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Newborn heart failure

Anne E Atkins, Beth A Tarini, Emily K Phillips, Amy R U L Calhoun
The Iowa Newborn Screening (NBS) Program began screening for very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) in 2003. Untreated VLCAD can lead to liver failure, heart failure, and death. Current confirmatory testing recommendations by the American College of Medical Genetics (ACMG) for VLCAD list molecular and functional analysis (i.e., fibroblast fatty acid oxidation probe) as optional. This can lead to misclassification of VLCAD carriers as false positives. Iowa implemented a comprehensive VLCAD confirmatory testing algorithm at the beginning of 2016 that included both molecular and fibroblast analysis...
February 5, 2019: Journal of Community Genetics
R I Stryuk, C A Burns, M P Filippov, Ya V Brytkova, I V Borisov, E L Barkova, T A Gomova, E A Kozina, O A Nagirnyak
AIM: the Aim of the BEREG Registry was to analyze the prevalence and structure of cardiovascular diseases, associated comorbid conditions and assess their effects on pregnancy and perinatal outcomes in real clinical practice. MATERIALS AND METHODS: In Tula city regional perinatal center the observation study named "Assessment of the clinical condition of the pregnant women with cardiovascular disease during gestation, at child delivery, at the early postpartum period and at twelve months after childbirth and assesment of perinatal outcomes, condition of the fetus and the newborn and the quality of treatment of these patients groups...
February 14, 2018: Terapevticheskiĭ Arkhiv
Patrícia Janeiro, Rita Jotta, Ruben Ramos, Cristina Florindo, Fátima V Ventura, Laura Vilarinho, Isabel Tavares de Almeida, Ana Gaspar
Fatty acid β-oxidation (FAO) disorders have a wide variety of symptoms, not usually evident between episodes of acute decompensations. Cardiac involvement is frequent, and severe ventricular arrhythmias are suspected of causing sudden death. Expanded newborn screening (ENS) for these disorders, hopefully, contribute to prevent potentially acute life-threatening events. In order to characterize acute decompensations observed in FAO-deficient cases identified by ENS, a retrospective analysis was performed, covering a period of 9 years...
January 7, 2019: European Journal of Pediatrics
Jeffrey L Platt, Lori J West, Richard E Chinnock, Marilia Cascalho
The newborn infant with severe cardiac failure owed to congenital structural heart disease or cardiomyopathy poses a daunting therapeutic challenge. The ideal solution for both might be cardiac transplantation if availability of hearts was not limiting and if tolerance could be induced, obviating toxicity of immunosuppressive therapy. If one could safely and effectively exploit neonatal tolerance for successful xenotransplantation of the heart, the challenge of severe cardiac failure in the newborn infant might be met...
December 11, 2018: Xenotransplantation
Maria Weinkouff Pedersen, Kristian Ambjørn Groth, Kristian Havmand Mortensen, John Brodersen, Claus Højbjerg Gravholt, Niels Holmark Andersen
A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life...
January 2019: Cardiology in the Young
Víctor Lago Leal, Luis Martínez Cortés, Carlos Seco Del Cacho
Congenital high airway obstruction syndrome (CHAOS) is a rare sequence due to a complete obstruction of the fetal airway that blocks the larynx or trachea, either intrinsic atresia or extrinsic compression (e.g., congenital neck mass). Despite the true incidence of CHAOS is unknown, an incidence of 1 per 50,000 newborns is described. If any obstruction occurs in the tracheobronchial airway, this secretion cannot be extricated. Because of this situation, a knock-on effect starts: the enlargement of the lungs squeezes the heart and great veins, what results in a replacement of the heart to the center of the chest becoming small and dysfunctional...
July 2018: Indian Journal of Radiology & Imaging
Shuyun Wang, Shan Huang, Lianghui Gong, Zhize Yuan, Joshua Wong, Jeffrey Lee, Ming-Sing Si
Newborns with critical congenital heart disease are at significant risk of developing heart failure later in life. Because treatment options for end-stage heart disease in children are limited, regenerative therapies for these patients would be of significant benefit. During neonatal cardiac surgery, a portion of the thymus is removed and discarded. This discarded thymus tissue is a good source of MSCs that we have previously shown to be proangiogenic and to promote cardiac function in an in vitro model of heart tissue...
2018: Stem Cells International
Jacquelyn M Walejko, Jeremy P Koelmel, Timothy J Garrett, Arthur S Edison, Maureen Keller-Wood
During late gestation, the fetal heart primarily relies on glucose and lactate to support rapid growth and development. While numerous studies describe changes in heart metabolism to preferentially utilize fatty acids a few weeks after birth, little is known about metabolic changes of the heart within the first day following birth. Therefore, we used the ovine model of pregnancy to investigate metabolic differences between the near-term fetal and the newborn heart. Heart tissue was collected for metabolomic, lipidomic, and transcriptomic approaches from the left and right ventricles and intraventricular septum in 7 fetuses at gestational day 142 and 7 newborn lambs on the day of birth...
October 9, 2018: American Journal of Physiology. Endocrinology and Metabolism
Paraskevi Kazakou, Marianna Theodora, Christina Kanaka-Gantenbein, Evangelia Zapanti, Helen Bouza, Chrysa Petropoulou, George Daskalakis, Stavroula A Paschou, Eleni Anastasiou
A 33-year-old Caucasian woman was referred at 24 + 3 weeks of gestation due to fetal tachycardia and hydrops. She had an uncomplicated pregnancy 16 years previously and was on levothyroxine after total thyroidectomy for Graves' disease 6 years previously, when she developed moderate exophthalmos. Laboratory evaluation revealed appropriate thyroid function for this time of gestation: thyroid stimulating hormone (TSH) 1.7 μU/ml (1-3), fT4 18.53 pmol/l (12-22), with positive antibodies: anti-TPO 157 U/ml (<35), TSH receptor antibodies (TRAb) 171...
October 2018: Case Reports in Women's Health
Katherine H Campbell, Jessica L Illuzzi, Henry C Lee, Haiqun Lin, Heather S Lipkind, Lisbet S Lundsberg, Christian M Pettker, Xiao Xu
BACKGROUND: This study aims to examine hospital variation in both maternal and neonatal morbidities and identify institutional characteristics associated with hospital performance in a combined measure of maternal and neonatal outcomes. METHODS: Using the California Linked Birth File containing data from birth certificate and hospital discharge records, we identified 1 322 713 term births delivered at 248 hospitals during 2010-2012. For each hospital, a risk-standardized rate of severe maternal morbidities and a risk-standardized rate of severe newborn morbidities were calculated after adjusting for patient clinical risk factors...
September 24, 2018: Birth
Shotaro Kai, Yasuyuki Fujita, Yuka Sato, Saki Kido, Nobuhiro Hidaka, Ichiro Sakamoto, Kiyoko Kato
Pregnancy after mechanical valve replacement involves high risk. Maternal valve thrombosis and hemorrhagic complications are associated with lethal outcomes; therefore, strict anticoagulant therapy is needed. Our patient was 26-year-old primiparous woman. She had undergone aortic valve replacement with a mechanical valve at 4 years of age and had used warfarin 3 mg per day since then. Because of her desire for a baby, she stopped warfarin and conceived spontaneously. She was referred to our hospital. After being informed of her choices, unfractionated heparin (UFH) administration was started...
September 19, 2018: Journal of Maternal-fetal & Neonatal Medicine
Jing-Jing Li, Yuan Liu, Si-Yuan Xie, Guo-Dong Zhao, Ting Dai, Hong Chen, Lan-Fang Mu, Hai-Ying Qi, Jia Li
BACKGROUND: Pulse oximetry screening for critical congenital heart disease (CHD) is inapplicable to high altitude due to the variedly decreased arterial saturations and rare complex CHD. We examined the incidence and spectrum of CHD in newborns using echocardiography at high altitude and followed up their outcomes. METHODS: A total of 1337 babies were studied. Echocardiography was performed in 1002 asymptomatic newborns (3-5 days). In the same period, retrospectively studied 394 newborns (≤2 days) admitted to the NICU where echocardiograph was performed in 335...
January 1, 2019: International Journal of Cardiology
M A Riedijk, A M König-Jung, J B M van Woensel, F H Kruisinga
BACKGROUND: Upper airway abnormalities in the newborn are associated with obstructive breathing but not with mixed apneas. A tracheostomy is necessary to treat severe obstructive apneas but will not have an effect on the central part of the mixed apneas. As far as we know, this is the first case report describing disappearance of severe mixed apneas after tracheostomy in a 7-week-old infant. CASE PRESENTATION: We report a case of a white female neonate with anatomical upper airway abnormalities and severe mixed apneas with desaturations needing respiratory support...
September 4, 2018: Journal of Medical Case Reports
Canan G Nebigil, Laurent Désaubry
Congenital heart disease is the most common birth defect, affecting 1.35 million newborns every year. Heart failure is a primary cause of late morbidity and mortality after myocardial infarction. Heart development is involved in several rounds of epithelial-to-mesenchymal transition (EMT) and mesenchymal-to-epithelial transition (MET). Errors in these processes contribute to congenital heart disease, and exert deleterious effects on the heart and circulation after myocardial infarction. The identification of factors that are involved in heart development and disease, and the development of new approaches for the treatment of these disorders are of great interest...
August 22, 2018: Trends in Cardiovascular Medicine
Alexandra Rodrigues, Andreia Forno, Edite Costa, Alberto Berenguer, Carla Pilar, Rui Loureiro, Duarte Rufino, Andreia Barros, Filomena Teixeira
Infantile hepatic haemangioma (IHH) is a rare vascular tumour that is potentially lethal due to its associated complications, including heart failure, hepatic failure, hypothyroidism and abdominal compartment syndrome. The authors report a case of an asymptomatic diffuse IHH in a newborn male, which was presented as an incidental finding at the time that the patient was diagnosed with pyloric stenosis. The patient was treated with increasing doses of propranolol that were well tolerated. With the regression of the IHH by the time that the patient reached one year of age, there was a significant imagiologic improvement...
August 2018: Oxford Medical Case Reports
Francesco Macchini, Valerio Gentilino, Ernesto Leva, Steven Rothenberg
An extralobar pulmonary sequestration (EPS) associated with a contralateral aorto-to-pulmonary vein fistula is rare. We report the case of a female newborn with left EPS fed by an artery originating from the distal thoracic aorta and, symmetrically on the controlateral side, an artery shunting in the inferior right pulmonary vein. Echocardiography showed dilatation of the left atrium. On the 34th day since birth (weight 4500 g), the patient was operated on thoracoscopically. The EPS was closed with a 3-mm sealing system, divided and removed...
July 5, 2018: Interactive Cardiovascular and Thoracic Surgery
Giovanna Mangili, Elena Garzoli, Youcef Sadou
Congenital heart disease (CHD) is the most common neonatal congenital malformation. The variety and severity of clinical presentation depend on the cardiac structures involved and their functional impact. The management of newborns with CHD requires a multidisciplinary approach, in which the nutritional aspect plays an important role. An adequate caloric intake during either preand post-surgical period, in fact, improves the outcome of these patients. In addition, the failure to thrive of these children in childhood has been related to long-term cognitive delay (attention deficit disorders, aggressive behaviour and poor social and emotional development)...
May 23, 2018: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
Inês Alves, Tânia Martins, Ana Luísa Neves, Esmeralda Rodrigues, Ana Teixeira, Caldas Afonso, Helena Pinto
Dilated cardiomyopathy is the most common form of cardiomyopathy and the main cause of cardiac transplantation in children and in adults. Infants and children have a wider spectrum of etiologies, hampering their identification. The most frequent initial manifestation of dilated cardiomyopathy is symptomatic heart failure during exercise or at rest (although many patients are asymptomatic). Some causes are potentially reversible, therefore the investigation should be carefully planned and immediately performed after diagnosis...
July 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
Shawn F L Hanson, Michael H Terry, Dafne T Moretta, Gordon G Power, Sean M Wilson, Farzana Alam, Fakhrul Ahsan, Arlin B Blood, Paresh C Giri
INTRODUCTION: Pulmonary hypertension (PH) is a potentially deadly disease for infants and adults with few existing medical interventions and no cure. In PH, increased blood pressure in the pulmonary artery eventually leads to heart failure. Fasudil, an antagonist of Rho-kinase, causes vasodilation leading to decreased systemic artery pressure and pulmonary artery pressure (PAP). This study compared the effects of fasudil administered as either an intravenous infusion or inhaled aerosol in newborn lambs...
September 2018: Journal of Cardiovascular Pharmacology and Therapeutics
Don K Nakayama
Evolving from the development of heart-lung machines for open-heart surgery, extracorporeal membrane oxygenation has reemerged as a rescue modality for patients with acute respiratory failure that cannot be supported by conventional modes of ventilation. The history of extracorporeal membrane oxygenation begins with the discovery of heparin, fundamental to the success of extracorporeal circulation and membrane lungs. Engineers and scientists created suitable artificial membranes that allowed gas exchange while keeping gas and blood phases separate...
April 1, 2018: American Surgeon
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