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Inlet Ventricular Septal Defect

Carine Pavy, Olivier Ghez
The association of complete atrial ventricular septal defect (cAVSD) with tetralogy of Fallot remains a complex malformation that involves both inlet and outlet components of the ventricles. The surgical treatment used to be, in the 1980s, a staged repair strategy due to the high mortality rate of first primary repairs (PRs). However, nowadays, PRs have better outcomes. Although double-patch procedures are widely performed, this article describes the single-patch approach for cAVSD repair with transatrial-transpulmonary repair of the Fallot component with preservation of the pulmonary valve...
2016: Multimedia Manual of Cardiothoracic Surgery: MMCTS
Olivier Villemain, Damien Bonnet, Lucile Houyel, Mathieu Vergnat, Magalie Ladouceur, Virginie Lambert, Zakaria Jalal, Pascal Vouhé, Emre Belli
The management of double-outlet right ventricle associated with anatomically noncommitted ventricular septal defect constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical vs univentricular repair still remain to be established. Between 1993 and 2011, 36 consecutive patients presenting with double-outlet right ventricle or noncommitted ventricular septal defect (21 inlet, 10 muscular, and 5 central perimembranous) and 2 adequately sized ventricles underwent surgical repair at 2 centers...
2016: Seminars in Thoracic and Cardiovascular Surgery
Aline Wolter, Sina Nosbüsch, Andreea Kawecki, Jan Degenhardt, Christian Enzensberger, Oliver Graupner, Carina Vorisek, Hakan Akintürk, Can Yerebakan, Markus Khalil, Dietmar Schranz, Jochen Ritgen, Rüdiger Stressig, Roland Axt-Fliedner
OBJECTIVE: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH. METHODS: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC)...
June 2016: Prenatal Diagnosis
Masamichi Ono, Jelena Kasnar-Samprec, Alfred Hager, Julie Cleuziou, Melchior Burri, Constantin Langenbach, Alessia Callegari, Martina Strbad, Manfred Vogt, Jürgen Hörer, Christian Schreiber, Rüdiger Lange
OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92...
March 23, 2016: European Journal of Cardio-thoracic Surgery
M A Ali, T Tazmin, T Latif, S A Haque, M A Hossain, M N Islam, R H Khan, M A Hoque
Cantrell's Pentalogy is a rare congenital malformation consists of supraumbilical abdominal wall defect, defect in the lower part of sternum, agenesis of anterior portion of diaphragm, an absence of the diaphragmatic part of the pericardium and intracardiac malformation. This case report presents a female neonate, who was born at 40 weeks of gestation weighing 2400 gm and was admitted 4 hours after delivery with the complaints of something coming out from chest. On physical examination her vital signs were within normal limit, she had a systolic murmur on heart at lower left sternal area and there was a vascular structure present on the upper part of abdomen which was pulsatile and pulsation was synchronized with cardiac pulsation...
January 2016: Mymensingh Medical Journal: MMJ
Minghui Zou, Yanfei Wang, Hujun Cui, Li Ma, Shengchun Yang, Yuansheng Xia, Weidan Chen, Xinxin Chen
BACKGROUND: The aim of this study was to review the early and mid-term outcomes of the total cavopulmonary connection (TCPC) procedure and evaluate risk factors for prolonged pleural effusions. METHODS: The clinical records of 82 consecutive patients, who underwent a TCPC operation between January 2008 and December 2013, were reviewed for incidence of prolonged pleural effusions, duration of ventilation time and pleural drainage, length of intensive care unit (ICU) stay, and early and mid-term morbidity and mortality...
January 2016: Journal of Thoracic Disease
Matthew Lewis, Jonathon Ginns, Christian Schulze, Matt Lippel, Paul Chai, Emile Bacha, Donna Mancini, Marlon Rosenbaum, Maryjane Farr
BACKGROUND: Adults with congenital heart disease (CHD) are at increased risk for adverse outcomes after heart transplantation (HT). However, small cohorts have constrained the identification of factors associated with poor prognosis. We hypothesized that number of sternotomies and bystander organ dysfunction would be associated with an increased risk for early death after HT. METHODS AND RESULTS: We performed a retrospective observational study of all adult CHD patients who underwent HT at our institution from January 1997 to January 2014...
July 2016: Journal of Cardiac Failure
Waseem Cossor, Vivian Wei Cui, David A Roberson
BACKGROUND: Ventricular septal defect (VSD) is the most common congenital cardiac anomaly. Accurate assessment is critical for planning treatment. Recent advances in three-dimensional (3D) echocardiography have improved image quality and ease of use. METHODS: The feasibility and accuracy of three specific 3D echocardiographic protocols to demonstrate en face views of VSDs were analyzed in a retrospective review of 100 consecutive patients. Sixty-four patients underwent transthoracic echocardiography and 36 transesophageal echocardiography...
September 2015: Journal of the American Society of Echocardiography
Robert H Anderson, Anne E Sarwark, Diane E Spicer, Carl L Backer
Holes between the ventricles are the commonest congenital cardiac malformations. As yet, however, there is no consensus as to how they can best be described and categorized. In this, our third exercise in cardiac anatomy, we address the issue of classification of ventricular septal defects. We begin our demonstration by analysing the normal heart. We show that the larger part of the ventricular septum is made up of its muscular component. The membranous part accounts for only a small portion, which is located centrally within the cardiac base...
2014: Multimedia Manual of Cardiothoracic Surgery: MMCTS
Murat Saygı, Hasan Tahsin Tola, Alper Güzeltaş, Ender Odemiş
A 14-year-old male patient presented with cyanosis and tiredness. The patient had undergone a Glenn procedure at age 12 following the echocardiographic determination of a double inlet left ventricle, ventriculoarterial discordance, moderate valvular-subvalvular pulmonary artery stenosis, non-restrictive inlet ventricular septal defect and right ventricle hypoplasia; his oxygen saturation was 70%. Echocardiography evaluation showed retrograde flow from the vena cava superior to the innominate vein and a left superior vena cava (LSVC) opening into the coronary sinus (CS)...
October 2014: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Zhong Qun Zhu, Hai Fa Hong, Hui Wen Chen, Hai Bo Zhang, Zhao Hui Lu, Jing Hao Zheng, Guo Cheng Shi, Jing Fen Liu
BACKGROUND: The intraatrial conduit (IAC) Fontan procedure is one of the Fontan modifications and is usually not considered the first choice. In this large series, we report our experience of the IAC Fontan procedure for the treatment of a functional single ventricle and review its indications, techniques, and clinical outcomes. METHODS: Between 2009 and 2013, 101 patients with a functional single ventricle underwent an IAC Fontan procedure. The median surgical age was 44 months and weight was 15 kg...
January 2015: Annals of Thoracic Surgery
Robert E Poelmann, Adriana C Gittenberger-de Groot, Rebecca Vicente-Steijn, Lambertus J Wisse, Margot M Bartelings, Sonja Everts, Tamara Hoppenbrouwers, Boudewijn P T Kruithof, Bjarke Jensen, Paul W de Bruin, Tatsuya Hirasawa, Shigeru Kuratani, Freek Vonk, Jeanne M M S van de Put, Merijn A de Bakker, Michael K Richardson
During cardiogenesis the epicardium, covering the surface of the myocardial tube, has been ascribed several functions essential for normal heart development of vertebrates from lampreys to mammals. We investigated a novel function of the epicardium in ventricular development in species with partial and complete septation. These species include reptiles, birds and mammals. Adult turtles, lizards and snakes have a complex ventricle with three cava, partially separated by the horizontal and vertical septa. The crocodilians, birds and mammals with origins some 100 million years apart, however, have a left and right ventricle that are completely separated, being a clear example of convergent evolution...
2014: PloS One
Carla Frescura, Gaetano Thiene
The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to hearts not amenable to biventricular repair, namely hearts with two ventricles unable to sustain separately pulmonary and systemic circulations in sequence. In the latter definition, even hearts with one hypoplastic ventricle are considered "functional" univentricular hearts. They include pulmonary/aortic atresia or severe stenosis with hypoplastic ventricle, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of right ventricular cavity...
2014: Frontiers in Pediatrics
J Gallego-Galiana, A L Aguilar-Shea
We report the case of a 46 year old asymptomatic male whose chest X-ray showed a right aortic knob and in the physical examination he had a systolic murmur with a palpable left parasternal heave. On performing the transesophageal echocardiography and MRI a ventricular septal defect (VSD) was observed which only requires periodic echocardiographic reviews. VSD is the most common congenital heart disease. The VSD may be membranous, inlet, trabecular, or infundibular. The clinical signs may range from only anodyne with a left pansystolic parasternal murmur on physical examination, up to lung hyper-flow which may lead to heart failure...
April 2012: Semergen
Robert H Anderson, Diane E Spicer, Nigel A Brown, Timothy J Mohun
The past decades have seen immense progress in the understanding of cardiac development. Appreciation of precise details of cardiac anatomy, however, has yet to be fully translated into the more general understanding of the changing structure of the developing heart, particularly with regard to formation of the septal structures. In this review, using images obtained with episcopic microscopy together with scanning electron microscopy, we show that the newly acquired information concerning the anatomic changes occurring during separation of the cardiac chambers in the mouse is able to provide a basis for understanding the morphogenesis of septal defects in the human heart...
August 2014: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
Mohammed H Alghamdi, M O Galal, Fahad Al-Habshan, Mansour Al-Mutairi
A 9.5-month-old boy with Down syndrome, weighing 4.8 kg, presented with history of failure to thrive. Clinically, he had symptoms and signs of congestive heart failure. His echocardiogram showed a large perimembranous ventricular septal defect (pmVSD) with some inlet extension covered by a large aneurysmal tissue with multiple right ventricular (RV) exits. Additionally, he had hypothyroidism and Hirschsprung disease. Instead of closing the VSD surgically, the VSD was successfully closed utilizing an 8 × 6 mm duct occluder...
April 2014: Journal of the Saudi Heart Association
S Turan, O M Turan, A Desai, C R Harman, A A Baschat
OBJECTIVE: A four-dimensional (4D) fetal echocardiographic technique utilizing spatiotemporal image correlation, tomographic ultrasound imaging display (STIC-TUI echo) and color Doppler has previously been shown to be effective in displaying the examination planes constituting the extended cardiac examination. The aim of this study was to evaluate the performance of this first-trimester STIC-TUI echo technique in identifying complex congenital heart disease (CHD) in high-risk pregnancies...
November 2014: Ultrasound in Obstetrics & Gynecology
Liu Du, Hong-ning Xie, Li-juan Li, Yun-xiao Zhu, Mei-fang Lin, Ju Zheng
OBJECTIVE: To evaluate the association between fetal ventricular septal defects (VSD) and chromosomal abnormalities. METHODS: The 214 fetuses diagnosed VSD in the First Affiliated Hospital of Sun Yat-sen University from January 2008 to September 2011 were included. The VSD were categorized into 3 types: perimembranous, muscular and mixed (the defect could not be classified because the dimensions were larger than 5 mm) type. The perimembranous defect was subdivided into inlet and outlet subtypes...
November 2013: Zhonghua Fu Chan Ke za Zhi
Victor O Morell, Peter D Wearden
The optimal surgical management of patients with transposition of the great arteries with a ventricular septal defect and pulmonary stenosis remains challenging. When compared to other surgical options, the Nikaidoh procedure results in a 'more normal' anatomic result, with better alignment of the right and left ventricular outflow tracts. Also, the pulmonary outflow is less likely to be compressed by the sternum, a major issue associated with the Rastelli repair. This technique is especially useful in the presence of an inlet or restrictive ventricular septal defect, a hypoplastic right ventricle, a straddling atrioventricular valve and/or anomalous coronary anatomy interfering with a distal right ventricular outflow tract incision...
January 1, 2008: Multimedia Manual of Cardiothoracic Surgery: MMCTS
Antonio F Corno, Pramod R Kandakure, Ramana Rao V Dhannapuneni, Gordon Gladman, Prem Venugopal, Nelson Alphonso
INTRODUCTION: A multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs). MATERIALS AND METHODS: From 2004 to 2012 17 consecutive children (3 premature, 14 infants), mean age 3.2 months (9 days-9 months), mean body weight 4.2 kg (3.1-6.1 kg), with multiple VSDs underwent Pulmonary Artery Banding (PAB) with an adjustable FloWatch-PAB(®). Associated cardiac anomalies included patent ductus arteriosus (1), aortic coarctation (2), hypoplastic aortic arch (2), and left isomerism (3)...
2013: Frontiers in Pediatrics
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