keyword
https://read.qxmd.com/read/38213379/late-onset-22q11-2-deletion-syndrome-with-mild-cardiac-phenotype-a-unique-adult-presentation-diagnosed-at-45-years-of-age
#21
Anasofia Elizondo-Plazas, Graciela Areli Lopez-Uriarte, Jose Gerardo Gonzalez-Gonzalez, Rene Rodriguez-Gutierrez, Laura Martinez-Villarreal, Angel Sebastian Trevino-Juarez, Camilo Daniel Gonzalez-Velazquez
This case report presents a detailed exploration of an adult-onset 22q11 deletion syndrome, a rare genetic disorder typically diagnosed in children. The report highlights the diagnostic challenges posed by this atypical presentation, emphasizing the need for clinicians to consider such conditions in differential diagnoses, especially in adults. This case is remarkable for its late onset and mild symptoms, which significantly deviated from the common pediatric presentation, including hypocalcemia due to hypoparathyroidism and a fenestrated atrial septal defect without significant hemodynamic implications...
December 2023: Curēus
https://read.qxmd.com/read/38166574/major-pneumothorax-during-pediatric-cardiac-mri-procedure-under-general-anesthesia-step-by-step-analysis-and-importance-of-a-well-known-environment-and-material
#22
JOURNAL ARTICLE
Quentin Delhez, Laurent Bairy, John Mitchell, Adrien Maseri
BACKGROUND: To perform step-by-step analysis of the different factors (material, anesthesia technique, human, and location) that led to major pneumothorax during an infrequent pediatric cardiac MRI and to prevent its occurrence in the future. Anesthesia equipment used in a remote location is often different than those in operating rooms. For magnetic resonance imaging (MRI), ventilation devices and monitors must be compatible with the magnetic fields. During cardiac MRI numerous apneas are required and, visual contact with the patient is limited for clinical evaluation...
January 2, 2024: BMC Anesthesiology
https://read.qxmd.com/read/38162156/wolf-hirschhorn-syndrome-with-hyperparathyroidism-a-case-report-and-a-narrative-review-of-the-literature
#23
JOURNAL ARTICLE
Changqing Xia, Dibyendu Kumar, Bei You, Deanna L Streck, Lisa Osborne, James Dermody, Jie-Gen Jiang, Beth A Pletcher
Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion condition. The WHS core phenotype includes developmental delays, intellectual disabilities, seizures, and distinctive facial features. Various other comorbidities have also been reported, such as hearing loss, heart defects, as well as eye problems and kidney problems. In this report, we present a case of WHS accompanied by hyperparathyroidism and hypercalcemia, which has not been previously reported. A girl was born at 37 weeks of gestation by vaginal delivery...
December 2023: Journal of Pediatric Genetics
https://read.qxmd.com/read/38125818/the-risk-of-pediatric-cardiovascular-diseases-in-offspring-born-to-mothers-with-systemic-lupus-erythematosus-a-nationwide-study
#24
JOURNAL ARTICLE
Jong Ho Cha, Jae Kyoon Hwang, Young-Jin Choi, Jae Yoon Na
BACKGROUND: Systemic lupus erythematosus (SLE), a common autoimmune disease predominantly affecting women, has been linked to various complications during pregnancy. The transfer of anti-Ro/SSA antibodies from SLE-affected mothers to their offspring can lead to neonatal lupus and cardiac issues. This study investigated the association between maternal SLE and the risk of pediatric cardiovascular disorders. METHODS: The study utilized South Korea's National Health Insurance Service (NHIS) database, covering 3,505,737 children born between 2007 and 2017 and tracked until 2020...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/38099635/ecpr-for-prolonged-pediatric-cardiac-arrest-an-outcome-without-major-neurological-compromise
#25
Laura Gutiérrez-Soriano, Eduardo Becerra Zapata, Nicolas Maya Trujillo, German Andres Franco Gruntorad, Pedro Hurtado Peña
Pediatric in-hospital cardiac arrest (IHCA) has been reported in 1-3% of pediatric intensive care unit (ICU) admissions and up to 6% of children admissions to the cardiac ICU. In the last 25 years, the survival to hospital discharge after pediatric IHCA has improved from 9% to 13.7% up to 35%. The improvement in outcomes was attributed in part to the application of ECMO as a rescue strategy when prolonged conventional CPR cannot restore spontaneous circulation. We report a case of a 4-month-old patient with a history of ventricular and septal defects, with left to right shunt and enlargement of left heart chambers that underwent surgery for the closure of the atrial and septal defects, and experienced complications that led to the use of ECMO in response to a prolonged cardiac arrest...
December 2023: Journal of Extra-corporeal Technology
https://read.qxmd.com/read/38094186/case-report-a-novel-combination-of-anomalies-in-a-patient-with-22q11-2-deletion-syndrome
#26
Connor Byeman, Ravi Ashwath
A frequently occurring genetic disorder, 22q11.2 deletion syndrome can manifest with various abnormalities. The range of cardiac anomalies associated with this syndrome is extensive, with conotruncal defects being the most prevalent. In this study, we report the case of a patient with a unique combination of anatomical abnormalities such as crisscross pulmonary arteries, a cervical aortic arch with coarctation of the aorta, and a ventricular septal defect. The patient underwent initial surgical intervention, which resulted in significant clinical improvement...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/38088201/an-8-year-old-boy-with-scimitar-syndrome-presentation-with-gastroesophageal-reflux-symptoms-and-concurrent-associations-including-ectopic-herniated-liver
#27
JOURNAL ARTICLE
Kadir Han Alver, Zozan Ayaz, Muhammed Tekinhatun
No abstract text is available yet for this article.
December 13, 2023: Pediatric Pulmonology
https://read.qxmd.com/read/38074278/pattern-of-congenital-heart-defects-among-children-in-jazan-region-saudi-arabia-a-five-year-hospital-based-study
#28
JOURNAL ARTICLE
Abdellh A Names, Ahmad Y Alqassim, Mohammad A Jareebi, Mohammed A Aldhamdi, Alaa A Abutaleb, Abdulaziz A Alwani, Mohammed O Shami, Ghadeer A Alneel, Abdurahman H Alhazmi, Hassan M Gohal, Basel A Hadad, Hamad M Alaksham
BACKGROUND: Congenital heart defects (CHDs) are the leading cause of death and morbidity in children under one year around the world. This study explores the prevalence, patterns, and interventions of congenital heart defects in children in the Jazan region, Saudi Arabia. METHODS: This was a descriptive cross-sectional study, conducted in the Jazan region, Saudi Arabia. We looked back at all of the CHD patients who went to the two tertiary hospitals in the Jazan region between January 2017 and December 2021 and collected and analyzed their background information and diagnosis...
October 2023: Journal of Family Medicine and Primary Care
https://read.qxmd.com/read/38059228/neurodevelopmental-assessment-of-children-with-congenital-heart-diseases-using-trivandrum-developmental-screening-chart
#29
JOURNAL ARTICLE
Punam Uke, Sarika Bhimrao Gaikwad, Keerti Swarnkar, Varsha Lamture, Prashant Khartade
OBJECTIVES: Congenital heart diseases (CHDs) are one of the most important congenital anomalies in children which have high-risk for neurodevelopment delay. This study was conducted to determine the proportion of developmental delay in children with CHD and comparison of delay between acyanotic and cyanotic heart diseases in children. MATERIALS AND METHODS: A cross-sectional study was conducted on children admitted in pediatric ward of rural hospital from 6 month to 6 years of age who are diagnosed with CHD by 2D ECHO and further classified into acyanotic congenital heart disease (ACHD) and cyanotic congenital heart disease (CCHD)...
2023: Journal of Neurosciences in Rural Practice
https://read.qxmd.com/read/38024556/maximum-surgical-blood-ordering-schedule-in-common-pediatric-cardiac-surgeries-in-a-tertiary-center
#30
JOURNAL ARTICLE
Maryam Sotoudeh Anvari, Seyedeh Zohreh Hashemi, Mohammadreza Mirzaaghayan, Alireza Abdollahi, Mohammad Taghi Haghi Ashtiani, Abbas Akbari
BACKGROUND & OBJECTIVE: Unnecessary pre-operative ordering of red blood cells (RBCs) in elective surgeries increases costs and waste of blood inventory. Maximum surgical blood order schedule (MSBOS) is a helpful strategy in the estimation of blood units needed for surgery and the prevention of overconsumption. In this study, an MSBOS for pediatric cardiac surgeries is designed. METHODS: In this cross-sectional study, we included all pediatric patients who underwent elective cardiac surgery in Children's Medical Center in Tehran, Iran, from March 21, 2019, to September 22, 2019...
2023: Iranian Journal of Pathology
https://read.qxmd.com/read/37998510/minimally-invasive-surgery-for-simple-congenital-heart-defects-preserving-aesthetics-without-jeopardizing-patient-safety
#31
JOURNAL ARTICLE
Mauro Lo Rito, Ylenia Claudia Maria Brindicci, Mario Moscatiello, Alessandro Varrica, Matteo Reali, Antonio Saracino, Massimo Chessa, Tommaso Aloisio, Giuseppe Isgrò, Alessandro Giamberti
Minimally invasive surgeries for pediatric patients have been proposed for decades, with different approaches in mind. Minimal right axillary thoracotomy (MRAT), proposed two decades ago, allows the preservation of patients' safety alongside faster aesthetic and functional recovery. The MRAT did not become widely adopted due to the prejudice that to follow a minimally invasive approach, safety and efficacy must be compromised. With this study, we aim to compare MRAT to the standard median sternotomy approach with a focus on safety and clinical outcomes...
November 6, 2023: Journal of Cardiovascular Development and Disease
https://read.qxmd.com/read/37984644/impact-of-gore-cardioform-asd-occluder-on-atrial-and-ventricular-electro-mechanics-in-a-pediatric-population
#32
JOURNAL ARTICLE
Biagio Castaldi, Giuseppe Santoro, Angela Di Candia, Pietro Marchese, Massimiliano Cantinotti, Alessandra Pizzuto, Silvia Scalera, Silvia Garibaldi, Jennifer Fumanelli, Domenico Sirico, Giovanni Di Salvo
BACKGROUND: Transcatheter closure is the first-line treatment for ostium secundum atrial septal defect (ASD). The GORE® Cardioform ASD Occluder (GCA) is potentially innovative compared to other self-centering devices. The aim of this study was to compare the mechanical changes in atrial and ventricular properties before and after GCA implantation. METHODS: All consecutive patients aged <18 years performing isolated ASD closure with a single GCA device were enrolled from two centers...
November 18, 2023: American Journal of Cardiology
https://read.qxmd.com/read/37965404/the-burden-of-neonatal-referrals-on-a-pediatric-cardiology-service-a-local-center-experience
#33
JOURNAL ARTICLE
Walaa S Al Maddallah, Yasser A Bhat, Abdulrahman Al Mesned, Abdullah Al Qwaee, Mohammad Ahmad Hassan, Ali Al Akhfash
BACKGROUND: Congenital heart disease (CHD) is a common occurrence in live births, with some exhibiting critical congenital heart disease; therefore, cardiology services should be available around the clock to ensure timely diagnosis and management. This study aims to describe the workload and the need for pediatric cardiac services in a maternity hospital for newborn referrals. Moreover, the study describes the indications for neonatal cardiology consultations. METHODS: The prospective cohort study was conducted over four months, from January to April 2022, in the Prince Sultan Cardiac Center Al Qassim region of Saudi Arabia...
October 2023: Curēus
https://read.qxmd.com/read/37937527/comparison-of-ultrafast-and-fast-track-extubation-after-secundum-atrial-septal-defect-surgery-in-pediatric-age-group
#34
JOURNAL ARTICLE
Salih Özçobanoğlu, Emel Gündüz, Nazan Ülgen Tekerek
BACGROUND: Patients who underwent ultrafast track on the operating table and fast track extubation in the pediatric intensive care unit for 2 to 6 hours after secundum atrial septal defect surgery in the pediatric age group were compared. METHODS: Between January 2013 and February 2017, 60 pediatric patients (24 boys, 36 girls; Mean age 7.5 ± 4.6 years) whose secundum atrial defect was closed were retrospectively analyzed. The patients were separated as those who were extubated on the operating table (Group1,n = 28) and those extubated in the pediatric intensive care unit within 2-6 hours postoperatively (Group2,n = 32)...
November 8, 2023: Acta Chirurgica Belgica
https://read.qxmd.com/read/37927770/crochetage-the-forgotten-electrocardiographic-sign
#35
Carol Fernandez Hazim, Mohammed Shaban, Dessiree Cordero, Ana P Urena Neme, Miguel A Rodriguez Guerra
Acquired complete heart block is a rare but severe arrhythmia caused by various factors such as infections, medications, and autoimmune conditions. Atrial septal defect (ASD) is a common congenital heart defect, with larger ASDs possibly causing symptoms such as fatigue, shortness of breath, and frequent respiratory infections. In some cases, high-grade atrioventricular block with ASD can occur; however, the exact incidence is not well established. We report a rare case of a 15-year-old male presenting with acute dizziness...
October 2023: Curēus
https://read.qxmd.com/read/37924347/cardiovascular-anomalies-in-patients-with-tessier-syndrome-a-systematic-review
#36
REVIEW
Shiva Nazari, Ali Vaezi, Shervin Mossavarali, Kimia Ghanavati, Akbar Shafiee
Tessier clefts are skeletal and soft tissue abnormalities of a neonate's facial structures. They could be classified as syndromic and non-syndromic clefts, which can be attributed to disruptions in fetal development and genetic mutations, respectively. Reported cases of these clefts typically document the presence of additional abnormalities associated with these clefts. In this systematic review, we analyzed reports of Tessier clefts accompanied by cardiovascular anomalies, as one of the commonly encountered anomalies...
November 4, 2023: European Journal of Pediatrics
https://read.qxmd.com/read/37914855/electrocardiographic-characteristics-in-438-neonates-with-atrial-septal-defects
#37
JOURNAL ARTICLE
Anna Maria Dehn, Maria Munk Pærregaard, Anna Sellmer, Sofie Dannesbo, Elisabeth Blixenkrone-Møller, Anne-Sophie Sillesen, Anna Axelsson Raja, Kasper Karmark Iversen, Henning Bundgaard, Alex Hørby Christensen, Vibeke Hjortdal
Arrhythmias and electrocardiographic (ECG) abnormalities are common among patients with atrial septal defects (ASDs). We studied a large cohort of neonates with ASDs to investigate whether ECG abnormalities are present at this early stage or develop later, secondary to hemodynamic changes. We analyzed the echocardiograms and ECGs from the Copenhagen Baby Heart Study, a population-based cohort study. We compared ECG characteristics of 438 neonates with secundum ASDs to 1314 matched controls. In subgroup analyses, we investigated whether electrocardiographic characteristics were associated with age at examination...
March 2024: Pediatric Cardiology
https://read.qxmd.com/read/37876958/fibronectin-enhances-attachment-of-human-adipose-derived-mesenchymal-stem-cells-into-polytetrafluoroethylene-patch-during-surgical-closure-of-the-atrial-and-ventricular-septal-defect
#38
JOURNAL ARTICLE
I Gde Rurus Suryawan, Anudya Kartika Ratri, Andrianto Andrianto, Meity Ardiana, Ricardo Adrian Nugraha
BACKGROUND: Polytetrafluoroethylene (PTFE) patch is commonly used during surgical closure for atrial septal defect (ASD) and ventricular septal defect (VSD). However, this patch has several limitations such as its inability to grow or remodel, especially in children and young adults. To tackle these limitations, we have tried to use fibronectin and human adipose-derived mesenchymal stem cells (hAMSCs) in the PTFE patch. OBJECTIVE: To understand the impact of fibronectin to enhance hAMSCs cell-to-cell adherence and cell-to-patch surface attachment into PTFE patches used in the surgical closure of ASD or VSD...
2023: Annals of Pediatric Cardiology
https://read.qxmd.com/read/37868716/atrial-septal-defect-closure-is-associated-with-improved-clinical-status-in-patients-%C3%A2-10%C3%A2-kg-with-bronchopulmonary-dysplasia
#39
JOURNAL ARTICLE
Melissa K Webb, Milenka Cuevas Guaman, S Kristen Sexson Tejtel, Neil Cambronero, Ryan D Coleman, Corey A Chartan, Betul Yilmaz Furtun, Shaine A Morris, Nidhy P Varghese, Natalie M Villafranco
Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post-ASD closure improvement is secondary to the expected course in BPD patients or related to the closure itself. A novel BPD-ASD score was created to assess patients' clinical status (higher score = worse disease) and applied to 10 BPD-ASD inpatients weighing ≤ 10 kg who underwent ASD closure. The score and its subcomponents were retrospectively calculated serially ranging from 8 weeks pre- to 8 weeks post-intervention, and pre- and post-intervention score slopes were created...
October 2023: Pulmonary Circulation
https://read.qxmd.com/read/37849572/anesthetic-management-of-inguinal-hernia-surgery-using-a-second-generation-supraglottic-airway-in-a-patient-with-trisomy-18-a-case-report
#40
Shintaro Akimaru, Toshiyuki Nakanishi, Tatsuya Hasegawa, Kazuya Sobue
Children with trisomy 18 have abnormal airway anatomy, making their airway management challenging. Only a few papers have comprehensively described and discussed the use of supraglottic airway devices in patients with trisomy 18. We present a case of a 20-month-old boy with trisomy 18 who was scheduled for open repair of the right inguinal hernia. He had micrognathia, a short neck, and an atrial septal defect but was in a clinically stable condition. A supraglottic airway device was inserted under general anesthesia...
September 2023: Curēus
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