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Sidhu Maninder - - 2010
The management of adult congenital heart disease is challenging and poses specific problems. We report a patient with ventricular septal defect and pulmonary stenosis who underwent successful repair and coronary artery bypass grafting at the age of 76 years.
Yang Eric H - - 2011
A 28-year-old Cambodian man with a history of congenital heart disease presented with a 6-month history of increasing fatigue, night sweats, and weight loss. His surgical history included two Blalock-Taussig shunts, ventricular septal defect closure, and placement of a pulmonary valve conduit via a Rastelli procedure. Echocardiographic and cardiac computed ...
Siminelakis Stavros - - 2010
Cardiovascular disease in patients with congenital hypopituitarism is not rare; however, there is a lack of reports referring to cardiac interventions in such patients. We present a 76-year-old man with complete pituitary hormone deficiency, who presented with recurrent events of unstable angina. He had a significant stenosis of the left ...
Carsin A - - 2010
We report the case of a 5-year-old girl with persistent chest X-ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common ...
Keijzer Richard - - 2010
Congenital diaphragmatic hernia (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances ...
Cho Seung Hyun - - 2010
In this report, we describe a very rare case of a congenital subaortic left ventricular aneurysm in an 80-year-old patient. Cardiac computed tomography imaging is a reliable and noninvasive tool for diagnosis and follow-up of subaortic aneurysms.
Shankarappa Ravindranath Khandenahally - - 2011
Balloon mitral valvotomy (BMV) is a safe and effective therapy for rheumatic mitral stenosis. During the procedure, the coiled portion of 0.025 in. stainless steel BMV guidewire broke off within the giant left atrium (LA). Attempts to retrieve the guidewire with various snares failed as it was moving freely within the ...
Vaidyanathan Karthik - - 2010
Isolated absent pulmonary valve syndrome is a very rare entity. We report the case of a four-year-old boy who had congenital absent pulmonary valve with a thin and aneurysmal right ventricular infundibulum. The histological picture was suggestive of Uhl's anomaly. This association of partial right ventricular Uhl's anomaly with absent ...
Gartner Robyn D - - 2010
Tetralogy of Fallot (TOF) represents the most common form of cyanotic congenital heart disease, accounting for 6.8% of all congenital heart disease. As surgical techniques and medical management of patients with TOF have improved, most affected patients are reaching adulthood. Though surgical outcomes are favorable (<2% early mortality rate), adults ...
Yon James R - - 2010
We report the case of a 21-year-old man who presented with a hilar mass and whose condition was diagnosed as a congenital anomalous communication between the bronchial and pulmonary artery. We review his lung vascular embryology and anatomy that led to this rare systemic to pulmonary arterial shunt, describe other ...
Samardhi Himabindu - - 2010
A 15-year-old girl gave a recent history of dyspnoea and "funny turns". She had congenital aortic stenosis, previous valvotomies, a mechanical valve replacement, permanent pacemaker, atrial tachyarrhythmias, impaired ventricular function, systemic hypotension, pulmonary hypertension, and anxiety. The diagnosis of diaphragmatic flutter was delayed due to all the differential diagnoses and ...
Babaoglu Kadir - - 2010
Crossed pulmonary arteries are rare anomalies of the pulmonary trunk and its branches. In this anomaly, the ostium of the left pulmonary artery originates superiorly and to the right of the right pulmonary artery. This anomaly is usually associated with other congenital cardiac and extracardiac diseases. We report three neonates ...
Singhal Pawan - - 2010
Congenital coronary artery fistulas (CAFs) are uncommon abnormalities. A connection between the left main stem and main pulmonary artery is extremely rare. Congenital CAFs are frequently associated with another congenital heart disease. Associated anomalies include atrial septal defect, tetralogy of Fallot, patent ductus arteriosus, ventricular septal defect, and pulmonary atresia. ...
Beiras-Fernandez Andres - - 2010
Congenital anomalies of the pericardium are extremely rare and often difficult to diagnose. We describe a 66-year-old woman presenting with multivessel coronary artery disease and right heart insufficiency complicated by an extremely left-rotated heart due to a congenital absence of left pericardium. A multidetector row computerized tomographic study was performed ...
Diller G-P - - 2010
Pulmonary arterial hypertension (PAH) is a common problem in adult patients with congenital heart disease. We review available data on aetiology, clinical presentation, prognosis and management of PAH in this setting. In addition, we discuss general management strategies and emerging disease-targeting therapies.
Yonekawa Yasuhiro - - 2010
A case of remarkable vertebrobasilar insufficiency due to congenital multiple occlusive cerebro-vascular anomalies is presented. Anomalies are of the anterior and posterior circulation, presumably induced by "segmental vulnerability" and modified by infection of recurrent tonsillitis. Symptoms of repeated faintness and black-out attacks and deterioration of cognitive function compromised the quality ...
Lentini Salvatore - - 2010
We present the case of a young patient with severe congenital musculoskeletal abnormalities. associated with different pathologies, with involvement of the arterial tree on both the systemic and pulmonary circulation, and involvement of the lungs due to thoracic hypoplasia. The presence of such extensive pathology raises the question of the ...
Matsumoto Kazuhiro - - 2010
CT angiography (CTA) based on the data acquired by multidetector-row CT (MDCT) is an established, minimally invasive modality for imaging peripheral arteries. CTA has been used to assess peripheral arterial disease before treatment, and it has replaced conventional angiography for the diagnostic evaluation of peripheral arteries. MDCT can optimize both ...
Ayad Ramy F - - 2010
Congenital pulmonic stenosis (PS) rarely presents in patients over the age of 55 years. A stable asymptomatic course into late adulthood is the usual history of mild to moderate PS. Balloon valvuloplasty has become the procedure of choice for congenital PS, especially in children and young adults. There are rare ...
Kogon Brian E - - 2010
Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and ...
Argueta-Morales I Ricardo - - 2009
Congenital unilateral pulmonary venous atresia is a rare anatomical defect. Patients present with pulmonary symptoms, and diagnosis may be elusive. Pulmonary arterial wedge angiography is the gold standard with which to achieve diagnosis, and surgical intervention is often required for symptomatic patients.
Popov Aron-Frederik - - 2009
Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown-ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart ...
Lee Kwang-Jong - - 2009
We report a rare case of coexisting pulmonary artery sling, congenital tracheal stenosis, and dextrocardia caused by right lung hypoplasia. Successful treatment of severe postoperative tracheomalacia was achieved by aortopexy, aiming displacement of the aortic arch across orthogonally in front of the trachea due to dextrocardia. The aim of this ...
Sanders Lucas H A - - 2009
Congenital hypofibrinogenemia is a rare condition. We believe that cardiac surgery using cardiopulmonary bypass in a patient with congenital hypofibrinogenemia has not been reported before. We discuss the management in a patient with this condition who successfully underwent an aortic valve replacement and coronary artery bypass grafting.
Dragulescu Andreea - - 2009
Congenital pulmonary vein stenosis (PVS) presents as an isolated lesion or in association with other congenital heart anomalies. The most extensive forms of the disease are uniformly fatal because neither surgical repair nor transcatheter therapy results in long-term relief of the stenosis. A case is presented involving single-ventricle physiology associated ...
Leblanc Jacques G - - 2009
BACKGROUND: The field of congenital heart disease is constantly evolving through better understanding of the disease itself, albeit its history, prevalence, genetics, and follow-up. Concurrently surgical techniques and approaches have been developed, modified, and refined. DATA SOURCES: The impact of interventional pediatric cardiology has been dramatic. The field of catheter-based ...
Lim Z S - - 2009
Adolescent and adults with congenital heart disease (CHD) represent a special group of individuals. They often require regular assessment of their cardiac anatomy and functional hemodynamics. Most children with repaired CHD surviving to adulthood are either corrected or fully repaired and may not require long term follow-up. However, there are ...
Berdat Pascal A - - 2009
Although U-clip anastomoses were studied for hemodynamics and patency, their potential for unimpeded growth after congenital cardiovascular surgery has not been investigated yet. In 53 children aged 2.1+/-3.3 years operated on between March 1998 and August 2005 growth of U-clip (U) vs. polypropylene running sutured (P) anastomoses in coarctation repair ...
Mataciunas Mindaugas - - 2009
We report a mildly symptomatic 12-year-old boy with a very rare congenital anomaly-isolated unilateral pulmonary vein atresia. Diagnosis was made using CT angiography and its role in diagnosis is discussed.
Malikov Sergueï - - 2009
Congenital (primary) neonatal abdominal aortic aneurysm (AAA) is an extremely rare truncular arterial abnormality among numerous congenital vascular malformations. Only seven cases have been reported as congenital origin in newborns. This report presents the case of a male infant in whom a 33-mm congenital AAA was diagnosed prenatally and was ...
Rame J Eduardo - - 2009
Congenital heart disease can predispose individuals to pulmonary vascular remodeling as a result of the abnormality in pulmonary blood flow and pressure that accompanies the specific congenital defect being considered. Pulmonary arterial hypertension associated with congenital heart defects is an important determinant of functional capacity and survival, especially when the ...
Wald Rachel M - - 2009
Individuals with repaired tetralogy of Fallot (TOF) comprise a substantial proportion of the current adult congenital heart disease population. Pulmonary regurgitation (PR) is one of the most prevalent postoperative sequelae, but timing of pulmonary valve replacement (PVR) in the asymptomatic TOF patient remains controversial. We sought to explore thresholds for ...
Yuan Shi-Min - - 2009
Although total repair of some congenital heart defects is possible in young children, palliative procedures still play an important role in relieving patients' symptoms, particularly in emergent settings, when total correction or surgical repair is not available. However, the concepts and taxonomies of the palliative procedures for congenital heart diseases ...
Sawyer Taylor - - 2009
Generalized arterial calcification of infancy (GACI) is a rare genetic disorder consisting of diffuse arterial calcification and intimal proliferation. The disease typically results in progressive arterial stenosis and frequently leads to death from myocardial ischemia by 6 months of life. Affected infants are usually diagnosed before birth or in the ...
Hammad Aiman M - - 2009
Pulmonary artery aneurysms (PAAs) are uncommon entities. PAAs are caused mostly by trauma (often iatrogenic), infections and Behcet's disease (BD). Less common causes are pulmonary hypertension, congenital heart disease and neoplasm. BD is a multisystem disorder presenting with recurrent oral and genital ulcerations, as well as ocular involvement, and PAA ...
Ong Thida - - 2009
Children undergoing congenital heart surgery are at risk for prolonged mechanical ventilation and length of hospital stay. We investigated the prognostic value of pulmonary dead space fraction as a non-invasive, physiologic marker in this population. In a prospective, cross-sectional study, we measured pulmonary dead space fraction in 52 intubated, pediatric ...
Margulis Alexander - - 2009
Congenital melanocytic nevi of neuroectodermal origin are composed primarily of melanocytes and occasional neural elements. A subset of large/giant congenital melanocytic nevi is characterized by neuroidal differentiation resembling the histological pattern of neurofibroma (neuroid congenital melanocytic nevi). The authors report the case of a male infant born with a neuroid ...
Fraga Jose Carlos - - 2009
Long segment congenital tracheal stenosis (LSCTS), associated with complete tracheal rings, is a rare condition, difficult to manage and historically associated with high mortality rate. We report two pairs of identical twins all affected by LSCTS successfully treated by sliding tracheoplasty. All had severe respiratory distress. Three infants had left ...
Karadag Bilgehan - - 2009
Chest pain in a young person without cardiovascular risk factors is usually attributed to noncoronary causes; however, if the history suggests ischemic pain, the potential presence of unusual cardiovascular abnormalities should not be disregarded. The present case describes a young man with solitary congenital ostial atresia of right coronary artery, ...
Padalino Massimo A - - 2010
We report a rare case of a neonate with congenital giant aortic aneurysm associated to cleft sternum, who underwent surgical repair. The patient died on postoperative Day 5 from cardiac arrest. Autopsy revealed a circumferential subendocardial myocardial infarction and misdiagnosed coronary ostial anomalies. A critical analysis of this unfortunate case ...
Kruse Lisa - - 2009
Congenital vascular alterations of the normal adult arterial pattern have been associated with multiple congenital limb deformities including clubfoot and vertical talus. Investigators have observed absence of the anterior tibial artery and dorsalis pedis artery in most patients with clubfoot, and absence of the posterior tibial artery in all patients ...
Jonas Richard A - - 2009
Outcomes for surgical repair of tetralogy of Fallot with pulmonary stenosis are often considered to be a useful benchmark for the assessment of congenital surgical programs. This procedure, including preoperative assessment and postoperative management, falls in the middle of the spectrum of difficulty of all the congenital cardiac procedures. Within ...
Wright Cameron D - - 2009
Congenital tracheal surgery is uncommon and few centers have enough experience to make meaningful conclusions about treatment. Short-segment congenital tracheal stenosis is treated by tracheal resection, whereas long-segment stenosis is treated by slide tracheoplasty. Tracheomalacia is treated most commonly by aortopexy if due to simple vascular compression, division of an ...
Chowdhury Ujjwal K - - 2009
A 28-year-old woman with congenital aneurysm of the left atrial appendage was successfully treated by aneurysmectomy and is reported for its rarity. The exact diagnosis is relatively easy to make by contrast echocardiography, magnetic resonance imaging, and/or computerised tomographic angiography, provided the possibility is entertained. Because of supraventricular arrhythmias and ...
Rousou Anthony J - - 2009
Unilateral pulmonary artery agenesis is a rare congenital anomaly that is commonly associated with additional cardiovascular abnormalities. We report a case of intrapulmonary hemorrhage and hemoptysis in a patient with left-sided pulmonary artery agenesis as well as the first description of this anomaly in a patient with a diverticulum of ...
Tsai Ming-Horng - - 2009
Congenital tracheal stenosis (CTS) is a rare disease associated with high mortality and morbidity, especially if it is associated with intracardiac anomalies or vascular rings. Heliox, a "carrier gas" with low density, has been used in pediatric intensive care units for treating acute respiratory failure due to obstructive airway disease. ...
Marcus K A - - 2009
Congenital coronary artery anomalies are a well-recognized risk factor for sudden cardiac death in children as well as young adults, mostly during or immediately after intense exertion on the athletic field. Because these malformations are amenable to surgical treatment, timely identification is crucial. Unfortunately, antemortem diagnosis is notoriously difficult, partly ...
Katsinelos P - - 2008
Congenital afibrinogenemia is a rare genetic disorder characterized by the complete absence of functional fibrinogen. We report a 22-year-old female who developed nephrogenic arterial hypertension and intestinal ischemia due to congenital afibrinogenemia-associated angiopathy of large abdominal arteries. We describe, for the first time, the capsule findings and discuss the pathophysiology ...
Lee Hsing-Yuan - - 2008
Congenital atresia or extreme hypoplasia of individual pulmonary veins is a rare condition that is usually asymptomatic if it only involves 1 or 2 segments of the lungs. It may be fatal if it occurs in combination with other complex cardiac defects. The patients often present with recurrent pulmonary infections, ...
Zegdi Rachid - - 2008
OBJECTIVE: Congenital mitral valve regurgitation (MVR) is a rare disease occurring in infancy or childhood. Although congenital MVR has been described in adults, no surgical series has been reported so far. We describe here a 6-year surgical experience of congenital MVR in adults at a single institution. METHODS: We reviewed ...
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