1/182. Autosomal dominant secundum atrial septal defect with various cardiac and noncardiac defects: a new midline disorder.We report on a Lebanese family in which 12 persons had an atrial septal defect and various cardiac and noncardiac anomalies. Cardiac anomalies are left axis deviation of QRS, right bundle branch block, atrial fibrillation, wolff-parkinson-white syndrome, nodal atrioventricular rhythm, aortic stenosis, pulmonic valve stenosis, mitral stenosis (lutembacher syndrome), and low implantation of the tricuspid valve (Ebstein disease). Noncardiac abnormalities consisted specially of the presence of hypertelorism, cleft lip, and pectus excavatum. This combination appears to constitute a hitherto undescribed autosomal dominant midline disorder of the heart and upper half of the body with almost full penetrance and variable expressivity. The mutation does not map to any known locus involved in atrial septal defect or conduction block.- - - - - - - - - - ranking = 1keywords = stenosis (Clic here for more details about this article) |
2/182. Pitfalls of undetected patent foramen ovale in off-pump cases.We describe pitfalls of a hitherto undetected patent foramen ovale during the conduct of an off-bypass coronary revascularization. Manipulation of the heart resulted in right-to-left shunt and severe desaturation requiring institution of cardiopulmonary bypass to close the patent foramen ovale and complete the revascularization.- - - - - - - - - - ranking = 2.9848641970357keywords = coronary (Clic here for more details about this article) |
3/182. Ebstein's anomaly associated with severe valvar pulmonary stenosis: successful palliation with balloon pulmonary valvuloplasty in an adult.We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention.- - - - - - - - - - ranking = 1.6666666666667keywords = stenosis (Clic here for more details about this article) |
4/182. Transcatheter closure of a patent foramen ovale following mitral valve replacement.We report the successful closure of a postoperative patent foramen ovale in a patient who underwent coronary artery bypass grafting and mitral valve replacement for severe mitral insufficiency. The postoperative course was complicated by severe hypoxemia due to a large patent foramen ovale. The patient underwent transcatheter closure with the Das Angel Wings transcatheter occluder (Microvena Corporation, White Bear Lake, MN) with immediate improvement.- - - - - - - - - - ranking = 2.9848641970357keywords = coronary (Clic here for more details about this article) |
5/182. Molecular and clinical characterization of a patient with duplication of 1p36.3 and metopic synostosis.Chromosome 1p duplications are rare. There have been only 11 reported cases of isolated 1p duplication, all of which were proximal, interstitial duplications. We present a patient with a terminal duplication of 1p (1p36.3). To our knowledge, this is the first such reported case. Our patient presented with metopic synostosis, rectal stenosis, atrial septal defect, and mildly delayed gross motor development. Molecular characterization using microsatellite marker analysis and fluorescence in situ hybridization (FISH) revealed an area of duplication between p58 and D1S2893, approximately 13 cM in size. We compare our patient's clinical findings with the clinical phenotype found in patients with the corresponding deletion of 1p36.3 and discuss the role of gene dosage in other deletion/duplication syndromes.- - - - - - - - - - ranking = 0.33333333333333keywords = stenosis (Clic here for more details about this article) |
6/182. Repair of cor triatriatum associated with partially unroofed coronary sinus.A young patient with cor triatriatum, secundum atrial septal defect, persistent left superior vena cava, partially unroofed coronary sinus, and moderate tricuspid regurgitation was documented to have both a left-to-right shunt from the common pulmonary venous chamber to the right atrium and a right-to-left shunt from the partially unroofed coronary sinus to the left atrium. Resection of the membrane dividing the left atrium, closure of the atrial septal defect and the partially unroofed coronary sinus with pericardial patches, and a tricuspid annuloplasty resulted in an excellent hemodynamic result.- - - - - - - - - - ranking = 20.89404937925keywords = coronary (Clic here for more details about this article) |
7/182. extracorporeal membrane oxygenation discontinuation despite technically successful reoperation: A case report.death remains a probable outcome of pediatric cardiac extracorporeal membrane oxygenation (ECMO) despite increasing efforts to improve the results. On venoarterial ECMO, in an obviously hopeless situation, the decision to withdraw a life supporting measure resulting in the sudden death of a child places a heavy burden on the team. After valvulotomy of critical aortic stenosis in a prenatally diagnosed term neonate, ECMO had to be installed during postoperative resuscitation. Despite technically successful homograft implantation while on ECMO complicated by postoperative bleeding, advancing multiorgan failure resulted in ECMO withdrawal. As shown in this case report, exact termination criteria are lacking but are necessary to prevent increasing team and resource related conflicts in pediatric cardiac ECMO.- - - - - - - - - - ranking = 0.33333333333333keywords = stenosis (Clic here for more details about this article) |
8/182. Device closure of an atrial septal defect following successful balloon valvuloplasty in a neonate with critical pulmonary valve stenosis and persistent cyanosis.Persistent cyanosis after successful balloon valvuloplasty for neonatal critical pulmonary valve stenosis is often related to poor right ventricular compliance and right-to-left shunting at the atrial level. A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt.- - - - - - - - - - ranking = 1.6666666666667keywords = stenosis (Clic here for more details about this article) |
9/182. A case of acute pulmonary embolism and acute myocardial infarction with suspected paradoxical embolism after laparoscopic surgery.We describe the case of a 59-year-old Japanese man who had an acute pulmonary embolism in addition to acute myocardial infarction after a laparoscopic cholecystectomy. The posterior descending coronary artery was totally occluded. and direct percutaneous transluminal balloon angioplasty was performed. The pulmonary embolism was diagnosed by lung perfusion scanning and was treated with anticoagulant therapy. A patent foramen ovale and right-to-left atrial shunting of blood were detected by contrast transesophageal echocardiography. Paradoxical embolism is a rare complication of pulmonary embolism and may have been responsible for the acute myocardial infarction in our patient.- - - - - - - - - - ranking = 2.9848641970357keywords = coronary (Clic here for more details about this article) |
10/182. Lutembacher's syndrome: recognition by echocardiography.The typical echocardiographic features of mitral stenosis in association with an enlarged right ventricle and abnormal septal motion are generally consistent with pulmonary hypertension and functional tricuspid regurgitation. In the absence of clinical and echocardiographic features of pulmonary hypertension, the combination of mitral stenosis and "volume overload" of the right ventricle should alert the clinician to the diagnosis of Lutembacher's syndrome. This report discusses a patient with these echocardiographic findings in whom the diagnosis of Lutembacher's syndrome was confirmed by cardiac catheterization.- - - - - - - - - - ranking = 0.66666666666667keywords = stenosis (Clic here for more details about this article) |
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