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Cardiology in the Young

Khadijah Maghrabi, Sanjiv Gandhi, Kevin C Harris
Aorto-left ventricular tunnel is a rare congenital heart lesion, with an incidence of <0.1% of all CHD. We present a unique case of a valvar aorto-left ventricular tunnel in a neonate, in belief that our findings may shed some light on the developmental origins of this lesion.
November 29, 2016: Cardiology in the Young
Mehnaz Atiq, Fatima Ali, Sulaiman B Hasan
Aneurysm of the sinus of Valsalva is a rare congenital cardiac anomaly. It occurs as an outpouching that progresses like a windsock, and it may rupture producing aortic regurgitation, cardiac tamponade, congestive heart failure, conduction abnormalities, and stroke. We describe a case of rupture of the sinus of Valsalva into the interventricular septum producing a large dissecting aneurysm. Despite the location, it did not produce a conduction abnormality.
November 22, 2016: Cardiology in the Young
Roland Fiszer, Karol Zbroński, Małgorzata Szkutnik
To date, there are no reported cases of the Amplatzer Duct Occluder II: Additional Sizes' use in percutaneous closure of an aortopulmonary window. We report a case of percutaneous closure of an aortopulmonary window in a 4.5-month-old, 6 kg child. Owing to the patient's low weight, high risk of damage to the pulmonary valve, as well as the possibility of aortic and pulmonary artery obstruction, classic implants were deemed unsuitable and a decision was made to use the Amplatzer Duct Occluder II: Additional Sizes...
November 21, 2016: Cardiology in the Young
David M Kwiatkowski, Elizabeth Price, David M Axelrod, Anitra W Romfh, Brian S Han, Scott M Sutherland, Catherine D Krawczeski
BACKGROUND: Acute kidney injury after cardiac surgery is a frequent and serious complication among children with congenital heart disease (CHD) and adults with acquired heart disease; however, the significance of kidney injury in adults after congenital heart surgery is unknown. The primary objective of this study was to determine the incidence of acute kidney injury after surgery for adult CHD. Secondary objectives included determination of risk factors and associations with clinical outcomes...
November 21, 2016: Cardiology in the Young
Puneet Bhatla, Ralph S Mosca, Justin T Tretter
Rapid prototyping is quickly gaining utility in various complex forms of CHD. In properly selected cases, these printed models provide detailed anatomical understanding that help guide potential surgical and cardiac catheterisation interventions. We present a case of a tunnel-like ventricular septal defect referred for surgical repair, where the decision to obtain a three-dimensional printed model helped in better understanding of the anatomy, leading to delaying, and hopefully avoiding altogether, surgical repair...
November 21, 2016: Cardiology in the Young
Arash Salavitabar, Eric S Silver, Leonardo Liberman
Risk stratification of patients with Wolff-Parkinson-White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation.
November 21, 2016: Cardiology in the Young
Yiğit Çanga, Tolga S Güvenç, Mehmet B Karataş, Ali N Çalık, Tolga Onuk, Veysel O Tanık, Barış Güngör, Osman Bolca
BACKGROUND: Coronary artery anomalies are a heterogeneous group of congenital disorders presenting with a wide spectrum of symptoms, ranging from vague chest pain to sudden cardiac death. Despite available data, there is no consensus about the classification, nomenclature, and outcomes of coronary anomalies in the normally connected heart. In this study, we aimed to investigate clinical and angiographic characteristics of coronary arterial anomalies, as well as the frequency of atherosclerotic involvement in anomalous coronaries, diagnosed at a tertiary referral centre...
November 15, 2016: Cardiology in the Young
Arshid Mir, Benjamin Stam, Charles Sperrazza
Coarctation of the abdominal aorta is a rare condition with a handful of cases reported in infancy. We report the case of a 4-month-old infant with severe abdominal coarctation who presented with dilated cardiomyopathy. Following successful transcatheter-based stenting of her abdominal aorta, her cardiomyopathy resolved.
November 14, 2016: Cardiology in the Young
Stella Mazurova, Martin Magner, Vendula Kucerova-Vidrova, Alzbeta Vondrackova, Viktor Stranecky, Anna Pristoupilova, Josef Zamecnik, Hana Hansikova, Jiri Zeman, Marketa Tesarova, Tomas Honzik
Cardiomyopathy is a common manifestation in neonates and infants with mitochondrial disorders. In this study, we report two cases manifesting with fatal mitochondrial hypertrophic cardiomyopathy, which include the third known patient with thymidine kinase 2 deficiency and the ninth patient with alanyl-tRNA synthetase 2 deficiency. The girl with thymidine kinase 2 deficiency had hypertrophic cardiomyopathy together with regression of gross motor development at the age of 13 months. Neurological symptoms and cardiac involvement progressed into severe myopathy, psychomotor arrest, and cardiorespiratory failure at the age of 22 months...
November 14, 2016: Cardiology in the Young
Guillermo Ventosa-Fernández, Carolina Pérez-Negueruela, Javier Mayol, Marina Paradela, José M Caffarena-Calvar
BACKGROUND: The surgical treatment for complex forms of d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction remains controversial. In this study, we describe the classical surgical options - namely, the Rastelli procedure and the "réparation à l'étage ventriculaire" - and present our experience with the modified Nikaidoh procedure with early and short-term follow-up results. METHODS: Between 2007 and 2014, four patients with d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction underwent surgical repair at our institution by means of a modified Nikaidoh procedure...
November 14, 2016: Cardiology in the Young
Philippe M Adjagba, Gaston Habib, Nancy Robitaille, Yves Pastore, Marie-Josée Raboisson, Daniel Curnier, Nagib Dahdah
: Purpose Sickle cell disease is known to cause various degrees of vasculopathy, including impact on heart function. The aims of this single-centre, retrospective study were to assess cardiac chamber size and function and the relationship with haematological indices such as haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin, lactate dehydrogenase in sickle cell disease. METHODS: Right ventricle and left ventricle diastolic diameters, left ventricle mass estimate, left ventricle shortening fraction, myocardial performance index, and an index of myocardial relaxation (E/E') were calculated and correlated with haematological parameters...
November 14, 2016: Cardiology in the Young
Marianna Fabi, Francesca Lami, Maurizio Zompatori
A 12-year-old boy with a right atrium endocardial mass was initially diagnosed as having Lemierre's syndrome on the basis of previous mastoiditis and jugular vein and cerebral venous thrombosis. Lack of response to antibiotics, persistent high fever with chills, acute-phase reactants, and peripheral arterial pseudoaneurysms made us reconsider the diagnosis. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, Hughes-Stovin syndrome was diagnosed. Hughes-Stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses...
November 11, 2016: Cardiology in the Young
Syed Javed H Zaidi, Rupali Gandhi, Saad Siddiqui
Pulmonary artery, and rarely aortic, calcifications have been reported in sporadic case reports in the recipient twin of twin-to-twin transfusion syndrome. This presentation is more likely to be secondary to the haemodynamic alterations in the recipient twin, but must be differentiated from idiopathic infantile arterial calcification as the clinical implications, treatment, and prognosis may be drastically different.
November 11, 2016: Cardiology in the Young
Maryanne Caruana, Victor Grech
BACKGROUND: The improved survival of patients born with CHD has led to increasing interest in research on quality of life of adult survivors. We report the findings of the first study in Malta carried out to investigate quality of life in adults with CHD under follow-up. METHODS: A self-reporting questionnaire modelled on the basis of the European Health Interview Survey 2008, including questions on mental health and vitality, was administered to consecutive adult CHD outpatients, aged 16 years and over, between May, 2013 and May, 2014...
November 11, 2016: Cardiology in the Young
Brady S Moffett, Rocky Tsang, Curt Kennedy, Ron A Bronicki, Ayse Akcan-Arikan, Paul A Checchia
BACKGROUND: Sequential nephron blockade using intravenous chlorothiazide is often used to enhance urine output in patients with inadequate response to loop diuretics. A few data exist to support this practice in critically ill infants. METHODS: We included 100 consecutive patients <1 year of age who were administered intravenous chlorothiazide while receiving furosemide therapy in the cardiac ICU in our study. The primary end point was change in urine output 24 hours after chlorothiazide administration, and patients were considered to be responders if an increase in urine output of 0...
November 11, 2016: Cardiology in the Young
Kayi Eliacik, Ali Kanik, Nurullah Bolat, Hilal Mertek, Baris Guven, Ulas Karadas, Buket Dogrusoz, Ali Rahmi Bakiler
Chest pain in adolescents is rarely associated with cardiac disease. Adolescents with medically unexplained chest pain usually have high levels of anxiety and depression. Psychological stress may trigger non-cardiac chest pain. This study evaluated risk factors that particularly characterise adolescence, such as major stressful events, in a clinical population. The present study was conducted on 100 adolescents with non-cardiac chest pain and 76 control subjects. Stressful life events were assessed by interviewing patients using a 36-item checklist, along with the Children's Depression Inventory and Spielberger's State-Trait Anxiety Inventory for children, in both groups...
November 10, 2016: Cardiology in the Young
Paul C Helm, Elisabeth J Sticker, Roland Keuchen, Marc-André Koerten, Gerhard-Paul Diller, Oktay Tutarel, Ulrike M M Bauer
BACKGROUND: Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients' assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans. METHODS: The present study was based on an online survey conducted in cooperation with patient organisations...
November 10, 2016: Cardiology in the Young
Niall Johnston, David Coleman, Colin J McMahon
We describe the case of an 8-week-old infant with late presentation of severe refractory atypical Kawasaki disease. In addition to developing giant coronary arterial aneurysms and coronary thrombosis, she formed extensive bilateral arterial aneurysms throughout her systemic circulation.
November 10, 2016: Cardiology in the Young
David G Greenhouse, Roger E Breitbart, Christopher W Baird
Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.
November 10, 2016: Cardiology in the Young
Kevin D Hill, Bryan H Goldstein, Michael J Angtuaco, Patricia Y Chu, Gregory A Fleming
OBJECTIVE: The aim of this study was to describe previously unrecognised or under-recognised adverse events associated with Melody® valve implantation. BACKGROUND: In rare diseases and conditions, it is typically not feasible to conduct large-scale safety trials before drug or device approval. Therefore, post-market surveillance mechanisms are necessary to detect rare but potentially serious adverse events. METHODS: We reviewed the United States Food and Drug Administration's Manufacturer and User Facility Device Experience (MAUDE) database and conducted a structured literature review to evaluate adverse events associated with on- and off-label Melody® valve implantation...
November 10, 2016: Cardiology in the Young
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