Coronary pathology of inherited generalized arterial calcification of infancy; a case report
Generalized arterial calcification of infancy (GACI), or idiopathic infantile arterial calcification, is a rare autosomal-recessive disease recognized as an inherited disorder characterized by severe pathologic calcification of large- and medium-sized arteries accompanied by smooth muscle cell (SMC) hyperplasia leading to vascular obstruction1. The prognosis is extremely poor with 85% of affected infants dying within the first 6months of life. Loss of function mutations in the ectonucleotide pyrophosphatase phosphodiesterase 1 (ENPP1) gene is recognized as the main defect associated with GACI [1]. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - May 18, 2018 Category: Cardiology Authors: Duccio Federici, Sho Torii, Matteo Ciuffreda, Lorenzo Galletti, Luca Lorini, Ezio Bonanomi, Andrea Gianatti, Maria Iascone, Joohyung Park, Liang Guo, Maria E. Romero, Frank D. Kolodgie, Giulio Guagliumi, Renu Virmani Tags: Case Report Source Type: research
Comparing fist size to heart size is not a viable technique to assess cardiomegaly
Several medical textbooks state that a human heart is approximately the size of that person's fist. Stating that a heart has the size of the corpse's fist is thought to signify that the heart size is normal. We formulate two hypotheses that are tested in this article. First, we hypothesize that in cases without cardiomegaly, volumes of the hand and the heart are not significantly different. Second, we hypothesize that in cases of cardiomegaly, the heart volume statistically significantly exceeds that of the hand. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - May 7, 2018 Category: Cardiology Authors: Garyfalia Ampanozi, Eileen Krinke, Patrick Laberke, Wolf Schweitzer, Michael J. Thali, Lars C. Ebert Tags: Original article Source Type: research
WITHDRAWN: PKP2 as a main cause of Arrhythmogenic right ventricular dysplasia in Iran: An update of the mutations around the world
This article has been withdrawn at the request of the author. The Publisher apologizes for any inconvenience this may cause. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - May 5, 2018 Category: Cardiology Authors: Nejat Mahdieh, Nasim Najafi, Mahdieh Soveizi, Sedigheh Saeidi, Fereidoon Noohi, Majid Maleki, Bahareh Rabbani Source Type: research
PKP2 as a main cause of Arrhythmogenic right ventricular dysplasia in Iran: An update of the mutations around the world
PKP2 mutations are the main cause of ARVC/D. Plakophilin-2 is a component of desmosomes and important for myocyte interactions. Here, genetic study of Iranian patients with ARVC/D is presented for the first time. An update of PKP2 mutations and in silico analyses of the reported variants are also presented.Clinical evaluations, pedigree diagram, 2-dimentional echocardiography, 12 lead ECG, signal averaged ECG and genetic investigations were performed for enrolled patients. A comprehensive literature survey of the previously reported mutation was conducted to present an update of PKP2 mutations. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - May 5, 2018 Category: Cardiology Authors: Nejat Mahdieh, Nasim Najafi, Mahdieh Soveizi, Sedigheh Saeidi, Fereidoon Noohi, Majid Maleki, Bahareh Rabbani Source Type: research
Editorial Board
(Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - May 1, 2018 Category: Cardiology Source Type: research
Table of Contents/Barcode
(Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - May 1, 2018 Category: Cardiology Source Type: research
Comparing coronary artery fibromuscular dysplasia with coronary atherosclerosis: from clinical to histopathological characteristics
Fibromuscular dysplasia(FMD) is a non-inflammatory and non-atherosclerotic disease that leads to aneurysm, dissection, and vascular occlusion. FMD is commonly involved with renal artery (most commonly) and internal carotid artery, while the vertebral artery, iliac artery, subclavian artery, and visceral artery are seldom involved. Patients with FMD are usually young women [1]. Although the first case was reported 75years ago, the knowledge of FMD remains insufficient [2]. Genetic factors have been found associated with this disease, for example, the phosphatase and actin regulator 1 (PHACTR1) is related to FMD in some fami...
Source: Cardiovascular Pathology - April 29, 2018 Category: Cardiology Authors: Xiumeng Hua, Xiao Chen, Kai Chen, Songqing Lai, Ran Gao, Yiqing Hu, Jiangping Song Source Type: research
A case of vascular Ehlers-Danlos Syndrome with a cardiomyopathy and multi-system involvement
We present an unusual case of vascular Ehlers-Danlos Syndrome with distinctive physical characteristics and a cardiomyopathy with features suggesting isolated left ventricular non-compaction. The cardiac features represent the first report of a cardiomyopathy associated with a mutation in the COL3A1 gene. This case also illustrates the multi-system nature of Ehlers-Danlos Syndrome and the complexity of managing patients with the vascular subtype. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 24, 2018 Category: Cardiology Authors: Nick Si Rui Lan, Michael Fietz, Nicholas Pachter, Vincent Paul, David Playford Tags: Clinical Case Report Source Type: research
A case of Vascular Ehlers-Danlos Sydrome with a cardiomyopathy and multi-system involvement
We present an unusual case of vascular Ehlers-Danlos Syndrome with distinctive physical characteristics and a cardiomyopathy with features suggesting isolated left ventricular non-compaction. The cardiac features represent the first report of a cardiomyopathy associated with a mutation in the COL3A1 gene. This case also illustrates the multi-system nature of Ehlers-Danlos Syndrome and the complexity of managing patients with the vascular subtype. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 24, 2018 Category: Cardiology Authors: Nick Si Rui Lan, Michael Fietz, Nicholas Pachter, Vincent Paul, David Playford Tags: Case Report Source Type: research
Molecular and cellular insights into the pathogenesis of coronary artery ectasia
Coronary artery ectasia describes a local or diffuse dilatation of the epicardial coronary arteries. This review summarizes the molecular and cellular mechanisms involved in the pathogenesis of coronary artery ectasia. Better identification of the pathophysiologic steps will shed light into the clinical significance and may have direct implications for the management strategies of this disease. Additionally, understanding the underlying etiology may help to improve treatment modalities specific to coronary artery ectasia. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 16, 2018 Category: Cardiology Authors: Selcuk Ozturk, Ertan Yetkin, Johannes Waltenberger Tags: Review article Source Type: research
Thromboembolic Findings in Patients with Heart Failure at Autopsy
The risk of thromboembolic events is increased in patients with heart failure (HF); however, few studies have reported thromboembolic findings in HF patients who have undergone autopsy. (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 13, 2018 Category: Cardiology Authors: Isabella Sales de Macedo, Layara Fernanda Lipari Dinardi, Thiago Vicente Pereira, Lyna Kyria Rodrigues de Almeida, Thaisa Silveira Barbosa, Luiz Alberto Benvenuti, Silvia Moreira Ayub-Ferreira, Edimar Alcides Bocchi, Victor Sarli Issa Tags: Original Article Source Type: research
Reasons for missing noncompaction in myopathies and vice versa
Feature article (Source: Cardiovascular Pathology)
Source: Cardiovascular Pathology - April 12, 2018 Category: Cardiology Authors: Josef Finsterer, Claudia St öllberger Tags: Original Article Source Type: research
