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https://www.readbyqxmd.com/read/29808750/perioperative-and-anesthetic-considerations-in-truncus-arteriosus
#1
Rishi Parikh, Michael Eisses, Gregory J Latham, Denise C Joffe, Faith J Ross
Truncus arteriosus is a congenital cardiac lesion in which failure of embryonic truncal septation results in a single semilunar valve and single arterial trunk providing both pulmonary and systemic circulations. Most patients with this lesion are symptomatic in the neonatal period with cyanosis and/or congestive heart failure and undergo complete repair in the first weeks of life. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with truncus arteriosus.
May 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29754396/the-upper-pouch-in-oesophageal-atresia-shows-proportional-growth-during-late-fetal-life
#2
R B Tröbs, M Nissen, J Wald
Oesophageal atresia with trachea-oesophageal fistula is a rare foregut malformation that requires surgery soon after birth. Prenatal ultrasound diagnosis is based on the presence of polyhydramnios, a small or non-visible fetal stomach and the blind ending oesophagus, called the upper pouch (1). Neonates present with salivation, coughing, choking and attacks of cyanosis. A diagnosis of oesophageal atresia is confirmed by inserting a nasogastric tube and a thoraco-abdominal X-ray. This article is protected by copyright...
May 12, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29707370/successful-repair-of-neonatal-tricuspid-regurgitation-due-to-chordae-rupture
#3
Jun-Yen Pan, Chu-Chuan Lin, Jen-Ping Chang
Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgitation (TR) due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion, successful early repair was achieved with artificial chordae implantation. The unique pathophysiology and the therapeutic strategy of this situation will be described.
March 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29629694/effect-of-gastric-lavage-on-meconium-aspiration-syndrome-and-feed-intolerance-in-vigorous-infants-born-with-meconium-stained-amniotic-fluid-a-randomized-control-trial
#4
RANDOMIZED CONTROLLED TRIAL
Shrishail Gidaganti, M M A Faridi, Manish Narang, Prerna Batra
OBJECTIVE: To compare the incidence of meconium aspiration syndrome and feed intolerance in infants born through meconium stained amniotic fluid with or without gastric lavage performed at birth. SETTING: Neonatal unit of a teaching hospital in New Delhi, India. DESIGN: Parallel group unmasked randomized controlled trial. PARTICIPANTS: 700 vigorous infants of gestational age ≥34 weeks from through meconium stained amniotic fluid...
March 15, 2018: Indian Pediatrics
https://www.readbyqxmd.com/read/29518833/-clinical-and-genetic-characteristics-of-congenital-myasthenia-syndrome-with-episodic-apnea-caused-by-chat-gene-mutation-a-report-of-2-cases
#5
Z M Liu, F Fang, C H Ding, W H Zhang, J Deng, C H Chen, X Wang, J Liu, Z Li, X L Jia, J S Zeng, S Y Qian
Objective: To investigate the clinical and genetic features of congenital myasthenia syndrome with episodic apnea (CMS-EA) caused by gene mutation of choline acetyltransferase (CHAT) Methods: The clinical data of 2 patients with congenital myasthenia syndrome were collected, and both were diagnosed from 2013 to 2015 in Beijing Children's Hospital, Capital Medical University. The clinical features and gene mutation characteristics were analyzed, and the patients were followed-up for therapeutic efficacy. Results: The two patients (case 1 and case 2) had the onset soon after birth and at 3 months after birth respectively...
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29456944/choanal-atresia-birth-without-breath
#6
Vikas Sinha, Samanth Talagauara Umesh, Sushil G Jha, Swati Dadhich
Bilateral Choanal atresia is a medical emergency. Corrective surgery is the mainstay of the treatment. Hegar's dilator was used in all cases to break the bony/membranous atretic plate. The 22 cases of choanal atresia all operated by the first author were included in this study. Eight cases were 1-5 year old with bilateral choanal atresia and all required immediate surgery as they had repeated attacks of respiratory distress and cycle of cyanosis. Eight cases were of CHARGE Syndrome. All the cases were operated under general anaesthesia...
March 2018: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/29361049/a-persistent-giant-eustachian-valve-functional-tricuspid-atresia-in-a-newborn
#7
Konstantinos Sideris, Janez Vodiskar, Ruediger Lange, Julie Cleuziou
Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis. Echocardiographic examination showed a giant Eustachian valve moving towards the tricuspid valve during late systole and leading to complete obstruction of the inflow. Blood flow was redirected through the persistent foramen ovale, producing a right-to-left shunt...
January 18, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29326774/the-burden-of-truncus-arteriosus-in-an-urban-city-in-africa-how-are-we-fairing
#8
Barakat Adeola Animasahun, Aminat Titilayo Ogunlana, Henry Olusegun Gbelee
Background: The true incidence of truncus arteriosus in underdeveloped countries is difficult to determine. This is due largely to underreporting as a result of nonavailability of technologically advanced facilities to make definitive diagnosis prenatally. There is a lack of data on the profile and outcome of patients with persistent truncus arteriosus (PTA) in Nigeria. This study aims to document the demographic characteristics, mode of presentation, indications for echocardiography, associated anomalies, average age at diagnosis, and outcome of patients with truncus arteriosus in our center...
October 2017: Heart Views: the Official Journal of the Gulf Heart Association
https://www.readbyqxmd.com/read/29316539/cyanosis-and-stroke-due-to-functional-cor-triatriatum-dexter-in-a-neonate
#9
Rachel L León, Nicholas B Zaban, Marcus S Schamberger, Chang Y Ho, Ulrike Mietzsch
Small remnants of the right valve of the sinus venosus are commonly found in adults, but the incidence and risk associated with these embryonic remnants in neonates are not well studied. The following report describes a cyanotic neonate with a large Eustachian valve remnant creating a functional cor triatriatum dexter who was initially diagnosed with persistent pulmonary hypertension of the newborn. The cyanosis in this infant improved over the first postnatal week with conservative management, but she suffered multifocal subcortical stroke, likely related to her intracardiac shunt...
2018: Neonatology
https://www.readbyqxmd.com/read/29301186/clinical-course-of-infants-with-congenital-heart-disease-who-developed-thyroid-dysfunction-within-100-days
#10
Hye Jin Lee, Hyeoh Won Yu, Gi Beom Kim, Choong Ho Shin, Sei Won Yang, Young Ah Lee
PURPOSE: We investigated the clinical course of infants with congenital heart disease (CHD) who experienced thyroid dysfunction within 100 days of birth. METHODS: We performed retrospective medical reviews of 54 CHD patients (24 male patients) who underwent a thyroid function test (TFT) between January 2007 and July 2016. Data were collected on birth history, diagnosis of CHD, underlying chromosomal or genetic abnormalities, medication history, surgery, ventilator care, and exposure to iodine contrast media (ICM)...
December 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29296069/tuberous-sclerosis-presenting-as-neonatal-cyanosis-because-of-rhabdomyoma-causing-tricuspid-valve-obstruction-needing-a-blalock-taussig-shunt
#11
Monther Obeidat, Yazan Qawasmeh, Hani Tarawneh, Ibrahim Sawalhah, Ala'a Tawalbeh
We report a newborn female baby who presented at 6 hours of age with cyanosis without any signs of respiratory distress. Cardiovascular and systemic examination was unremarkable apart from cyanosis (saturation 75%). An echocardiogram showed multiple echogenic and homogeneous masses in the interventricular septum, one of which was big and protruding through the tricuspid valve causing right ventricular inflow obstruction. There was a small atrial septal defect (ASD) shunting right to left and patent ductus arteriosus (PDA) shunting left to right...
January 2018: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/29286939/clinical-profile-and-pattern-of-congenital-heart-disease-in-infants-of-diabetic-mother-and-infants-of-non-diabetic-mother-at-a-tertiary-care-hospital
#12
Suraiya Begum, Sanjoy Kumar Dey
OBJECTIVES: To assess the clinical manifestations and pattern of congenital heart diseases (CHD) in infants of diabetic mothers (IDMs) and infants of non-diabetic mothers. METHODS: A prospective cross sectional study was carried out at tertiary care center over a period of thirty months. All neonates were included in this study if CHD were identified by echocardiography within this study period. Chi-square test and Fisher's exact test were used to compare between groups...
2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/29062561/transposition-of-the-great-arteries-and-coarctation-of-the-aorta-in-an-infant-presenting-with-bronchiolitis-an-incidental-finding
#13
Khaloud S Al-Mukhaini, Ala M Mohamed
The transposition of the great arteries (TGA) is a complex congenital heart disease which usually presents as cyanosis in neonates with limited mixing between the systemic and pulmonary circulatory systems. A delayed diagnosis of TGA can lead to ventricular failure. We report a six-week-old infant who was admitted to the paediatric Intensive Care Unit of the Royal Hospital, Muscat, Oman, in 2016 for bronchiolitis. During admission, she was incidentally diagnosed with TGA and coarctation of the aorta. Postnatal screening, including the use of pulse oximetry, plays a significant role in avoiding a late diagnosis of TGA; however, this screening tool is not widely used in Oman...
August 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28794091/a-rare-cause-of-early-neonatal-cyanosis-absent-right-pulmonary-artery
#14
Avadhesh Joshi, Manish Kumar, Sumitha Arun, Mane Manisha Sheshrao
Unilateral absent right pulmonary artery is a rare developmental anomaly that usually presents in late childhood and adolescence as recurrent respiratory tract infections, dyspnoea and haemoptysis. We report a case of a 2-day-old baby with respiratory distress and differential cyanosis. Echocardiogram showed pulmonary hypertension with absent right pulmonary artery. The findings were confirmed by CT angiogram. The baby improved with pulmonary vasodilators and antifailure medications.
August 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28745124/dysfunction-of-the-foetal-arterial-duct-results-in-a-wide-spectrum-of-cardiovascular-pathology
#15
Marc Gewillig, Stephen C Brown, Mieke Roggen, Benedicte Eyskens, Ruth Heying, Patrice Givron, Bjorn Cools, Luc de Catte
OBJECTIVE: Foetal ductal problems may have various cardiopulmonary consequences. This study aimed to identify the spectrum of ductus arteriosus (DA) dysfunction (closure, constriction, kinking, aneurysm and thrombosis) and the resultant clinical and echocardiographic presentation in foetuses and neonates. METHODS AND RESULTS: This is a retrospective analysis of serial pre- and post-natal data of 27 cases of foetal ductal dysfunction diagnosed at a median gestational age of 33 weeks (range 20-39)...
December 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28688570/congenital-nasal-obstruction-in-infants-a-retrospective-study-and-literature-review
#16
REVIEW
Vijay A Patel, Michele M Carr
OBJECTIVES: To identify etiologies of congenital nasal obstruction and describe clinical practice patterns in the evaluation, diagnosis, and treatment of symptomatic infants. METHODS: An electronic chart review from 1/1/2006-10/1/2016 for all patients with a diagnosis of nasal obstruction within the first six months of life using ICD-9 and 10 codes 478.19 and J34.89. RESULTS: A total of 34 patients were evaluated by the Division of Otolaryngology for this chief complaint...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28633270/surgical-correction-for-a-neonate-with-ebstein-s-anomaly-associated-with-tetralogy-of-fallot
#17
Ling-Yi Wei, Jin-Chung Shih, En-Ting Wu, Shyh-Jye Chen, Yih-Sharng Chen, Shu-Chien Huang
Ebstein's anomaly in combination with tetralogy of Fallot (TOF) is rare. We report a male infant with these combined anomalies who presented with severe cyanosis, heart failure, and respiratory distress after birth. Biventricular repair was successfully performed with 1-stage correction of his ventricular septal defect (VSD), right ventricular outflow tract (RVOT) obstruction, and tricuspid regurgitation. The downward displaced tricuspid valve was also restored to the normal annulus position. The infant recovered well...
July 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28566837/massive-biventricular-rhabdomyoma-in-a-neonate
#18
Manish Pandey, Rimlee Dutta, Shyam S Kothari
Rhabdomyoma is a well characterised entity in a neonate. Herein, we report a massive biventricular rhabdomyoma in a neonate presenting with cyanosis and congestive heart failure which was confirmed on autopsy. The report is for documentation of an unusually large tumour.
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28566834/the-utility-of-computed-tomographic-angiography-in-a-neonate-on-extracorporeal-membrane-oxygenation-with-extreme-cyanosis-after-blalock-taussig-shunt
#19
Jess D Rames, Minoo N Kavarana, U Joseph Schoepf, Anthony Marcus Hlavacek
A modified Blalock-Taussig shunt (mBTS) is often employed to provide pulmonary blood flow in neonates that are born with cyanotic congenital heart defects. However, acute shunt thrombosis can occur in the postoperative period, resulting in profound cyanosis. In this case report, we describe the utility of computed tomographic angiography (CTA) in the management of a neonate with extreme cyanosis after placement of a mBTS while on extracorporeal membrane oxygenation. Using CTA, several small clots were identified in the shunt as well as stenosis of the left pulmonary artery; neither of which were identified with echocardiography...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28491157/infracardiac-type-total-anomalous-pulmonary-venous-return-with-obstruction-and-dilatation-of-portal-vein
#20
Felipe Aluja Jaramillo, Cristian Hernandez, Juan Pablo Garzón, Angela Paola Sánchez Herrera, Martha Lucia Velasco Morales
Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection, is a congenital cardiovascular malformation that presents itself in the neonatal period, with cyanosis and tachypnea. There are 4 types of TAPVR with the mixed type being the least common. Any type of TAPVR may be associated with obstruction as result of flow redirection through the liver parenchyma before it may return to the heart, but infracardiac is the most common one. We report a case of a 10-hour-old female, with a mixed (cardiac and infracardiac) TAVPR with obstruction, that showed drainage to the coronary sinus and the portal vein, as the other classic findings in TAVPR, made with computed tomography angiography and echocardiogram...
June 2017: Radiology Case Reports
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