1/11. Reduced left ventricular dimension and normalized atrial natriuretic hormone level after repair of aortic coarctation in an adult.Although unusual in the older patient, coarctation of the aorta can be an occult cause of cardiomyopathy. This report describes a 53-year-old man with new-onset heart failure symptoms, global left ventricular (LV) dysfunction, and underlying aortic coarctation. Surgical correction resulted in reduced LV size, resolution of symptoms, and normalization of atrial natriuretic hormone levels.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
2/11. Expanding indications for the Ross procedure.The pulmonary autograft procedure has been shown to provide excellent hemodynamic results in young patients with aortic pathology. However, the use of this procedure in those with more complex aortic disease has not been extensively evaluated. The purpose of this report is to present the application of the Ross procedure in a 21-year-old man with extensive acquired aortic root pathology, both subannular and supraannular, and prosthetic valve dysfunction after two previous procedures.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
3/11. Ebstein's anomaly with coarctation of the aorta. An unusual association.Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solitus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a DeVega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
4/11. Precoronary stenosis after stage I palliation for hypoplastic left heart syndrome.We report a patient with stenosis of the native ascending aorta after palliation of hypoplastic left heart syndrome and aortic atresia. We describe the approach to diagnosis, temporary support with extracorporeal membrane oxygenation, and successful surgical reintervention. Stenosis of the native ascending aorta is an important, potentially reversible cause of acute, early postoperative ventricular dysfunction.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
5/11. Balloon angioplasty of critical coarctation in a 970-gram premature infant.We describe a 970-gram infant born prematurely at 28 weeks gestation. On the third day of life, she presented in heart failure with Shone's syndrome and severe coarctation of the aorta. Despite initiation of intravenous prostaglandins, she developed progressive severe biventricular dysfunction and acidosis. Balloon angioplasty of the coarctation was successfully performed on the fifth day of life, with a dramatic improvement in ventricular function. At seven weeks of age, after improvement of ventricular function, she underwent surgical resection of the coarctation and arch advancement. At six months of age she is thriving and developing normally.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
6/11. Midaortic syndrome in the fetus and premature newborn: a new etiology of nonimmune hydrops fetalis and reversible fetal cardiomyopathy.Nonimmune hydrops fetalis is the final common pathway of many conditions that ultimately result in fetal anasarca. Even after extensive evaluation, the etiology of a small percentage of cases of hydrops remains unknown. We present a case of midaortic syndrome, also known as abdominal coarctation syndrome, in a fetus with hydrops and a severe cardiomyopathy. The clinical manifestations of midaortic syndrome in this fetus and premature newborn, including malignant hypertension and reversible cardiomyopathy, are detailed. The fetal pathophysiology of midaortic syndrome remains speculative, but likely includes fetal hypertension as the cause of cardiac dysfunction. To our knowledge, this is the first report of midaortic syndrome as an etiology for nonimmune hydrops fetalis.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
7/11. Dilated cardiomyopathy: an unusual presentation of aortic coarctation in an infant.Coarctation of the aorta commonly presents in infancy as congestive heart failure, or later in childhood as hypertension or as a heart murmur. However, we experienced a unique infant case of isolated coarctation presenting with acute decompensation of a dilated cardiomyopathy, which recovered completely 8 months postoperatively. Our report highlights the previously unreported presentation of coarctation in infancy as a dilated cardiomyopathy. It also implies that before we label any patient presenting with a dilated cardiomyopathy as an idiopathic cardiomyopathy, we must exclude all possible specific causes of myocardial dysfunction because many such specific cardiomyopathies are curable and very rewarding, just like our patient.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
8/11. Chronic pseudoaneurysm and coarctation of the aorta: a rare delayed complication of trauma.We report an unusual case of pseudoaneurysm and coarctation of the descending thoracic aorta after trauma. The coarctation of aorta resulted in hypertension, severe left ventricular dysfunction, and symptoms of congestive heart failure. Surgical bypass resulted in control of blood pressure and improvement of heart failure symptoms. The mechanism of aortic injury leading to the development of this rare combination is discussed.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
9/11. digeorge syndrome with isolated aortic coarctation and isolated ventricular septal defect in three sibs with a 22q11 deletion of maternal origin.digeorge syndrome was diagnosed in an infant who had an interrupted aortic arch, hypoparathyroidism, and low T lymphocyte numbers. Two siblings had heart defects that are not commonly described in digeorge syndrome (a membranous ventricular septal defect and coarctation of the aorta respectively). These siblings did not have evidence of thymic dysfunction or hypoparathyroidism. Chromosome analysis showed that the mother, whose cardiovascular examination was normal, and her three offspring with heart defects had a 22q11 interstitial deletion, which was confirmed by molecular analysis. This family suggests that 22q11 deletions can cause apparently isolated heart defects and that the range of these defects may be wider than previously recognised. Once the genes that are deleted in this family are characterised they will be useful candidate genes in the investigation of isolated cardiac malformations.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
10/11. renal insufficiency in williams syndrome.A 19-year-old patient with williams syndrome and renal cystic dysplasia is presented. This young man has had gradual deterioration of renal function and recurrent, acute episodes of dehydration secondary to a concentrating defect. A review revealed several reports of renal dysfunction in patients with williams syndrome, but this complication has not been sufficiently emphasized. We recommend that all patients with williams syndrome undergo periodic ultrasound examination and renal function evaluation.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
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