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Palliation for children with valve disease

Damien Kenny
Congenital heart interventions are now replacing surgical palliation and correction in an evolving number of congenital heart defects. Right ventricular outflow tract and ductus arteriosus stenting have demonstrated favorable outcomes compared to surgical systemic to pulmonary artery shunting, and it is likely surgical pulmonary valve replacement will become an uncommon procedure within the next decade, mirroring current practices in the treatment of atrial septal defects. Challenges remain, including the lack of device design focused on smaller infants and the inevitable consequences of somatic growth...
March 29, 2018: Korean Circulation Journal
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
Jacek Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Bogusław Mazurek, Lidia Tomkiewicz-Pająk
BACKGROUND: Second-stage palliation with hemi-Fontan or bidirectional Glenn procedures has improved the outcomes of patients treated for single-ventricle heart disease. The aim of this study was to retrospectively analyze risk factors for death after second-stage palliation of single-ventricle heart and to compare therapeutic results achieved with the hemi-Fontan and bidirectional Glenn procedures. MATERIAL AND METHODS: We analyzed 60 patients who had undergone second-stage palliation for single-ventricle heart...
September 11, 2017: Cardiovascular Ultrasound
Mathieu Vergnat, Boulos Asfour, Claudia Arenz, Philipp Suchowerskyj, Benjamin Bierbach, Ehrenfried Schindler, Martin Schneider, Victor Hraska
OBJECTIVES: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. METHODS: From 2003 to 2015, 193 consecutive patients were included...
September 1, 2017: European Journal of Cardio-thoracic Surgery
Michael V Di Maria, Meryl S Cohen, Matthew A Harris, Mark A Fogel, Kevin K Whitehead
BACKGROUND: Children with single ventricle heart disease are at risk for developing systemic to pulmonary arterial collateral vessels that adversely impact short-term outcomes, although the effect on long-term outcomes remains unclear. Collateral flow (CollF) can be quantified using cardiac magnetic resonance (CMR) flow quantification. The velocity-time integral (VTI), obtained from spectral Doppler tracings, has been used in "runoff" lesions like aortic regurgitation to quantify insufficiency...
April 2015: World Journal for Pediatric & Congenital Heart Surgery
Mark Ruzmetov, Karl F Welke, Dale M Geiss, Randall S Fortuna
OBJECTIVES: Tricuspid valve regurgitation (TR) is a common finding in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation due to annular dilatation. The purpose of this study was to determine the incidence of severe TR and to evaluate the effect of surgically treated tricuspid valve (TV) disease on overall survival. METHODS: Between December 1988 and December 2012, 86 patients with HLHS underwent a Norwood procedure (mean age/weight, 11...
September 2014: Journal of Cardiac Surgery
Bas Bergmans, Janneke A E Kammeraad, Leon N A van Adrichem, Lonneke M Staals
We report on a case where craniosynostosis surgery for a left-sided coronal synostosis was performed successfully on an 11-month old infant with a hypoplastic left ventricle with a dysplastic mitral valve, double outlet right ventricle, transposition of the great arteries, atrial septal defect, multiple ventricular septal defects, and surgically applied pulmonary banding. Craniosynostosis surgery is considered high-risk surgery, because of possible sudden and extensive blood loss, and is usually performed in cardiopulmonary healthy children...
July 2014: Paediatric Anaesthesia
Christián Kreutzer, Jacqueline Kreutzer, Guillermo O Kreutzer
The first successful total right heart bypass via atriopulmonary anastomosis (APA) were reported in 1971 for patients with tricuspid atresia. At the Children's Hospital of Buenos Aires, the cohort of such procedures started in July, when the first fenestrated right heart by pass was performed, with the interposition of a homograft between the right atrial appendage and the main pulmonary artery. In the second patient, instead of placing a homograft, the APA was achieved with the patient's own pulmonary root harvested from the outflow tract of the right ventricle...
2013: Frontiers in Pediatrics
Jonathan W Cramer, Peter J Bartz, Pippa M Simpson, Steven D Zangwill
Turner syndrome (TS), a genetic abnormality affecting 1 in 2,500 people, is commonly associated with congenital heart disease (CHD). However, the surgical outcomes for TS patients have not been well described. This study reviewed the spectrum of CHD in TS at the authors' center. The authors report outcomes after coarctation of the aorta (CoA) repair or staged palliation of hypoplastic left heart syndrome (HLHS) and then compare the surgical outcomes with those of non-TS patients undergoing like repair. This retrospective chart review was conducted at the Children's Hospital of Wisconsin from 1999 to 2011...
February 2014: Pediatric Cardiology
Bhavesh Thakkar, Tarun Madan, A J Ashwal
Recurrent or residual right ventricular outflow tract obstruction after early surgical repair of congenital heart disease is one of the most frequent indications for either surgical or transcatheter reintervention. Transcatheter stent implantation across the stenotic right ventricular outflow tract or conduit is a safe and effective alternative to surgical reintervention. However, chronic deleterious effects of pulmonary regurgitation can potentially counterbalance the early improvement in clinical and hemodynamic parameters, sometimes necessitating further intervention...
July 2013: Journal of Invasive Cardiology
István Hartyánszky, András Kollár, Krisztina Kádár, László Ablonczy, Edit Novák, Attila Tóth
INTRODUCTION: The surgical strategy to manage multilevel left ventricular outflow tract diseases is changing constantly, however, the Ross-procedure has remained a standard method for 45 years. AIM: The aim of the study was to analyze early and mid-term results of Ross-procedure in congenital heart defects (single surgeon's results). METHODS: From 2001 until 2011 a total of 63 patients (age, 28 days-21 years; mean: 10 years, weight 3.4-96 kg; mean, 8...
February 10, 2013: Orvosi Hetilap
Raffaella Mormile, Ilaria Quadrini, Umberto Squarcia
Pediatric Cardiology as a discipline has been proposed to have been born on August 26, 1938, when Robert Gross at the age of 33 years, successfully ligated a patent ductus arteriosus of a 7 years girl at the Children's Hospital in Boston. In November 1944, Helen Taussig convinced Alfred Blalock to anastomose the left subclavian artery to the left pulmonary artery after Robert Gross had declined to cooperate with her. About the 1950s, at the University of Minneapolis, Clarence Walton Lillehei worked on a controlled "crossed circulation" in which the cardiopulmonary bypass machine was another human, generally one of the patient's parents...
February 2013: Clinical Cardiology
Patrick O Myers, Mustafa Cikirikcioglu, Cécile Tissot, Jan T Christenson, Maurice Beghetti, Afksendiyos Kalangos
BACKGROUND AND AIM OF THE STUDY: Triple valve replacement has poor early and long-term results, particularly in children, and few data are available on triple valve repair. Herein are reported the single-center, long-term results of combined aortic, mitral, and tricuspid valve repair in rheumatic children. METHODS: Ten children (mean age 12 +/- 3 years) with severe rheumatic aortic, mitral, and tricuspid regurgitation underwent triple valve repair over a 17-year period, using a tailored cusp extension to repair the aortic valve, and ring annuloplasty and Carpentier's techniques to repair the mitral and tricuspid valves...
September 2012: Journal of Heart Valve Disease
Bari Murtuza, Nathalie Dedieu, Alejandro Vazquez, Matthew Fenton, Michael Burch, Tain-Yen Hsia, Victor T Tsang, Martin Kostolny
OBJECTIVES: Previous studies have indicated that results for orthotopic heart transplantation (OHT) in patients with surgically palliated hypoplastic left heart (HLHS) are worse compared with patients with other forms of congenital heart disease (CHD) or acquired cardiomyopathy (CM) as well as those undergoing primary OHT for HLHS. In light of the decreasing donor pool for transplantation and increasing numbers of palliated HLHS patients with improving survival, we sought to review our results for OHT in surgically palliated HLHS patients and failing Glenn or Fontan circulations...
March 2013: European Journal of Cardio-thoracic Surgery
Edward J Hickey, Christopher A Caldarone, Brian W McCrindle
"Hypoplastic left heart syndrome" is an unsatisfactory term describing lethal underdevelopment of the left ventricle (LV). It represents the more severe end of a spectrum of LV hypoplasia, mandating single-ventricle palliation or cardiac transplantation. Less severe "borderline" ventricular hypoplasia may instead allow various biventricular therapeutic strategies and better long-term outcomes. In this review, we consider factors causing and modifying the abnormal development of the LV. LV hypoplasia is typically seen in association with left ventricular outflow tract obstruction, itself part of a spectrum of related defects with common etiologies...
January 3, 2012: Journal of the American College of Cardiology
Katharina E Thom, Andreas Hanslik, Christoph Male
Advances in medical and surgical care have resulted in improved survival of patients with congenital heart disease (CHD). Parallel to these progresses, an increasing number of immediate and long-term complications have been recognized. One important complication in CHD is the development of thrombosis. Children with a single functional cardiac ventricle usually require sequential steps of surgery: the initial Blalock-Taussig shunts (BTS) during the neonatal period, followed by the Glenn shunt, and finally, the Fontan shunt, the "definitive palliative" procedure...
October 2011: Seminars in Thrombosis and Hemostasis
Waldemar F Carlo, Kathleen E Carberry, Jeffrey S Heinle, David L Morales, E Dean McKenzie, Charles D Fraser, David P Nelson
OBJECTIVE: With improving operative mortality for staged palliation of hypoplastic left heart syndrome, interstage death accounts for an increasing proportion of hypoplastic left heart syndrome mortality. We investigated risk factors for death or cardiac transplantation during the interstage period between bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome. METHODS: Patients with hypoplastic left heart syndrome who underwent bidirectional Glenn between August 1995 and June 2007 were screened...
September 2011: Journal of Thoracic and Cardiovascular Surgery
Wilson Lam, Richard A Friedman
Improved surgical outcomes in children have led to a growing population of adults with congenital heart disease. Rhythm disturbances in the adult congenital heart disease (ACHD) patient can be intrinsic to the anomaly or acquired after palliation. Tachyarrhythmias, either supraventricular or ventricular, and bradyarrhythmias, either sinus node dysfunction or atrioventricular block, may occur frequently. Technological advances in intervention and surgical approaches have led to prophylactic and therapeutic reduction in arrhythmias...
April 2011: Methodist DeBakey Cardiovascular Journal
Minh Thanh Bui, Oswin Grollmus, Mohamedou Ly, Anca Mandache, Elie Fadel, Benoit Decante, Alain Serraf
OBJECTIVE: Patients with idiopathic pulmonary hypertension are at risk for right-sided heart failure and sudden death. Despite improvement in pharmacologic management, some still require lung transplantation. Potts anastomosis has been demonstrated as a good palliation in children to alleviate symptoms and medical therapy despite desaturation in the lower part of the body. Young adult patients with pulmonary hypertension and isosystemic pressure remain at risk, particularly at exercise...
November 2011: Journal of Thoracic and Cardiovascular Surgery
Bahaaldin Alsoufi, Cedric Manlhiot, Mamdouh Al-Ahmadi, Zohair Al-Halees, Brian W McCrindle, Ahmed Yehia Mousa, Yasser Al-Heraish, Avedis Kalloghlian
OBJECTIVES: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. METHODS: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection...
July 2011: Journal of Thoracic and Cardiovascular Surgery
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