keyword
https://read.qxmd.com/read/38536194/-pregnancy-management-in-a-patient-with-long-qt-syndrome-type-2-lqt2-carrier-of-the-variant-kcnh2-trp100x
#1
JOURNAL ARTICLE
Greta Hametner, Martin Osranek, Corinna Brunckhorst, Argelia Medeiros Domingo
Long QT syndrome (LQTS) is a congenital ion channel disorder causing prolonged ventricular repolarization and presents on surface ECG with a prolonged QTc interval. This condition predisposes to ventricular arrhythmias and also sudden cardiac death. LQTS without appropriate therapy during pregnancy and the postnatal phase poses an additionally increased risk of sudden cardiac death due to physiological changes associated with gestation. We present a case report of a 30-year-old pregnant woman with known long QT syndrome Type 2 (LQT2) and discuss the management in cardiological practice...
February 2024: Praxis
https://read.qxmd.com/read/38535107/objective-quantification-of-bilateral-bubble-contrast-echocardiography-correlates-with-systemic-oxygenation-in-patients-with-single-ventricle-circulation
#2
JOURNAL ARTICLE
Ashley Phimister, Chana Bushee, Monica Merbach, Sai Alekha Challa, Amy Y Pan, Andrew D Spearman
Bubble contrast echocardiography is commonly used to diagnose pulmonary arteriovenous malformations (PAVMs) in single ventricle congenital heart disease (CHD), yet previous studies inconsistently report a correlation between bubble echoes and oxygenation. In this study, we sought to re-evaluate the correlation between bubble echoes and oxygenation by assessing total bilateral shunting and unilateral shunting. We conducted a single-center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent a cardiac catheterization and bubble echocardiogram during the same procedure from 2011 to 2020...
March 1, 2024: Journal of Cardiovascular Development and Disease
https://read.qxmd.com/read/38532336/pathfinder-chd-prospective-registry-on-adults-with-congenital-heart-disease-abnormal-ventricular-function-and-or-heart-failure-as-a-foundation-for-establishing-rehabilitative-prehabilitative-preventive-and-health-promoting-measures-rationale-aims-design-and
#3
JOURNAL ARTICLE
Sebastian Freilinger, Harald Kaemmerer, Robert D Pittrow, Stefan Achenbach, Stefan Baldus, Oliver Dewald, Peter Ewert, Annika Freiberger, Matthias Gorenflo, Frank Harig, Christopher Hohmann, Stefan Holdenrieder, Jürgen Hörer, Michael Huntgeburth, Michael Hübler, Niko Kohls, Frank Klawonn, Rainer Kozlik-Feldmann, Renate Kaulitz, Dirk Loßnitzer, Friedrich Mellert, Nicole Nagdyman, Johannes Nordmeyer, Benjamin A Pittrow, Leonard B Pittrow, Carsten Rickers, Stefan Rosenkranz, Jörg Schelling, Christoph Sinning, Mathieu N Suleiman, Yskert von Kodolitsch, Fabian von Scheidt, Ann-Sophie Kaemmerer-Suleiman
BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD...
March 26, 2024: BMC Cardiovascular Disorders
https://read.qxmd.com/read/38531747/perioperative-sildenafil-therapy-in-pulmonary-hypertension-associated-with-congenital-cardiac-disease-an-updated-meta-analysis
#4
REVIEW
Shajie Ur Rehman Usmani, Syed Umar Hasan, Syed Hasan Ali, Syeda Hoorulain Ahmed, Neha Pervez, Muhammad Danial, Owais Khan, Dominique L Tucker, M Mujeeb Zubair
To provide an updated meta-analysis to evaluate the efficacy and safety of sildenafil on pediatric patients with pulmonary hypertension (PH) associated with congenital heart disease (CHD). To assess the efficacy and safety of sildenafil, five outcomes, time duration of post-operative need for mechanical ventilation, time duration of post-operative ICU stay, length of hospitalization (LOH), the incidence of mortalities and pulmonary arterial pressure to aortic pressure ratio (PAP/AoP) were regarded as primary efficacy outcomes...
March 25, 2024: Asian Journal of Surgery
https://read.qxmd.com/read/38531627/variant-characterisation-and-clinical-profile-in-a-large-cohort-of-patients-with-ellis-van-creveld-syndrome-and-a-family-with-weyers-acrofacial-dysostosis
#5
JOURNAL ARTICLE
Umut Altunoglu, Adrian Palencia-Campos, Nilay Güneş, Gozde Tutku Turgut, Julian Nevado, Pablo Lapunzina, Maria Valencia, Asier Iturrate, Ghada Otaify, Rasha Elhossini, Adel Ashour, Asmaa K Amin, Rania F Elnahas, Elisa Fernandez-Nuñez, Carmen-Lisset Flores, Pedro Arias, Jair Tenorio, Carlos Israel Chamorro Fernández, Yeliz Güven, Elif Özsu, Beray Selver Eklioğlu, Marisol Ibarra-Ramirez, Birgitte Rode Diness, Birute Burnyte, Houda Ajmi, Zafer Yüksel, Ruken Yıldırım, Edip Ünal, Ebtesam Abdalla, Mona Aglan, Hulya Kayserili, Beyhan Tuysuz, Victor Ruiz-Pérez
BACKGROUND: Ellis-van Creveld syndrome (EvC) is a recessive disorder characterised by acromesomelic limb shortening, postaxial polydactyly, nail-teeth dysplasia and congenital cardiac defects, primarily caused by pathogenic variants in EVC or EVC2 . Weyers acrofacial dysostosis (WAD) is an ultra-rare dominant condition allelic to EvC. The present work aimed to enhance current knowledge on the clinical manifestations of EvC and WAD and broaden their mutational spectrum. METHODS: We conducted molecular studies in 46 individuals from 43 unrelated families with a preliminary clinical diagnosis of EvC and 3 affected individuals from a family with WAD and retrospectively analysed clinical data...
March 26, 2024: Journal of Medical Genetics
https://read.qxmd.com/read/38531618/high-sensitive-troponint-interleukin-8-and-interleukin-6-link-with-post-surgery-risk-in-infant-heart-surgery
#6
JOURNAL ARTICLE
Elin M Thorlacius, Juho Keski-Nisula, Maria Vistnes, Tiina Ojala, Mattias Molin, Mats Synnergren, Birgitta S Romlin, Sven-Erik Ricksten, Håkan Wåhlander, Albert Gyllencreutz Castellheim
BACKGROUND: This study focuses on biomarkers in infants after open heart surgery, and examines the association of high-sensitive troponin T (hs-cTnT), interleukin-6 (IL-6), and interleukin-8 (IL-8) with postoperative acute kidney injury (AKI), ventilatory support time and need of vasoactive drugs. METHODS: Secondary exploratory study from a double-blinded clinical randomized trial (Mile-1) on 70 infants undergoing open heart surgery with cardiopulmonary bypass (CPB)...
March 26, 2024: Acta Anaesthesiologica Scandinavica
https://read.qxmd.com/read/38530796/automated-isochronal-late-activation-mapping-for-substrate-characterization-in-patients-with-repaired-tetralogy-of-fallot
#7
JOURNAL ARTICLE
Eduardo Arana-Rueda, Juan Acosta, Manuel Frutos-López, Juan-Antonio Sánchez-Brotons, Carmen González de la Portilla-Concha, Pastora Gallego, Alonso Pedrote
AIMS: Slow conduction (SC) anatomical isthmuses (AIs) are the dominant substrate for monomorphic ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTF). This study aimed to evaluate the utility of automated propagational analysis for the identification of SC-AI in patients with rTF. METHODS AND RESULTS: Consecutive rTF patients undergoing VT substrate characterization were included. Automated isochronal late activation maps (ILAM) were obtained with multielectrode HD Grid Catheter...
March 1, 2024: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://read.qxmd.com/read/38530365/rab35-is-required-for-embryonic-development-and-kidney-and-ureter-homeostasis-through-regulation-of-epithelial-cell-junctions
#8
JOURNAL ARTICLE
Kelsey R Clearman, Napassawon Timpratoom, Dharti Patel, Addison B Rains, Courtney J Haycraft, Mandy J Croyle, Jeremy F Reiter, Bradley K Yoder
BACKGROUND: Rab35 is a member of a GTPase family of endocytic trafficking proteins. Studies in cell lines have indicated that Rab35 participates in cell adhesion, polarity, cytokinesis, and primary cilia length and composition. Additionally, sea urchin Rab35 regulates actin organization and is required for gastrulation. In mice, loss of Rab35 in the CNS disrupts hippocampal development and neuronal organization. Outside of the CNS, the functions of mammalian Rab35 in vivo are unknown...
March 26, 2024: Journal of the American Society of Nephrology: JASN
https://read.qxmd.com/read/38530019/congenital-cardiac-anomalies-in-non-syndromic-cleft-lip-and-cleft-palate-patients-a-systematic-review-and-meta-analysis
#9
JOURNAL ARTICLE
Bindey Kumar, Priyankar Singh, Alok Ranjan, Tulika Singh, Nimmi Singh, Kriti, Swati Singh, Siddharth Singh, Navin Mishra, Arbind Kumar Sharma
The aim was to establish a specific and definite connection between non-syndromic orofacial cleft patients and associated congenital heart disease (CHD). Following PRISMA guidelines, selective databases were searched for data collection. Studies showing a definite association of CHD with orofacial cleft were included, and studies non-specific of the association of orofacial cleft with CHD were excluded. Data extraction criteria were study design, frequency of CHD in overall non-syndromic orofacial cleft and in specific cleft type, and most prevalent congenital cardiac anomaly...
March 26, 2024: Congenital Anomalies
https://read.qxmd.com/read/38529652/diagnostic-strategy-for-suspected-unilateral-absence-of-the-pulmonary-artery
#10
JOURNAL ARTICLE
Van Luong Hoang, Viet Anh Lam, Thanh Nguyen Pham
UNLABELLED: <p>Background: Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly. OBJECTIVE: To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam. METHODS: All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue...
March 19, 2024: Current medical imaging
https://read.qxmd.com/read/38528794/bicuspidisation-of-unicuspid-stenotic-pulmonary-valve-in-a-nine-year-old-male
#11
JOURNAL ARTICLE
Florin Anghel, Catalin Constantin Badiu, Alain J Poncelet
We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure...
March 26, 2024: Cardiology in the Young
https://read.qxmd.com/read/38527508/-diagnosis-and-treatment-of-arrhythmogenic-cardiomyopathy-in-children
#12
JOURNAL ARTICLE
W Q Ye, Y Y Xiao, X K Jiang, M Jin, X F Wang, W W Ding
Objective: To summarize the clinical manifestations, experiences in diagnosis and treatment of arrhythmogenic cardiomyopathy (ACM) in children. Methods: A retrospective analysis of the clinical manifestations, laboratory tests, radiological features, treatment and follow-up results was conducted in 11 children diagnosed with ACM at the center of congenital heart disease, Beijing anzhen hospital from May 2010 to March 2022. Results: A total of 11 patients aged 2 to 16 years, including 5 males and 6 females were diagnosed with ACM...
March 25, 2024: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://read.qxmd.com/read/38526305/bovine-aortic-arch-with-an-aberrant-left-vertebral-artery-in-a-3-year-old-boy-with-vacterl-association-a-case-report
#13
JOURNAL ARTICLE
Jesús Martínez-García, Sara F Ordorica-Sandoval, Erick Rivera-Sainz, Manuel A Beltrán-Salas, Nidia León-Sicairos, Adrian Canizalez-Roman
BACKGROUND The VACTEREL association is an acronym that includes vertebral malformations (V), anal atresia (A), cardiac defects (C), tracheoesophageal fistula (TE), renal defects (R), and limb malformations (L). The aortic arch is the section between the ascending aorta and the descending aorta, where some variants have been described, such as the right aortic arch and bovine aortic arch, among others. A rare presentation in the Natsis classification is the "type X" where a bovine aortic arch and anomalous origin of the left vertebral artery are present...
March 11, 2024: American Journal of Case Reports
https://read.qxmd.com/read/38524995/percutaneous-balloon-dilation-of-the-pulmonary-valve-in-a-79-year-old-with-congenital-pulmonary-valve-stenosis
#14
Matthew C Schwartz, Jorge Alegria, Joseph Paolillo, Markus Scherer
• Congenital valvar PS is a rare cause of heart failure in the geriatric population. • Transcatheter PV dilation is an effective treatment of congenital PS in older adults. • Cardiac CT can be very useful in the evaluation of suspected valvar PS in adults.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524992/a-tale-of-three-chambers-cor-triatriatum-sinistrum
#15
Maliha Butt, Husban Halim, Ataul Qureshi, Ryan Koene, Su Aung, Tara Karamlou, Ashish Saini
• Cor triatriatum is an important cause of HF and atrial arrhythmias in young adults. • Misdiagnosis can cause inappropriate interventions and delay in surgical treatment. • Integrated imaging is useful in describing anatomy and associated abnormalities. • Collaboration between hospitals and cardiac specialties helps prevent poor outcomes.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524991/pulmonary-veins-not-always-where-you-expect-them
#16
Kathryn I Sunthankar, Angela J Weingarten, Thomas P Doyle, George T Nicholson, Sudeep D Sunthankar
• Multimodality imaging is important in patients with ACHD and challenging anatomy. • Complex congenital anatomy and physiology requires multidisciplinary discussions. • Image-guided percutaneous intervention may be warranted after TAPVR repair.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524988/percutaneous-closure-of-a-ruptured-sinus-of-valsalva-aneurysm-under-transesophageal-echocardiography-guidance
#17
Xi E Li, Doff B McElhinney, George K Lui, Daniel E Clark, Jennifer P Woo
• SOVA is a rare cardiac anomaly. • Ruptured SOVA carries a high mortality rate. • SOVA often coexists with other congenital lesions, most commonly VSD and bicuspid AV. • Ruptured SOVA needs repair; percutaneous repair is a safe alternative to surgery. • Echo plays a vital role in both diagnosing SOVA and guiding percutaneous closure.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524987/double-chambered-right-ventricle-in-adulthood-a-case-series
#18
Richard Joseph Malone, Everett Randall Henderson, Zachary Ryan Wilson, Matthew R McMullan, Thomas N Skelton, William F Campbell, Michael R McMullan
• DCRV is a rare cardiac anomaly leading to subvalvular RVOT obstruction. • DCRV is most frequently found in children but can occur in adults. • Adults with DCRV are often initially misdiagnosed. • Echocardiography is the first-line imaging modality to visualize this anomaly. • Providers should be aware of this condition and the associated image findings.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524979/double-aortic-arch-with-ligamentous-remnant-of-left-arch-a-rare-cause-of-dysphagia-in-an-adult
#19
Muhammad Najih Liaqath Ali, Samantha Fitzsimmons, James Shambrook, Reginald Ragunathan Iruthayanathan
• Thoracic vascular rings are congenital anomalies with varied symptomatology. • Suspect and rule out vascular ring in unexplained upper airway/esophageal obstruction. • Include additional AA and pulmonary branch views in ACHD echo protocol. • Cross-sectional cardiac imaging helps in unambiguous surgical planning.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524968/large-secundum-atrial-septal-defect-managed-conservatively-in-an-adult-dog
#20
Carl Toborowsky, Lauren E Markovic
• ASDs can be medically managed in dogs if interventional options are not selected. • 3D modeling can be used for canine congenital heart disease. • 3D imaging helps with preprocedural planning for ASD.
March 2024: CASE: Cardiovascular Imaging Case Reports
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