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Hypoplastic heart

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https://www.readbyqxmd.com/read/29223842/interstage-evaluation-of-homograft-valved-right-ventricle-to-pulmonary-artery-conduits-for-palliation-of-hypoplastic-left-heart-syndrome
#1
Nefthi Sandeep, Rajesh Punn, Sowmya Balasubramanian, Shea N Smith, Olaf Reinhartz, Yulin Zhang, Gail E Wright, Lynn F Peng, Lisa Wise-Faberowski, Frank L Hanley, Doff B McElhinney
OBJECTIVE: Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling. METHODS: This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015...
November 8, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29215158/congenital-diaphragmatic-hernia-with-heart-defect-has-a-high-risk-for-hypoplastic-left-heart-syndrome-and-major-extra-cardiac-malformations-10-year-national-cohort-from-finland
#2
Johanna Hautala, Emma Karstunen, Annukka Ritvanen, Risto Rintala, Ilkka P Mattila, Juha Räsänen, Pertti K Suominen, Tiina Ojala
INTRODUCTION: Congenital diaphragmatic hernia (CDH) has a well-known risk of congenital heart defects with poor prognosis. This study was conducted to determine the national total prevalence and prenatal detection rates of CDH with heart defects and its association with major extra-cardiac malformations and to further evaluate the impact of the heart defect severity on survival. MATERIAL AND METHODS: A 10-year national cohort was derived from four national registries, including live births, stillbirths, and terminations of pregnancy for fetal anomalies...
December 7, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/29181203/conducting-prolonged-general-anesthesia-without-intravenous-access-in-a-child-with-hypoplastic-left-heart-syndrome
#3
Phat T Dang, Binjon Sriratana
Children with chronic medical conditions often need multiple intravenous (IV) access instances during their hospitalizations, both peripheral and central. Obtaining a working IV in this patient population undergoing general anesthesia can be challenging. In our case report, we describe a method of administering general anesthesia in an infant with partially repaired hypoplastic left heart syndrome without IV access.
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/29165381/factors-associated-with-the-need-for-and-the-impact-of-extracorporeal-membrane-oxygenation-in-children-with-congenital-heart-disease-during-admissions-for-cardiac-surgery
#4
Salvatore Aiello, Rohit S Loomba
INTRODUCTION: This study aimed to determine factors associated with the need for extracorporeal membrane oxygenation (ECMO) in children with congenital heart disease (CHD) during admission for cardiac surgery (CS). A secondary aim was to determine how ECMO impacted length, cost, and mortality of the admission. METHODS: Data from the Kids' Inpatient Database (KIDS) were utilized. Admissions with CHD under 18 years of age with cardiac surgery were included. Need for ECMO in these admissions was then identified...
November 22, 2017: Children
https://www.readbyqxmd.com/read/29163663/an-unusual-and-rare-form-of-truncus-arteriosus-in-an-asymptomatic-woman
#5
Maryam Nabati
Persistent truncus arteriosus is a rare congenital cardiac malformation. It is characterized by a single great artery arising from the heart which supplies the aorta, the origin of coronary arteries and pulmonary arteries. Without surgery, prognosis is poor and 90% of these patients die before one year of age. We report a rare case of an asymptomatic 35-year-old woman with uncorrected persistent truncus arteriosus and hypoplastic right and left pulmonary arteries. Hypoplastic branch pulmonary arteries prevented the development of severe pulmonary arterial hypertension...
November 2017: Ultrasound: Journal of the British Medical Ultrasound Society
https://www.readbyqxmd.com/read/29141840/cerebral-oxygenation-measurements-by-magnetic-resonance-imaging-in-fetuses-with-and-without-heart-defects
#6
Mette H Lauridsen, Niels Uldbjerg, Tine B Henriksen, Olav B Petersen, Brian Stausbøl-Grøn, Niels B Matthiesen, David A Peters, Steffen Ringgaard, Vibeke E Hjortdal
BACKGROUND: Children with major congenital heart defects are risking impaired cerebral growth, delayed cerebral maturation, and neurodevelopmental disorders. We aimed to compare the cerebral tissue oxygenation of fetuses with major heart defects to that of fetuses without heart defects as estimated by the magnetic resonance imaging modality T2*. T2* is low in areas with high concentrations of deoxyhemoglobin. METHODS AND RESULTS: At gestational age mean 32 weeks (early) and mean 37 weeks (late), we compared the fetal cerebral T2* in 28 fetuses without heart defects to that of 15 fetuses with major heart defects: transposition of the great arteries (n=7), coarctation of the aorta/hypoplastic aortic arch (n=5), tetralogy of Fallot (n=1), hypoplastic right heart (n=1), and common arterial trunk (n=1)...
November 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29118890/a-novel-technique-for-the-administration-of-sub-ambient-oxygen-in-the-operating-room
#7
Asad A Khawaja, Marco Corridore, Joseph D Tobias
Hypoplastic left heart syndrome (HLHS) is a common form of congenital heart disease with abnormal development of left-sided structures of the heart. As excessive pulmonary blood flow is common prior to palliative surgical procedures, therapeutic maneuvers may be required to decrease pulmonary blood flow and augment systemic cardiac output. Respiratory manipulations to optimize the ratio of pulmonary to systemic blood flow may include techniques to induce hypercarbia or the delivery of sub-ambient concentrations of oxygen (FiO2 less than 0...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29096870/hypoplastic-left-heart-syndrome-is-not-a-predictor-of-worse-intermediate-mortality-post-fontan
#8
Billie-Jean Martin, Kandice Mah, Luke Eckersley, Joyce Harder, Charissa Pockett, Daryl Schantz, John Dyck, Mohammed Al Aklabi, Ivan M Rebeyka, David B Ross
BACKGROUND: An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation. METHODS: All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival...
October 30, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29081324/coarctation-of-the-aorta-stenting-via-glidesheath-slender-in-a-newborn-with-recoarctation-early-after-a-norwood-operation
#9
Katarzyna Gendera, Julie Cleuziou, Daniel Tanase
In this article, we report on a newborn with hypoplastic left heart syndrome in whom recoarctation of the aorta was treated with a bare metal stent (Cook Formula 414 Stent) in the early postoperative period after a Norwood procedure. To reduce the risk for scarring and occluding the femoral artery the stent was implanted via 5F Glidesheath Slender sheath.
October 30, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29063727/nurse-driven-analgesia-and-sedation-in-pediatric-patients-with-univentricular-hearts-requiring-extracorporeal-life-support-after-first-stage-palliation-surgery-a-pilot-study
#10
Jörg Michel, Michael Hofbeck, Ines Gerbig, Vanya Icheva, Ellen Heimberg, Walter Jost, Christian Schlensak, Matthias Kumpf, Gunnar Blumenstock, Felix Neunhoeffer
BACKGROUND: Few data are available regarding requirements of sedation and analgesia in children during extracorporeal life support. AIMS: The aim of this study was to evaluate if children with functionally univentricular hearts on extracorporeal life support after first-stage palliation surgery have higher requirement of analgesics and sedatives compared with children without extracorporeal life support using a goal-directed nurse-driven analgesia and sedation protocol...
December 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29057564/the-effect-of-fetal-hemodynamics-on-fetal-growth-in-single-ventricle-and-transposition-of-the-great-arteries
#11
Tarek Alsaied, Stephanie Tseng, Eileen King, Eunice Hahn, Allison Divanovic, Mounira Habli, James Cnota
INTRODUCTION: As birth weight is a critical predictor of neonatal congenital heart defect (CHD) outcomes, the common problem of poor fetal growth in this population is clinically important but not well understood. The impact of fetal hemodynamics on fetal growth and birth weight in CHD has not been assessed. Combined cardiac output (CCO) has been used to evaluate patients with structurally normal heart but rarely in CHD. Middle cerebral artery and umbilical artery pulsatility indices (PIs) have been used to evaluate the relative distribution of circulation in patients with CHD...
October 23, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29049702/aortic-root-and-hemiarch-replacement-in-a-patient-with-loeys-dietz-and-hypoplastic-left-heart-syndromes
#12
Eltayeb Mohamed Ahmed, Alexandru Ciprian Visan, Graham Stuart, Serban Stoica
A 15-year-old patient with hypoplastic left heart syndrome underwent 3-stage palliation by the age of 3 years. He was later diagnosed with Loeys-Dietz syndrome. On follow-up imaging, the neoaorta was dilated at 50 mm in diameter. He underwent aortic root replacement with a composite valve conduit and hemiarch replacement, using a boat-shaped Dacron graft. The uncertainty of how univentricular circulation would tolerate long bypass time steered us away from a total arch replacement. His postoperative recovery was uneventful...
October 11, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29047321/perioperative-and-anesthetic-considerations-in-pulmonary-atresia-with-intact-ventricular-septum
#13
Stephen Gleich, Gregory J Latham, Denise Joffe, Faith J Ross
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation...
October 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29035962/effectiveness-of-interstage-home-monitoring-on-healthcare-outcomes-in-infants-with-hypoplastic-left-heart-syndrome-a-systematic-review-protocol
#14
Chelsey Andries, Michelle Palokas
What is the effectiveness of home monitoring on interstage survival rates, weight gain, hospital admissions and cost savings in infants with hypoplastic left heart syndrome?
October 2017: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/29016838/the-hand1-frameshift-a126fs-mutation-does-not-cause-hypoplastic-left-heart-syndrome-in-mice
#15
Beth A Firulli, Kevin P Toolan, Jade Harkin, Hannah Millar, Santiago Pineda, Anthony B Firulli
Aims: To test if a human Hand1 frame shift mutation identified in human samples is causative of hypoplastic left heart syndrome (HLHS). Methods and results: HLHS is a poorly understood single ventricle congenital heart defect that affects two to three infants in every 10 000 live births. The aetiologies of HLHS are largely unknown. The basic helix-loop-helix transcription factor HAND1 is required for normal heart development. Interrogation of HAND1 sequence from fixed HLHS tissues identified a somatic frame-shift mutation at Alanine 126 (NP_004812...
August 31, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28993061/risk-comparison-for-prenatal-use-of-analgesics-and-selected-birth-defects-national-birth-defects-prevention-study-1997-2011
#16
Julia D Interrante, Elizabeth C Ailes, Jennifer N Lind, Marlene Anderka, Marcia L Feldkamp, Martha M Werler, Lockwood G Taylor, James Trinidad, Suzanne M Gilboa, Cheryl S Broussard
PURPOSE: To compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects. METHODS: We used data from the National Birth Defects Prevention Study (1997-2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Adjusted odds ratios (aORs) were calculated to examine associations with 16 birth defects...
October 2017: Annals of Epidemiology
https://www.readbyqxmd.com/read/28976608/cerebrovascular-response-to-maternal-hyperoxygenation-mh-in-fetuses-with-hypoplastic-left-heart-syndrome-hlhs-depends-upon-gestational-age-ga-and-baseline-cerebrovascular-resistance
#17
Anita Szwast, Mary Putt, J William Gaynor, Daniel Licht, Jack Rychik
OBJECTIVES: Compared to normal fetuses, HLHS fetuses have smaller brain volumes and more brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce brain injury in these fetuses. This study investigates whether gestational age (GA) and baseline cerebrovascular resistance impact response to MH in HLHS fetuses. METHODS: The middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed in 43 HLHS fetuses...
October 4, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28971557/prenatal-diagnosis-of-hypoplastic-left-heart-and-coarctation-of-the-aorta-with-color-doppler-fine
#18
L Yeo, R Romero
No abstract text is available yet for this article.
October 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28964428/surgical-interventions-in-infants-born-preterm-with-congenital-heart-defects-an-analysis-of-the-kids-inpatient-database
#19
Jagdish Desai, Sanjeev Aggarwal, Steven Lipshultz, Prashant Agarwal, Paulos Yigazu, Riddhiben Patel, Samantha Seals, Girija Natarajan
OBJECTIVE: To evaluate short-term outcomes in infants born preterm with congenital heart defects (CHDs) and the factors associated with surgery, survival, and length of hospitalization in this population. STUDY DESIGN: We analyzed data from infants born preterm (gestational age <37 weeks) enrolled in the multicenter Kids' Inpatient Database of the Healthcare Cost and Utilization Project who were admitted to the hospital within 30 days after birth. Infants with atrial septal defects were excluded...
December 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28960425/two-cases-of-prenatally-diagnosed-membranous-and-muscular-ventricular-septal-aneurysms
#20
Olga Ivanitskaya, Elena Andreeva, Natalia Odegova
A ventricular septal aneurysm is a rare heart defect located in the muscular or membranous part of the septum. Muscular ventricular septal aneurysms are usually isolated, with a favorable prognosis. Membranous ventricular septal aneurysms are often associated with other heart anomalies, could result in serious complications, and may require surgical treatment. We describe 2 cases of prenatally diagnosed ventricular septal aneurysms: an isolated membranous ventricular septal aneurysm with a good outcome, which was initially misdiagnosed as an atrioventricular septal defect; and a muscular ventricular septal aneurysm associated with a hypoplastic aortic arch and severe hydrocephaly, which resulted in termination of the pregnancy...
September 29, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
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