bluuebunny/crossref_metadata_2025_split huggingface dataset:
Haemophagocytic lymphohistiocytosis after heart transplantation: a case report
Authors: Danielsson Christian , Karason Kristjan , Dellgren Göran 
Published: May 2020

Abstract

Background
Haemophagocytic lymphohistiocytosis (HLH) is an uncommon but serious systemic inflammatory response with high mortality rates. It can be triggered by malignancy or infectious agents, often in the context of immunosuppression. Literature covering HLH in heart transplantation (HTx) is scarce.

Case summary
A 25-year-old male with a history of celiac disease underwent HTx at Sahlgrenska Hospital in 2011 due to giant cell myocarditis and was treated with tacrolimus, mycophenolate mofetil (MMF), and prednisolone. He developed several episodes of acute cellular rejections (ACR) during the first 3 post-HTx years, which subsided after addition of everolimus. In May 2017, the patient was admitted to the hospital due to fever without focal symptoms. He had an extensive inflammatory reaction, but screening for infectious agents was negative. Haemophagocytic lymphohistiocytosis was discussed early, but first dismissed since two bone marrow biopsies revealed no signs of haemophagocytosis. Increasing levels of soluble IL-2 were considered confirmative of the diagnosis. Even with intense immunosuppressant treatment, the patient deteriorated and died in progressive multiorgan failure within 2 weeks of the symptom onset.

Discussion
A 25-year-old HTx recipient with an extensive inflammatory response, fulfilled criteria for HLH, but the diagnosis was delayed due to normal bone marrow biopsies. A background with autoimmune reactivity and immunosuppressive therapy may have contributed to HLH, but the actual trigger was not identified. Haemophagocytic lymphohistiocytosis can occur in HTx recipients in the absence of malignancy, identifiable infectious triggers and signs of haemophagocytosis. Early diagnosis and intervention are likely to be of importance for a favourable outcome.
Cardiac tamponade due to primary hypothyroidism: acute management and approach to prevent recurrence—a case report
Authors: Maddali Vikas Reddy , Miryala Srikar , Bellamkonda Yagna Sreekanth , Nagula Praveen 
Published: May 2020

Abstract

Background
Hypothyroidism is a common endocrine disorder resulting from deficiency of thyroid hormone, with iodine deficiency remains the foremost cause. It is more common in women with increasing incidence in the elderly. The manifestations of hypothyroidism results from the hypometabolism in the body at cellular level and affects all organs. Although there can be an incidental diagnosis of the disorder, the presentation with cardiac signs and symptoms is rare. We report a case of primary hypothyroidism with dysmorphic features manifesting as massive pericardial effusion with cardiac tamponade at presentation.

Case summary
A female aged 20 years presented with lethargy, constipation, and dyspnoea of 6 months duration. On examination, she was short-statured and had dysmorphic features with hypotension, raised jugular venous pressure (JVP), muffled heart sounds, and thyroid stimulating hormone &gt;100 uIU/mL. Chest X-ray showed cardiomegaly and 2DEcho confirmed cardiac tamponade for which emergency pericardiocentesis was done.

Discussion
Cardiovascular manifestations in hypothyroidism are dyspnoea and decreased exercise tolerance. Bradycardia, diastolic hypertension, cardiomegaly, and non-pitting or pitting peripheral oedema may be seen on physical examination. Mild pericardial effusion is common and generally asymptomatic. Massive pericardial effusion being manifested at presentation primarily as a sign of hypothyroidism is rare. A few cases have been mentioned in the literature in India and western population. Rarely, hypothyroidism presents with massive pericardial effusion resulting in cardiac tamponade as in our case.
Extensive coil embolization of a giant coronary artery aneurysm in an octogenarian: a case report
Authors: Ahmed Talha , Chahal Diljon , Shkullaku Melsjan , Gupta Anuj 
Published: May 2020

Abstract

Background
Coronary artery aneurysms (CAA) are often diagnosed incidentally on coronary angiography or imaging modalities done for other reasons. ‘Giant’ CAA by definition exceeds 20 mm in diameter or four times the diameter of normal coronary artery. The management of patients with CAAs is challenging due to poorly understood mechanism, variable presentation, and lack of clear-cut societal recommendations. Though conservative management is preferred in asymptomatic patients, massive size or interval growth may make intervention necessary.

Case summary
We describe a case of successful coil embolization of a giant coronary aneurysm in an elderly 84-year-old male. Patient, who presented for a follow-up computed tomography angiography to evaluate a previously repaired abdominal aortic aneurysm 2 years back, was found to have interval growth of right coronary artery aneurysm from 4 cm in diameter to 7 × 8 cm in its greatest dimensions. The rationale for treatment was to prevent sudden death from continued growth and eventual rupture of aneurysm in addition to potential risk of thromboembolism and compression of adjacent structures.

Discussion
This case demonstrates the safe and successful use of extensive coil embolization technique to treat a ‘giant’ CAA in an elderly patient when surgical risks were prohibitive.
Case report: adult-onset manifesting heterozygous glycogen storage disease type IV with dilated cardiomyopathy and absent late gadolinium enhancement on cardiac magnetic resonance imaging
Authors: Lyo Shawn , Miles Jeremy , Meisner Jay , Guelfguat Mark 
Published: May 2020

Abstract

Background
Glycogen storage disease type IV (GSD IV; Andersen’s disease) is a rare autosomal recessive disease caused by mutation in the GBE1 gene. Presentation of GSD IV varies on a continuum of severity and symptomatology ranging from neonatal death to mild adult-onset disease with variable involvement of hepatic, muscular, neurologic, dermatologic, and cardiac systems. Cardiomyopathy seen in GSD IV is also heterogeneous and its appearance on cardiac magnetic resonance imaging (CMR) is rarely described.

Case summary
A 29-year-old man without previous medical history was admitted to our facility multiple times over 2 years for focal sensorimotor deficits, gout arthropathy, chronic hyperlactataemia and hyperuricaemia, and severe decompensated non-ischaemic cardiomyopathy complicated by episodes of thromboembolic organ infarction. Echocardiography and CMR showed severe biventricular failure with the presence of intraventricular thrombi with increased right ventricular trabeculation and absent late gadolinium enhancement. He underwent muscle biopsy which showed prominent glycogen in skeletal muscle followed by genetic testing showing a single heterozygous splicing mutation c.993-1G&gt;T found at the junction of intron 7 and exon 8 of the GBE1 gene which had not previously been reported and was predicted to be pathologic. He was referred to a tertiary care centre with glycogen storage disease specialists but expired prior to establishing care at that facility.

Discussion
Discovery of GSD IV in our patient was unexpected due to a highly variant clinical presentation. Our case stresses the clinical heterogeneity of GSD IV and the importance of genetic sequencing studies in the evaluation of potential glycogen storage disease.
Late presentation of left atrial appendage erosion and perforation by an Amplatzer™ Amulet™ closure device: a case report
Authors: Wilkins Ben , Carranza Christian L , Søndergaard Lars , De Backer Ole 
Published: May 2020

Abstract

Background
Percutaneous left atrial appendage (LAA) closure may reduce the risk of cardioembolic stroke in patients with non-valvular atrial fibrillation. Given the prophylactic nature of the procedure, identifying and managing complications are paramount.

Case summary
A 73-year-old man presented 14 months after percutaneous LAA closure with syncope and acute pericardial tamponade which required surgical exploration and haemostasis; the most temporally remote account of this complication albeit amongst very few case reports. Tissue erosion by the Amplatzer™ Amulet™ LAA closure device (Abbott, Plymouth, MN, USA) was noted at two separate anatomical locations, corresponding to the device disc and lobe, which has not been described previously.

Discussion
This case report highlights the anatomical relationship between the LAA and its surrounding structures, and the importance of recognizing the risk of late device erosion.
Infectious stentitis after treatment of coarctation of the aorta: a case report
Authors: van der Zwaan Heleen B , Sieswerda Gertjan Tj , Krings Gregor J , Voskuil Michiel 
Published: May 2020

Abstract

Background
Aortitis is a rare condition that can be caused by inflammatory or infectious aetiologies. The clinical presentation of aortitis includes a heterogeneous range of symptoms and clinical signs.

Case summary
We present a 53-year-old man whose medical history included presence of a ventricular septal defect, a bicuspid aortic valve, and coarctation of the aorta. The coarctation was treated with percutaneous stent implantation. One and a half years later, he presented to our hospital with complaints of fatigue, night sweats, and shivers. Physical examination revealed a fever, tachycardia, and hypertension. Imaging studies showed no signs of endocarditis. Positron emission tomography–computed tomography (PET–CT) showed an increase in 18F-fluorodeoxyglucose uptake at the distal end of the stent in the descending aorta. Blood cultures revealed a Streptococcus gordonii and antibiotic treatment was adjusted accordingly. The patients’ functional status improved quickly, the fever resolved, and the laboratory markers of inflammation returned to normal.

Discussion
Aortitis is extremely rare after stent implantation. Risk factors for aortitis include congenital vascular malformation and stent implantation. Computed tomography is currently the imaging study of choice for aortitis, while PET–CT seems ideal for identification of stent infection. Mortality associated with infectious aortitis ranges from 21% to 44%, with generally higher mortality if managed with antibiotics alone. The differential diagnosis of stent infection should be taken into account in patients presenting with fever and chills after previous stent procedures.
False-positive troponin elevation due to an immunoglobulin-G-cardiac troponin T complex: a case report
Authors: Akhtar Zaki , Dargan James , Gaze David , Firoozi Sami , Collinson Paul , Shanmugam Nesan 
Published: May 2020

Abstract

Background
Troponin is a crucial biomarker for the diagnosis of an acute coronary syndrome (ACS). It rises in response to myocardial injury from significant acute myocardial ischaemia caused by obstructive coronary artery disease [‘classical’ myocardial infarction (MI)]. However, raised levels have also been noted in conditions not recognized as classical ACS. This may include MI with non-obstructed coronary arteries such as takotsubo cardiomyopathy and other acute or chronic conditions such as pulmonary embolus or chronic kidney disease. This is commonly labelled as a ‘falsely elevated’ troponin although there is some myocardial strain to explain the rise, such as an increase in cardiac oxygen demand. True ‘falsely elevated’ troponin, characterized by a persistent elevation in the absence of cardiac injury does occur and thought to be secondary to an immunoglobulin-troponin complex (macrotroponin).

Case summary
A 53-year-old gentleman with a background of diabetes, hypertension, hypercholesterolaemia, and hepatitis B was admitted with chest pain and persistently elevated cardiac troponin T (cTnT) levels. Investigations revealed unobstructed coronary arteries and a structurally normal, well-functioning heart. Subsequent biochemical analysis found the persistently elevated cTnT secondary to macrotroponin T.

Discussion
Macrotroponin, an immunoglobulin-troponin bound complex should be considered as a differential diagnosis when the biochemistry is not reflective of the clinical picture. Early recognition requires effective collaboration with the biochemistry laboratory for accurate diagnosis.
The mysterious needle in the heart: a case report
Authors: Mahon Ciara , Gatehouse Peter , Baksi John , Mohiaddin Raad H 
Published: May 2020

Abstract

Background
A 53-year-old female with dyspnoea and atypical chest pain. Her electrocardiogram demonstrated a left bundle branch block, transthoracic echocardiogram demonstrated a mildly impaired left ventricle ejection fraction, and coronary angiogram revealed unobstructed coronary arteries. She was referred for cardiovascular magnetic resonance (CMR) for structural and functional assessment. Her imaging revealed an unexpected finding of an off-resonance artefact within the ventricle wall. This material was secondary to a ferromagnetic material.

Case summary
Chest X ray and computer tomography confirmed a needle-shaped structure in the ventricle wall. Understanding the basis of this off-resonance artefact aided in a new diagnosis, raised questions on the origin of the material, patient safety, and implementation of corrective strategies to optimize image acquisition.

Discussion
The continued development of CMR is revolutionizing our ability to establish diagnosis and guide patient treatment. The CMR sequences can be prone to artefact. This case highlights the importance of understanding the basis of CMR artefacts.
Cardiac tamponade due to ruptured coronary-pulmonary artery fistula aneurysm: a case report
Authors: Hijikata Sadahiro , Sakurai Kaoru , Maeba Satoru , Azegami Koji 
Published: May 2020

Abstract

Background
Coronary artery fistulas are rare and most commonly asymptomatic; however, they can become enlarged and rupture in some cases.

Case summary
We report a case of a 51-year-old woman who was brought to our hospital unconscious in an ambulance. Cardiac tamponade caused by the rupture of an aneurismal coronary-pulmonary artery fistula (CPAF) was detected by contrast-enhanced computed tomography and confirmed by invasive coronary angiography. Due to prompt diagnosis and subsequent surgical intervention, the patient’s condition was rapidly improved, and she was discharged from the hospital.

Discussion
Coronary-pulmonary artery fistula aneurysm rupture requires rapid diagnosis and treatment, and thus, in cases with cardiac tamponade and coronary aneurysm, CPAF aneurysm rupture should be considered.
Mechanical tricuspid balloon ‘valvuloplasty’ in a prohibitive risk patient with cardiogenic shock: a case report
Authors: Hanna Nader , Carmona Andres , Crespo Hector , Cubeddu Roberto J 
Published: May 2020

Abstract

Background
We present a complex case of a failing tricuspid mechanical valve prosthesis in a patient with refractory cardiogenic shock at prohibitive risk for surgery in whom balloon ‘valvuloplasty’ resulted in immediate haemodynamic improvement in valve function.

Case summary
A 67-year-old woman with remote history of endocarditis s/p tricuspid valve repair and mechanical aortic valve replacement was referred for second opinion and management of new severe symptomatic tricuspid valve stenosis resulting in progressive debilitating congestive heart failure (HF). The patient was approved by the heart team to undergo redo open heart for surgical repair of the tricuspid valve. Intraoperative technical challenges were met to repair the tricuspid valve. In turn, the native valve was resected and a 33 mm On-X mechanical valve prosthesis. The patient’s post-operative course was complicated by recurrent haemoptysis, prolonged mechanical respiratory support, acute kidney injury, and cardiogenic shock. Surgical re-exploration to address the dysfunctional mechanical tricuspid valve was felt to be prohibitive. Structural heart team was consulted. Cardiac catheterization was recommended to ascertain and confirm findings. The patient was transferred to the cardiac catheterization laboratory. Initial fluoroscopic examination of the heart confirmed the echocardiographic results of an immobile septal leaflet of the recently implanted mechanical tricuspid valve. An 8 × 40 mm Mustang OTW angioplasty balloon was then advanced across the mechanical valve and inflated gradually at nominal pressure. A single inflation resulted in successful restoration of valve leaflet function.

Discussion
To the best of our knowledge, this is the first balloon ‘valvuloplasty’ on a mechanical On-X valve in the tricuspid position.
Zero-iodinated contrast retrograde percutaneous coronary interventions of chronic total occlusions using gadolinium and imaging guidance: a case report of a patient with severe anaphylaxis to iodinated contrast
Authors: Gupta Ankur , Neupane Saroj , Basir Mir , Alaswad Khaldoon 
Published: May 2020

Abstract

Background
Gadolinium-based contrast agents have been used as an alternative to iodinated contrast agents for simple percutaneous coronary interventions (PCIs) in patients with obstructive coronary artery disease. Their use has been limited in complex PCI due to poor image quality with gadolinium contrast. Significant dilution of gadolinium is required in an effort to avoid malignant ventricular arrhythmias. Further, the recommended amount of gadolinium contrast that can be used is very limited.

Case summary
We describe a case of patient with severe anaphylaxis to iodinated contrast agents despite pre-exposure prophylaxis with steroids and histamine blockers who underwent intravascular imaging-guided PCI of chronic total occlusions (CTOs) of left anterior descending and dominant left circumflex arteries using gadolinium contrast.

Discussion
In patients with anaphylaxis to iodinated contrast agents (i) complex coronary interventions including CTO PCI can be successfully performed without use of iodinated contrast, and (ii) combination of intravascular ultrasound guidance and gadolinium-based contrast agents can be safely and effectively used to perform complex PCI.
A case report of an iatrogenic coronary cameral fistula treated by retrograde percutaneous coronary intervention
Authors: Ohayon Paul , Matta Anthony , Boudou Nicolas 
Published: May 2020

Abstract

Background
Acquired coronary cameral fistula is an extremely rare condition that involves an abnormal communication between a coronary artery and a cardiac chamber. It usually occurs after chest trauma or cardiovascular interventions, such as percutaneous coronary intervention (PCI) and is associated with various outcomes, ranging from a stable status to haemodynamic instability. Acquired coronary cameral fistula frequently arises from the right coronary artery and drains generally into the right ventricle.

Case summary
We report the unusual case of a 56-year-old male patient referred to an invasive cardiology centre for a suspected left anterior descending (LAD) coronary–left ventricular (LV) fistula resulting from a primary PCI for an anterior ST-elevation myocardial infarction. Here, the confirmed LAD–LV fistula was successfully treated by retrograde PCI with covered stent implantation. Clinical and angiographic outcomes were favourable at 1-month follow-up.

Discussion
Coronary cameral fistula can be a severe complication of primary PCI. Various treatment strategies can be considered based on haemodynamic status and anatomical features. In the case described herein, the use of a retrograde approach led to permanent fistula closure and complete revascularization.
Cardioembolic stroke in a young male with cor triatriatum sinister: a case report
Authors: Amara Richard S , Lalla Rakhee , Jeudy Jean , Hong Susie Nam 
Published: May 2020

Abstract

Background
Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly defined by a fibromuscular membrane which bisects the left atrium. Cor triatriatum sinister has been associated with cardioembolic stroke through mechanisms including stagnation of blood flow within the left atrium, an association with atrial fibrillation (AF), and/or an accompanying atrial septal defect (ASD) or patent foramen ovale. We describe a case highlighting the role that CTS may play in cardioembolic stroke, provide high-quality computed tomography angiography and two- and three-dimensional echocardiography of the CTS membrane, and outline management strategies for this uncommon clinical scenario.

Case summary
A 35-year-old man with no prior medical history presented with acute onset weakness and aphasia. He was found to have an embolic stroke with left M1 and A1 occlusions and received tissue plasminogen activator followed by mechanical thrombectomy with successful recanalization. A thorough stroke workup revealed CTS with an associated ASD as well as potential protein C deficiency. He was managed with indefinite anticoagulation with apixaban.

Discussion
This is the 13th reported case of CTS associated with stroke. In most previous cases evidence of blood stasis or frank thrombus was associated with the CTS membrane, and/or existing AF was noted. In this case, none of these were identified, particularly highlighting the surreptitious risk of CTS. In addition, the presence of potential protein C deficiency in this case compounded the risk for thromboembolism and factored into multidisciplinary management decisions.
A case report of a coronary myocardial bridge with impaired full-cycle ratio during dobutamine challenge
Authors: Usui Eisuke , Maehara Akiko , Ali Ziad A , Moses Jeffrey W 
Published: May 2020

Abstract

Background
A myocardial bridge (MB) is a coronary variant in which an epicardial coronary artery tunnels through the myocardial band. Although MBs have been reported to cause ischaemia, physiological assessment of an MB has not been fully established.

Case summary
We encountered a case with exertional chest pain who underwent coronary angiography showing an MB at the mid-left anterior descending artery with systolic compression. Optical coherence tomography showed an MB defined as a homogeneous intermediate intensity surrounding the epicardial artery. The full-cycle ratio, defined as the lowest ratio of distal coronary pressure (Pd) to aortic pressure (Pa) during the entire cardiac cycle, measured 0.89 at rest and 0.73 with intravenous dobutamine of 20 µg/kg/min with a distinctive waveform pattern (early diastolic Pd drop) during a dobutamine challenge. Metoprolol succinate dosage was increased. The patient has been free from chest pain for 7 months after the discharge.

Discussion
Optical coherence tomography may contribute to anatomical detections of MBs. Because a systolic compression of the MB and release of the vascular lumen during early diastole leads to an early steep pressure loss, early diastolic Pd drop should be one of the specific haemodynamic characteristics of MBs. On the other hand, in a severe atherosclerotic stenosis, Pd drop is typically observed in late diastole, which could be differentiated from that of MBs. Because full-cycle ratio reflects the whole cardiac cycle including early diastole, this might be more useful than other physiological indices for detection of MB-related ischaemia induced by a dobutamine challenge.