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https://www.readbyqxmd.com/read/29666009/in-vitro-validation-of-a-self-driving-aortic-turbine-venous-assist-device-for-fontan-patients
#1
Kerem Pekkan, Ibrahim Basar Aka, Ece Tutsak, Erhan Ermek, Haldun Balim, Ismail Lazoglu, Riza Turkoz
BACKGROUND: Palliative repair of single ventricle defects involve a series of open-heart surgeries where a single-ventricle (Fontan) circulation is established. As the patient ages, this paradoxical circulation gradually fails, because of its high venous pressure levels. Reversal of the Fontan paradox requires an extra subpulmonic energy that can be provided through mechanical assist devices. The objective of this study was to evaluate the hemodynamic performance of a totally implantable integrated aortic-turbine venous-assist (iATVA) system, which does not need an external drive power and maintains low venous pressure chronically, for the Fontan circulation...
March 11, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29662811/management-of-coronary-sinus-ostial-atresia-during-a-staged-operation-of-a-functional-single-ventricle
#2
Seung Ri Kang, Won Kyoun Park, Bo Sang Kwon, Jae Kon Ko, Hyun Woo Goo, Jeong-Jun Park
Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly...
April 2018: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29614908/two-cases-of-aortic-root-replacement-after-fontan-completion
#3
Kenji Suzuki, Takahiko Sakamoto, Narutoshi Hibino, Shigeyuki Aomi, Toshiharu Shin'oka, Kenji Yamazaki
Aortic root dilatation is a well-known complication in patients with congenital aortic valve malformation, tetralogy of Fallot, or a double outlet right ventricle. We report two rare patients who underwent composite graft replacement of the aortic root with a mechanical valve, the so-called Bentall-type operation, after Fontan completion. The pathological examination on the resected aortic wall revealed mucoid degeneration in tunica media and elastic fiber fragmentation. Our report emphasizes the need for close observation of these patients over a long-term period...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29609888/impact-of-hemodynamics-and-fluid-energetics-on-liver-fibrosis-after-fontan-operation
#4
Phillip M Trusty, Zhenglun Wei, Jack Rychik, Pierre A Russo, Lea F Surrey, David J Goldberg, Mark A Fogel, Ajit P Yoganathan
OBJECTIVE: The staged Fontan procedure has shown promising short-term outcomes in patients with single ventricles. However, Fontan-associated liver disease is a marked problem as patients age. The purpose of this study is to investigate the relationship between hemodynamics and liver fibrosis in patients undergoing the Fontan. METHODS: A total of 33 patients undergoing the Fontan with liver fibrosis were included in this study. Cardiac magnetic resonance and phase-contrast cardiac magnetic resonance data, as well as catheterization measurements and liver biopsies, were obtained for each patient...
March 7, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29544424/association-of-habitual-activity-and-body-mass-index-in-survivors-of-congenital-heart-surgery-a-study-of-children-and-adolescents-with-tetralogy-of-fallot-transposition-of-the-great-arteries-and-fontan-palliation
#5
Michael L O'Byrne, Michael G McBride, Stephen Paridon, Elizabeth Goldmuntz
BACKGROUND: Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease. METHODS: A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29492393/transplantation-in-the-single-ventricle-population
#6
Louise A Kenny, Fabrizio DeRita, Mohamed Nassar, John Dark, Louise Coats, Asif Hasan
The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair...
January 2018: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29492392/overview-of-adult-congenital-heart-transplants
#7
Roosevelt Bryant, David Morales
Transplantation for adult patients with congenital heart disease (ACHD) is a growing clinical endeavor in the transplant community. Understanding the results and defining potential high-risk patient subsets will allow optimization of patient outcomes. This review summarizes the scope of ACHD transplantation, the mechanisms of late ventricular dysfunction, the ACHD population at risk of developing heart failure, the indications and potential contraindications for transplant, surgical considerations, and post-transplant outcomes...
January 2018: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29473337/acoustic-radiation-force-impulse-of-the-liver-after-fontan-operation-correlation-with-cardiopulmonary-exercise-test
#8
Michael V Di Maria, Lindsey Silverman, Adel K Younoszai, Mariana L Meyers, Kelley E Capocelli, Michael R Narkewicz
BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort...
February 23, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29470938/diagnostic-performance-of-mr-elastography-for-liver-fibrosis-in-children-and-young-adults-with-a-spectrum-of-liver-diseases
#9
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
https://www.readbyqxmd.com/read/29464511/computational-investigation-of-a-self-powered-fontan-circulation
#10
Marcus W Ni, Ray O Prather, Giovanna Rodriguez, Rachel Quinn, Eduardo Divo, Mark Fogel, Alain J Kassab, William M DeCampli
Children born with anatomic or functional "single ventricle" must progress through two or more major operations to sustain life. This management sequence culminates in the total cavopulmonary connection, or "Fontan" operation. A consequence of the "Fontan circulation", however, is elevated central venous pressure and inadequate ventricular preload, which contribute to continued morbidity. We propose a solution to these problems by increasing pulmonary blood flow using an "injection jet" (IJS) in which the source of blood flow and energy is the ventricle itself...
February 20, 2018: Cardiovascular Engineering and Technology
https://www.readbyqxmd.com/read/29464136/anesthetic-management-of-pheochromocytoma-resection-in-adults-with-single-ventricle-physiology
#11
Giancarlo Suffredini, Natalia Diaz-Rodriguez, Krishnan Chakravarthy, Aarti Mathur, Heather K Hayanga, Steve M Frank, Richard E Ringel, Stephen Freiberg, Viachaslau M Barodka, Jochen Steppan
Survival rates for patients with palliated congenital heart disease are increasing, and an increasing number of adults with cyanotic congenital heart disease (CCHD) might require surgical resection of pheochromocytoma-paraganglioma (PHEO-PGL). A recent study supports the idea that patients with a history of CCHD and current or historical cyanosis might be at increased risk for developing PHEO-PGL. We review the anesthetic management of two adults with single-ventricle physiology following Fontan palliation presenting for PHEO-PGL resection and review prior published case reports...
December 8, 2017: Curēus
https://www.readbyqxmd.com/read/29448010/improved-outcomes-in-management-of-hypoplastic-left-heart-syndrome-associated-with-congenital-diaphragmatic-hernia-an-algorithmic-approach
#12
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. 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 was 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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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