1,869 results match your criteria left-sided congenital

Uncrossed corticospinal tract in health and genetic disorders: Review, case report and clinical implications.

Eur J Neurol 2021 May 5. Epub 2021 May 5.

University Hospital of the LMU Munich, Department of Neurology, Marchioninistr. 15, 81377, Munich, Germany.

Background: Crossing pathologies of the corticospinal tract (CST) are rare and often associated with genetic disorders. However, they can be present in healthy humans and lead to ipsilateral motor deficits when a lesion to motor areas occurs. Here we review historical and current literature of CST crossing pathologies and present a rare case of asymmetric crossing of the CST. Read More

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Prognostic Factors in Recurrent Congenital Muscular Torticollis.

Malays Orthop J 2021 Mar;15(1):43-47

Department of Orthopaedic Surgery, Faculty of Medicine Siriraj Hospital, Bangkok, Thailand.

Introduction: Congenital muscular torticollis (CMT), primarily resulting from unilateral shortening and fibrosis of the sternocleidomastoid muscle. One of the common surgical complications is recurrent deformity. However, the associations between unipolar or bipolar release, age of the patient, and the recurrence of the disease are unclear. Read More

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Thoracoscopic repair of congenital diaphragmatic hernia in neonates: findings of a multicenter study in Japan.

Surg Today 2021 Apr 20. Epub 2021 Apr 20.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.

Purpose: We compared the efficacy of thoracoscopic repair (TR) with that of open repair (OR) for neonatal congenital diaphragmatic hernia (CDH).

Methods: The subjects of this multicenter retrospective cohort study were 524 infants with left-sided isolated CDH, diagnosed prenatally, and treated at one of 15 participating hospitals in Japan between 2006 and 2018. The outcomes of infants who underwent TR and those who underwent OR were compared, applying propensity score matching. Read More

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Predicting Language Outcome After Left Hemispherotomy: A Systematic Literature Review.

Neurol Clin Pract 2021 Apr;11(2):158-166

Department of Pediatric Neurology and Developmental Medicine (KL, MS), University Children's Hospital Tübingen, Germany; Clinic for Neuropediatrics and Neurorehabilitation (MS), Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany; Pediatric Neurology (KL), Inselspital University Children's Hospital, University of Bern, Switzerland; and Department of Neuropediatrics (SEB), University Children's Hospital Zurich, Switzerland.

Objective: Hemidecortication is a therapeutic option in patients with drug-resistant structural epilepsy. If surgery is performed early enough in left-hemispheric pathology, the plasticity of the developing brain may enable the right hemisphere to take over language-if this has not occurred before surgery. A systematic overview of potential predictors of language outcome after left hemidecortication in children is warranted. Read More

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Risk factors for mortality in infants with congenital diaphragmatic hernia: a single center experience.

Wien Klin Wochenschr 2021 Mar 30. Epub 2021 Mar 30.

Comprehensive Centre for Paediatrics, Division of Neonatology, Paediatric Intensive Care & Neuropaediatrics, Department of Paediatric and Adolescent Medicine, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.

Background: Despite current progress in research of congenital diaphragmatic hernia, its management remains challenging, requiring an interdisciplinary team for optimal treatment.

Objective: Aim of the present study was to evaluate potential risk factors for mortality of infants with congenital diaphragmatic hernia.

Methods: A single-center chart review of all patients treated with congenital diaphragmatic hernia over a period of 16 years, at the Medical University of Vienna, was performed. Read More

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Cervicothoracic Neurenteric Cyst With Contralateral Diaphragmatic Hernia: An Unusual Combination.

Pediatr Dev Pathol 2021 Mar 24:10935266211004253. Epub 2021 Mar 24.

Division of Newborn Medicine, Department of Pediatrics, University of Pittsburgh School of Medicine, UPMC Children's Hospital of Pittsburgh, UPMC Magee-Womens Hospital, Pittsburgh, Pennsylvania.

We describe a premature male infant who died from complications resulting from two malformations: a large left-sided diaphragmatic hernia and a right-sided cervicothoracic neurenteric cyst. The findings of the first limited prenatal ultrasound led to the incorrect diagnosis of right-sided diaphragmatic hernia. Vertebral anomalies, commonly associated with neurenteric cysts, and an intrathoracic stomach, were not identified until autopsy examination. Read More

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Reproducibility of Lung and Liver Volume Measurements on Fetal Magnetic Resonance Imaging in Left-Sided Congenital Diaphragmatic Hernia.

Fetal Diagn Ther 2021 23;48(4):258-264. Epub 2021 Mar 23.

Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA,

Introduction: Congenital diaphragmatic hernia (CDH) affects 1 in 3,000 live births and is associated with significant morbidity and mortality.

Methods: A review of fetal magnetic resonance imaging (MRI) examinations was performed for fetuses with left CDH and normal lung controls. Image review and manual tracings were performed by 4 pediatric radiologists; right and left lung volumes in the coronal and axial planes as well as liver volume above and below the diaphragm in the coronal plane were measured. Read More

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Splenogonadal fusion: aiding detection and avoiding radical orchidectomy.

Ann R Coll Surg Engl 2021 Mar 19. Epub 2021 Mar 19.

Chelsea and Westminster Hospital, London, UK.

Splenogonadal fusion is a rare benign congenital anomaly in which there is an abnormal connection between the gonad and the spleen. It was first described over 100 years ago with limited reports in the literature since then. Its similarity in presentation to testicular neoplasia poses a significant challenge in diagnosis and management, often resulting in radical orchidectomy. Read More

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Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia.

BMC Pediatr 2021 Mar 11;21(1):120. Epub 2021 Mar 11.

Departments of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.

Background: To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors.

Methods: Children with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Read More

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Recurrent congenital diaphragmatic hernia: A single center experience.

Pediatr Med Chir 2021 Feb 22;43(1). Epub 2021 Feb 22.

Department of Paediatric Surgery, Medical University of Warsaw.

Congenital Diaphragmatic Hernia (CDH) occurs in 1:4000 live births. It's morbidity and mortality rates are significant. Recurrence is one of the recognized complications occurring after a successful initial repair. Read More

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February 2021

Two cases of different genetic variants of alveolar capillary dysplasia associated with left-sided obstructive CHDs.

Cardiol Young 2021 Mar 5:1-3. Epub 2021 Mar 5.

Congenital Heart Center, Spectrum Health Helen DeVos Children's Hospital, Grand Rapids, MI, USA.

Alveolar capillary dysplasia with misalignment of the pulmonary veins is an uncommon disorder that affects the lung vasculature development in the neonatal period and leads to pulmonary hypertension. We describe two patients with alveolar capillary dysplasia associated with left-sided obstructive heart defects with two different genetic variants. Our cases highlight the importance of early recognition of this disease in the setting of persistent and supra-systemic pulmonary hypertension despite surgical correction of the associated lesions. Read More

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Congenital lateral facial teratoma: A case report and review of literature.

Afr J Paediatr Surg 2021 Apr-Jun;18(2):99-103

Department of Pediatric Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Facial teratomas are uncommon tumours in children that distort the face and may be associated with functional problems. They are less common than cervical teratomas though they are often grouped together and considered an emergency due to their tendency to cause respiratory compromise. They tend to be large and cause cosmetic issues; hence usually noticed early and medical help sought promptly by parents. Read More

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Wide unroofed coronary sinus and cryptogenic stroke: a case report.

Eur Heart J Case Rep 2020 Dec 7;4(6):1-6. Epub 2020 Dec 7.

Division of Cardiology, Department of Medicine, Albert Einstein Medical Center, 5501 Old York Road Unit 3, Philadelphia, PA 19141, USA.

Background : Unroofed coronary sinus (UCS) is a rare congenital cardiac anomaly in which there is complete or partial absence of the roof of the coronary sinus (CS) resulting in a communication between the right and left atria. There are four types of UCS described in the literature. While usually asymptomatic and discovered incidentally on imaging, UCS can be the source of a brain abscess or paradoxical embolism. Read More

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December 2020

Asymmetric Hapln1a drives regionalised cardiac ECM expansion and promotes heart morphogenesis in zebrafish development.

Cardiovasc Res 2021 Feb 22. Epub 2021 Feb 22.

Department of Biomedical Science, University of Sheffield, Western Bank, Sheffield, UK.

Aims: Vertebrate heart development requires the complex morphogenesis of a linear tube to form the mature organ, a process essential for correct cardiac form and function requiring coordination of embryonic laterality, cardiac growth, and regionalised cellular changes. While previous studies have demonstrated broad requirements for extracellular matrix (ECM) components in cardiac morphogenesis, we hypothesised that ECM regionalisation may fine tune cardiac shape during heart development.

Methods And Results: Using live in vivo light sheet imaging of zebrafish embryos we describe a left-sided expansion of the ECM between the myocardium and endocardium prior to the onset of heart looping and chamber ballooning. Read More

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February 2021

Exploratory Assessment of Levosimendan in Infants With Congenital Diaphragmatic Hernia.

Pediatr Crit Care Med 2021 Feb 12. Epub 2021 Feb 12.

All authors: Department of Neonatology and Pediatric Intensive Care Medicine, University Children's Hospital Bonn, Bonn, Germany.

Objectives: Infants with congenital diaphragmatic hernia frequently suffer from cardiac dysfunction and pulmonary hypertension during the postnatal course. With the use of the inodilator levosimendan, a therapeutic approach is available in situations with catecholamine-refractory low-cardiac-output failure and severe pulmonary hypertension.

Design: Retrospective single-center cohort study. Read More

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February 2021

Case Report: A Rare Case of Right-Sided Papillary Fibroelastoma in a 1-Year-Old With Congenital Heart Disease.

Front Cardiovasc Med 2020 27;7:624219. Epub 2021 Jan 27.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United States.

Cardiac papillary fibroelastomas (PFEs) are the most common primary benign cardiac tumors, although they are somewhat unusual in children and typically seen on the left-sided cardiac valves. A 10-week-old patient was found to have a partial atrioventricular canal defect, with associated tricuspid and mitral regurgitation. He was medically managed until 1 year of age, when surgical correction was done. Read More

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January 2021

Hypoplastic left heart syndrome: current modalities of treatment and outcomes.

Indian J Thorac Cardiovasc Surg 2021 Jan 11;37(Suppl 1):26-35. Epub 2020 Mar 11.

Department of Pediatric and Congenital Heart Surgery, Kokilaben Dhirubhai Ambani Hospital, Four Bungalows, Andheri (West), Mumbai, 400053 India.

Hypoplastic left heart syndrome is a constellation of malformations which result from the severe underdevelopment of any left-sided cardiac structures. Once considered to be universally fatal, the prognosis for this condition has tremendously improved over the past four decades since the work of William Norwood in the early 1980s. Today, a staged surgical approach is applied for palliating this distinctive cohort of patients, in which they undergo three operative procedures in the first 10 years of their life. Read More

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January 2021

Aortopulmonary window, left coronary artery aneurysm, and redundant aortic valve leaflet in a calf: the use of multimodal imaging to diagnose congenital heart disease.

J Vet Cardiol 2021 Apr 16;34:48-54. Epub 2021 Jan 16.

University of Wisconsin Veterinary Medical Teaching Hospital, Madison, WI 53706, USA.

A 2-month-old male Holstein calf was presented for evaluation of a continuous systolic murmur. A grade V/VI left basilar continuous murmur and a grade IV/VI right basilar continuous murmur was auscultated upon evaluation with increased respiratory effort, wheezes, and crackles. Multimodality diagnostics were performed on this patient for further workup and included transthoracic and transesophageal echocardiography, fluoroscopy guided angiography, and gross necropsy with histopathology. Read More

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Corneal ulcer secondary to ectropion in lamellar Ichthyosis: A rare congenital disorder.

Saudi J Ophthalmol 2020 Jan-Mar;34(1):59-61. Epub 2020 Nov 22.

Department of Ophthalmology, BARC Hospital, Mumbai, India.

We present a rare case of Lamellar Ichthyosis with bilateral ectropion with left sided corneal ulcer with descemetocele in a four-month-old female child, the youngest ever reported. Ichthyosis is a group of skin disorders characterized by the presence of fish-like scales all over the body (Mushriff, 2016; Turgut ., 2009). Read More

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November 2020

Thoracoscopic Repair of Congenital Diaphragmatic Hernia in Preterm Neonate at 1 Kilogram.

European J Pediatr Surg Rep 2021 Jan 28;9(1):e13-e16. Epub 2021 Jan 28.

Department of Neonatology, Chelsea and Westminster Hospital National Health Service Foundation Trust, London, United Kingdom of Great Britain and Northern Ireland.

We report the first successful primary thoracoscopic repair of congenital diaphragmatic hernia (CDH) in a preterm infant born at 28 weeks of gestation weighing 1,043 g. Left-sided CDH was incidentally diagnosed on postnatal chest X-ray on day 1. The neonate subsequently underwent thoracoscopic repair with primary closure of the defect on day 8 weighing 1,150 g. Read More

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January 2021

Reduced-port surgery for right paraduodenal hernia in an adult patient: A case report and review of the literature.

Asian J Endosc Surg 2021 Jan 24. Epub 2021 Jan 24.

Department of Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.

Paraduodenal hernia (PDH) is a congenital internal hernia caused by an abnormal rotation of the midgut during embryotic life; it is characterized as right- or left-sided according to the hernial sac location. Herein, we describe a case of a 30-year-old male patient who was preoperatively diagnosed with right PDH and underwent successful laparoscopic repair using reduced-port surgery (RPS). The patient presented with postprandial nausea, vomiting, and upper abdominal pain. Read More

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January 2021

A late presenting left-sided congenital diaphragmatic hernia repair complicated by postoperative chylothorax: A case report.

Clin Case Rep 2021 Jan 11;9(1):350-354. Epub 2020 Nov 11.

Department of Pediatric Surgery Kanti children's Hospital Kathmandu Nepal.

Management of chylothorax after repair of late presenting congenital diaphragmatic hernia is debatable. Conservative management in the form of close monitoring of chylous output with nutritional support appears convincing to surgery. Read More

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January 2021

Identification of patients at risk of sudden cardiac death in congenital heart disease: The PRospEctiVE study on implaNTable cardIOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD).

Heart Rhythm 2021 May 16;18(5):785-792. Epub 2021 Jan 16.

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands. Electronic address:

Background: Sudden cardiac death (SCD) is the main preventable cause of death in patients with adult congenital heart disease (ACHD). Since robust risk stratification methods are lacking, we developed a risk score model to predict SCD in patients with ACHD: the PRospEctiVE study on implaNTable cardIOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD) risk score model.

Objective: The purpose of this study was to prospectively study predicted SCD risk using the PREVENTION-ACHD risk score model and actual SCD and sustained ventricular tachycardia/ventricular fibrillation (VT/VF) rates in patients with ACHD. Read More

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Safety of Transseptal Puncture for Access to the Left Atrium in Infants and Children.

Pediatr Cardiol 2021 Mar 16;42(3):685-691. Epub 2021 Jan 16.

Department of Pediatric Cardiology and Intensive Care Medicine, Georg August University Medical Center, Robert-Koch-Str. 40, 37075, Göttingen, Germany.

Transseptal puncture (TSP) is a standard procedure to obtain access to the left heart. However, data on TSP in infants and children particularly with congenital heart defects (CHD) is sparse. Safety and efficacy of TSP in infants and children < 18 years with normal cardiac anatomy and with CHD were assessed. Read More

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Evidence that FGFRL1 contributes to congenital diaphragmatic hernia development in humans.

Am J Med Genet A 2021 03 14;185(3):836-840. Epub 2021 Jan 14.

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.

Fibroblast growth factor receptor-like 1 (FGFRL1) encodes a transmembrane protein that is related to fibroblast growth factor receptors but lacks an intercellular tyrosine kinase domain. in vitro studies suggest that FGFRL1 inhibits cell proliferation and promotes cell differentiation and cell adhesion. Mice that lack FGFRL1 die shortly after birth from respiratory distress and have abnormally thin diaphragms whose muscular hypoplasia allows the liver to protrude into the thoracic cavity. Read More

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A case series of three patients with unilateral disconnected pulmonary artery supplied by an ipsilateral patent ductus arteriosus: neonatal ductal stenting as palliation to preserve pulmonary arterial patency.

Eur Heart J Case Rep 2020 Dec 18;4(6):1-7. Epub 2020 Nov 18.

Congenital Cardiology, Leeds General Infirmary, Great George Street, Leeds, UK.

Background : Disconnected branch pulmonary arteries with a systemic arterial origin of the disconnected vessel is a rare, but well-described entity. Most will have ductal tissue connecting the pulmonary artery to the aorta.

Case Summary : We describe in this paper the haemodynamic result in three neonates presenting with ductal origin of a single branch pulmonary artery in the context of trans-catheter stenting procedures to maintain or re-recruit vessel patency. Read More

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December 2020

Genetic Etiology of Left-Sided Obstructive Heart Lesions: A Story in Development.

J Am Heart Assoc 2021 Jan 12;10(2):e019006. Epub 2021 Jan 12.

Division of Cardiology Department of Pediatrics Duke University School of Medicine Durham NC.

Congenital heart disease is the most common congenital defect observed in newborns. Within the spectrum of congenital heart disease are left-sided obstructive lesions (LSOLs), which include hypoplastic left heart syndrome, aortic stenosis, bicuspid aortic valve, coarctation of the aorta, and interrupted aortic arch. These defects can arise in isolation or as a component of a defined syndrome; however, nonsyndromic defects are often observed in multiple family members and associated with high sibling recurrence risk. Read More

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January 2021

Laparoscopic abdominal perineal rectal resection for rectal cancer with a horseshoe kidney using preoperative 3D-CT angiography: a case report.

BMC Surg 2021 Jan 6;21(1):15. Epub 2021 Jan 6.

Department of Surgery, Suwa Red Cross Hospital, 5-11-11-50, Kogan-dori, Suwa, Nagano, 392-8510, Japan.

Background: A horseshoe kidney is a congenital malformation involving the fusion of the bilateral kidneys and is often accompanied by anomalies of the ureteropelvic and vascular systems. When performing resection of colorectal cancer in a patient with horseshoe kidney, damage to the ureter or excessive renal arteries should be avoided. To achieve this purpose, comprehensive preoperative anatomical assessments and surgical planning are important. Read More

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January 2021

Partial anomalous pulmonary venous return after orthotopic heart transplantation case report.

BMC Cardiovasc Disord 2021 Jan 6;21(1). Epub 2021 Jan 6.

Department of Advanced Cardiopulmonary Therapies and Transplantation, Center for Advanced Heart Failure, The University of Texas Health Science Center at Houston/Memorial Hermann Hospital - Texas Medical Center, 6400 Fannin St., Suite 2350, Houston, TX, 77030, USA.

Background: Partial anomalous pulmonary venous return (PAPVR) is a congenital heart defect. Reports of repair and treatment in pediatric cases have been published, but incidence of PAPVR in adults is not common. To our knowledge, there has not been a diagnosis of left-sided PAPVR after a heart transplant an in adult patient. Read More

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January 2021

Preoperative false-negative transthoracic echocardiographic results in native valve infective endocarditis patients: a retrospective study from 2001 to 2018.

Cardiovasc Ultrasound 2021 Jan 2;19(1). Epub 2021 Jan 2.

Department of Infectious Diseases, Nanfang Hospital, Southern Medical University, Guangzhou, China.

Background: Infective endocarditis (IE) is a lethal disease that is difficult to diagnosis early. Although echocardiography is one of the most widely used diagnostic technique, it has limited sensitivity. This study surveyed the clinical features of IE patients who underwent surgery and compared transthoracic echocardiography and histological findings to explore the factors related to false-negative echocardiographic results. Read More

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January 2021