Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.
The double-chambered right ventricle (DCRV) is a rare congenital heart disease caused by hypertrophic anomalous muscle bands that divide the ventricular cavity, resulting in the formation of a high-pressure proximal chamber and other low-pressure distal one. Because of its evolving nature, its diagnosis is usually made during childhood/adolescence and very rarely during adulthood. It accounts for approximately 0.5-1% of patients with congenital heart disease. We report a case of a 50-year-old man who presented to us with acute inferior wall myocardial infarction (MI). Echocardiography revealed the features of DCRV and ventricular septal defect. Coronary angiogram revealed significant stenosis in right coronary artery. This is the first case where an adult patient with DCRV incidentally diagnosed after presentation with acute MI and had undergone successful coronary angioplasty.
Facklamia hominis is a rare causative organism of infective endocarditis (IE). Only few cases of infection due to F. hominis have been reported in the literature. We describe a case of IE due to Gram-positive, alpha-haemolytic, catalase-negative coccus F. hominis in an adult patient with rheumatic mitral stenosis. Isolated mitral stenosis is an uncommon valve lesion predisposing to IE. The following paper is being presented to emphasize the possibility of IE due to F. hominis, and laboratories need to be alert of the potential significance in appropriate clinical setting.
Familial homozygous hypercholesterolemia is a potentially dangerous risk factor that can result in premature coronary arterial disease in children and young adults. This can result in severe morbidity and premature death in young individuals. We also emphasise the need to screen first-degree relatives and extended family members, this playing an important role in early detection and treatment. Despite recent advances in treatment using lipid lowering agents, the disease remains a significant challenge.
DESCRIPTIONA 76-year-old woman was admitted with acute onset of breathlessness with lethargy since the last 3 days. She was a known patient with hypertension and diabetes. On clinical examination, she had an engorged neck vein, tachycardia and tachypnoea, hypotension, oxygen saturation of 92% in room air and right ventricular S3 gallop with pansystolic murmur in the left lower parasternal area. ECG showed sinus tachycardia with an S1Q3T3 pattern. Chest X-ray did not reveal any significant abnormality. There was no elevation of cardiac biomarkers. Echocardiography revealed the presence of right atrial and ventricular dilation, moderate tricuspid regurgitation, severe right ventricular dysfunction, regional wall motion abnormality of the basal and mid right ventricular free wall with apical hyper contractility (McConnell's sign), paradoxical septal motion and dilated inferior vena cava (figure 1A-C; videos 1-3). The CT pulmonary angiogram was suggestive of acute pulmonary embolism (APE) (figure 2). She was treated with infusion of streptokinase for 48 h, followed by an oral anticoagulant.McConnell's sign is the most distinct echocardiographic finding described in patients with APE.
Thymolipomas are rare anterior mediastinal tumors composed of mature adipose tissue and benign thymic tissue and they may rarely simulate cardiomegaly on chest radiograph. We report an adult male who presented with progressive dyspnea of 2 months' duration. Clinical examination was unremarkable. Chest radiograph showed enlarged cardiac silhouette. Computed tomography of chest revealed a giant anterior mediastinal noncontrast enhancing mass partially wrapping around the heart. A needle biopsy obtained lymphomatous material that was diagnosed as thymolipoma. The tumor was successfully removed en bloc through a median sternotomy. Histopathological examination confirmed thymolipoma. We emphasize the importance of considering mediastinal tumors as a differential diagnosis in patients with progressive dyspnea without any obvious cause and chest radiograph showing enlarged cardiac silhouette.
Coronary artery anomalies are found in 0.6%-1.5% of patients undergoing diagnostic coronary angiogram. Intervention in these patients poses a particular technical challenge secondary to the aberrancies in the vessel origin and course. From March 2011 to February 2013, 13 cases with complex coronary artery anomalies were observed among 2482 patients undergoing CAG (0.52%) at our cath lab. Only three patients had severe stenosis in the anomalous artery sufficient to require an intervention and had presented with myocardial infarction. PCI was performed successfully in these 3 patients two of which had anomalous left circumflex artery and the other having an anomalous right coronary artery.
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