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Mamas Mamas A - - 2008
Haemoptysis is a common feature of adults with congenitally malformed hearts suffering from Eisenmenger's syndrome. While this situation is often treated conservatively, it may not settle with such treatment. A further therapeutic option for these patients is embolisation of appropriate systemic-to-pulmonary collateral arteries. We discuss here our experience in treating ...
Takahashi Fumiyuki - - 2008
This report describes a case of isolated congenital spleen agenesis complicated by chronic thromboembolic pulmonary hypertension (CTPH) in a 44-year-old female patient. The patient had increasing exertional dyspnoea and thrombocytosis. An echocardiogram showed severe pulmonary hypertension and right ventricular hypertrophy, and contrast-enhanced chest CT revealed multiple thromboemboli within both pulmonary ...
Yalonetsky S - - 2009
Cyanotic congenital heart disease is associated with an increased risk of thromboembolism. We describe the case of sudden cardiac death caused by native aortic valve thrombosis in adult cyanotic patient. Such embolic complication was not previously described in cyanotic patient. It is also the first report of sudden cardiac death ...
Nakagawa Motoo - - 2008
PURPOSE: The pulmonary artery (PA) is involved in most congenital heart diseases; and in these patients it is necessary to evaluate precisely the PA configuration and development. The accuracy of 16 multidetector row computed tomography (16-MDCT) in evaluating the central PA was evaluated. MATERIALS AND METHODS: 16-MDCT and angiocardiography (ACG) ...
Bladt O - - 2008
Noncompaction of the ventricular myocardium is a cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. Non-compaction presents as an isolated form or in association with other congenital anomalies, such as obstruction of the right or left ventricular outflow tracts, complex cyanotic congenital heart ...
Karl Tom R - - 2008
Tetralogy of Fallot (TOF) is an important lesion for all pediatric and congenital heart surgeons. In designing the most appropriate operation for children with TOF, the postoperative physiology should be taken into account, both in the short and long term. The balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) ...
Kulkarni Snehal - - 2008
In a retrospective analysis of echocardiograms, the incidence of retroaortic innominate vein was found to be 0.55% amongst children with congenital heart disease. It was most commonly associated with tetralogy of Fallot and right aortic arch.
Yamamoto Toshiyuki - - 2008
A newborn male had an interstitial deletion of 16q21-q22.1 accompanying tetralogy of Fallot associated with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA), dysmorphic craniofacial features, failure to thrive, and severe psychomotor developmental delay. When the deletion in this patient and other reported patients are compared, the 16q22 region appears ...
Simsek Pelin Ozlem - - 2009
Congenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our ...
Jaeggi Edgar - - 2008
We describe two siblings of consanguineous parents with a prenatal diagnosis of a currently unique form of congenital cardiac disease characterized by superior-inferior atrial and ventricular arrangement, concordant atrioventricular and ventriculo-arterial connections with normal arterial relationships, and a bizarre topography of the ventricular outlets, with the arterial poles being displaced ...
Puchalski Michael D - - 2008
Pulmonary regurgitation (PR) is common after repair of congenital heart disease involving the right ventricular outflow tract. Because PR results in chronic right ventricular volume overload and associated morbidity and mortality, accurate assessment of its severity is important. The aim of this study was to compare echocardiography with the gold ...
Eren Nihan Kahya - - 2008
We report a 40-year-old man with an unoperated tetralogy of Fallot with pulmonary atresia and congenital aortic valvular stenosis. A well-developed coronary-pulmonary arterial collateral arising from the left main coronary artery was also demonstrated. Although rare cases of tetralogy of Fallot with congenital aortic stenosis has been reported, this is ...
Kalavrouziotis Georgios - - 2008
We describe the first application in Greece of a telemetrically adjustable device for pulmonary artery banding (FloWatch-PAB device) in a 2-month-old baby, weighing 3.6 kg, with congenital heart disease (complete atrioventricular septal defect with pulmonary hypertension). The surgical application of the device was easy, the postoperative course of the patient ...
Vefa Yildirim Selman - - 2008
Congenital pulmonary venous stenosis is a rare condition leads to progressive pulmonary hypertension and heart failure. It may be isolated or associated with other cardiac malformations. The condition ranges from hypoplasia of individual pulmonary veins to constriction of the intima of the venoatrial junction. We present a case of congenital ...
Baden W - - 2008
Bridging bronchus (BB) is a rare, congenital bronchial anomaly that is frequently associated with congenital cardiac malformations, especially left pulmonary artery sling. It represents an anomalous bronchus to the right originating from the left main bronchus. Discrimination from other bronchial anomalies is important, since BB is frequently associated with bronchial ...
Törer Birgin - - 2008
A congenital tracheobronchial stenosis is a rare obstructive lesion of the airway characterized by intrinsic narrowing of a segment of the trachea and bronchi. In this report, we present the smallest premature infant with a congenital tracheobronchial stenosis who has been successfully treated with balloon dilatation and placement of a ...
Ruano Rodrigo - - 2008
OBJECTIVE: The aim of the study was to evaluate the potential of fetal pulmonary artery (PA) diameters to predict perinatal death and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH). STUDY DESIGN: In this prospective observational study, observed PA (main, right, and left) diameters were measured at the level ...
Lee Meng-Luen - - 2008
We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). From January 1990 to January 2007, a total ...
Hoover Wynton - - 2008
Congenital porto-caval fistulas are uncommon vascular malformations with a varied clinical presentation beginning in infancy and spanning late adulthood. We report a 14-year-old male who presented with dyspnea and cough. His past medical history was significant for a chronic non-immune hemolytic anemia, thrombocytopenia, coagulopathy and a learning disability. He was ...
- - 2008
Separate guidelines are needed for determining the optimal timing of intervention in children with congenital heart diseases in India, because of their frequent late presentation, undernutrition and co-existing morbidities. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 26th ...
Kulkarni Snehal
In a retrospective analysis of echocardiograms, the incidence of retroaortic innominate vein was found to be 0.55% amongst children with congenital heart disease. It was most commonly associated with tetralogy of Fallot and right aortic arch.
Pitsis Antonis A - - 2008
Congenital left ventricular (LV) diverticula are rare findings, particularly when first diagnosed in adulthood. We describe successful surgical repair of an isolated congenital apical LV diverticulum associated with an abnormal submitral apparatus in a young adult who received his diagnosis following a peripheral embolism. We intraoperatively identified an abnormally originating ...
Pigozzi Fabio - - 2008
Sudden death in athletes is an extremely rare event yet no less tragic for its infrequency. Up to 90% of these deaths are due to underlying cardiovascular diseases and therefore categorized as sudden cardiac death (SCD). The causes of SCD among athletes are strongly correlated with age. In young athletes ...
Singh Anurag - - 2008
Aortopulmonary (AP) window is an extremely rare congenital anomaly involving a communication between the ascending aorta and pulmonary artery. Two-dimensional transthoracic echocardiography is traditionally used in the diagnosis but has limitations. We report the usefulness of three-dimensional transthoracic echocardiography in the assessment of AP window in two newborns studied by ...
Munárriz A - - 2008
Congenital aneurysm of the left atrial appendage is quite infrequent. Most instances are asymptomatic. Patients can report a variety of symptoms, one of the most frequent being onset of auricular tachyarrhythmia. Various imaging techniques are useful in diagnosis and allow the differential diagnosis with other pathologies. We describe the case ...
Osanai Toshihisa - - 2008
Persistent sciatic artery (PSA) is a very rare, but clinically significant, congenital anomaly. PSA is known to undergo aneurysmal formation; however, there have been no previous reports of a soft-tissue sarcoma arising from a PSA. We describe a 79-year-old woman with a 10 year history of a slowly growing mass ...
Bret-Zurita Montserrat - - 2008
Persistence of the primitive hepatic venous plexus (PPHVP) is an uncommon anomaly that can present associated with complex congenital heart disease and inferior vena cava (IVC) development anomalies and complicate endovascular and surgical procedures. We report a case of PPHVP in a patient with complex congenital heart disease with unexplained ...
Lee Edward Y - - 2007
The early diagnosis and complete anatomic evaluation of pulmonary artery sling, a congenital vascular anomaly in which left pulmonary artery arises from the right pulmonary artery, is paramount for proper patient management, because patients with this disorder frequently have other congenital anomalies resulting in high morbidity and mortality. Until recently, ...
Nomura Norikazu - - 2007
Bronchial compression due to a dilated pulmonary artery is a troublesome problem in the surgical management of infants or children with congenital heart disease. We experienced an infantile case of ventricular septal defect and prolonged respiratory insufficiency caused by right bronchial compression and left pulmonary hypoplasia. Anterior translocation of the ...
Lee Young-Soo - - 2007
Cor triatriatum dexter is a rare congenital anomaly caused by persistence of the right valve of the embryonic sinus venosus, and this results in septation of the right atrium. Three-dimensional echocardiography is a novel technique that can be used to identify and characterize this entity. Here, we report two women ...
Gamillscheg Andreas - - 2008
After surgical repair of an aortic coarctation a term infant presented with severe pulmonary hypertension and cyanosis unresponsive to treatment including extracorporeal membrane oxygenation. The atypical clinical course became apparent once the accompanying diagnosis of congenital alveolar capillary dysplasia with misalignment of the pulmonary veins had been established at autopsy. ...
Yang Hua - - 2008
We determined the utility of continuous wave (CW) Doppler for quantification of pulmonary insufficiency (PI) confirmed by pulmonary angiography in patients with postoperative adult congenital heart disease. A total of 41 patients with PI were divided into two groups on the basis of PI severity by pulmonary angiography: group A ...
Wong S F - - 2007
OBJECTIVES: Congenital heart disease is associated with high mortality and morbidity rates, being the most life-threatening defect in the first month of postnatal life and accounting for approximately half of all childhood mortality from birth defects. Despite this, the prenatal detection rate for congenital outflow tract anomalies by ultrasound imaging ...
Sahin Tayfun - - 2008
Cor triatriatum dextrum is a rare congenital malformation, usually associated with complex right heart abnormalities, characterized by a membrane that divides the right atrium into two chambers. It is considered the result of the incomplete and abnormal regression of the embryonic right valve of the sinus venosus. With an incomplete ...
Fairley Sarah L - - 2008
Tetralogy of Fallot (TOF) is the commonest form of cyanotic congenital heart defect after infancy [Brickner ME, Hillis LD, Lange RA. Congenital Heart Disease in Adults-Second of Two Parts. NEJM 2000; 342(5):334-342.]. There are few studies assessing the risk of surgical correction in adult patients and long-term survival into the ...
Tangren Christopher M - - 2007
We report a case of a patient with a congenitally bicuspid aortic valve and extreme obesity who developed severe aortic stenosis. She dramatically improved after the combined use of balloon valvuloplasty and Roux en Y gastric bypass. Gastric bypass surgery has promise for patients with congenital cardiac disease whose treatment ...
Gocmen Rahsan - - 2007
The radiological features of a 7-year-old boy with congenital absence of the portal vein, pathologically proven congenital hepatic fibrosis, double inferior vena cava, ventricular septal defect, vertebral anomalies, crossed fused renal ectopia, and facial anomalies with pathological correlation are reported. This association between congenital absence of the portal vein and ...
Nieves Omar N - - 2007
Tetralogy of Fallot is the most common cyanotic congenital heart disease in children. It is usually detected in early infancy due to symptoms related to cyanosis and right to left shunting of blood through a large ventricular septal defect. Uncorrected it is associated to a very poor prognosis, with only ...
Kling David E - - 2007
Congenital diaphragmatic hernia (CDH) is a congenital malformation that occurs with a frequency of 0.08 to 0.45 per 1,000 births. Children with CDH are born with the abdominal contents herniated through the diaphragm and exhibit an associated pulmonary hypoplasia which is frequently accompanied by severe morbidity and mortality. Although the ...
Ramos Simone G - - 2007
An 8-year-old girl was evaluated for a mass in the left pulmonary lobe. Her clinical history was remarkable for an intermittent dry cough since the age of 2, with recurrent episodes of purulent sputum and fever. She underwent left lower lobectomy, which was found to be a bronchioloalveolar carcinoma arising ...
Lee Meng-Luen - - 2008
A 10-year-old boy, who had suffered Kawasaki disease 7 years ago, presented chest pain, pale face, and cold sweating in the stress (99m)technetium-methoxyisobutylisonitrile ((99m)Tc-MIBI) single-photon emission computed tomography (SPECT), which showed reversible perfusion abnormality indicative of myocardial ischemia involving the right coronary artery. Angiography identified congenital coronary arteriovenous fistula (CAVF) ...
Ordovás Karen G - - 2007
PURPOSE: To compare the relative severity of stenoses of right or left pulmonary arteries with differences in flow to each lung after repair of congenital heart disease (CHD). MATERIALS AND METHODS: A total of 15 patients with postoperative congenital heart disease underwent MRI to evaluate branch pulmonary artery stenoses. Spin-echo ...
Monajemzadeh, M
Objective: Tetralogy of fallot (TOF) is one of the most common forms of cyanotic congenital heart disease (CHD). The aim of this study was determination of demography, associated anomalies, peripheral pulmonary stenosis (PPS), blood grouping, Rh typing, operation results and complications in TOF. Material & Methods: The records of 270 ...
Diller Gerhard-Paul - - 2007
Pulmonary arterial hypertension of variable degree is commonly associated with adult congenital heart disease. Depending on size and location of the underlying cardiac defect as well as on repair status, pulmonary arterial hypertension may present with or without reversed shunting and associated cyanosis (ie, Eisenmenger syndrome). We review available data ...
Diluna Michael L - - 2007
Faciogenital dysplasia, also known as Aarskog-Scott syndrome (AAS), is an X-linked dominant congenital disorder characterized by multiple facial, musculoskeletal, dental, neurological and urogenital abnormalities, ocular manifestations, congenital heart defects, low IQ and behavioral problems. Here we describe an unusual presentation of dysplastic carotid artery, basilar artery malformation or occlusion and ...
Yaliniz H - - 2007
Congenital true aneurysms of left atrial appendage are very rare. Abnormal cardiac silhouette on the chest roentgenograph is usually an important clue for the diagnosis. They are commonly associated with supraventricular arrhythmias and life-threatening systemic embolization. We report here the surgically-corrected case of a patient with congenital left atrial appendage ...
Arai Lynda R - - 2007
Laryngotracheo-esophageal cleft is a rare congenital anomaly that results from complete or partial failure of the development of the tracheoesophageal septum. The presenting symptoms include stridor, respiratory distress, and coughing or cyanotic episodes with feeding. There are four classifications for laryngeal clefts; the severity depends on the type present. We ...
Dodd Jonathan D - - 2007
The purpose of this pictorial essay is to review the 64-MDCT appearance of congenital anomalies of the origins of the coronary arteries in adults. Increasing use of MDCT for cardiac imaging of adults requires familiarity with the cross-sectional appearance of congenital coronary artery anomalies visualized with noninvasive imaging techniques. Many ...
Kovacikova L - - 2007
We describe successful use of enteral sildenafil following surgery for congenital heart disease in three cases. One infant after repair of ventricular septal defect and aortic coarctation had pulmonary hypertension non-responsive to nitric oxide, another infant and 3.5 year child following palliative surgery for congenital heart disease with univentricular physiology ...
Hares Dominic Llewelyn - - 2007
We describe the use of a new vascular occlusion device to embolize vessels whose flow is detrimental to cardiac function in a variety of clinical situations in both adults and children with congenital cardiac disease. Our series includes four cases in which we have successfully occluded large venous vessels, to ...
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