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Sotto il Monte Giovanni XXIII, Italy

Myers P.O.,University of Geneva | Tissot C.,Unit of Pediatric Cardiology | Cikirikcioglu M.,University of Geneva | Kalangos A.,University of Geneva
Thoracic and Cardiovascular Surgeon | Year: 2011

Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass. © Georg Thieme Verlag KG Stuttgart New York. Source

Manieri S.,Unit of Pediatrics | Adurno G.,Unit of Cardioanesthesiology | Iorio F.,Unit of Cardiosurgery | Tomasco B.,Unit of Cardiosurgery | Vairo U.,Unit of Pediatric Cardiology
Minerva Pediatrica | Year: 2013

Congenital left ventricular diverticulum is a rare condition. When found, it is usually accompanied by other intracardiac malformations, so that again further examination is indicated. Furthermore, it is usually associated with thoracoabdominal wall defect, as seen in the spectrum of Cantrell's pentalogy, a congenital anomaly consisting of a lower sternal defect, diastasis recti, pericardial defect, anterior diaphragmatic hernia and cardiac malformation. This paper reports a case of Cantrell's Syndrome with left ventricular diverticulum. Successful total correction of ventricular diverticulum, ventricular defects and diastasi recti, was performed at two years of age. The clinical features, ambryology and surgical management of these defects are discussed. Source

Amado M.,Hospital Unit of Portimao | Calado M.A.,Hospital Unit of Portimao | Ferreira R.,Unit of Pediatric Cardiology
BMJ Case Reports | Year: 2014

Marfan syndrome (MFS) is a genetic disorder of the connective tissue which rarely manifests in the neonatal period and has an ominous prognosis. A case of a first female offspring of healthy parents is described here. The pregnancy was uneventful and the mother had a term caesarean delivery. At birth, some dysmorphic signs became apparent, such as loose redundant skin, dolichocephaly, frontal bossing, deeply sunken eyes, micrognathia, contractures of the elbows, arachnodactyly and hip dysplasia. The echocardiogram showed a mitral and tricuspid valve regurgitation and a long aortic arch. The diagnosis of neonatal MFS came forward and genetic studies revealed a de novo mutation in the fibrillin 1 (FBN1) gene. At 6 months, due to a progressive worsening of the cardiac pathology, she was submitted to mitral valvuloplasty. She is now 2 years and 10 months old, which is a remarkable feat for a child suffering from this condition. Copyright 2014 BMJ Publishing Group. All rights reserved. Source

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