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Single ventricle

Frank W DiPaola, Kurt R Schumacher, Caren S Goldberg, Joshua Friedland-Little, Aishwarya Parameswaran, Jonathan R Dillman
OBJECTIVES: Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. METHODS: Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available...
October 17, 2016: European Radiology
Manavi Tyagi, Theodora Fteropoulli, Catherine S Hurt, Shashivadan P Hirani, Lorna Rixon, Anna Davies, Nathalie Picaut, Fiona Kennedy, John Deanfield, Shay Cullen, Stanton P Newman
OBJECTIVE: We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. METHODS: A total of 310 patients participated in this study. Patients were classified into four structural complexity groups - tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions...
October 18, 2016: Cardiology in the Young
Aabid Hussain Mir, Zulfiqar Ali, Bashir Ahmad Dar, Imtiaz A Naqash, Samreena Bashir
Congenital heart defects are associated with various physiological disturbances. They pose anesthetic challenges for both cardiac and noncardiac surgeries. Atrioventricular septal defects are due to a developmental failure in the separation of atria and the ventricles into separate chambers and failure in the separation of mitral and tricuspid valves. We present a case of a child (1½ years), weighing 10 kg, diagnosed as congenital hydrocephalus who was planned for ventriculoperitoneal shunt. Child was having an oxygen saturation of 76% on room air...
September 2016: Anesthesia, Essays and Researches
Wonho Kim, Ramon Quesada, Melanie B Schernthaner, Raul E Herrera, Juan-Carlos Zevallos, Victor N Becerra, Juan M Acuña, Barry T Katzen
OBJECTIVE: We investigated whether successful revascularization of total occlusion of a large lower-extremity artery is associated with improvement of left ventricular (LV) diastolic function. BACKGROUND: Total occlusion of a large lower-extremity artery might affect the systemic vascular resistance and increase the afterload, because the left ventricle must work harder to eject blood into a smaller vascular bed. Chronic elevation of afterload is a cause of LV diastolic dysfunction...
October 15, 2016: Journal of Invasive Cardiology
Ruilan Zhang, Zhenggang Zhang, Michael Chopp
Hypoxic/ischemic injury is the single most important cause of disabilities in infants, while stroke remains a leading cause of morbidity in children and adults around the world. The injured brain has limited repair capacity, and thereby only modest improvement of neurological function is evident post injury. In rodents, embryonic neural stem cells in the ventricular zone generate cortical neurons, and adult neural stem cells in the ventricular-subventricular zone of the lateral ventricle produce new neurons through animal life...
October 14, 2016: Journal of Cerebral Blood Flow and Metabolism
Chaeyoun Oh, Joong Kee Youn, Ji-Won Han, Gi Beom Kim, Hyun-Young Kim, Sung-Eun Jung
INTRODUCTION: The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC). CLINICAL FINDINGS: We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue...
October 2016: Medicine (Baltimore)
Manchu Chaithanya, Suresh Sr
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
R S Monteiro, D P Dob, M R Cauldwell, M A Gatzoulis
Women with a single ventricle circulation palliated with the Fontan operation require specialist multidisciplinary management. We report 14 such cases with successful pregnancies and detail the pathophysiology encountered. A combined obstetric and cardiac service between Chelsea and Westminster Hospital and Royal Brompton Hospital provides care for women with heart disease, and maintains a prospective database of referred women. We searched this database for women with a known Fontan circulation and reviewed the case notes and electronic patient records between January 1994 and December 2015...
September 7, 2016: International Journal of Obstetric Anesthesia
Bui Quoc Thang, Tatsuya Furugaki, Motoo Osaka, Yutaka Watanabe, Shinya Kanemoto, Fuminaga Suetsugu, Yuji Hiramatsu
PURPOSE: There is less certainty regarding the best strategy for treating neonates with functional single ventricle (SV) and hypoplastic aortic arch. We have applied a modified extended aortic arch anastomosis (EAAA) and main pulmonary artery banding (PAB) as an initial palliation in neonates with transverse arch hypoplasia and assessed the mid-term outcomes. METHODS: In total, 10 neonates with functional SV and extensive hypoplasia or interruption of the arch underwent a modified EAAA (extended arch anastomosis with a subclavian flap) concomitant with main PAB through a thoracotomy without cardiopulmonary bypass...
October 11, 2016: Annals of Thoracic and Cardiovascular Surgery
Hung-Yi Lai, Ching-Hsin Lee, Ching-Yi Lee
BACKGROUND: For patients suffering from primary brain injury, monitoring intracranial pressure alone is not enough to reflect the dynamic intracranial condition. In our previous study, a segment of the pressure-volume curve can be expressed by the parabolic regression model with single indicator "a". The aim of this study is to evaluate if the indicator "a" can reflect intracranial conditions. METHODS: Patients with traumatic brain injury, spontaneous intracranial hemorrhage, and/or hydrocephalus who had external ventricular drainage from January 2009 to February 2010 were included...
2016: PloS One
Edith V Sullivan, Barton Lane, Dongjin Kwon, M J Meloy, Susan F Tapert, Sandra A Brown, Ian M Colrain, Fiona C Baker, Michael D De Bellis, Duncan B Clark, Bonnie J Nagel, Kilian M Pohl, Adolf Pfefferbaum
Structural MRI of volunteers deemed "normal" following clinical interview provides a window into normal brain developmental morphology but also reveals unexpected dysmorphology, commonly known as "incidental findings." Although unanticipated, these anatomical findings raise questions regarding possible treatment that could even ultimately require neurosurgical intervention, which itself carries significant risk but may not be indicated if the anomaly is nonprogressive or of no functional consequence. Neuroradiological readings of 833 structural MRI from the National Consortium on Alcohol and NeuroDevelopment in Adolescence (NCANDA) cohort found an 11...
October 8, 2016: Brain Imaging and Behavior
Min-Seok Kim, Hong-Gook Lim, Woong Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly. SUBJECTS AND METHODS: Forty-eight patients with Ebstein's anomaly who underwent open heart surgery between 1982 and 2013 were included. Median age at operation was 5.6 years (1 day-42.1 years). Forty-five patients (93.7%) demonstrated tricuspid valve (TV) regurgitation of less than moderate degree. When the patients were divided according to Carpentier's classification, types A, B, C, and D were 11, 21, 12, and 4 patients, respectively...
September 2016: Korean Circulation Journal
Mineto Kamata, Corey Stiver, Aymen Naguib, Dmitry Tumin, Joseph D Tobias
OBJECTIVES: The objectives of this study were to evaluate the effect of ventricular morphology on perioperative outcomes during Fontan surgery. DESIGN: Retrospective cohort study. SETTING: Single standing, not-for-profit pediatric hospital. PARTICIPANTS: A total of 72 patients who underwent Fontan surgery using cardiopulmonary bypass without aortic cross-clamp between January 1, 2009 and December 31, 2014. INTERVENTIONS: None...
July 19, 2016: Journal of Cardiothoracic and Vascular Anesthesia
Carlos M Mery, R Michael Nieto, Luis E De León, Shaine A Morris, Wei Zhang, John L Colquitt, Iki Adachi, Lauren C Kane, Jeffrey S Heinle, E Dean McKenzie, Charles D Fraser
BACKGROUND: Predictors for single ventricle palliation (SVP) or successful biventricular repair (BVR) in patients with borderline left-side heart structures are not well defined. The goal was to evaluate the role of echocardiography and intracardiac exploration in determining feasibility of BVR. METHODS: All neonates surgically treated from 1995 to 2015 with mitral valve (MV), aortic valve, or left ventricle end-diastolic dimension z score of -2 or less for whom management was controversial were included...
October 4, 2016: Annals of Thoracic Surgery
Štěpán Havránek, Tomáš Paleček, Petr Kuchynka, Ivana Vítková
Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a rare condition characterised by progressive fibrofatty replacement of the myocardium of the left ventricle in combination with arrhythmias of left ventricular origin. ALVC has been linked to autosomal dominant mutations of genes encoding desmosomal proteins, similarly to the classic arrhythmogenic right ventricular cardiomyopathy with which it also shares pathological and prognostic features. It seems that isolated left or right ventricular abnormalities represent two extremes of the spectrum of clinical manifestations of a single disease: arrhythmogenic cardiomyopathy...
2016: Vnitr̆ní Lékar̆ství
Jun-Yen Pan, Chu-Chuan Lin, Jen-Ping Chang
Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging...
September 2016: Acta Cardiol Sin
Brett Cronin, Robin Robbins, Timothy Maus
OBJECTIVE: To assess the feasibility of pulmonary artery catheter placement using transesophageal echocardiography inclusive of a description of the technique. DESIGN: A prospective, proof-of-concept study. SETTING: Single university hospital. PARTICIPANTS: Twenty patients with chronic thromboembolic pulmonary hypertension scheduled for pulmonary thromboendarterectomy. INTERVENTIONS: Pulmonary artery catheters were placed in 20 patients solely by transesophageal echocardiographic guidance...
July 12, 2016: Journal of Cardiothoracic and Vascular Anesthesia
Yong-Hee Park, Da-Hye Yoo, Eun-Hee Kim, In-Kyung Song, Ji-Hyun Lee, Hee-Soo Kim, Woong-Han Kim, Jin-Tae Kim
This study aimed to investigate the optimal transducer level for accurate measurement of atrial and pulmonary arterial pressures in the supine position for patients with functional single ventricle. Contrast-enhanced chest computed tomographic images of 108 patients who underwent either the bidirectional cavopulmonary shunt (BCPS) placement or the Fontan procedure were reviewed. Vertical distances from the skin of the back to the uppermost levels of fluid in the single atrium or the pulmonary artery confluence and their ratios to the greatest anteroposterior (AP) diameter of the thoracic cage were determined...
October 1, 2016: Pediatric Cardiology
Johanna Calderon, Christian Stopp, David Wypij, David R DeMaso, Michael Rivkin, Jane W Newburger, David C Bellinger
OBJECTIVES: To investigate the long-term impact of early-term birth (37-38 weeks' gestation) relative to full-term birth (≥39 weeks' gestation) on neurodevelopmental and psychiatric outcomes in adolescents with single-ventricle congenital heart disease (CHD). STUDY DESIGN: This cross-sectional cohort study analyzed retrospective medical records from full term adolescents with single-ventricle CHD who underwent the Fontan procedure. Participants underwent neurodevelopmental and psychiatric evaluations, as well as structural brain magnetic resonance imaging...
September 27, 2016: Journal of Pediatrics
Danica Grujicic, Luigi Maria Cavallo, Teresa Somma, Rosanda Illic, Mihailo Milicevic, Savo Raicevic, Milica Skender Gazibara, Alessandro Villa, Dragan Savic, Domenico Solari, Paolo Cappabianca
BACKGROUND: Primary intraventricular meningiomas (IVMs) make up 0.5-5% of all intracranial meningiomas and represent one of the most challenging lesions in neurosurgery. METHODS: Between 1990 and 2013, 42 patients (30 female, 12 male, mean age of 43.6 years) underwent the resection of their IVM. The removal was performed by posterior-parietal approach in 19 of the 40 lateral ventricle tumors, and one in the third ventricle meningioma. The transcallosal approach was used for three meningiomas, while other lesions underwent temporal (7 cases) and temporo-parietal approaches (12 pts) respectively...
September 26, 2016: World Neurosurgery
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