Search Results
Results 201 - 250 of 599
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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 &amp; 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 ...
Adam Margaret P - - 2006
Mowat-Wilson syndrome (MWS) is a relatively newly described multiple congenital anomaly/mental retardation syndrome. Haploinsufficiency of a gene termed ZFHX1B (also known as SIP1) on chromosome 2 is responsible for this condition, and clinical genetic testing for MWS recently became available. The majority of reports in the literature originate from Northern ...
Vaideeswar Pradeep - - 2006
Pulmonary arterial endarteritis is a rare event even in patients with congenital heart disease. In this paper, the authors report two cases of pulmonary endarteritis diagnosed at autopsy. The first was a 3-month-old male infant with absence of congenital heart defect (who had had neonatal sepsis). This patient presented with ...
Canter Jeffrey A - - 2007
Increased pulmonary artery pressure (PAP) can complicate the postoperative care of children undergoing surgical repair of congenital heart defects. Endogenous NO regulates PAP and is derived from arginine supplied by the urea cycle. The rate-limiting step in the urea cycle is catalyzed by a mitochondrial enzyme, carbamoyl-phosphate synthetase I (CPSI). ...
Cleuziou Julie - - 2006
Substantial long-term morbidity after a successful operation in complex congenital heart defects is a matter of concern. We present a patient with a giant ascending aortic aneurysm 18 years after repair of a double-outlet right ventricle with pulmonary stenosis. Our report emphasizes the need for ongoing follow-up into adulthood.
Kameyama Kotaro - - 2006
A 36-year-old woman presented with left chest pain and frequent symptoms of upper respiratory infection. Chest roentgenograms revealed a left pneumothorax and apical bulla, and hyperlucency in the left pulmonary field. She was diagnosed with congenital bronchial atresia associated with a left spontaneous pneumothorax. A thoracoscopy-assisted left superior segmentectomy was ...
Tsai Shang-Yueh - - 2006
A temporal correlation (TC) mapping method is proposed to help bolus chasing during dynamic contrast-enhanced (DCE) MRI of complex pulmonary circulation (CPC) in patients with congenital heart disease. DCE-MRI was performed on five healthy male subjects (23-24 years old) and 25 patients (nine males and 16 females, 0.25-44 years old), ...
Chadha Rajiv - - 2006
A two-month-old girl was operated for a left-sided eventration of the diaphragm. At surgery, a mass representing an extralobar pulmonary sequestration (EPS) was found inferior to the left lobe of the lung and was excised. Histopathological examination of the resected specimen showed presence of a type II congenital cystic adenomatoid ...
Kenny D - - 2007
Spontaneous neonatal arterial thrombosis is rare in the neonatal period. Four cases of neonatal arterial thrombosis presenting with suspected congenital heart disease are reported. The urgency for a correct diagnosis in this setting and the need for active treatment for a remediable condition are emphasised. These treatment options are discussed.
Liu Peter S - - 2007
Congenital supravalvular aortic stenosis (SVAS) is a rare cause of congenital left ventricular outflow obstruction and pressure overload, with a diffuse form that often extends into the aortic arch, and can be associated with stenoses in other vascular territories. Knowledge of the extent of disease involvement can be complementary to ...
Taori K - - 2006
A left atrial aneurysm is a rare cardiac anomaly. The etiology is usually congenital, but it can also occur as an acquired pathology secondary to mitral valve disease or a degenerative process. We report a case which, on routine PA chest radiography, presented as cardiomegaly with a bulge on the ...
Sokol Jenni - - 2006
OBJECTIVE: The objective of the study was to examine the size and growth of fetal pulmonary artery diameters in congenital diaphragmatic hernia and assess their correlation with postnatal survival and morbidity outcomes. STUDY DESIGN: Prospective antenatal echocardiographic examination of fetal branch pulmonary arteries were correlated with postnatal survival and respiratory ...
Said Salah A M - - 2006
Congenital solitary coronary artery fistulas (CAFs) in adults are uncommon anomalies, which by themselves may resemble the whole spectrum of cardiac presentations from asymptomatic behavior to life-threatening and catastrophic events with syncope or shock and even sudden death. It may take decades to collect a reasonable series of patients in ...
Lee Andrew C - - 2006
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. The mortality of untreated ALCAPA has been estimated to range from 35% to greater than 85% ...
Welter Stefan - - 2006
A rare case of a patient with progressive dyspnoea due to atrial compression between ascending and descending aorta is demonstrated. After neoadjuvant chemoradiation for a locally advanced nonsmall cell lung cancer stage IIIb, he had a left-sided pneumonectomy. The underlying problem for cardiac compression was the extreme mediastinal shift reinforced ...
Okeke Linus I - - 2006
Urethral duplication (UD) is a rare congenital anomaly. In UD, there is in addition to a normally positioned urethra an accessory urethra arising from the bladder, bladder neck or the proximal urethra and terminating anywhere between the base of the penis along the ventral surface and the glans penis. The ...
Sambasivan Arathi - - 2006
OBJECTIVES: Children with congenital heart disease experience both hemorrhagic and thrombotic complications. In this report the authors test the hypothesis that hypoxemia is associated with altered sensitivity to activated protein C (aPC) in pediatric patients with congenital heart lesions. DESIGN: A retrospective genetic registry review, with statistical evaluation of factors ...
Baburaj A K - - 2006
Isolated congenital intrapericardial aneurysm of the left atrium (LA) or the left atrial appendage (LAA) is a rare anomaly [Zhao 1999]. The patient usually presents with features of systemic embolization, serious arrhythmia, and severe congestive cardiac failure as a complication, which can be fatal. Once the condition is diagnosed, surgery ...
Weber Oliver M - - 2006
Cardiovascular magnetic resonance (CMR) has become the method of choice in the evaluation of a number of questions in congenital heart disease. In addition to morphology, modern CMR techniques allow the visualization of function and flow in a temporally resolved manner. Among the pathologies where these methods play a major ...
Kohl Thomas - - 2006
OBJECTIVE: In order to assess the effect of deliberately delayed percutaneous fetoscopic tracheal occlusion on survival of fetuses with life-threatening congenital diaphragmatic hernia. METHODS: Eight fetuses with life-threatening congenital diaphragmatic hernia underwent fetoscopic tracheal balloon occlusion between 29 + 0 and 32 + 4 weeks of gestation. Delayed occlusion was ...
Schmitz Michael L - - 2006
The fixed incidence of congenital heart defects and improved survival have resulted in increasing numbers of adults with congenital heart disease (CHD) who have undergone complex repairs and/or palliations. Eventually, there will be more adults with CHD than children. They will require cardiac surgical interventions associated with progression of their ...
Ohtsuka Takashi - - 2006
Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly usually diagnosed in childhood. We report a case of isolated UAPA in an adult without any other cardiovascular anomaly. The patient was admitted for repeated progressive hemoptysis, which we treated with embolization of the bronchial artery three times, ...
Manghat N E - - 2005
The quality of the imaging of the main coronary arteries and side branches provided by multidetector row computed tomography (MDCT) may have importance when assessing congenital coronary artery anomalies. This review discusses the rationale for using MDCT for this indication and examines the advantages and disadvantages of the technique. Examples ...
Singh Harminder - - 2005
We present a unique case of idiopathic pulmonary vein stenosis in an adult who presented with progressive dyspnea and severe pulmonary arterial hypertension. Magnetic resonance imaging confirmed the diagnosis. The patient was treated by balloon angioplasty and is well at 6 months post-treatment follow-up. The etiology of pulmonary vein stenosis ...
Yo Chia-Hung - - 2005
The ring-sling complex is a rare developmental anomaly combining pulmonary artery sling and complete tracheal ring. The abnormal left pulmonary artery is a sling-like structure that surrounds the trachea and produces upper airway obstruction. Complete tracheal ring is associated with tracheal stenosis which often leads to respiratory distress. This article ...
Serban Marinela - - 2006
Cor triatriatum sinister is a rare congenital anomaly characterized by the presence of a fibromuscular membrane dividing the left atrium into two chambers: one entering the four pulmonary veins, the other connecting to the mitral valve. The extent of the communication between the two chambers and the presence of associated ...
Ackerman Kate G - - 2005
Congenital diaphragmatic hernia and other congenital diaphragmatic defects are associated with significant mortality and morbidity in neonates; however, the molecular basis of these developmental anomalies is unknown. In an analysis of E18.5 embryos derived from mice treated with N-ethyl-N-nitrosourea, we identified a mutation that causes pulmonary hypoplasia and abnormal diaphragmatic ...
Boll Daniel T - - 2005
The aim of this work was to assess magnetic resonance (MR) perfusion patterns of chronic, non-embolic pulmonary diseases of congenital and neoplastic origin and to compare the findings with results obtained with pulmonary, contrast-enhanced multislice computed tomography (CT) imaging to prove that congenital and neoplastic pulmonary conditions require MR imaging ...
Takiguchi Makoto - - 2005
Though congenital tracheal stenosis in infants with congenital heart disease is uncommon, congestive heart failure is often deteriorated by respiratory symptoms. We report an infant having a diagnosis of congenital tracheal stenosis complicated with congenital heart disease who underwent pericardial patch tracheoplasty after the arterial switch operation for the transposition ...
Ulucam Melek - - 2005
The giant congenital intrapericardial aneurysmal dilatation of the left atrial appendage without mitral valve disease is a very rare condition that is generally diagnosed in older patients. The problem is usually accompanied with supraventricular rhythm disorders and life-threatening systemic thromboembolism. Complete surgical correction is possible, and it should be performed ...
Tagliarini José V - - 2005
The congenital stenosis of pyriform aperture is an unusual cause of neonatal nasal obstruction. It is due to bony overgrowth of the nasal lateral process of the maxilla. Initially this narrowest part of nasal airway was considered an isolated deformity; subsequently the congenital Stenosis of pyriform aperture was thought to ...
Sihoe Alan D L - - 2005
Alveolar capillary dysplasia with misalignment of pulmonary vessels is an uncommon congenital cause of persistent pulmonary hypertension of the newborn. It is universally fatal, and diagnosis is entirely dependent upon surgical lung biopsy. We present a case of alveolar capillary dysplasia with misalignment of pulmonary vessels occurring in a full-term ...
Ziada H M - - 2005
Nostril stenosis is an uncommon deformity and its aetiology is variable. The shape alteration and nostril asymmetry may have negative aesthetic and functional effects on nostril stenosis patients. Five patients were included in this study and followed up for 24 months; four of these had congenital nostril stenosis and one ...
BaƟpinar Osman - - 2005
Congenital aneurysms of the muscular interventricular septum without ventricularseptal defect are extremely rare. We describe a five-month-old girl patient with congenital aneurysm, pathological ST changes and minimal right ventricular outflow tract obstruction. The electrocardiogram showed Wolf-Parkinson-White syndrome, pathological ST changes and combined ventricular hypertrophy criteria. The transthoracic echocardiography and cardiac ...
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