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on crisis and cardiogenic shock, who was successfully salvaged by VA-ECMO support.
Pheochromocytoma and paraganglioma is a rare disease with a prevalence of 0.2-0.6% in hypertensive patients from outpatient.
A 21-year-old man complained of blood pressure elevation over one year and persistent hyperhidrosis near 5 years. SARS-CoV inhibitor In local hospital, a mass in the pericardial space with abundant blood flow was observed via echocardiography and confirmed under minimally invasive thoracotomy. With suspicion of malignant cardiac mass, the patient was recommended to transfer for further diagnosis and treatment. Combining evaluation for blood and urinary catecholamine levels, somatostatin receptor imaging, and iodine-131 metaiodobenzylguanidine scintigraphy, he was confirmed with the diagnosis of cardiac paraganglioma with blood supply from the right coronary artery identified via angiography. The cardiac tumour was then surgically resected and confirmed with a pathological diagnosis of paraganglioma. Subsequent genetic test suggested succinate dehydrogenase complex iron sulfur subunit B (SDHB) gene mutation. At 5-month follow-up, the patient was recovered with normal levels of blood catecholamines and catecholamine metabolites.
Cardiac paraganglioma should be considered and evaluated in hypertensive patients with cardiac mass, even in non-typical population. Given a potential risk of developing malignancies, close follow-up is significant in patients with SDHB gene mutations.
Cardiac paraganglioma should be considered and evaluated in hypertensive patients with cardiac mass, even in non-typical population. Given a potential risk of developing malignancies, close follow-up is significant in patients with SDHB gene mutations.
Transcatheter approach for large and complex atrial septal defects may represent a therapeutic challenge, particularly when the postero-inferior rim is deficient and floppy.
Here, we describe a successful catheter-based closure of a large (>30 mm) secundum atrial septal defect associated with postero-inferior rim deficiency in a 35-year-old female with congestive heart failure using pulmonary vein slide-out assisted implantation technique.
Inferior-posterior rim deficiency is a well-known risk factor for device instability or embolization. Transcatheter closure may represent a safe and effective alternative to the traditional surgical approach provided that modified implantation techniques are employed.
Inferior-posterior rim deficiency is a well-known risk factor for device instability or embolization. Transcatheter closure may represent a safe and effective alternative to the traditional surgical approach provided that modified implantation techniques are employed.
Spontaneous retroperitoneal haemorrhage (SRH) is a rare cause of retroperitoneal haemorrhage in patients who are on anticoagulants or antiplatelet agents or both.
We report here a rare and catastrophic complication of use of anticoagulants and antiplatelet drugs in a case undergoing coronary angioplasty. The patient had multiple coronary risk factors and developed acute myocardial infarction with pulmonary oedema and hypotension during hospitalization for treatment of lower respiratory tract infection and diabetic ketoacidosis. He underwent successful angioplasty of the culprit vessel but later developed hypotension attributable to retroperitoneal haemorrhage. No bleeding site was identified despite extensive evaluation of the aorta and iliac vessels.
A diagnosis of SRH is considered when a patient on anticoagulants or antiplatelet drugs develops retroperitoneal haemorrhage without any specific identifiable site of bleeding in the retroperitoneum. Diffuse vasculopathy and atherosclerosis or vasculitis ohe leak or surgical decompression in cases of abdominal compartment syndrome.
The prevalence of culture negative infective endocarditis (IEC) is reported as 2-7% though this figure may be as high as 70% in developing countries.
This higher rate will, at least in part, be due to reduced diagnostic facilities though some data suggests higher rates even when appropriate cultures were taken. The frequency is significantly elevated in patients who have already been exposed to antibiotics prior to blood cultures.
A rare cause of culture negative IEC is the HACEK group of organisms that are normal habitants of the oropharyngeal flora and account for 1-3% of native valve endocarditis.
(A. aphrophilus) is a member of the HACEK group of organisms.
A 32-year-old gentleman with a previous bioprosthetic aortic valve presented with a 1-week history of diarrhoea, vomiting, malaise, and weight loss. He was awaiting redo surgery for stenosis of the bioprosthesis, which had been inserted aged 17 for aortic stenosis secondary to a bicuspid valve. The initial blood tests revealed liver andtrates the importance of the 16S rRNA gene sequencing for HACEK infection diagnosis and appropriate antibiotic treatment.
Leigh syndrome is a neurodegenerative disorder caused by mitochondrial dysfunction with both phenotypic and genetic heterogeneity. Mitochondrial impairments are usually demonstrated by skeletal muscle biopsy. We report a case of Leigh syndrome diagnosed by endomyocardial biopsy (EMB), not by skeletal muscle biopsy.
At aged 7 months, the patient had delayed motor development. He developed metabolic acidosis triggered by an infection with elevated lactate and pyruvate values in serum and cerebrospinal fluid when he was 1 year old. T2-weighted imaging on magnetic resonance imaging of the brain revealed bilateral hyperintensity in midbrain and dorsal pons. Biopsied skeletal muscle did not show evidence of mitochondrial disease. Left ventricular hypertrophy, bilateral putamen hyperintensity in T2-weighted imaging and a lactate peak in the right basal ganglia in single voxel spectroscopy, and a convulsive seizure appeared at the age of 12, 15, and 16, respectively. When he was 17 years old, biopsied myocardium showed cytoplasmic vacuolization and a marked proliferation of mitochondria within myofibrils pathologically. Respiratory chain enzyme activity of the biopsied myocardium showed decreased activity of complex I. Genetic testing revealed an m.14453 A>G mutation on the MT-ND6 gene. He was finally diagnosed with Leigh syndrome. Administration of oral 5-aminolevulinic acid reduced the frequency of seizures.
EMB led to the diagnosis of Leigh syndrome. Efforts to find and conduct the biopsy of affected organs are important to diagnose mitochondrial disease. EMB is a useful diagnostic method when there is a difficulty in diagnosing mitochondrial disease by skeletal muscle biopsy.
EMB led to the diagnosis of Leigh syndrome. Efforts to find and conduct the biopsy of affected organs are important to diagnose mitochondrial disease. EMB is a useful diagnostic method when there is a difficulty in diagnosing mitochondrial disease by skeletal muscle biopsy.
Hypertrophic obstructive cardiomyopathy (HOCM) is sometimes concomitant with atrial fibrillation (AF) and exacerbates heart failure symptoms. Although optimal medication for the reduction of left ventricular outflow tract (LVOT) obstruction and the maintenance of sinus rhythm should be considered, it is difficult to control the symptoms permanently.
A 45-year-old man, diagnosed with HOCM, presented with progressive dyspnoea on exertion, which significantly deteriorated during episodes of paroxysmal AF, despite optimal medical therapy. On echocardiography, we found LVOT obstruction with a peak pressure gradient of 98 mmHg, concomitant with redundant mitral valve leaflets, which caused significant systolic anterior motion (SAM). Since he declined open surgery, we selected a combination of catheter interventions, AF ablation, and alcohol septal ablation (ASA). After the AF ablation, the occurrence of AF significantly decreased, and there was no recurrence after the ASA. link2 By 6 months, the plasma N-terminal pro-B-type natriuretic peptide level had decreased from 1022 to 124 pg/mL, the peak pressure gradient of LVOT decreased from 98 to 12 mmHg, and the left atrium volume decreased from 203 to 178.4 mL. The improvement in the SAM was visualized on echocardiography and was haemodynamically corroborated by the four-dimensional (4D)-flow cardiac magnetic resonance (CMR).
The treatment of drug-refractory HOCM concomitant with paroxysmal AF needs both septal reduction and the maintenance of sinus rhythm, which can be accomplished through transcatheter interventions. Moreover, the detailed intra-ventricular haemodynamic assessment in HOCM patients can be explored using the 4D-flow CMR.
The treatment of drug-refractory HOCM concomitant with paroxysmal AF needs both septal reduction and the maintenance of sinus rhythm, which can be accomplished through transcatheter interventions. Moreover, the detailed intra-ventricular haemodynamic assessment in HOCM patients can be explored using the 4D-flow CMR.
The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the pathogen responsible for the now pandemic disease, coronavirus disease (COVID-19). link3 A number of reports have emerged suggesting these patients may present with signs and symptoms consistent with ST-segment elevation myocardial infarction without coronary artery occlusion.
We report an international case series of patients with confirmed COVID-19 infection who presented with suspected ST-segment elevation myocardial infarction. Three patients with confirmed COVID-19 presented with electrocardiogram criteria for ST-segment elevation myocardial infarction. No patient had obstructive coronary disease at coronary angiography. Post-mortem histology in one case demonstrated myocardial ischaemia in the absence of coronary atherothrombosis or myocarditis.
Patients with COVID-19 may present with features consistent with ST-segment elevation myocardial infarction and patent coronary arteries. The prevalence and clinical outcomes of this condition require systematic investigation in consecutive unselected patients.
Patients with COVID-19 may present with features consistent with ST-segment elevation myocardial infarction and patent coronary arteries. The prevalence and clinical outcomes of this condition require systematic investigation in consecutive unselected patients.
Immune checkpoint inhibitors (ICIs) can cause cardiac immune-related adverse events (irAEs), including pericarditis. Cardiovascular events related to pericardial irAE are less frequent, but fulminant forms can be fatal. However, the diagnosis and treatment strategies for pericardial irAE have not established.
A 58-year-old man was diagnosed with advanced non-small-cell lung cancer and nivolumab was administered as 5th-line therapy. Eighteen months after the initiation of nivolumab, the patient developed limb oedema and increased body weight. Although a favourable response of the cancer was observed, pericardial thickening and effusion were newly detected. He was diagnosed with irAE pericarditis after excluding other causes of pericarditis. Nivolumab was suspended and a high-dose corticosteroid was initiated. However, right heart failure (RHF) symptoms were exacerbated during the tapering of corticosteroid because acute pericarditis developed to steroid-refractory constrictive pericarditis. To suppress sustained inflammation of the pericardium, infliximab, a tumour necrosis factor-alfa inhibitor, was initiated.
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