Lutembacher's syndrome: A rare combination of congenital and acquired heart disease – A case report and review of literature

Lutembacher's syndrome is defined as the rare combination of congenital atrial septal defect and acquired mitral stenosis. The haemodynamic effects of this syndrome are a result of the interplay between the relative effects of the atrial septal defect and mitral stenosis. Mitral stenosis augments the left to right shunt through the atrial septal defect. The definition of Lutembacher's syndrome has undergone many changes. The earliest description in medical literature was found in a letter written by anatomist Johann Friedrich Meckel to Albrecht von Haller in 1750.In 1916, Lutembacher described his first case of this syndrome, involving a 61-year-old woman, and he attributed the mitral valvular lesion to congenital mitral stenosis. Because the mitral stenosis was, in fact, rheumatic in aetiology, the syndrome was defined eventually as a combination of congenital atrial septal defect and acquired, almost always rheumatic, mitral stenosis.Keywords:Lutembacher's syndrome, congenital heart disease, valvular heart disease,  atrial septal defect, mitral stenosi

Tetralogy of Fallot and Atrial Septal Defect in a White Bengal Tiger Cub (Panthera tigris tigris)

A 3-week-old female white Bengal Tiger cub (Panthera tigris tigris) presented with acute onset tachypnoea, cyanosis and hypothermia. The cub was severely hypoxaemic with a mixed acid–base disturbance. Echocardiography revealed severe pulmonic stenosis, right ventricular hypertrophy, high membranous ventricular septal defect and an overriding aorta. Additionally, an atrial septal defect was found on necropsy, resulting in the final diagnosis of Tetralogy of Fallot with an atrial septal defect (a subclass of Pentalogy of Fallot). This report is the first to encompass arterial blood gas analysis, thoracic radiographs, echocardiography and necropsy findings in a white Bengal Tiger cub diagnosed with Tetralogy of Fallot with an atrial septal defect

51 year-old male with dyspnea and hypoxia

AbstractWith continued advancements in medical practice, physicians are caring for more adult patients with congenital heart diseases and their sequelae. We report a 51 year-old with obstructive sleep apnea presenting with dyspnea, hypoxia and pulmonary hypertension, found to have a congenital atrial septal defect. The patient had symptomatic improvement following percutaneous closure of his ostium secundum atrial septal defect

Interrupted inferior vena cava and partial anomalous pulmonary venous return with atrial septal defect in a 38-year-old adult: a case report

We present a woman having congenital anomalies of the inferior vena cava and partial anomalous pulmonary venous return from the right lung with atrial septal defect in a 38-year-old. Congenital anomalies of inferior vena cava are rare. They are seen more often in young males. If there are not other anomalies, they are latent for a long time. Peripheral venous thrombosis, chronic venous insufficiency, dyspnea and fatigue are often the first symptoms of these anomalies. Surgical repair of atrial septal defect with partial anomalous pulmonary venous return include provision of durably unobstructed systemic and pulmonary venous pathways, closure of the atrial septal defect, and avoidance of arrhythmias. The diagnosis has been determined by compression ultrasonography with color doppler assessment, multidetector computed tomography angiography and echocardiography

Spontaneous resolution of atrial and ventricular septal defects in Malta

Congenital heart disease (CHD) is the commonest congenital malformation, and ventricular septal defect (VSD) and atrial septal defect (ASD) are the commonest forms of CHD. This study was undertaken to determine rates or spontaneous closure or lesions diagnosed at echocardiography after detection or a murmur in Maltese patients born in 1990-94. A significant excess or ASD and VSD was found in Malta, and this was attributed to early echocardiographic diagnosis or small defects, prior to spontaneous closure. A high spontaneous closure rate was round for both ASD and VSD. Rate of closure for both defects was initially high, and tapered off at about 5 to 7 years or age for both lesions. ASDs in excess of 8 mm in diameter at presentation also underwent spontaneous resolution, which is contrary to the known natural history or these defects. Larger VSDs were shown to have a smaller likelihood or closing (p=0.04). Parents and patients can be reassured that spontaneous closure is very likely to occur in the vast majority of these conditions.peer-reviewe

Early postoperative interventional ASD-closure for severe atrial right to left shunt in a neonate with common arterial trunk

Although closure of an atrial septal defect (ASD II) with an occluding device in the first year of life is not a routine procedure, it is a feasible treatment, even in neonates. Case reports on the off-label use of Amplatzer devices have been repeatedly published, but there are no reports on using the Amplatzer Duct Occluder (ADO) to close an atrial septal defect in a neonate. We report on a successful catheter closure of an ASD II with ADO in a severely cyanotic neonate, seven days after surgical repair of common arterial trunk. Due to progressive cyanosis and clinical signs of right ventricular failure, which developed after common arterial trunk repair, the neonate underwent cardiac catheterization. Diastolic filling impairment of the right ventricle (right ventricle hypertrophy, pulmonary regurgitation, and residual right ventricle outflow tract obstruction) was thought to be the cause of impaired right ventricle diastolic filling, resulting in the right-to-left shunt at the atrial level. Under transesophageal echocardiographic guidance, ADO was delivered through a 5 French sheath into the atrial septal defect. Amplatzer duct occluder closed the defect and proved to be stable in position after disconnection. During the procedure, the child was stable and then transferred to the intensive care unit with significantly improved oxygen saturation. This is the first report on placing a duct occluder in the atrial septal position, which is a novel procedure for-small neonates

Atrial septal defect

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Atrial Septal Defect

Atrial Septal Defects (ASDs) are relatively common both in children and adults. Recent reports of increase in the prevalence of ASD may be related use of color Doppler echocardiography. The etiology of the ASD is largely unknown. While the majority of the book addresses closure of ASDs, one chapter in particular focuses on creating atrial defects in the fetus with hypoplastic left heart syndrome. This book, I hope, will give the needed knowledge to the physician caring for infants, children, adults and elderly with ASD which may help them provide best possible care for their patients

A comparison of clinical paediatric murmur assessment with echocardiography

Objective: To compare the clinical acumen of paediatric cardiovascular examination between various hospital paediatrician grades. Design: Prospective data collection of clinical and echocardiography findings on paediatric echocardiography referrals. Setting and patients: All paediatric patients (birth - 14 years) referred for echocardiography, in a regional hospital catering for the island population of Malta. Echocardiography was carried out by three paediatricians with tertiary training in this technique. Main outcome measures: Pre-echocardiography clinical diagnoses were compared with echocardiography results according to grade of referring hospital doctor (ranging from houseman to consultant). Both normal and abnormal hearts at echocardiography were included. Results: Echocardiographers had the highest clinical accuracy and the highest attempts at reaching a clinical diagnosis. Accuracy and attempts at diagnosis decreased as doctor's hospital grade decreased, from consultant to houseman. Ventricular septal defect was the most easily diagnosed lesion. Atrial septal defect was often misdiagnosed as pulmonary stenosis.peer-reviewe