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palliative heart surgery

Nathaly M Sweeney, Shareef A Nahas, Shimul Chowdhury, Miguel Del Campo, Marilyn C Jones, David P Dimmock, Stephen F Kingsmore, Rcigm Investigators
Congenital diaphragmatic hernia (CDH) results from incomplete formation of the diaphragm leading to herniation of abdominal organs into the thoracic cavity. CDH is associated with pulmonary hypoplasia, congenital heart disease and pulmonary hypertension. Genetically, it is associated with aneuploidies, chromosomal copy number variants, and single gene mutations. CDH is the most expensive non-cardiac congenital defect: Management frequently requires implementation of Extracorporeal Membrane Oxygenation (ECMO), which increases management expenditures 2...
March 16, 2018: Cold Spring Harbor Molecular Case Studies
Faisal Junejo, Mohammad Irfan Akhtar, Mohammad Hamid, Syed Shabbir Ahmed, Fauzia Minai, Muneer Amanullah
OBJECTIVE: To compare the intravenous boluses and intravenous continuous infusion of tranexamic acid (TXA) to reduce postoperative bleeding in cyanotic congenital heart disease surgeries. STUDY DESIGN: Single-blinded randomised clinical trial. PLACE AND DURATION OF STUDY: Anaesthesia Department, The Aga Khan University Hospital, Karachi, from July 2016 to April 2017. METHODOLOGY: Sixty patients of cyanotic congenital heart disease, undergoing either palliative or corrective surgery involving cardiopulmonary bypass (CPB), were recruited...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
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
Mio Noma, Muneaki Matsubara, Chiho Tokunaga, Tomomi Nakajima, Bryan James Mathis, Hiroaki Sakamoto, Yuji Hiramatsu
BACKGROUND: Although pulmonary artery banding (PAB) is a common palliative procedure for pediatric heart malformation, there are concerns of pressure overload and concomitant immune reactions in the right ventricle causing postsurgical complications such as pericardial effusion. At this time, no clear guidelines as to potential risk factors or procedural contraindications have been widely disseminated. Therefore, a study was undertaken to examine wide-ranging factors to find potential biomarkers for postsurgical pericardial effusion formation risk...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
W M Crescini, A Muralidaran, I Shen, A LeBlan, J You, C Giacomuzzi, M M Treggiari
BACKGROUND: Acute normovolemic hemodilution (ANH) is considered safe and effective in decreasing perioperative transfusion in paediatric populations undergoing high blood-loss surgeries. We determined the association between ANH and the intraoperative use of allogeneic blood products in paediatric cardiac surgery patients. METHODS: This is a single-centre retrospective cohort study including paediatric patients between 0 and 36 months of age undergoing surgical repair or palliation of their cardiac defect with the use of cardiopulmonary bypass between November 2013 and November 2014...
March 4, 2018: Acta Anaesthesiologica Scandinavica
Wolf B Kratzert, Eva K Boyd, Johanna C Schwarzenberger
Survival of adults with congenital heart disease (CHD) has improved significantly over the last 2 decades, leading to an increase in hospital and intensive care unit (ICU) admissions of these patients. Whereas most of the ICU admissions in the past were related to perioperative management, the incidence of medical emergencies from long-term sequelae of palliative or corrective surgical treatment of these patients is rising. Intensivists now are confronted with patients who not only have complex anatomy after congenital cardiac surgery, but also complex pathophysiology due to decades of living with abnormal cardiac anatomy and diseases of advanced age...
November 16, 2017: Journal of Cardiothoracic and Vascular Anesthesia
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
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
Jun Won Kim, Chang-Ki Hong, Yoon Jin Cha, Se Hoon Kim, Chang-Ok Suh, Kyu-Sung Lee
BACKGROUND: Chordomas are rare tumors showing locally aggressive nature and high rates of local recurrences. Distant metastases are infrequently reported and cardiac metastases are extremely rare. CASE DESCRIPTION: A 32-year-old woman had been diagnosed with a large chordoma involving the clivus. She received a total removal of the tumor and adjuvant intensity-modulated radiotherapy. Four and a half years after the surgery, she presented with multifocal lesions in the ventricles and the right atrium, although an open thoracotomy revealed chronic inflammation...
February 14, 2018: World Neurosurgery
Hugo R Martinez, Stephanie M Ware, Marcus S Schamberger, John J Parent
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy. In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female)...
September 2017: Progress in Pediatric Cardiology
Bhavna Kakkar, Munisha Agarwal, Aakanksha Sehgal, Neelam Prasad Govil
Glenn shunt is an anastomosis between superior vena cava and right pulmonary artery to palliate patients with single ventricle physiology of the heart. Chylothorax is a frequent and troublesome complication after the creation of this shunt, which if not controlled with medical management, might require pleurodesis, and thoracic duct ligation. Video-assisted thoracoscopic surgery (VATS) causes less postoperative pain, earlier mobilization, lower overall morbidity, a shortened hospital stay with reduced cost, and a cosmetic incision...
January 2018: Saudi Journal of Anaesthesia
Felix Neunhoeffer, Michael Hofbeck, Martin Ulrich Schuhmann, Jörg Fuchs, Christian Schlensak, Martin Esslinger, Ines Gerbig, Vanya Icheva, Ellen Heimberg, Matthias Kumpf, Jörg Michel
OBJECTIVE: Although infants following major surgery frequently require RBC transfusions, there is still controversy concerning the best definition for requirement of transfusion in the individual patient. The aim of this study was to determine the impact of RBC transfusion on cerebral oxygen metabolism in noncardiac and cardiac postsurgical infants. DESIGN: Prospective observational cohort study. SETTING: Pediatric critical care unit of a tertiary referral center...
February 5, 2018: Pediatric Critical Care Medicine
Margarita Brida, Gerhard-Paul Diller, Michael A Gatzoulis
The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol...
January 30, 2018: Circulation
Akihiko Higashida, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Tomohiro Nakata, Hideto Ozawa, Kenichi Kurosaki, Hajime Ichikawa
OBJECTIVES: To assess the feasibility of hybrid Stage I palliation consisting of bilateral pulmonary artery bandings and ductal stenting for patients with 2 ventricular cavities and hypoplastic left heart structures. METHODS: Eleven consecutive patients who underwent hybrid Stage I palliation between 2010 and 2017 were enrolled. The diagnoses were interrupted aortic arch/coarctation of the aorta, ventricular septal defect and significant left ventricular (LV) outflow tract obstruction in 5 patients, critical aortic stenosis and reduced LV contraction in 3 patients and hypoplastic left heart complex in 3 patients...
January 24, 2018: Interactive Cardiovascular and Thoracic Surgery
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
Keti Vitanova, Cornelius Leopold, Jelena Pabst von Ohain, Cordula Wolf, Elisabeth Beran, Rüdiger Lange, Julie Cleuziou
BACKGROUND:  Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease. Although it is thought to be a simple operation, it is associated with significant morbidity and mortality. METHODS:  Data for all neonates who underwent surgery for a systemic-to-pulmonary artery shunt between 2000 and 2016 were reviewed. The study endpoints were shunt failure and shunt-related mortality. Shunt failure was defined as a shunt dysfunction because of thrombosis or stenosis requiring intervention or reoperation; shunt mortality was defined as death because of a shunt dysfunction...
January 19, 2018: Thoracic and Cardiovascular Surgeon
Takashi Kido, Kyoichi Nishigaki, Yoichi Kawahira, Koji Kagisaki, Kazuki Tanimoto, Eiji Ehara, Yosuke Murakami
OBJECTIVES: This study aimed to review the clinical outcomes of staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. METHODS: Six extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation underwent staged cardiac surgery between 2005 and 2017. The median birthweight was 895 g (range 620-990 g), and the median gestational age was 28 weeks (range 23-31 weeks)...
January 9, 2018: Interactive Cardiovascular and Thoracic Surgery
Hayley S Hancock, Ken Pituch, Karen Uzark, Priya Bhat, Carly Fifer, Maria Silveira, Sunkyung Yu, Suzanne Welch, Janet Donohue, Ray Lowery, Ranjit Aiyagari
Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge...
April 2018: Cardiology in the Young
Wolfgang Boettcher, Matthias Redlin, Frank Dehmel, Katharina Graefe, Mi-Young Cho, Helmut Habazettl, Marian Kukucka
OBJECTIVES: In paediatric cardiac surgery, body weight-adjusted miniaturized cardiopulmonary bypass (CPB) circuits within a comprehensive blood-sparing approach can reduce transfusion requirements. Haemodilution resulting from mixing the patient's blood with a CPB crystalloid solution may be reduced to the extent that asanguineous priming becomes possible. Therefore, we adopted asanguineous priming in our clinical routine. Our goal was to report the effects of asanguineous priming on transfusion requirements, clinical features associated with transfusion and effects on in-hospital morbidity...
January 2, 2018: European Journal of Cardio-thoracic Surgery
Timothy Colen, Shelby Kutty, Richard B Thompson, Edythe Tham, Andrew S Mackie, Ling Li, Dongngan T Truong, Michiko Maruyama, Jeffrey F Smallhorn, Nee Scze Khoo
BACKGROUND: Tricuspid regurgitation (TR) is an important risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS), yet the evolution of tricuspid valve (TV) dysfunction in HLHS is poorly understood. This study sought to examine changes in TV function in HLHS between the first two stages of surgical palliation and to determine the mechanism of TR at the time of stage two surgery-bidirectional cavopulmonary anastomosis (BCPA). METHODS: We prospectively investigated 44 infants at two time points-prior to Norwood-Sano (T1 - median age 5...
December 29, 2017: Journal of the American Society of Echocardiography
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