- Catecholamine-Induced Cardiomyopathy in Pheochromocytoma: How to Manage a Rare Complication in a Rare Disease? [Journal Article]
- HMHorm Metab Res 2018 Sep 18
- Pheochromocytomas and paragangliomas (PHEOs) are rare neuroendocrine tumors. Clinical manifestations include different cardiovascular signs and symptoms, which are related to excessive secretion of c...
Pheochromocytomas and paragangliomas (PHEOs) are rare neuroendocrine tumors. Clinical manifestations include different cardiovascular signs and symptoms, which are related to excessive secretion of catecholamines. Catecholamine-induced cardiomyopathy in PHEO (CICMPP) is a rare but dreaded complication of PHEO. Once patient is diagnosed with this condition, the prognosis is worse and a surgical risk is much higher than expected. This article focuses on how catecholamines affect the heart and the pathophysiologic mechanism of CICMPP. The cardiovascular responses to catecholamine depend mostly on which catecholamine is released as well as the amount of catecholamine that is released. The acute release of norepinephrine and epinephrine from PHEO increases heart rate, systemic vascular resistance, myocardial contractility, and reduces venous compliance. The excessive adrenergic stimulation by catecholamine results in severe vasoconstriction and coronary vasospasm, myocardial ischemia, and subsequently damage, and necrosis. Chronically elevated catecholamine levels lead to significant desensitization of cardiac β-adrenoceptors. The increased levels of the enzyme β-adrenoceptors kinase (βARK) in the heart seems to mediate these biochemical and physiological changes that are consistently correlated with attenuated responsiveness to catecholamine stimulation. Through these mechanisms different types of cardiomyopathy (CMP) can be formed. This review discusses extensively the 3 types of cardiomyopathies that can be present in a PHEO patient. It also provides the clinical presentation and diagnostic and therapeutic algorithm in managing patients with CICMPP.
- An Introduction to the Mathematical Modeling in the Study of Cancer Systems Biology. [Review]
- CICancer Inform 2018; 17:1176935118799754
- CONCLUSIONS: In cancer research, outlining mathematical modeling of experimentally obtained data snapshots of molecular species may provide insights into a better understanding of the multiple biochemical circuits. Recent discoveries have provided support for the existence of complex cancer progression in dynamics that span from a simple 1-dimensional deterministic system to a stochastic (ie, probabilistic) or to an oscillatory and multistable networks. Further research in mathematical modeling of cancer progression, based on the evolving molecular kinetics (time series), could inform a specific and a predictive behavior about the global systems biology of vulnerable tumor cells in their earlier stages of oncogenesis. On this footing, new preventive measures and anticancer therapy could then be constructed.
- Long-term outcomes of surgical treatment for intravascular leiomyomatosis. [Journal Article]
- JFJ Formos Med Assoc 2018 Sep 11
- CONCLUSIONS: One-stage operation to completely remove IVL is feasible and with good long-term outcomes, which is recommended if the patient can tolerate the operation. Concomitant intrapulmonary tumors can be followed up watchfully except when associated with pleural effusion or the pathology indicating trend of increasing malignancy.
- The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations. [Review]
- JCJ Clin Med 2018 Sep 13; 7(9)
- Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. They are most commonly identified in the head and neck, being most freque...
Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. They are most commonly identified in the head and neck, being most frequent in the carotid body, followed by jugulotympanic paraganglia, vagal nerve and ganglion nodosum, as well as laryngeal paraganglia. Abdominal sites include the well-known urinary bladder tumors that originate in the Organ of Zuckerkandl. However, other unusual sites of origin include peri-adrenal, para-aortic, inter-aortocaval, and paracaval retroperitoneal sites, as well as tumors in organs where they may not be expected in the differential diagnosis of neuroendocrine neoplasms, such as thyroid, parathyroid, pituitary, gut, pancreas, liver, mesentery, lung, heart and mediastinum. The distinction of these lesions from epithelial neuroendocrine neoplasms is critical for several reasons. Firstly, the determination of clinical and biochemical features is different from that used for epithelial neuroendocrine tumors. Secondly, the genetic implications are different, since paragangliomas/pheochromocytomas have the highest rate of germline susceptibility at almost 40%. Finally, the characterization of metastatic disease is unique in these highly syndromic lesions. In this review, we summarize updated concepts by outlining the spectrum of anatomic locations of paragangliomas, the importance of morphology in establishing the correct diagnosis, the clinical implications for management, and the impact of genetics on the distinction between multifocal primary tumors compared with malignant disease.
- A meta-analysis of the prevalence of cardiac valvulopathy in hyperprolactinemic patients treated with Cabergoline. [Journal Article]
- JCJ Clin Endocrinol Metab 2018 Sep 11
- CONCLUSIONS: Treatment with low dose cabergoline in hyperprolactinemia appears to be associated with an increased prevalence of tricuspid regurgitation. The clinical significance of this is unclear and requires further investigation. 51.
- Neurological manifestations of atrial myxoma: A retrospective analysis. [Journal Article]
- OLOncol Lett 2018; 16(4):4635-4639
- Atrial myxoma is the most common type of primary cardiac tumor and it is closely associated with stroke in adults. Early diagnosis and treatment of atrial myxomas is essential for the prevention of e...
Atrial myxoma is the most common type of primary cardiac tumor and it is closely associated with stroke in adults. Early diagnosis and treatment of atrial myxomas is essential for the prevention of embolic events. The aim of the present study was to assess neurological complications associated with atrial myxoma. The neurological signs of atrial myxoma were retrospectively assessed in individuals who underwent treatment at West China Hospital (Chengdu, China) and The Affiliated Hospital of Hainan Medical University (Haikou, China), between March 2003 and February 2015. A total of 130 patients with atrial myxoma were included and 22 (17%) exhibited neurologic signs. These patients were aged 39.9±12.6 years (range, 13-78 years) and there were 13 female and 9 male patients. Ischemic cerebral infarct constituted the dominant clinical symptom (68.2%) and 3 patients exhibited concomitant cardiac manifestations. Atrial myxoma was diagnosed by echocardiography in all patients. Irregular surface of atrial myxomas was associated with a high risk of embolic events. The patients with myxoma successfully underwent surgery with no mortality recorded. In conclusion, atrial myxomas frequently manifest as cerebral infarction in individuals without cardiovascular risk factors. These tumors more commonly affect the middle cerebral artery. Irregular surface of myxomas appears to be associated with embolic events. Echocardiography may improve the diagnosis and early treatment of atrial myxomas.
- Clinical presentation and treatment of cardiac myxoma in 153 patients. [Journal Article]
- MMedicine (Baltimore) 2018; 97(37):e12397
- Cardiac myxoma (CM) is the most common benign cardiac tumor. We retrospectively reviewed our single center experience in 153 patients with CM over a period 25 years.From November 1993 to May 2017, 15...
Cardiac myxoma (CM) is the most common benign cardiac tumor. We retrospectively reviewed our single center experience in 153 patients with CM over a period 25 years.From November 1993 to May 2017, 153 patients were operated in our institution with diagnosis of a CM. In all patients preoperative, perioperative, and postoperative data were recorded including the long-term follow-up. All patients followed up in the outpatient's clinics and echocardiography at regular intervals.Mean age 59 ± 12 years old. There were 104 women and 49 men. Preoperative clinical manifestations of the patients were hemodynamic consequences (47.7%), asymptomatic (46.4%), systemic embolism (4.5%), systemic manifestations-fever (0.7%), and hemoptysis (0.7%). The most common location of CM was in the left atrium in 82.4% patients. Mean tumors diameter was 4.5 ± 1.9 cm. In addition, we were observed that the age of the patients have differences between sex groups women versus men, 60.3 and 54.8 years old respectively (P = .02). On the other hand the tumor size have not differences between the sex groups (P = .56). Combine operations were performed in 24 (15.7%) patients. New cerebrovascular accident was observed in 2 patients post-op. Mean in-hospital stay was 8.02 ± 2.8 days. In-hospital mortality was 1 patient (0.7%) (from sepsis). During median follow-up 3.7 ± 4.3 years CM recurrence was identified in 5 (3.3%) patients.Surgical resection of CMs contributes in an excellent prognosis and associated with low complications and recurrences rate. Regular long-term follow-up is recommended in all patients with CM.
- The optimal anticoagulant therapy for mechanical heart valves in a gallbladder cancer patient with hepatic metastases: A case report. [Journal Article]
- MMedicine (Baltimore) 2018; 97(37):e12368
- CONCLUSIONS: LMWH may represent a reasonable alternative regarding the inhibition of thrombus and bleeding in MHVs carriers with cancer and hepatic metastases.
- Primary cardiac natural killer/T-cell lymphoma, a very rare form of lymphoma. [Journal Article]
- ACAsian Cardiovasc Thorac Ann 2018 Sep 12; :218492318798230
- Extranodal natural killer/T-cell lymphoma is a rare non-Hodgkin lymphoma that is divided into nasal, non-nasal, and aggressive/leukemia subtypes, according to anatomic origin and clinical manifestati...
Extranodal natural killer/T-cell lymphoma is a rare non-Hodgkin lymphoma that is divided into nasal, non-nasal, and aggressive/leukemia subtypes, according to anatomic origin and clinical manifestations, with each subtype carrying a different prognosis. We present a case of primary cardiac natural killer/T-cell lymphoma with extension to other organs in a 38-year-old man, to highlight the role of imaging in categorizing nasal versus non-nasal types. This distinction has relevant implications for patient care because the non-nasal type has a much lower survival rate.
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- Serotonin contribution to cardiac valve degeneration: new insights for novel therapies? [Review]
- PRPharmacol Res 2018 Sep 09
- Heart valve disease (HVD) is a complex entity made by different pathological processes that ultimately lead to the abnormal structure and disorganization of extracellular matrix proteins resulting to...
Heart valve disease (HVD) is a complex entity made by different pathological processes that ultimately lead to the abnormal structure and disorganization of extracellular matrix proteins resulting to dysfunction of the leaflets. At its final evolutionary step, treatments are limited to the percutaneous or surgical valve replacement, whatever the original cause of the degeneration. Understanding early molecular mechanisms that regulate valve interstitial cells remodeling and disease progression is challenging and could pave the way for future drugs aiming to prevent and/or reverse the process. Some valve degenerative processes such as the carcinoid heart disease, drug-induced valvulopathy and degenerative mitral valve disease in small-breed dogs are clearly linked to serotonin. The carcinoid heart is typically characterized by a right-sided valve dysfunction, observed in patients with carcinoid tumors developed from serotonin-producing gut enterochromaffin cells. Fenfluramine or ergot derivatives were linked to mitral and aortic valve dysfunction and share in common the pharmacological property of being 5-HT2B receptor agonists. Finally, some small-breed dogs, such as the Cavalier King Charles Spaniel are highly prone to degenerative mitral valve disease with a prevalence of 40% at 4 years-old, 70% at 7 years-old and 100% in 10-year-old animals. This degeneration has been linked to high serum serotonin, 5-HT2B receptor overexpression and SERT downregulation. Through the comprehension of serotonergic mechanisms involved into these specific situations, new therapeutic approaches could be extended to HVD in general. More recently, a serotonin dependent/ receptor independent mechanism has been suggested in congenital mitral valve prolapse through the filamin-A serotonylation. This review summarizes clinical and molecular mechanisms linking the serotonergic system and heart valve disease, opening the way for future pharmacological research in the field.