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https://www.readbyqxmd.com/read/28743383/successful-atrioventricular-valve-repair-improves-long-term-outcomes-in-children-with-unbalanced-atrioventricular-septal-defect
#1
Edward Buratto, Xin Tao Ye, Christian P Brizard, Johann Brink, Yves d'Udekem, Igor E Konstantinov
BACKGROUND: Atrioventricular valve regurgitation is a significant cause of morbidity and mortality in patients with unbalanced atrioventricular septal defect. However, knowledge of the outcomes of atrioventricular valve repair in children with unbalanced atrioventricular septal defect and univentricular physiology is limited. METHODS: We conducted a retrospective review of all patients with unbalanced atrioventricular septal defect treated with single-ventricle palliation who underwent atrioventricular valve surgery at The Royal Children's Hospital...
June 28, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28734439/coronary-sinus-orifice-atresia-after-the-fontan-completion-unique-repair-technique
#2
Shinya Yokoyama, Takeo Yonekura, Noboru Nishiwaki, Shigeki Taniguchi
We report a successful unroofing of the coronary sinus via a unique technique. Our patient was diagnosed with single ventricle morphology. Pulmonary artery banding and bidirectional cavopulmonary shunt were performed at 1 and 6 months old, respectively. Ordinary univentricular repair strategy was performed; extracardiac total cavopulmonary connection was achieved at 18 months old. Catheterization performed 1 year after the Fontan procedure revealed the coronary sinus orifice atresia, diffuse coronary artery stenosis, and sinusoid formation of the coronary system...
August 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28734437/two-pumps-for-single-ventricle-mechanical-support-for-establishment-of-biventricular-circulation
#3
Stanislav Ovroutski, Oliver Miera, Thomas Krabatsch, Felix Berger, Joachim Photiadis, Evgenii Potapov
A 31-year-old patient with a univentricular heart and double-inlet left ventricle, moderate pulmonary stenosis, and severe mitral valve regurgitation experienced irreversible heart failure after mitral valve replacement. "Biventricular" mechanical circulatory support was initiated. The systemic circulation was supported using the HeartWare ventricular assist device (HVAD) (HeartWare, Framingham, MA) pump in the usual manner. The second pump was inserted into the right atrium and connected to the pulmonary artery after closure of the pulmonary valve and atrial separation...
August 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28730300/health-related-quality-of-life-in-paediatric-patients-with-congenital-heart-defects-association-with-the-type-of-heart-defect-and-the-surgical-technique
#4
A Heusch, H J Kahl, K O Hensel, G Calaminus
The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL. METHODS: Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected...
July 20, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28727893/outcome-after-prenatal-and-postnatal-diagnosis-of-complex-congenital-heart-defects-and-the-influence-of-genetic-anomalies
#5
Katya De Groote, Ellen Vanhie, Ellen Roets, Paul Ramaekers, Hans De Wilde, Joseph Panzer, Kristof Vandekerckhove, Thierry Bove, Katrien François, Koen Van Herck, Daniël De Wolf
OBJECTIVE: Determine prenatal detection rate, mortality and association with genetic abnormalities in patients with severe CHD. METHOD: Single center retrospective study in patients with severe CHD diagnosed pre or postnatally (2006 to 2014). RESULTS: 567 patients were included, 176 (31%) after prenatal diagnosis, with large differences in prenatal detection rate among CHD types. Coarctation (24%), tetralogy of Fallot (21%) and univentricular heart (19%) were the most prevalent CHD...
July 20, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28720202/a-novel-surgical-approach-to-mechanical-circulatory-support-in-univentricular-infants
#6
Avihu Z Gazit, Orlando Petrucci, Peter Manning, Mark Shepard, Sirine Baltagi, Kathleen Simpson, Chesney Castleberry, Charles Canter, Pirooz Eghtesady
BACKGROUND: Historically, the options for mechanical circulatory support in infants, particularly those with single-ventricle physiology, have been limited and outcomes have generally been poor. We report a new approach implemented for long-term support in a series of such patients. METHODS: This study is a single-center case series of 7 patients with single-ventricle physiology after stage 1 palliation supported with mechanical circulatory support using a novel technique, between May 2014 and September 2015...
July 15, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#7
Constanze Pfitzer, Paul C Helm, Hannah Ferentzi, Lisa-Maria Rosenthal, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions)...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28701613/management-of-iatrogenic-pulmonary-artery-injury-during-pulmonary-artery-banding
#8
Neeti Makhija, Shivani Aggarwal, Sachin Talwar, Suruchi Ladha, Deepanwita Das, Usha Kiran
Pulmonary Artery banding (PAB) is limited to selected patients who cannot undergo primary repair due to complex anatomy, associated co-morbidities, as a part of staged univentricular palliation, and for preparing the left ventricle prior to an arterial switch operation. We report a catastrophic iatrogenic complication in which the pulmonary artery was injured during the PAB. We discuss its multi-pronged management.
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28701598/the-blalock-and-taussig-shunt-revisited
#9
REVIEW
Usha Kiran, Shivani Aggarwal, Arin Choudhary, B Uma, Poonam Malhotra Kapoor
The systemic to pulmonary artery shunts are done as palliative procedures for cyanotic congenital heart diseases ranging from simple tetralogy of Fallots (TOFs)/pulmonary atresia (PA) to complex univentricular hearts. They allow growth of pulmonary arteries and maintain regulated blood flow to the lungs till a proper age and body weight suitable for definitive corrective repair is reached. We have reviewed the BT shunt with its anaesthtic considerations and management of associated complications.
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28696884/intra-atrial-y-graft-fontan-for-univentricular-heart-with-discontinuous-pulmonary-arteries
#10
Rajesh Sharma, Sudip Dutta Baruah, Ashutosh Marwah, Chandra Prakash Singh Chauhan
The Y-graft Fontan as described today suffers from the disadvantage of being hostage to restrictions imposed upon the design of the limbs of the Y by existent cardiac anatomy. We describe a patient with discontinuous pulmonary arteries following a prior Glenn shunt, who underwent Fontan completion using an intra-atrial Y-limb placement for recruitment of the discontinuous pulmonary artery. Intracardiac placement of the limb(s) of the Y-graft could potentially increase the applicability of this Fontan modification without being constrained by external cardiac anatomy...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28696882/risk-factors-for-mortality-in-reoperations-for-pediatric-and-congenital-heart-surgery-in-a-developing-country
#11
Carlos A Villa-Hincapie, Marisol Carreno-Jaimes, Carlos E Obando-Lopez, Jaime Camacho-Mackenzie, Juan P Umaña-Mallarino, Nestor F Sandoval-Reyes
BACKGROUND: The survival of patients with congenital heart disease has increased in the recent years, because of enhanced diagnostic capabilities, better surgical techniques, and improved perioperative care. Many patients will require reoperation as part of staged procedures or to treat grafts deterioration and residual or recurrent lesions. Reoperations favor the formation of cardiac adhesions and consequently increase surgery time; however, the impact on morbidity and operative mortality is certain...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28696873/modified-aortic-uncrossing-procedure-a-novel-approach-for-norwood-palliation-of-complex-univentricular-congenital-heart-disease-with-a-circumflex-aorta
#12
Roosevelt Bryant, William Wallen, Raheel Rizwan, David L Morales
The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The "aortic uncrossing procedure" described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28696869/feasibility-of-biventricular-repair-in-right-dominant-unbalanced-atrioventricular-septal-defect-a-new-echocardiographic-metric-to-refine-surgical-decision-making
#13
Ignacio Lugones, María Fernanda Biancolini, Julio César Biancolini, Ana M S de Dios, Germán Lugones
BACKGROUND: Unbalanced forms of atrioventricular septal defect continue to be challenging and present poor surgical outcomes. Echocardiographic indicators such as atrioventricular valve index, right ventricle/left ventricle inflow angle, and size of the ventricular septal defect have been identified as relevant discriminators that may guide surgical strategy. Our purpose is to describe another metric to refine surgical decision-making. METHODS: We outline a geometrical description of the anatomic features of atrioventricular septal defect and describe equations that help explain the interplay between the main echocardiographic variables...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28659205/-the-standard-mid-pregnancy-anomaly-scan-in-the-netherlands-what-is-its-effect
#14
M Gijtenbeek, M C Haak
- The mid-pregnancy anomaly scan was introduced into the Netherlands in 2007. The scan is performed at 18- 21 weeks of pregnancy.- The detection rate of open spina bifida is 94%.- In the Netherlands, 60% of all congenital heart defects are diagnosed prenatally compared with 35-40% in other countries. There is a strong relationship between the severity of the heart defect and the detection rate, as more than 95% of all univentricular heart defects are detected.- The detection rate of isolated cleft lip and cleft palate has increased from 32% to 87%...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28651676/anomalous-origin-of-the-left-coronary-artery-from-the-right-pulmonary-artery-in-a-univentricular-heart
#15
Ziyad M Binsalamah, Diego A Lara, Emmett D McKenzie
Anomalous origin of the left coronary artery from the right pulmonary artery in single ventricles is a very rare congenital anomaly. Failure to recognise it preoperatively may lead to adverse outcomes, including death. We report the case of a neonate with a univentricular heart in the form of a double-outlet right ventricle, mitral atresia with discrete coarctation of the aorta, and an incidental intraoperative finding of an anomalous origin of the left coronary artery from the right pulmonary artery. The patient underwent a successful repair with an uneventful postoperative course...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28603940/outcome-of-prenatally-diagnosed-fetal-heterotaxy-a-systematic-review-and-meta-analysis
#16
REVIEW
Danilo Italo Pio Buca, Asma Khalil, Giuseppe Rizzo, Alessandra Familiari, Silvia Di Giovanni, Marco Liberati, Daniela Murgano, Alessandra Ricciardulli, Francesco Fanfani, Giovanni Scambia, Francesco D'Antonio
OBJECTIVES: To assess the perinatal outcomes of fetuses affected by heterotaxy. METHODS: Medline, Embase and Cinhal were searched. Only studies reporting a prenatal diagnosis of isomerism were included. The outcomes observed were: associated cardiac and extra-cardiac anomalies, fetal arrhythmias, abnormal karyotype, type of surgical repair and perinatal mortality. The analysis was stratified according the type of heterotaxy syndrome (left, LAI, and right, RAI, atrial isomerism)...
June 12, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28589407/changes-of-cerebral-oxygenation-in-sequential-glenn-and-fontan-procedures-in-the-same-children
#17
Yasunori Yagi, Masataka Yamamoto, Hitoshi Saito, Toshihiro Mori, Yuji Morimoto, Takayoshi Oyasu, Tsuyoshi Tachibana, Yoichi M Ito
Recently, it is common to perform the Fontan procedure after the Glenn procedure as surgical repair for the univentricular heart. How the brain oxygen saturation (rSO2) values change with the cardiac restoration and the process of growth during these procedures in individual children remains unknown. In this study, we retrospectively studied rSO2 data as well as the perioperative clinical records of 30 children who underwent both Glenn and Fontan procedures by the same surgeon in the same institute. The rSO2 was measured at the beginning and end of each procedure with an INVOS 5100C...
June 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28577664/feasibility-of-exercise-stress-echocardiography-and-myocardial-response-in-patients-with-repaired-congenital-heart-disease
#18
Babar S Hasan, Fatima I Lunze, Najveen Alvi, Keri M Shafer, Jonathan Rhodes
BACKGROUND: Exercise stress echocardiography (ESE) can unmask ventricular dysfunction in asymptomatic patients with congenital heart disease (CHD), but its acquisition and interpretation is often challenging, and the method has not been validated in CHD. This study aimed to evaluate the feasibility of ESE using Doppler imaging and to assess myocardial response to exercise in patients with biventricular (BiV) and univentricular (UniV) circulation after CHD repair. METHODS: In this single-center prospective study, we recruited 55 participants (17 females), median age 14 years (8-22 years)...
June 2017: American Heart Journal
https://www.readbyqxmd.com/read/28575303/are-more-extensive-procedures-warranted-at-the-time-of-aortic-arch-reoperation-%C3%A2
#19
Jeremy S Y Wong, Melissa G Y Lee, Johann Brink, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
OBJECTIVES: To determine the early and late outcomes of patients undergoing aortic arch reoperations. METHODS: The follow-up of 70 patients undergoing a second arch operation (excluding univentricular physiology) between 1979 and 2015 was reviewed. Median age at initial arch operation and second operation was 9 days (interquartile range: 5-35) and 10 months (interquartile range: 3-64), respectively. The most common indication for initial arch operation was coarctation in 79% (55/70)...
June 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28566825/catheter-hemodynamic-assessment-of-the-univentricular-circulation
#20
REVIEW
Oliver Stumper, Gemma Penford
Children with very complex congenital heart disease not amenable to biventricular repair are increasingly being considered for a palliative univentricular care pathway. This involves a staged surgical approach culminating in the Fontan circulation with passive pulmonary blood flow and added resistances. The catheter based hemodynamic assessment at all three stages of this palliation is described in detail. Frequent pitfalls, inherent limitations and potential errors are discussed and clinical examples are illustrated...
May 2017: Annals of Pediatric Cardiology
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