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Results 201 - 250 of 582
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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 ...
Heper Gulumser - - 2005
Congenital nonfamilial supravalvular aortic stenosis (SVAS) is relatively rare, its diffuse type being the least common. We present a 30-year-old woman with diffuse SVAS complicated with left ventricular apical aneurysm. We believe that subtle left ventricular myocardial ischemia or infarction and long-lasting severe pressure overload to the apical chamber caused ...
David R Hootnick - - 2004
Ischemic necrosis, which develops rarely after clubfoot surgery, may have a vascular etiology, since many idiopathic and neurogenic clubfeet have congenital deficiency of the anterior tibial and dorsalis pedis arteries. Dorsalis pedis deficiency is demonstrated more frequently in those clubfeet showing greater deformity. Substantial hypoplasia of the profunda femoris and ...
Altuntas Ali - - 2004
Choanal atresia is one of the most commonly observed congenital abnormalities of the nose. This defect is frequently associated with other congenital anomalies. One of the anomalies, which is rarely encountered is a Tessier's facial cleft 2. In this report, we present a 6-year-old girl who was brought to our ...
Chockalingam Anand - - 2004
Submitral aneurysm (SMA) is congenital outpouching of the left ventricular (LV) wall invariably occurring adjacent to the posterior leaflet of mitral valve. SMA is typically diagnosed in young adults who present with severe mitral regurgitation, heart failure, systemic embolism, and sudden cardiac death. African blacks account for the majority of ...
Gravereaux Edwin C. - - 2004
Congenital vascular anomalies are rare. The cardiovascular specialist should nevertheless be aware of the more common types of vascular anomalies and understand the implications for patient treatment and the likelihood of associated morbidity. The presentation of congenital arteriovenous malformations can range from asymptomatic or cosmetic lesions, to those causing ischemia, ...
Torres Javier - - 2004
LEOPARD syndrome is a neuroectodermal disorder presumed to result from an abnormality in neural crest cells. The acronym 'LEOPARD' is derived from the clinical features which include multiple lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retarded growth, and deafness. Given the multisystem nature of the disease process, several ...
Schaefer Katherine S - - 2004
The outflow tract (OFT) is abnormal in many congenital heart defects. One critical mechanism for morphogenesis of this complex structure is apoptosis. Chicken embryos (stages 19-38; ED4-10) stained with a fluorescent supravital lysosomal dye (LysoTracker Red; LTR) revealed the three-dimensional relationship between structural changes and apoptosis. The LTR staining peaked ...
Bellini C - - 2004
We report on three infants with congenital chylothorax (CC) and congenital pulmonary lymphangiectasia (CPL). CPL appears to be a characteristic pathological finding in CC. Through the use of lymphoscintigraphy and computed tomography, this study suggests that CC and CPL are strongly correlated entities and that the dysplasia of the lymphatic ...
Lin Pei-Chun - - 2004
Aneurysms of the subclavian artery are rare. Similarly, congenital absence of the internal carotid artery is an extremely rare vascular anomaly. In this report, we discuss the case of a 54 year-old woman with an unusual aneurysm of the right subclavian artery in association with congenital absence of the right ...
Kussman Barry D - - 2004
Compression of the paediatric airway is a relatively common and often unrecognized complication of congenital cardiac and aortic arch anomalies. Airway obstruction may be the result of an anomalous relationship between the tracheobronchial tree and vascular structures (producing a vascular ring) or the result of extrinsic compression caused by dilated ...
Griselli Massimo - - 2004
OBJECTIVE: Congenital heart defects with major aortopulmonary collateral arteries show marked variability in the size and distribution of native pulmonary arteries. We sought to classify the size and distribution of native pulmonary arteries and to determine their influence on surgical outcome. METHODS: Between 1989 and 2002, 164 patients underwent surgical ...
Yilmaz Mehmet Birhan - - 2003
Swyer-James syndrome is an uncommon disease with unknown aetiology. It is generally discovered on a chest radiograph as increased translucency involving one hemithorax because of diminished vascular markings. Pulmonary vascularization on the affected side is greatly reduced leading to radiological translucency. Myocardial bridging describes an angiographic entity, which is any ...
Yukiiri Kazushi - - 2003
Congenital malformation of the right atrium or the coronary sinus is rare, and cases are classified into 1 of the following 4 categories: (1) congenital enlargement of right atrium, (2) single diverticulum, (3) multiple diverticula of the right atrium, and (4) diverticulum of the coronary sinus. This report presents a ...
Konen Eli - - 2003
The term congenital pulmonary venolobar syndrome refers to a wide spectrum of pulmonary developmental anomalies that may appear singly or in combination. The main components of congenital pulmonary venolobar syndrome are hypogenetic lung (including lobar agenesis, aplasia, or hypoplasia), partial anomalous pulmonary venous return, absence of pulmonary artery, pulmonary sequestration, ...
Raas-Rothschild Annick - - 2003
The Stuve-Wiedemann syndrome (SWS) is a congenital bone dysplasia characterized by camptodactyly with ulnar deviation and congenital bowing of the long bones. Affected patients present with respiratory difficulties in the neonatal period or later and recurrent episodes of hyperthermia. The typical radiological findings are bowing of the long bones of ...
Angtuaco Sylvia S O - - 2003
The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right ...
Ishikawa Shiro - - 2003
Pulmonary hypoplasia is involved in patients with various surgical diseases. The aim of this study was to evaluate the clinical usefulness of measurement of the chest/trunk-length ratio (C/T) for predicting pulmonary hypoplasia in patients with congenital anomalies, with the exception of mass-like lesions in the thorax such as diaphragmatic hernia ...
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