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https://www.readbyqxmd.com/read/29448010/improved-outcomes-in-management-of-hypoplastic-left-heart-syndrome-associated-with-congenital-diaphragmatic-hernia-an-algorithmic-approach
#1
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival of neonates with CDH and HLHS is only 1-5%. We review our experience with CDH and HLHS and compare our outcomes to published literature. METHODS: Retrospective review of all neonates with CDH and HLHS at our institution over a 10 year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies and outcomes were reviewed and an algorithmic approach is proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29433966/temporal-changes-in-liver-stiffness-after-fontan-operation-results-of-serial-magnetic-resonance-elastography
#2
Alexander Egbe, William R Miranda, Heidi M Connolly, Arooj R Khan, Mohamad Al-Otaibi, Sudhakar K Venkatesh, Douglas Simonetto, Patrick Kamath, Carole Warnes
BACKGROUND: The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes. METHODS: Retrospective review of Fontan patients who had >1 liver MRE, 2010-2016. Annual change in MRE-LS was the quotient of the difference between baseline and subsequent MRE-LS, and the interval between scans...
February 9, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29431283/predictive-value-of-presuperior-cavopulmonary-anastomosis-cardiac-catheterization-at-increased-altitude
#3
Michael V Di Maria, Matthew Mulvahill, James Jaggers, David Dunbar Ivy, Adel K Younoszai
OBJECTIVE: Infants with single ventricle physiology typically undergo cardiac catheterization prior to superior cavopulmonary anastomosis (SCPA) to assess operative suitability. Predictors of poor outcome at sea level include elevated pulmonary artery pressure (mPAP), indexed pulmonary vascular resistance (PVRi), age <3 months, significant atrioventricular valve regurgitation, among others. Increased altitude has vasoconstrictive effects on the pulmonary vasculature, which may affect pre-SCPA hemodynamics and outcomes...
February 12, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29430429/extracardiac-conduit-fontan-operation-with-reduction-aortoplasty-for-left-pulmonary-artery-compression-after-a-norwood-procedure-in-a-patient-with-double-inlet-left-ventricle
#4
Jae Won Song, Woong Han Kim, Jae Gun Kwak, Ji Young Park
Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure...
February 2018: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29428622/targeted-increase-in-pulmonary-blood-flow-in-a-bidirectional-glenn-circulation
#5
Samuel L Casella, Aditya Kaza, Pedro Del Nido, James E Lock, Audrey Marshall
Objective In patients with a Glenn shunt and unilateral pulmonary vascular abnormalities, supplemental pulmonary blood flow may improve antegrade flow in the target lung and may permit eventual Fontan completion, while mitigating the risks of uncontrolled additional flow to the contralateral lung. We attempted this by inserting a small aortopulmonary shunt into the affected pulmonary artery and banding the central pulmonary artery. .Methods We performed a retrospective review of all patients who underwent this procedure from 2000-2015...
February 8, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29421006/mechanism-for-temporal-changes-in-exercise-capacity-after-fontan-palliation-role-of-doppler-echocardiography
#6
Alexander Egbe, Arooj R Khan, William R Miranda, Naser M Ammash, Carole A Warnes, Sameh S Said, Nathaniel W Taggart, Emmanuel Akintoye, Gruschen R Veldtman, Heidi M Connolly
BACKGROUND: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o2). METHODS: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o2 and LV function indices...
February 2018: American Heart Journal
https://www.readbyqxmd.com/read/29406387/in-silico-thrombin-and-factor-xa-generation-profiles-in-adult-patients-after-fontan-operation
#7
Matthew Gissel, Lidia Tomkiewicz-Pajak, Piotr Podolec, Piotr Hoffman, Olga Trojnarska, Magdalena Lipczyńska, Anetta Undas, Kathleen E Brummel-Ziedins
: Single-ventricle defects are associated with increased risk of thromboembolic events. To analyze the prothrombotic potential in a long-term follow-up on Fontan patients via plasma contribution to thrombin and factor (F)Xa generation profiles. Thrombin and FXa generation was simulated from plasma concentrations of FII, FV, FVII, FVIII, FIX, FX, antithrombin and tissue factor (TF) pathway inhibitor from Fontan patients (n = 48) and healthy controls (n = 34). TF and thrombin-antithrombin complex (TAT) were measured by ELISA...
February 5, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29402692/life-after-surviving-fontan-surgery-a-meta-analysis-of-the-incidence-and-predictors-of-late-death
#8
REVIEW
C L Poh, Y d'Udekem
AIM: We now know that 20-40% of patients with a single ventricle will develop heart failure after the second decade post-Fontan surgery. However, we remain unable to risk-stratify the cohort to identify patients at highest risk of late failure and death. We conducted a systematic review of all reported late outcomes for patients with a Fontan circulation to identify predictors of late death. METHODS: We searched MEDLINE, Embase and PubMed with subject terms ("single ventricle", "Hypoplastic left heart syndrome", "congenital heart defects" or "Fontan procedure") AND ("heart failure", "post-operative complications", "death", "cause of death", "transplantation" or "follow-up studies") for relevant studies between January 1990 and December 2015...
December 20, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29396581/routine-cardiac-catheterization-prior-to-fontan-operation-is-it-a-necessity
#9
Bassel Mohammad Nijres, Joshua J Murphy, Karim Diab, Sawsan Awad, Ra-Id Abdulla
Prior to the Fontan procedure, patients with single ventricle physiology with Glenn shunt are typically referred for cardiac catheterization to assess hemodynamics and potentially provide interventional measures. Currently, echocardiography provides detailed information which together with other non-invasive imaging such as CT scan and MRI may obviate the need for routine cardiac catheterization prior to the Fontan procedure. In this study, we examine the findings in cardiac catheterization in this population to determine: (a) the accuracy of echocardiography in providing adequate information prior to the Fontan procedure, particularly in identifying those in need of per-catheter intervention, and (b) the percentage of patients requiring interventional procedures during cardiac catheterization...
February 3, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29372588/five-decades-of-the-fontan-operation-a-systematic-review-of-international-reports-on-outcomes-after-univentricular-palliation
#10
REVIEW
Laura S Kverneland, Peter Kramer, Stanislav Ovroutski
Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients' characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted...
January 25, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29372280/correction-to-routine-sildenafil-does-not-improve-clinical-outcomes-after-fontan-operation
#11
J Leslie Gaddis Collins, Mark A Law, Santiago Borasino, W Clinton Erwin, David C Cleveland, Jeffrey A Alten
The original version of this article unfortunately contained a mistake. The presentation of Table 2 was incorrect .The corrected table is given below.
January 25, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29358023/surgical-strategy-and-outcomes-for-the-delayed-diagnosis-of-pulmonary-atresia-with-intact-ventricular-septum
#12
Xiaomin He, Botao Gao, Guocheng Shi, Huiwen Chen, Xinwei Du, Zhiwei Xu, Jinfen Liu, Zhongqun Zhu, Jinghao Zheng
BACKGROUND: In the present study, we summarize the experiences and evaluate clinical outcomes for the delayed diagnosis of pulmonary atresia with intact ventricular septum (PAIVS) patients when undergoing an initial visit and diagnosis in our heart center. METHODS: Fifty-eight cases of delayed diagnosis of PAIVS in patients aged more than 6 months between January 2006 and June 2016 were reviewed in our hospital. The median age at initial diagnosis was 12.2 months (range, 6...
January 18, 2018: Journal of Cardiology
https://www.readbyqxmd.com/read/29340873/fontan-surgical-planning-previous-accomplishments-current-challenges-and-future-directions
#13
Phillip M Trusty, Timothy C Slesnick, Zhenglun Alan Wei, Jarek Rossignac, Kirk R Kanter, Mark A Fogel, Ajit P Yoganathan
The ultimate goal of Fontan surgical planning is to provide additional insights into the clinical decision-making process. In its current state, surgical planning offers an accurate hemodynamic assessment of the pre-operative condition, provides anatomical constraints for potential surgical options, and produces decent post-operative predictions if boundary conditions are similar enough between the pre-operative and post-operative states. Moving forward, validation with post-operative data is a necessary step in order to assess the accuracy of surgical planning and determine which methodological improvements are needed...
January 16, 2018: Journal of Cardiovascular Translational Research
https://www.readbyqxmd.com/read/29337121/the-society-of-thoracic-surgeons-congenital-heart-surgery-database-2018-update-on-outcomes-and-quality
#14
Jeffrey P Jacobs, John E Mayer, Sara K Pasquali, Kevin D Hill, David M Overman, James D St Louis, S Ram Kumar, Carl L Backer, Charles D Fraser, James S Tweddell, Marshall L Jacobs
The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry which captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency...
January 11, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29327115/investigation-of-an-appropriate-contrast-enhanced-ct-protocol-for-young-patients-following-the-fontan-operation
#15
Motoo Nakagawa, Yoshiyuki Ozawa, Norikazu Nomura, Sachiko Inukai, Ayano Shiba, Keita Sakurai, Masashi Shimohira, Yuta Shibamoto
PURPOSE: Children with congenital heart diseases (CHDs) may need to be followed up with contrast-enhanced CT following the Fontan operation because complications such as the occlusion of conduits may occur. The purpose of the present study was to develop an adequate contrast-enhanced CT protocol for children with CHD following the Fontan operation. MATERIALS AND METHODS: Between July 2012 and July 2017, 29 CT examinations for 26 patients aged 2-11 years (median 5 years) with CHD following the Fontan operation were performed using dual-source CT...
January 11, 2018: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/29326110/contemporary-management-and-outcomes-in-congenitally-corrected-transposition-of-the-great-arteries
#16
REVIEW
Shelby Kutty, David A Danford, Gerhard-Paul Diller, Oktay Tutarel
Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration...
January 11, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29318931/lessons-learned-from-a-critical-analysis-of-the-fontan-operation-over-three-decades-in-a-single-institution
#17
Luiz Fernando Caneo, Aida L R Turquetto, Rodolfo A Neirotti, Maria A Binotto, Leonardo A Miana, Carla Tanamati, Juliano G Penha, João B D Silveira, Thais M Alexandre E Silva, Fabio B Jatene, Marcelo B Jatene
BACKGROUND: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors. METHODS: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29315433/indications-for-and-results-of-axillary-arterio-venous-fistula-in-patients-with-a-functionally-single-ventricle-after-cavopulmonary-anastomosis
#18
Ivan A Yurlov, Vladimir P Podzolkov, Dmitry V Kovalev, Mikhail M Zelenikin, Victor B Samsonov, Nikolay S Chikin, Lyudmila A Yurpol'skaya, Tatyana V Mataeva
OBJECTIVES: This study aimed to investigate the indications for and the results of axillary arterio-venous fistula in patients with a functionally single ventricle at mid-term follow-up after bidirectional cavopulmonary connection. METHODS: From 2012 to 2017, 20 patients aged 21 ± 8.7 years underwent axillary arterio-venous fistula after bidirectional cavopulmonary connection in our institution. The interval between bidirectional cavopulmonary connection and axillary arterio-venous fistula was 13...
January 5, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29305367/embolic-stroke-in-a-postpartum-patient-with-fontan-physiology
#19
Katherine VanHise, Linda Li, Emily Smith, Niamh Condon
In cardiac physiology, single ventricle typically refers to the presence of a dominant systemic ventricle along with a hypoplastic ventricle. The Fontan operation is used to repair the single ventricle by directing deoxygenated systemic venous flow to the pulmonary arterial circulation. Normal pregnancy physiology, particularly increased intravascular volume, increased heart rate, increased cardiac output, hypercoagulability and decreased systemic vascular resistance, can exacerbate cardiac disease in patients with Fontan circulation, leading to pregnancy complications...
January 5, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29288017/improved-lung-perfusion-after-left-pulmonary-artery-patch-enlargement-during-the-norwood-operation
#20
Mona Salehi Ravesh, Jens Scheewe, Tim Attmann, Abdullah Al Bulushi, Marka-Jill Jussli-Melchers, Michael Jerosch-Herold, Dominik D Gabbert, Philip Wegner, Hans-Heiner Kramer, Carsten Rickers
BACKGROUND: Optimal pulmonary perfusion is crucial for a well-functioning Fontan circulation in patients with hypoplastic left heart syndrome (HLHS). To obtain an adequate size of the left pulmonary artery (LPA), patch enlargement is a routine part of Hemi-Fontan procedure in our center. However, LPA patch enlargement at the time of the modified Norwood procedure may have surgical advantages. Therefore, the aim of this study was to evaluate if anatomical and functional effects of the new approach are superior...
December 26, 2017: Annals of Thoracic Surgery
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