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Cureus Apr 2024Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent tumors arising from chromaffin cells of the adrenal medulla and extra-adrenal sympathetic paraganglia,...
Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent tumors arising from chromaffin cells of the adrenal medulla and extra-adrenal sympathetic paraganglia, respectively. PCCs commonly produce one or more catecholamines (epinephrine, norepinephrine, and dopamine), but rarely are they biochemically silent. PGLs on the other hand, generally do not produce catecholamines. They have the highest heritability of all adrenal tumors and are known to be associated with genetic mutations. Patients with hereditary tumors typically present at a younger age and with multifocal disease when compared to sporadic disease. Specific genetic mutations have been well established with hereditary syndromes involving PCC/PGLs. Further research has aimed to identify other mutations and delineate specific phenotypes associated with these mutations. A 34-year-old woman presented for evaluation following a laparoscopic appendectomy that identified a 4-cm well-differentiated neuroendocrine tumor on final pathology. Further work-up included a repeat CT scan followed by a Dotatate PET CT scan which revealed a large (7.3 x 5.8 cm) periaortic mass related to the left adrenal gland. Functional adrenal work-up was negative and her Chromogranin A level was 679 ng/mL. She did report intermittent chest tightness and palpitations but was otherwise asymptomatic. The patient subsequently underwent an exploratory laparotomy with left adrenalectomy and adjacent tumor resection as well as completion of right hemicolectomy with ileocolonic anastomosis. Surgical pathology revealed two distinct masses consistent with multifocal PCC. No residual tumor was found in the colectomy specimen and 24 lymph nodes were negative. She had an uneventful recovery and genetic testing showed a variant of uncertain significance for the POLE and VHL genes. She has received genetic counseling and will be enrolled in an appropriate surveillance protocol.
PubMed: 38813302
DOI: 10.7759/cureus.59295 -
Cureus Apr 2024Atrial fibrillation (AF) is a type of cardiac arrhythmia causing shortness of breath, lightheadedness, and palpitations. It may go unrecognized and asymptomatic among... (Review)
Review
Atrial fibrillation (AF) is a type of cardiac arrhythmia causing shortness of breath, lightheadedness, and palpitations. It may go unrecognized and asymptomatic among many patients. AF is not a potentially fatal arrhythmia; its hemodynamic, structural, and hemocoagulative effects have a significant impact on the standard of life, which can lead to various complications such as stroke. A stroke caused by AF leads to additional burdens on both patients and the global economy. Patients with AF can prevent strokes with oral anticoagulants; however, ensuring diligent adherence to medication is crucial for maximizing treatment efficacy. Since they have a lighter treatment load than warfarin, non-vitamin K antagonist oral anticoagulants (NOACs) are also recommended with better hope for medication adherence. Various anticoagulants such as warfarin and ximelagatran, among many more, are prescribed to patients who have the potential to reduce the incidence of stroke as well as alleviate their likelihood of developing other thromboembolic events that can decrease their quality of life. Economic and psychological burdens associated with diminished functionality can be prevented by anticoagulant therapy among AF patients, therefore reducing their economic and social burden. This is due to the negative association between stroke among AF patients and anticoagulation consumption.
PubMed: 38813294
DOI: 10.7759/cureus.59298 -
Heart Rhythm May 2024Management of atrial fibrillation is frequently geared toward improving symptoms. Yet, the magnitude of symptom-rhythm discordance is not well known in the setting of...
BACKGROUND
Management of atrial fibrillation is frequently geared toward improving symptoms. Yet, the magnitude of symptom-rhythm discordance is not well known in the setting of monitoring by ambulatory electrocardiography (AECG).
OBJECTIVE
We aimed to quantify the symptom-rhythm correlation (SRC) for atrial arrhythmia (atrial tachycardia/atrial fibrillation [AT/AF]) events.
METHODS
This was a retrospective cohort analysis of AECG data at a tertiary care center. All AECGs of ≥7 days with at least 1 AT/AF were included. Patient-triggered symptoms included shortness of breath, tiredness, palpitations, dizziness, or passing out with or without concurrent AT/AF. SRC was calculated for each patient. In addition, AT/AF-symptom association was evaluated at the event level by multivariable mixed effects logistic regression.
RESULTS
We identified 742 patients with qualifying AECG data; mean age was 64 years, 50% were female, and 22% had heart failure. The mean CHADS-VASc score was 2.5. There were 6289 symptomatic events and 6900 AT/AF episodes. Of symptomatic events, 1013 (16%) had shortness of breath, 839 (13%) tiredness, 2640 (42%) palpitations, 783 (12%) dizziness, and 93 (1%) passing out. Overall SRC was 0.39 (range, 0-1.0), but presence of AT/AF increased odds of symptoms by ∼8.3 times in adjusted analyses (P < .01). In multivariable analysis, prior AF rhythm control treatment and lower heart rate were associated with worse SRC (P < .01).
CONCLUSION
Whereas AT/AF events increase the chances of symptoms, there is poor overall correlation between symptomatic events and documented AT/AF. Patient factors and prior treatments influence SRC. An improved understanding of this relationship correlation is needed to optimize clinical outcomes and to improve the rigor of AF research.
PubMed: 38810920
DOI: 10.1016/j.hrthm.2024.05.041 -
American Family Physician May 2024Atrial fibrillation is a supraventricular arrhythmia that increases the risk of stroke and all-cause mortality. It is the most common cardiac dysrhythmia in adults in... (Review)
Review
Atrial fibrillation is a supraventricular arrhythmia that increases the risk of stroke and all-cause mortality. It is the most common cardiac dysrhythmia in adults in the primary care setting, and its prevalence increases with age. The U.S. Preventive Services Task Force concluded that there is insufficient evidence to assess the benefits and harms of screening asymptomatic adults older than 50 years for atrial fibrillation. Many patients with atrial fibrillation are asymptomatic, but symptoms can include palpitations, exertional dyspnea, fatigue, and chest pain. Diagnosis is based on history and physical examination findings and should be confirmed with 12-lead electrocardiography or other recording device. The initial evaluation should include transthoracic echocardiography; serum electrolyte levels; complete blood count; and thyroid, kidney, and liver function tests. Stroke risk should be assessed in patients with atrial fibrillation using the CHA2DS2-VASc score. Warfarin and direct oral anticoagulants reduce the risk of stroke by preventing atrial thrombus formation and subsequent cerebral or systemic emboli. Hemodynamically unstable patients, including those with decompensated heart failure, should be evaluated and treated emergently. Most hemodynamically stable patients should be treated initially with rate control and anticoagulation. Rhythm control, using medications or procedures, should be considered in patients with hemodynamic instability or in some patients based on risk factors and shared decision-making. Electrical cardioversion may be appropriate as first-line rhythm control. Conversion to sinus rhythm with catheter ablation may be considered in patients who are unable or unwilling to take rate or rhythm control medications long-term or if medications have been ineffective.
Topics: Humans; Atrial Fibrillation; Anticoagulants; Electrocardiography; Stroke; Electric Countershock; Aged; Risk Factors; Echocardiography; Middle Aged; Anti-Arrhythmia Agents; Female; Male; Catheter Ablation
PubMed: 38804754
DOI: No ID Found -
Cureus Apr 2024Coronary artery fistulas (CAFs) are rare vascular anomalies characterized by abnormal connections between coronary arteries and cardiac chambers or adjacent structures....
Coronary artery fistulas (CAFs) are rare vascular anomalies characterized by abnormal connections between coronary arteries and cardiac chambers or adjacent structures. Advances in cardiac interventions have led to an increasing recognition of acquired CAFs, which are typically congenital. We present a case of a 62-year-old male with a complex medical history, including hypertension, atrial fibrillation, and heart failure, who presented with exertional chest pain and palpitations. Diagnostic evaluation revealed a significant CAF originating from the right coronary artery (RCA) and terminating into the coronary sinus and right ventricle. Despite the absence of significant coronary artery occlusions, the fistula was deemed clinically significant due to its potential to cause myocardial ischemia. Management involved guideline-directed medical therapy and lifestyle modifications. This case underscores the importance of early recognition and appropriate management of CAFs to optimize patient outcomes. Further research is needed to better understand the natural history and optimal management strategies of CAFs.
PubMed: 38803750
DOI: 10.7759/cureus.59155 -
Cureus Apr 2024Atrial myxomas are the most common form of primary benign cardiac tumors. The left atrium is typically the most common location while right atrial myxomas are much rarer...
Atrial myxomas are the most common form of primary benign cardiac tumors. The left atrium is typically the most common location while right atrial myxomas are much rarer and only occur in about 15%-25% of all myxoma patients. Typically, left atrial myxomas have the ability to cause symptoms such as syncope. We report a case of a 67-year-old female who presented with complaints of palpitations, dizziness, and near-syncope that had been ongoing for about a year. Other causes of syncope were investigated and ruled out. A transthoracic echocardiogram (TTE) found a large 4.3 x 4.0 cm spherical mass in the right atrium which was confirmed by surgical resection and immunohistochemistry to be a myxoma. The patient's condition of syncope-like symptoms warrants elevating atrial myxomas to a higher position in the diagnostic differential.
PubMed: 38800188
DOI: 10.7759/cureus.59070 -
AACE Clinical Case Reports 2024Pheochromocytoma can recur years after curative surgical resection. Rarely, it may reoccur as metastasis. Here, we present a case of metastatic pheochromocytoma to the...
BACKGROUND/OBJECTIVE
Pheochromocytoma can recur years after curative surgical resection. Rarely, it may reoccur as metastasis. Here, we present a case of metastatic pheochromocytoma to the bones in a patient with neurofibromatosis type 1 (NF1), 8 years after initial resection of primary bilateral adrenal pheochromocytomas without metastases.
CASE REPORT
A 44-year-old woman presented with diffuse body pain and palpitations. Her past medical history included NF1 and hypertension. Eight years prior to her current presentation, she had undergone a bilateral adrenalectomy for the management of bilateral adrenal pheochromocytomas. Her plasma metanephrines normalized after surgery and remained normal at her 1-year postoperative visit. She was subsequently lost to follow-up until her current presentation. Our evaluation revealed significantly elevated urine and plasma metanephrines as well as innumerable DOTATATE avid lesions along the axial and perpendicular spine compatible with a metastatic neuroendocrine tumor. She was started on doxazosin and metoprolol and discharged home with a plan to be seen by Oncology to discuss systemic therapy.
DISCUSSION
Predicting malignant disease in patients with primary tumors without metastases is challenging. There is no single factor that can reliably predict tumor behavior. It is unknown if individuals with NF1, who have a genetic predisposition for developing pheochromocytomas, are at an increased risk of malignant disease.
CONCLUSION
Due to a lack of accurate predictors, annual biochemical testing is recommended after primary tumor resection and in patients with a genetic predisposition. Strict lifelong follow-up should be strongly considered due to a possible higher risk of malignant disease.
PubMed: 38799052
DOI: 10.1016/j.aace.2024.02.006 -
MedRxiv : the Preprint Server For... May 2024The risk of cardiovascular outcomes in the post-acute phase of SARS-CoV-2 infection has been quantified among adults and children. This paper aimed to assess a multitude...
BACKGROUND
The risk of cardiovascular outcomes in the post-acute phase of SARS-CoV-2 infection has been quantified among adults and children. This paper aimed to assess a multitude of cardiac signs, symptoms, and conditions, as well as focused on patients with and without congenital heart defects (CHDs), to provide a more comprehensive assessment of the post-acute cardiovascular outcomes among children and adolescents after COVID-19.
METHODS
This retrospective cohort study used data from the RECOVER consortium comprising 19 US children's hospitals and health institutions between March 2020 and September 2023. Every participant had at least a six-month follow-up after cohort entry. Absolute risks of incident post-acute COVID-19 sequelae were reported. Relative risks (RRs) were calculated by contrasting COVID-19-positive with COVID-19-negative groups using a Poisson regression model, adjusting for demographic, clinical, and healthcare utilization factors through propensity scoring stratification.
RESULTS
A total of 1,213,322 individuals under 21 years old (mean[SD] age, 7.75[6.11] years; 623,806 male [51.4%]) were included. The absolute rate of any post-acute cardiovascular outcome in this study was 2.32% in COVID-19 positive and 1.38% in negative groups. Patients with CHD post-SARS-CoV-2 infection showed increased risks of any cardiovascular outcome (RR, 1.63; 95% confidence interval (CI), 1.47-1.80), including increased risks of 11 of 18 post-acute sequelae in hypertension, arrhythmias (atrial fibrillation and ventricular arrhythmias), myocarditis, other cardiac disorders (heart failure, cardiomyopathy, and cardiac arrest), thrombotic disorders (thrombophlebitis and thromboembolism), and cardiovascular-related symptoms (chest pain and palpitations). Those without CHDs also experienced heightened cardiovascular risks after SARS-CoV-2 infection (RR, 1.63; 95% CI, 1.57-1.69), covering 14 of 18 conditions in hypertension, arrhythmias (ventricular arrhythmias and premature atrial or ventricular contractions), inflammatory heart disease (pericarditis and myocarditis), other cardiac disorders (heart failure, cardiomyopathy, cardiac arrest, and cardiogenic shock), thrombotic disorders (pulmonary embolism and thromboembolism), and cardiovascular-related symptoms (chest pain, palpitations, and syncope).
CONCLUSIONS
Both children with and without CHDs showed increased risks for a variety of cardiovascular outcomes after SARS-CoV-2 infection, underscoring the need for targeted monitoring and management in the post-acute phase.
PubMed: 38798448
DOI: 10.1101/2024.05.14.24307380 -
Children (Basel, Switzerland) May 2024Congenital long QT syndrome (LQTS) represents a disorder of myocardial repolarization characterized by a prolongation of QTc interval on ECG, which can degenerate into... (Review)
Review
Congenital long QT syndrome (LQTS) represents a disorder of myocardial repolarization characterized by a prolongation of QTc interval on ECG, which can degenerate into fast polymorphic ventricular arrhythmias. The typical symptoms of LQTS are syncope and palpitations, mainly triggered by adrenergic stimuli, but it can also manifest with cardiac arrest. At least 17 genotypes have been associated with LQTS, with a specific genotype-phenotype relationship described for the three most common subtypes (LQTS1, -2, and -3). β-Blockers are the first-line therapy for LQTS, even if the choice of the appropriate patients needing to be treated may be challenging. In specific cases, interventional measures, such as an implantable cardioverter-defibrillator (ICD) or left cardiac sympathetic denervation (LCSD), are useful. The aim of this review is to highlight the current state-of-the-art knowledge on LQTS, providing an updated picture of possible diagnostic algorithms and therapeutic management.
PubMed: 38790576
DOI: 10.3390/children11050582 -
Children (Basel, Switzerland) May 2024Dysfunctional breathing (DB) describes a respiratory condition that is mainly characterized by abnormal breathing patterns, affecting both children and adults, often... (Review)
Review
Dysfunctional breathing (DB) describes a respiratory condition that is mainly characterized by abnormal breathing patterns, affecting both children and adults, often leading to intermittent or chronic complaints and influencing physiological, psychological, and social aspects. Some symptoms include breathlessness; dizziness; palpitations; and anxiety, while its classification lies in breathing pattern disorders and upper airway involvement. Its prevalence among the pediatric population varies with a female overrepresentation, while the existence of comorbidities in DB, such as asthma, gastro-esophageal reflux, nasal diseases, and anxiety/depression, frequently leads to misdiagnosis or underdiagnosis and complicates therapeutic approaches. The basic diagnostic tools involve a detailed history, physical examination, and procedures such as structured light plethysmography, cardiopulmonary exercise testing, and laryngoscopy when a laryngeal obstruction is present. The management of DB presumes a multidimensional approach encompassing breathing retraining, disease-specific advice through speech and language therapy in the presence of laryngeal obstruction, psychotherapy for fostering self-efficacy, and surgical therapy in a structural abnormality. The current review was developed to provide a summary of classifications of DB and epidemiological data concerning the pediatric population, comorbidities, diagnostic tools, and therapeutic approaches to enhance the comprehension and management of DB in children.
PubMed: 38790551
DOI: 10.3390/children11050556