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https://www.readbyqxmd.com/read/29782307/cutaneous-manifestations-of-tuberous-sclerosis
#1
Mirjana Bakić, Marina Ratković, Branka Gledović, Balsa Vujović, Danilo Radunović, Vera Babić, Vladimir Prelević
Dear Editor, Tuberous sclerosis (TS) is an autosomal dominant multisystem disease, which occurs due to genetically determined hyperplasia of ectodermal and mesodermal cells. Clinical manifestations present on the skin and in the nervous system, kidneys, heart, and other organs. Recent studies estimate the incidence of TS at 1/6000 to 1/10,000 live births, and a prevalence in the general population of approximately 1 in 20,000 (1). There are two different genetic loci responsible for TS: 9q34 (TSC1-hamartin) and 16p13...
April 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29777134/zebrafish-vcap1x2-regulates-cardiac-contractility-and-proliferation-of-cardiomyocytes-and-epicardial-cells
#2
Fang-Chi Hsieh, Yu-Fen Lu, Ian Liau, Chien-Chang Chen, Chao-Min Cheng, Chung-Der Hsiao, Sheng-Ping L Hwang
Sarcomeric signaling complexes are important to sustain proper sarcomere structure and function, however, the mechanisms underlying these processes are not fully elucidated. In a gene trap experiment, we found that vascular cell adhesion protein 1 isoform X2 (VCAP1X2) mutant embryos displayed a dilated cardiomyopathy phenotype, including reduced cardiac contractility, enlarged ventricular chamber and thinned ventricular compact layer. Cardiomyocyte and epicardial cell proliferation was decreased in the mutant heart ventricle, as was the expression of pAKT and pERK...
May 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29770201/recent-advances-in-managing-septal-defects-ventricular-septal-defects-and-atrioventricular-septal-defects
#3
REVIEW
P Syamasundar Rao, Andrea D Harris
This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse...
2018: F1000Research
https://www.readbyqxmd.com/read/29762932/pulmonary-hypertension-as-a-possible-cause-of-paradoxical-low-flow-low-gradient-aortic-stenosis
#4
Yuta Watanabe, Haruhiko Higashi, Katsuji Inoue, Jun Aono, Takafumi Okura, Jitsuo Higaki, Shuntaro Ikeda
Paradoxical low-flow, low-gradient aortic stenosis (LFLG AS) is recognized as a subtype of aortic stenosis. A small left ventricular (LV) cavity with marked LV concentric remodeling leads to a reduced stroke volume in this condition. The case is reported of a paradoxical LFLG AS patient who was undergoing treatment for pulmonary hypertension (PH) and interstitial pneumonia associated with scleroderma. Echocardiography demonstrated enlargement of the right ventricle and a diminished LV cavity. Moreover, the aortic valve opening was restricted despite a preserved LV ejection fraction (61%)...
September 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29749646/left-and-right-ventricular-morphology-and-function-in-athletes-with-elevated-pulmonary-systolic-arterial-pressure
#5
Oana Mirea, Oana M Corîci, Octavian Istrătoaie, Ionuț Donoiu, Maria Iancău, Constantin Militaru
OBJECTIVES: The objective of the study was to evaluate the prevalence and potential impact of elevated pulmonary arterial systolic pressure (PASP) on left and right cardiac morphology and function in young elite athletes. METHODS: In total, 85 professional athletes (40 endurance, 20 strength, and 25 mixed training, mean age 17.8 ± 4.0) and 50 sedentary controls (mean age 18.6 ± 3.3) underwent transthoracic echocardiographic examination. Two-dimensional measurements of the right (RV) and left ventricle (LV) were obtained...
May 11, 2018: Echocardiography
https://www.readbyqxmd.com/read/29718159/mitral-regurgitation-anatomy-is-destiny
#6
Constantine L Athanasuleas, Alfred W H Stanley, Gerald D Buckberg
Mitral regurgitation (MR) occurs when any of the valve and ventricular mitral apparatus components are disturbed. As MR progresses, left ventricular remodelling occurs, ultimately causing heart failure when the enlarging left ventricle (LV) loses its conical shape and becomes globular. Heart failure and lethal ventricular arrhythmias may develop if the left ventricular end-systolic volume index exceeds 55 ml/m2. These adverse changes persist despite satisfactory correction of the annular component of MR. Our goal was to describe this process and summarize evolving interventions that reduce the volume of the left ventricle and rebuild its elliptical shape...
April 26, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29678339/usefulness-of-pulmonary-arterial-end-diastolic-forward-flow-late-after-tetralogy-of-fallot-repair-to-predict-a-restrictive-right-ventricle
#7
Shelby Kutty, Anne Marie Valente, Matthew T White, Kelsey Hickey, David A Danford, Andrew J Powell, Tal Geva
The functional significance of pulmonary arterial end-diastolic forward flow (EDFF) in patients with repaired tetralogy of Fallot (rTOF) is not fully understood, with conflicting reports regarding its associations with pulmonary regurgitation (PR), right ventricular (RV) size and function, and so-called restrictive RV physiology. To examine these associations, we retrospectively analyzed 399 patients with rTOF who had contemporaneous echocardiography (Echo) and cardiovascular magnetic resonance (CMR) studies...
March 7, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29668474/craniosynostosis-acrocephalosyndactyly-apert-syndrome-diagnosed-in-a-newborn
#8
Orhideja Stomnaroska, Dragan Danilovski, Sanja Ivanovska
We report a 10 days old newborn with brachycephaly, midfacial hypoplasia, syndactyly and broad distal phalanx of thumb and big toe. At the 20th gestational weeks an enlargement of the left cerebral ventricle and malformation of the fingers of the hands and toes were noticed on a regular ultrasound examination. The aforementioned malformations were observed at birth and at the age of 11 months. The large fontal was closed; the small one was palpable at the tip of the finger. Brachycephaly was evident with high full forehead, flat occiput, and irregular craniosynostosis especially at the coronal suture...
December 1, 2017: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/29658871/new-onset-ventricular-arrhythmias-in-patients-with-left-ventricular-dysfunction-after-coronary-surgery-incidence-risk-factors-and-prognosis
#9
Nan Cheng, Changqing Gao, Rong Wang, Ming Yang, Lin Zhang
BACKGROUND: The incidence, risk factors, and long-term prognosis of new-onset ventricular tachycardia (VT) and ventricular fibrillation (VF) after coronary artery bypass graft surgery (CABG) in patients with impaired left ventricular function have not been thoroughly examined. Methods: This study enrolled 612 consecutive patients with impaired left ventricular function (ejection fraction <50%) undergoing CABG at a single institution between March, 1996, and September, 2015. Outcomes were analyzed and compared, including in-hospital mortality and long-term survival...
March 26, 2018: Heart Surgery Forum
https://www.readbyqxmd.com/read/29619581/approach-to-undifferentiated-dyspnea-in-emergency-department-aids-in-rapid-clinical-decision-making
#10
Siva Nageswara Rao Guttikonda, Kiran Vadapalli
BACKGROUND: Diagnosis and management of patients presenting with acute dyspnea is one of the major challenges for physicians in emergency department (ED). A correct diagnosis is frequently delayed and difficult to ascertain, and clinical uncertainty is common, explaining the need for rapid diagnosis and a management plan. The primary aim of our study is to assess a diagnostic strategy using multiorgan point of care ultrasonography (USG) to differentiate patients presenting with acute dyspnea to ED into different diagnostic categories for timely management in a resource-limited setting...
April 4, 2018: International Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29588890/endoscopic-transsphenoidal-salvage-surgery-for-symptomatic-residual-cystic-craniopharyngioma-after-radiotherapy
#11
Enzo Emanuelli, Giuliana Frasson, Diego Cazzador, Daniele Borsetto, Luca Denaro
Objectives  Ideal treatment of craniopharyngiomas is still controversial. Radiotherapy (RT) is considered effective for recurrences or after subtotal tumor removal (STR). About 40 to 50% of patients may experience tumor cyst expansion soon after RT; in these cases, the role of salvage surgery is debated. Design  Operative video. Setting  Tertiary care center. Participants  An 11-year-old boy diagnosed with persistent craniopharyngioma. In 2015, the patient underwent right frontotemporal craniotomy for STR at another center, complicated by panhypopituitarism...
April 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29576908/rare-case-of-a-rapidly-enlarging-symptomatic-arachnoid-cyst-of-the-posterior-fossa-in-an-infant-a-case-report-and-review-of-the-literature
#12
Nobuyuki Takeshige, Tomoko Eto, Shinji Nakashima, Kiyohiko Sakata, Hisaaki Uchikado, Toshi Abe, Motohiro Morioka
Background: Intracranial arachnoid cysts are space-occupying lesions that typically remain stable or decrease in size over time. Cysts in infants younger than 1 year of age are remarkably different from those in older children and adults in terms of cyst localization and enlargement. Arachnoid cysts of the posterior fossa (PFACs) are very rare in infants and do not typically grow or present with clinical symptoms, such that surgical treatment is generally considered to be unnecessary...
2018: Surgical Neurology International
https://www.readbyqxmd.com/read/29570509/impact-of-obstructive-sleep-apnea-on-cardiac-organ-damage-in-patients-with-acute-ischemic-stroke
#13
Paola Mattaliano, Carolina Lombardi, Davide Sangalli, Andrea Faini, Barbara Corrà, Laura Adobbati, Giovanna Branzi, Davide Mariani, Vincenzo Silani, Gianfranco Parati
BACKGROUND AND PURPOSE: Both obstructive sleep apnea (OSA) and cardiac organ damage have a crucial role in acute ischemic stroke. Our aim is to explore the relationship between OSA and cardiac organ damage in acute stroke patients. METHODS: A total of 130 consecutive patients with acute ischemic stroke were enrolled. Patients underwent full multichannel 24-h polysomnography for evaluation of OSA and echocardiography to evaluate left ventricle (LV) mass index (LV mass/BSA, LV mass/height), thickness of interventricular septum (IVS) and posterior wall (LVPW), LV ejection fraction and left atrium enlargement...
March 22, 2018: Journal of Hypertension
https://www.readbyqxmd.com/read/29566549/mid-term-development-of-the-right-ventricle-in-competitive-athletes
#14
Patrick Krumm, Stefan Krauß, Stefanie Mangold, Tanja Zitzelsberger, Bernhard D Klumpp, Konstantin Nikolaou, Andreas M Nieß, Ulrich Kramer, Christof Burgstahler
Background Long-term intensive training induces physiological, morphological, and functional adaption of the athlete's heart. Purpose To evaluate the development of athlete's heart during a mid-term follow-up of competitive athletes using cardiac magnetic resonance (CMR). Material and Methods Eighteen competitive long-distance runners and triathletes (age 43 ± 13 years, 3 women) were prospectively examined in a longitudinal follow-up study 5.05 ± 0.6 years after baseline. CMR at 1.5-T was performed for functional and late gadolinium enhancement (LGE) imaging...
January 1, 2018: Acta Radiologica
https://www.readbyqxmd.com/read/29550261/anatomical-associations-and-radiological-characteristics-of-scimitar-syndrome-on-ct-and-mr
#15
Abdulrahman Masrani, Sebastian McWilliams, Sanjeev Bhalla, Pamela K Woodard
PURPOSE: To report the anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. MATERIALS AND METHODS: Retrospective review of the medical records between February 2001 and February 2016 was performed. To identify patients, radiological reports were queried for "Scimitar" or "partial anomalous pulmonary venous return." Patients with radiological findings of Scimitar syndrome were included. Patients without cross-sectional imaging were excluded...
February 3, 2018: Journal of Cardiovascular Computed Tomography
https://www.readbyqxmd.com/read/29480834/percutaneous-treatment-for-silicosis-induced-pulmonary-artery-stenosis-a-case-report-and-review-of-the-literature
#16
REVIEW
Ruifeng Zhang, Guofeng Ma, Xiaoling Xu, Li Liang
BACKGROUND: Exposure to crystalline silica results in silicosis with initiation and progression of pulmonary fibrosis. The impaired lung parenchyma leads to pulmonary arterial hypertension and increased pressure in the right ventricle of the heart. Usually, the silicosis may be followed by enlargement of hilar lymphnodes, but silicosis-induced pulmonary artery stenosis with severe pulmonary hypertension is rare. Percutaneous pulmonary artery stenting and balloon angioplasty were performed to relieve stenosis and pulmonary hypertension...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29476388/left-atrial-dilatation-in-systolic-heart-failure-a-marker-of-poor-prognosis-not-just-a-buffer-between-the-left-ventricle-and-pulmonary-circulation
#17
A Rossi, F L Dini, E Agricola, P Faggiano, G Benfari, P L Temporelli, C Cucco, L Scelsi, C Vassanelli, S Ghio
BACKGROUND: The relation between systolic pulmonary pressure (sPAP) and left atrium in patients with heart failure (HF) is unclear. Diastolic dysfunction, expressed as restrictive mitral filling pattern (RMP), and functional mitral regurgitation (FMR) are associated with both LA enlargement and increased sPAP. We aimed to evaluate whether atrial dilation might modulate the consequences of RMP and FMR on the pulmonary circulation of patients with HF with reduced ejection fraction (HFrEF)...
February 23, 2018: Journal of Echocardiography
https://www.readbyqxmd.com/read/29451444/survival-and-prognostic-factors-in-cats-with-restrictive-cardiomyopathy-a-review-of-90-cases
#18
Chiara Locatelli, Danitza Pradelli, Giulia Campo, Ilaria Spalla, Alice Savarese, Paola G Brambilla, Claudio Bussadori
Objectives Large studies focusing on restrictive cardiomyopathy (RCM) in the cat are scarce. The aims of this retrospective study were to describe epidemiological characteristics and to analyse prognostic factors affecting survival in cats with RCM. Methods The clinical archives of the Gran Sasso Veterinary Clinic (Milan, Italy) and of the cardiology unit of the Department of Veterinary Medicine (University of Milan, Italy) from 1997-2015 were reviewed for all cats diagnosed with RCM based on an echocardiographic examination (left atrial/bi-atrial enlargement, normal left ventricle wall thickness, normal or mildly decreased systolic function and restrictive left ventricle filling pattern with pulsed Doppler echocardiography)...
February 1, 2018: Journal of Feline Medicine and Surgery
https://www.readbyqxmd.com/read/29394347/prognostic-value-of-3-dimensional-echocardiographical-heart-volume-assessment-in-patients-scheduled-for-left-ventricular-assist-device-implantation
#19
Albert Otten, Stephan Kurz, Sibtain Anwar, Jevgenij Potapov, Christian Krall, Benjamin O'Brien, Helmut Habazettl, Thomas Krabatsch, Marian Kukucka
OBJECTIVES: Left ventricular assist device (LVAD) support is an increasingly important and successful therapeutic option for patients with end-stage heart failure. As chronic heart failure progresses, the left and right ventricles adapt by enlarging its volume and patients present for LVAD implantation with varying degrees of dilatation. By quantitatively assessing right ventricular (RV) and left ventricular (LV) volumes using 3D transoesophageal echocardiography and correlating the findings with clinical outcomes, we aim to investigate the prognostic value of LV and RV volumes for early survival after LVAD implantation...
January 30, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29246514/atrial-enlargement-in-the-athlete-s-heart-assessment-of-atrial-function-may-help-distinguish-adaptive-from-pathologic-remodeling
#20
REVIEW
Flavio D'Ascenzi, Francesca Anselmi, Marta Focardi, Sergio Mondillo
Intensive training is associated with hemodynamic changes that typically induce an enlargement of cardiac chambers, involving not only the ventricles but also the atria. The hearts of competitive athletes are characterized by increases in left and right atrial dimensions that have been interpreted as a physiologic adaptation to training. Conversely, some authors have hypothesized maladaptive remodeling; furthermore, the extent of left atrial dimensional remodeling may overlap atrial dilation observed in patients with cardiac disease, representing a challenge for clinicians...
February 2018: Journal of the American Society of Echocardiography
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