a dedicated Scientist. He made many significant and ground-breaking contributions to the fields of cardiac anatomy and embryology. He was devoted as a teacher, spending many hours with medical students, Residents, and Fellows, all of whom appreciated his regularly scheduled educational sessions. Those of us who were fortunate to know and spend time with him will always remember his great mind, his willingness to share his knowledge, and his ability to encourage spirited and fruitful discussions. His life was most productive, and he will long be remembered by many through his awesome and exemplary scientific contributions.His legacy continues to influence the current and future generations of surgeons and all providers of paediatric and congenital cardiac care through the invaluable archive he established at University of Florida in Gainesville: The University of Florida van Mierop Heart Archive. Undoubtedly, with these extraordinary contributions to the fields of cardiac anatomy and embryology, which were way ahead of his time, Professor van Mierop was a true giant in Paediatric Cardiology. The invaluable archive he established at University of Florida in Gainesville, The University of Florida van Mierop Heart Archive, has been instrumental in teaching medical students, Residents, Medical Fellows, and Surgical Fellows. Only a handful of similar archives exist across the globe, and these archives are the true legacy of giants such as Dr. van Mierop. We have an important obligation to leave no stone unturned to continue to preserve these archives for the future generations of surgeons, physicians, all providers of paediatric and congenital cardiac care, and, most importantly, our patients.

Vertebral artery dissection managed by interventional radiology.

A 21-year-old patient presented with sudden-onset headache, visual disturbance and left hand incoordination. She was diagnosed with a left vertebral artery dissection of the V3 segment resulting in multiple cerebellar and cerebral infarcts. There were no risk factors for dissection other than recent COVID-19 infection. She was treated initially with antiplatelets, followed by anticoagulation, but experienced recurrent ischaemia. Although guidance suggests endovascular repair may be beneficial for patients with cerebral artery dissection (CAD) who experience recurrent strokes on medical therapy, evidence is limited. After multidisciplinary team consideration of the individual patient anatomy and risks and benefits of different endovascular techniques, the patient was treated with endovascular coiling. At 10 months follow-up, she had no further strokes and improving neurological symptoms. The case highlighted COVID-19 as a potential trigger for CAD and the use of endovascular coiling in preventing catastrophic cerebral ischaemia in CAD refractive to medical therapy.

Cardiac MRI Detection of Left Ventricular Aneurysm in Children After Ventricular Assist Device Explantation.

Consensus is lacking regarding screening for complications such as ventricular aneurysms after ventricular assist device explant. We report two pediatric patients, status-post explantation, who developed true left ventricular apical aneurysms diagnosed by cardiac magnetic resonance imaging. Imaging also suggested laminated thrombus versus postoperative changes which were treated with antiplatelet therapy.

Screening for cerebrovascular disorder on the basis of family history in asymptomatic children.

Background: Cerebrovascular disorders represent a group of uncommon, heterogeneous, and complex conditions in children. We reviewed the screening practice for the detection of cerebrovascular disorder in asymptomatic children referred to our neurovascular service on the basis of a positive family history and parental and/or treating physician concern.Methods: Retrospective case-note review of referrals to our neurovascular service (July 2008-April 2018). Patients were included if the referral was made for screening, on the basis of a positive family history of cerebrovascular disorder. Symptomatic children, those with previous cranial imaging, or children under the care of a clinical geneticist (i.e. due to the child or their relative having HHT or mutations in KRIT1) were not eligible for inclusion.Results: Forty-one children were reviewed, 22 males (Median age 10.7 years, range 0.6-15.6 years). This represented 22% of the total number of referrals over a 10-year period. Twenty-nine children had an MRI/MRA brain. Twenty-eight children were referred due to a family history of intracranial aneurysm and/or subarachnoid haemorrhage, but only two had two first-degree relatives affected. Ten children were referred due to a family history of arteriovenous malformation. Three children were referred due to a family history of stroke. No cerebrovascular disease was detected during the study period (n = 29).Conclusions: Parental and/or physician concern generated a substantial number of referrals but no pathology was detected after screening. Whilst general screening guidance exists for the detection of intracranial aneurysms, consensus guidelines for the screening of children with a positive family history do not, but are required both to guide clinical practice and to assuage parental and/or physician concerns.

Takayasu's arteritis presenting as acute myocardial infarction: case series and review of literature.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu's arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu's arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu's arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu's arteritis within the paediatric community.

A case series of intracranial dural arteriovenous fistulae mimicking cervical myelitis: a diagnosis not to be missed.

OBJECTIVE: To describe the diagnostic features of intracranial dural arteriovenous fistulae (DAVF) presenting with cervical cord or brainstem swelling. METHODS: Retrospective case note and neuroimaging review of patients with angiographically confirmed DAVF diagnosed during January 2015-June 2020 at a tertiary neuroscience centre (Walton Centre NHS Foundation Trust, Liverpool, UK). RESULTS: Six intracranial DAVF causing cervical cord or brainstem oedema (all males aged 60-69 years) and 27 spinal DAVF (88% thoracolumbar) were detected over a 5.5-year period. Significantly more patients with intracranial DAVF received steroids for presumed inflammatory myelitis than those with spinal DAVF (5/6 vs 1/27, p = 0.0001, Fisher's exact test). Several factors misled the treating clinicians: atypical rostral location of cord oedema (6/6); acute clinical deterioration (4/6); absence (3/6) or failure to recognise (3/6) subtle dilated perimedullary veins on MRI; intramedullary gadolinium enhancement (2/6); and elevated CSF protein (4/5). Acute deterioration followed steroid treatment in 4/5 patients. The following features may suggest DAVF rather than myelitis: older male patients (6/6), symptomatic progression over 4 or more weeks (6/6) and acellular CSF (5/5). CONCLUSION: Intracranial DAVF are uncommon but often misdiagnosed and treated as myelitis, which can cause life-threatening deterioration. Neurologists must recognise suggestive features and consider angiography, especially in older male patients. Dilated perimedullary veins are an important clue to underlying DAVF, but may be invisible or easily missed on routine MRI sequences.

Retro-aortic brachiocephalic vein with azygous vein continuation: a rare vascular variant.

We present an infant with incidentally found retro-aortic left brachiocephalic vein draining directly into azygous vein. This rare vascular variant is asymptomatic but does have clinical implications with surgical and interventional procedures.

Optimized Computed Tomography Angiography Protocol for the Evaluation of Thrombus in Patients with Fontan Anatomy.

The Fontan procedure is the final stage in the palliative surgical approach to patients with single-ventricle physiology. These patients have an increased risk for thromboembolic disease in the Fontan circuit, which can be evaluated by chest computed tomography angiography (CTA) in acute settings. However, false-positive results are common secondary to unusual streaming patterns in the Fontan circuit. A biphasic CTA protocol was evaluated for the capability to clearly identify structures of the Fontan circuit that are critical for the evaluation of thromboembolic disease. The study was a retrospective chart review of Fontan patients with a chest CTA scan obtained between 2011 and 2017. Two pediatric cardiologists with additional training in cardiac CT imaging independently reviewed each CTA and awarded one point for each of 5 Fontan circuit structures clearly identified resulting in a score range of 0-5. A score of 0-2 considered not capable, 3-4 partially capable, and 5 capable to clearly identify critical structures of the Fontan circuit. During the study period, 46 CTA scans were performed on 21 patients. Of the CTA scans using a biphasic protocol, 62.5% (10/16) were considered capable to clearly identify all 5 critical structures of the Fontan circuit vs 27% (8/30) of the CTA scans using a monophasic protocol (p = 0.027). Overall our results suggest that the single-site biphasic CTA protocol has greater diagnostic capability to detect the presence of Fontan thromboembolic disease when compared to the more traditional monophasic CTA protocol. Future prospective studies are needed to confirm these findings.

Isolated Right Superior Cava Drainage to the Left Atrium: An Uncommon Etiology of Cyanosis.

Anomalous superior vena cava drainage is a rare congenital cardiac defect where the right superior vena cava anomalously drains into the left atrium causing a right to left shunt. We present a case of a 17-day-old male who presented with cyanosis without respiratory distress and was found to have a right superior vena cava draining anomalously into the left atrium.

Left Atrial Appendage Aneurysm Characterized by Multimodal Imaging.

We present a four-year-old female with an incidental finding of a congenital left atrial appendage aneurysm who underwent surgical resection with excellent results. This case highlights the importance of multimodal imaging in the diagnosis and characterization of this rare condition.

Impact of 3D Printouts in Optimizing Surgical Results for Complex Congenital Heart Disease.

Planning corrective and palliative surgery for patients who have complex congenital heart disease often relies on the assessment of cardiac anatomy using two-dimensional noninvasive cardiac imaging modalities (echocardiography, cardiac magnetic resonance imaging, and computed tomography scan). Advances in cardiac noninvasive imaging now include the use of three-dimensional (3D) reconstruction tools that produce 3D images and 3D printouts. There is scant evidence available in the literature as to what effect the availability of 3D printouts of complex congenital heart defects has on surgical outcomes. Surgical outcomes of study subjects with a 3D cardiac printout available and their paired control subject without a 3D cardiac printout available were compared. We found a trend toward shorter surgical times in the study group who had the benefit of 3D models, but no statistical significance was found for bypass time, cross-clamp time, total time, length of stay, or respiratory support. These preliminary results support the proposal that 3D modeling be made readily available to congenital cardiac surgery teams, for use in patients with the most complex congenital heart disease.

A single centre experience with an evolving approach for the repair of coarctation of the aorta.

BACKGROUND: Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention. METHODS: Retrospective chart review of 66 patients aged 0-1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4-232) and median weight was 3.08 kg (1.25-8.0). Forty-one (62%) patients underwent median sternotomy. RESULTS: There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%. CONCLUSION: We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.