Patent Foramen Ovale-associated Stroke and COVID-19 Vaccination.

Background: COVID-19 infection has been associated with paradoxical thromboembolism through a patent foramen ovale (PFO) and ischaemic stroke. Such events have not been reported after COVID-19 vaccination. The aim of the present study was to investigate PFO-associated stroke during the mass COVID-19 vaccination in Slovenia. Methods: This prospective study, conducted between 26 December 2020 and 31 March 2022, enrolled consecutive patients (≥18 years) with PFO-associated stroke referred for a percutaneous closure to a single interventional facility in Slovenia. Results: A total of 953,546 people aged between 18 and 70 years received at least one dose of a COVID-19 vaccine approved by the European Medicines Agency. Of the 28 patients presenting with PFO-associated stroke, 12 patients (42.9%) were vaccinated prior to the event, of whom nine were women and three were men, aged between 21 and 70 years. Stroke occurred within 35 days after vaccination in six patients (50%). Clinical presentation included motor dysphasia, paresis, vertigo, ataxia, paraesthesia, headache, diplopia and hemianopia. At hospital discharge, 11 patients (91.6%) had at least one residual ischaemic lesion. Conclusion: A temporal coincidence of COVID-19 vaccination and PFO-associated stroke has been described. A potential cause-effect relationship may only be hypothesised.

Platypnea-Orthodeoxia Syndrome Due to Patent Foramen Ovale Shunt Caused by Aortic Root Dilation

Background Orthodeoxia syndrome (OS) is a rare clinical condition characterized by oxygen desaturation noted in the upright position (orthodeoxia). OS can originate in the heart, lungs, abdomen, or elsewhere due to either an intracardiac (cardiac OS) or intrapulmonary shunt, or a ventilation-perfusion mismatch. Anecdotal reports suggest that the enlargement of the aortic root could interfere with atrial septal mobility (ASM) and cause patent foramen ovale (PFO) shunt. Case A 77-year-old male patient was admitted to the ICU with pneumonia due to COVID-19. During the evaluation, the patient presented orthodeoxia detected by pulse oximetry, with oxygen saturation (SpO2) of 96% in supine and 70% in upright positions. COVID-19 was treated with remdesivir and corticosteroids without orthodeoxia improvement. Decision-making Pulmonary embolism was ruled out with CT Angiography. Transthoracic Echocardiogram was also normal. A microbubble contrast-enhanced Transesophageal echocardiography was done and confirmed an aortic root dilatation with atrial septal mobility leading to a significant patent foramen ovale (PFO) shunt. The patient was submitted to interventional closure of the patent foramen ovale with the resolution of orthodeoxia. Conclusion Increased Mobility of the Atrial Septum in Aortic Root Dilation can be a cause of PFO shunt and orthodeoxia [Formula presented]Copyright © 2023 American College of Cardiology Foundation

A Case of Massive Pulmonary Embolus Extending into a Patent Foramen Ovale: An Impending Catastrophe

Background Massive pulmonary embolus (PE) is a life-threatening condition, however thrombus in transit in the setting of patent foramen ovale (PFO) poses catastrophic risk including systemic thromboembolism. Case An 88 year-old with history of COVID-related PE in 2020 previously on anticoagulation (AC) presented with chest pain & dyspnea. She was found to have lower extremity DVTs & extensive PE in the main pulmonary arteries & its branches. Transthoracic echo (TTE) revealed severe right ventricular dysfunction & right atrial (RA) thrombus in transit that extended into a PFO with right to left shunt. She was hemodynamically stable, but hypoxic on 4L/min of oxygen with a ProBNP 7712 pg/L, Troponin T 104 ng/dl, & pulmonary embolism severity (PESI) score of 104 (10% risk of 30 day mortality). Decision-making Due to the high PESI score & thrombus burden with risk of systemic thromboembolism, a multidisciplinary PE Response Team reached a consensus to pursue urgent mechanical thrombectomy. Inari FlowTriever system was successfully used for thrombectomy & retrieval of the RA clot in transit, with rapid improvement in right sided pressures. Repeat TTE showed no residual clot or shunting. Patient was placed on AC with plan for future PFO closure. Conclusion A multidisciplinary team approach was pivotal in managing this complex case with potential for hemodynamic compromise & systemic thromboembolism. We also demonstrate that mechanical thrombectomy is a feasible strategy for retrieving RA clot in transit. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

An Unusual Presentation of Right Atrial Myxoma: A Case Report.

A right atrial myxoma extending to the inferior vena cava with associated right-to-left shunting leading to systemic desaturation is an exceedingly rare clinical entity. The number of cases reported in the literature to date is not more than five. This case study presents a 45-year-old female who was referred to our center with symptoms of breathlessness, easy fatigability, generalized weakness, and central cyanosis. On routine blood investigation, polycythemia was detected. On preoperative 2D-transthoracic echocardiography, the diagnosis of right atrial mass was confirmed but the reason for cyanosis was not revealed. A tumor thrombus in a branch of the right lower pulmonary artery with a small locoregional pulmonary infarct along with right atrial myxoma was reported in computed tomography angiography of the heart and great vessels. On-table transesophageal echocardiography showed patent foramen ovale (PFO) with right-to-left shunting because of raised right atrial pressure and dynamic obstruction of the tricuspid valve by the mass. The myxoma was resected completely via right atrial approach along with primary closure of PFO. Postoperative period was uneventful, and the patient was discharged on day seven. At three-month follow-up, the patient was doing well.

Exertional desaturations after COVID-19: A case for PFO closure.

We present a case of a woman with past medical history notable for mild COVID-19 infection who presented with dyspnea on exertion, then developed progressively worsening exertional desaturations and was found to have a patent foramen ovale (PFO). Extensive cardiopulmonary testing revealed no clear alternate etiology for her symptoms. After much discussion, she underwent successful closure of the PFO with complete resolution of her symptoms and significantly improved exertional desaturation.

Unexplained hypoxia requiring prone ventilation in a patient with acute disseminated encephalomyelitis

Introduction: Acute Disseminated Encephalomyelitis (ADEM) is a rare, immune-mediated, demyelinating disorder of the central nervous system characterized by acute encephalopathy with neurologic deficits and MRI findings consistent with multifocal demyelination requiring immunosuppression for therapy.1,2 Patients seldom develop hypoxia during the course of the illness, requiring prone ventilation to improve oxygenation which is the first line of therapy and a proven standard of care in patients with ARDS.3,4We would like to present a case of ADEM where a patient developed unexplained hypoxia requiring prone ventilation. Case description: A 35-year-old male with no significant past medical history presented to our neuro-specialist centre with one day history of severe lower back pain associated with lower limb weakness and numbness. His symptoms, which commenced 10 days post his Covid vaccination, rapidly progressed over 2 days of hospital admission to involve right upper limb & facial weakness. MRI scan of brain and spine showed features of ADEM and pulse Methylprednisolone was initiated. CT thorax and abdomen on admission was unremarkable. He was transferred to the critical care unit in view of progressive ascending paralysis and was intubated on his 5th inpatient day due to involvement of respiratory muscles. Following 4 cycles of plasma exchange with albumin (day 6,7,9 and 10 of hospital admission), he developed unexplained hypoxic episodes which eventually resulted in sustained hypoxia, requiring 100% oxygen. Airway pressures and lung compliance were within normal range. Bedside ultrasound demonstrated good lung sliding in all lung fields and good left ventricular contractility with no evidence of right ventricular dilatation. There was no evidence of pericardial/pleural effusion. CT thorax repeated on day 9 showed no features of acute thromboembolic disease and there were no signs of lung parenchymal involvement. Formal echocardiography with bubble test showed normal heart with no evidence of patent foramen ovale. Multi-disciplinary discussions involving cardiology, respiratory, neurology teams and regional ECMO centre could not explain the enigma of impaired oxygenation. The patient responded well to 16 hours of prone ventilation on day 10 with decreasing oxygen requirements. In the subsequent 3 months of his inpatient stay, he was weaned off oxygen and was tracheostomised in view of his neurological illness. He continues to receive physiotherapy and neuro rehabilitation which had led to clinical improvement. Conclusion: The possible reason for hypoxia could be impaired tissue oxygenation post plasma exchange. However, it could be a coincidental finding and there is not much literature to explain this phenomenon and warrants further research.5.

Enterogenous Cyst and Glioblastoma: A Brief Histopathological Review of Two Uncommon Cystic Lesions of the Central Nervous System

Intracranial cystic lesions are common findings in cerebral imaging and might represent a broad spectrum of conditions. These entities can be divided into nonneoplastic lesions, comprising Rathke cleft cyst, arachnoid cyst, and colloid cyst, as well as neoplastic lesions, including benign and malignant components of neoplasms such as pilocytic astrocytoma, hemangioblastoma, and ganglioglioma. Surgical resection and histological evaluation are currently the most effective methods to classify cysts of the central nervous system. The authors report two uncommon cases presenting as cystic lesions of the encephalic parenchyma-a enterogenous cyst and a glioblastoma-and discuss typical histological findings and differential diagnosis.

COVID-19 AS A CAUSE OF COMA IN ADULT ONSET VANISHING WHITE MATTER DISEASE

A 34 year old female presented with fever, diarrhoea, confusion, and a decline in mobility, having recently tested positive for COVID-19. Her medical history included patent foramen ovale, primary ovarian failure and leukoencephalopathy which had been investigated in her 20s leading to a clinical diagnosis of Vanishing White Matter disease (VWM). On admission she deteriorated rapidly with decreased GCS requiring frequent critical care review. MRI demonstrated mild progression of the cerebral atrophy and slight increase in the 'cystic' changes in the white matter. Lumbar puncture was unremarkable. EEG demonstrated widespread cerebral dysfunction with frontotemporal emphasis but without seizure activity. She was managed with supportive care recovering to her baseline function within days. VWM is a leukodystrophy caused by recessive mutations in eukaryotic initiation factor 2B (eIF2b) complex. Adult onset presentations have been described which have a slowly progressive course. Patients with VWM are known to rapidly decline with fever, and mild head injury occasionally resulting in coma. Our case high-lights the potentially devastating effects of a neurotropic virus such as COVID-19 in VWM, and the need to consider rare genetic disorders in adult patients presenting with extensive white matter abnormalities on MRI and premature ovarian failure.

A Case Report of ECMO Patient with Cerebrovascular Complication Caused by a Broken Dual Lumen Cannula

Introduction: Dual-lumen cannula is used for extracorporeal membrane perfusion (ECMO) to support patients with ARDS due to COVID-19 as a bridge to lung recovery. It tends to be a longer support and there are several factors that can degrade the physical structure of the ECMO cannula and put the cannula at risk for breakage. Case Report: A 63-year-old woman was admitted to the hospital with COVID-19 pneumonia. Two days later, she was intubated and VV-ECMO was initiated due to treatment-resistant acute respiratory failure;a 28Fr CrescentTM dual-lumen cannula (Medtronic, MN) was inserted through the left subclavian vein and connected to a centrifugal oxygen pump with a centrifugal oxygenator. Within six weeks after onset, the patient was unable to be weaned from the ventilator and was transferred to our center with ECMO connected for consideration of lung transplantation. Two days after transfer, the patient developed acute aphasia, altered mental status, disturbed consciousness, and left arm seizures. A suction sound was heard from the left subclavian cannula insertion site and the ECMO bubble detector alarmed, but local inspection, chest X-rays, and CT scans of the brain and chest showed no obvious abnormalities. The patient was reintubated for encephalopathy and subsequently underwent a tracheostomy. The patient regained normal neurological function over the next 7 days. However, the air bubble sensor alarmed and suction sound was heard at the cannulation site again, and air bubbles were seen in the oxygenator. Due to concerns about air entrapment and cannula failure, we changed to a dual-canal VV-ECMO configuration using a right internal cervical and a left femoral cannula. The removed cannula had a 2 cm fracture distal to the skin insertion site. After resumption of ECMO, no new neurological episodes occurred. While awaiting lung transplantation, the patient died due to sepsis and multiple organ failure. An autopsy revealed a possible cause of cerebrovascular disease patent foramen ovale and air embolism to the brain. If a patient has been on ECMO for a long time and the bubble sensor warns of air detection, cannula breakage and impending air embolism should be suspected clinically, even if the defect is not found on examination and is not evident on imaging. If the COVID-19 epidemic continues, increased transport events may increase ECMO cannula breakage.

MANAGEMENT OF REFRACTORY SYMPTOMATIC PREMATURE VENTRICULAR CONTRACTIONS IN A PATIENT WITH LOUIS-DIETZ SYNDROME TYPE 3, EHLERS-DANLOS SYNDROME, AND SYSTEMIC LUPUS ERYTHEMATOSUS

Background: Louis-Dietz syndrome (LDS) Type 3 is a rare disorder caused by an autosomal-dominant mutation in SMAD-3, altering the TGF-β pathway. LDS Type 3 typically manifests as aortic aneurysms and early-onset osteoarthritis, however other dermatologic, cardiovascular, and skeletal abnormalities have been reported. Case: A 51-year-old woman was referred to the cardiology clinic for episodes of palpitations, syncope, chest pain, and shortness of breath during the COVID-19 pandemic. She had a history of congestive heart failure, cardiomyopathy, patent foramen ovale, atrial septal aneurysm, pre-COVID myocarditis, mitral valve prolapse, mitral regurgitation, and pericarditis. She also has a pertinent medical history of hypermobile Ehlers-Danlos syndrome (hEDS) and systemic lupus erythematosus (SLE). Her family and social history were remarkable for a daughter with SLE. Cardiopulmonary and general physical exams were remarkable for hypermobility. Evaluation with an ECG and Holter monitor showed normal sinus rhythm with unifocal premature ventricular contractions (PVCs) that correlated with her symptoms. Decision-making: The patient was initially managed un-successfully with beta and calcium channel blockers. Cardiac ablation was subsequently performed on a left ventricular septal focus with remote magnetic navigation using the Niobe system from Stereotaxis inc. (due to its low risk for cardiac perforation). At 6 months follow up, the patient exhibited an increase in left ventricular ejection fraction from 40-50% to 55-60%, fewer symptoms, and fewer PVCs. She was later diagnosed with a right internal carotid artery aneurysm that prompted genetic testing that was positive for LDS Type 3. Conclusion: This patient’s unique combination of illnesses required a multidisciplinary team for management. The Stereotaxis robotic system safely and successfully treated the patient’s PVCs and resulted in improvement of left ventricular function. Due to previous reports of arrhythmias associated with these connective tissue disorders, additional studies are necessary to understand the role of the SMAD-3 mutation, EDS, and SLE in contributing to arrhythmogenicity.

Platypnea-orthodeoxia syndrome years after acute pulmonary embolism: Two consecutive cases

Platypnea-Orthodeoxia syndrome (POS) is a rare condition in which dyspnoea and arterial oxygen desaturation are present in the upright position, while in the supine position, they are alleviated. It is observed in the presence of an anatomical (intra-or extracardiac) communication between the right and left heart causing a right-to-left shunt. POS is most frequently caused by a patent foramen ovale (PFO) and usually, the clinical assessment and a transthoracic echocardiograms with bubble study are enough to reach the diagnosis. The only possible treatment of POS is the percutaneous closure of the defect. We describe two cases of POS due to a PFO which manifested itself years after an episode of acute pulmonary embolism (PE), a finding never reported to date in the literature. Few cases describe the relationship between PE and POS, but these conditions may be more closely related than we currently think.

The Life of a Myxoma—Transoesophageal Images of the Progressive Growth of a Myxoma Over a 19-month Period

Introduction: A paucity in data exists regarding the expected growth rate of myxomas given the prompt transition to surgical intervention on initial discovery. Most case reports describe an initial normal echocardiogram compared with a subsequent echocardiogram revealing a myxoma at the time of diagnosis [1-3]. Uniquely, our case follows the sequential monitored growth of an atrial myxoma with transoesophageal images over a 19-month period. Case: This case describes a 71-year-old woman with a history of hypertension and hypercholesterolaemia admitted with a provoked deep vein thrombosis. This was complicated by bilateral pulmonary emboli and a presumed paradoxical embolus through a patent foramen ovale (PFO) resulting in a middle cerebral artery stroke. She was thrombolysed, then managed with rivaroxaban. During PFO closure, intraoperative transoesophageal echocardiogram (TOE) revealed a new left-sided atrial mass 1.0cm x 0.6cm in size, resulting in procedure abandonment. Uncertainty around diagnosis in addition to delays related to the COVID-19 pandemic led to serial imaging. The mass was monitored via TOE at 5, 11 and 19 months, revealing an initially stable then unexpected rapid progression to a size of 3.6cm x 2.9cm in the final 8 months. The patient had prompt excision of her myxoma and patch closure of interatrial septum. Discussion: This case exhibits a rare opportunity to appreciate the visual progression of an atrial myxoma and the unpredictable rapid growth over an 8-month period, highlighting the importance of interval monitoring of undifferentiated atrial lesions. [Formula presented]

Teleproctoring for Training in Structural Heart Interventions: Initial Real-World Experience During the COVID-19 Pandemic.

Background Proctoring represents a cornerstone in the acquisition of state-of-the-art cardiovascular interventions. Yet, travel restrictions and containment measures during the COVID-19 pandemic limited on-site proctoring for training and expert support in interventional cardiology. Methods and Results We established a teleproctoring setup for training in a novel patent foramen ovale closure device system (NobleStitch EL, HeartStitch Inc, Fountain Valley, CA) at our institution using web-based real-time bidirectional audiovisual communication. A total of 6 patients with prior paradoxical embolic stroke and a right-to-left shunt of grade 2 or 3 were treated under remote proctorship after 3 cases were performed successfully under on-site proctorship. No major device/procedure-related adverse events occurred, and none of the patients had a residual right-to-left shunt of grade 1 or higher after the procedure. Additionally, we sought to provide an overview of current evidence available for teleproctoring in interventional cardiology. Literature review was performed identifying 6 previous reports on teleproctoring for cardiovascular interventions, most of which were related to the current COVID-19 pandemic. In all reports, teleproctoring was carried out in similar settings with comparable setups; no major adverse events were reported. Conclusions Teleproctoring may represent a feasible and safe tool for location-independent and cost-effective training in a novel patent foramen ovale closure device system. Future prospective trials comparing teleproctoring with traditional on-site proctoring are warranted.

Fifty years of pediatric cardiology (1971-2021): from Fontan's surgery to the present day

Pediatric cardiology has matured profoundly over the last 50 years, paralleled by a similar development in pediatric cardiac surgery and cardiac anesthesia. This field of medicine provides structured and effective care for a very heterogeneous group of diseases including congenital heart disease, cardiomyopathies and heart failure, myocarditis, rheumatic heart disease, inherited and acquired arrhythmias, Kawasaki disease and more recently multisystemic inflammatory syndrome of children related to primary infection by SARS-CoV-2. This review summarizes achievements and results in selected topics of pediatric cardiology and cardiac surgery with focused attention to the diagnosis and management of congenital heart diseases.

Stroke and pulmonary embolism in moderate CoViD-19

Background: Venous thromboembolism, arterial thrombosis and thrombotic microangiopathy substantially contribute to increased morbidity and mortality in CoViD-19. We report a case of 56-year old man that presented with stroke and was found to have CoViD- 19 pneumonia complicated by pulmonary embolism (PE). Description of the case: A 55-year-old man with history of hypertension presented to the emergency department after a transient loss of consciousness. He was found to have left lateral hemianopia and lower right quadrantanopsia and head CT confirmed bilateral stroke in the posterior cerebral artery territory. MR angiography excluded atherosclerosis/dissection of the vertebral and basilar artery and a positive nasopharingeal swab PCR test revealed SARS-CoV-2 infection. The patient was admitted and ASA 100 mg and enoxaparin 40 mg per day were started. He experienced dry cough and fever and 10 days after admission presented atypical chest pain. CT Angiography revealed multiple confined ground glass opacities with segmental bilateral PE. Therapeutic dose of enoxaparin was started and after 5 days switched to edoxaban 60 mg per day. The patient progressively recovered and a complete work up excluded patent foramen ovale and any other cause predisponing to combined presence of venous and arterial thrombosis Conclusions: CoViD-19 has presented many diagnostic challanges in patients with neurologic and respiratory findings: thromboembolic disease may even be the initial or unique presentation. The early recognition of these phenotipes of the disease play a dramatic role in the CoViD-19 management.