Late endocarditis of Amplatzer atrial septal occluder device in a child.

Bacterial endocarditis following atrial septal defect closure using Amplatzer device in a child is extremely rare. We report a 10-year-old girl who developed late bacterial endocarditis, 6 years after placement of an Amplatzer atrial septal occluder device. Successful explantation of the device and repair of the resultant septal defect was carried out using a homograft patch. The rare occurrence of this entity prompted us to highlight the importance of long-term follow up, review the management and explore preventive strategies for similar patients who have multiple co-morbidities and a cardiac device. A high index of suspicion is warranted particularly in pediatric patients.

Ischemic gall bladder perforation: a complication of type A aortic dissection.

Malperfusion of end organs occurs in 20% to 40% patients with acute type A aortic dissection. Because irreversible ischemia is a time-dependent event, expedient diagnosis and treatment are necessary. We herein report successful surgical management of a patient with acute type A aortic dissection causing transient gut ischemia and a rare gall bladder perforation. We implemented one-stage surgical and laparoscopic management approach for the diagnosis and treatment. Increased awareness of this complication and appropriate use of available diagnostic tools may improve the outcome in similar patients. Patients with aortic dissection complicated by visceral ischemia require a prompt sequential and rational multidisciplinary approach for successful management.

Inflammatory myofibroblastic tumor of the right atrium.

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity and is associated with distinct clinical, pathological and molecular features. The clinical behavior, natural history, biological potential, management and prognosis of such tumors are unclear. We present herewith an adolescent girl who presented with similar entity involving the junction of the right atrium and the inferior vena cava (IVC) in association with thrombocytosis and IVC thrombosis leading to obstruction of blood flow. Diagnostic tools included imaging and immuno-histopathology studies. Surgical management included resection of the tumor and thrombo-embolectomy of the IVC under cardiopulmonary bypass. This case is unique due to association of complete obstruction of IVC caused by the strategic location of the tumor, thrombosis of vena cava and association of thrombocytosis. These features have not been reported yet in relation to the cardiac IMT. This report will help in better understanding and management of similar cases in terms of planning cannulation of femoral veins or application of total hypothermic circulatory arrest during cardiopulmonary bypass and prompt us to look for recurrence or metastasis during follow up using echocardiography and laboratory investigations. The possibility of IMT should be kept in the differential diagnosis of cardiac tumors especially in children and adolescents.

Papillary fibroelastoma of the aortic valve--a case report and literature review.

The prevalence of primary cardiac tumour ranges from 0.0017-0.28% and papillary fibroelastoma is rare but not uncommon benign cardiac neoplasm. Currently, with the advent of higher-resolution imaging technology especially transoesophageal echocardiography such cases being recognized frequently. The clinical presentation of these tumours varies from asymptomatic to severe ischaemic or embolic complications. We herein, present a 50-year-old female patient with a papillary fibroelastoma of the aortic valve arising from the endocardium of the right coronary cusp very close to the commissure between the right and non-coronary cusps. The patient presented with angina-like chest pain and was investigated using echocardiography and CT angiographic modalities in addition to the usual investigations. The differential diagnosis considered was a thrombus, myxoma, Lambl's excrescence and infective vegetation. The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve. The patient recovered well. A review of the literature suggests that the cardiac papillary fibroelastoma is a rare but potentially treatable cause of embolic stroke and other fatal complications, therefore, a strong suspicion; appropriate use of imaging modality, preoperative anticoagulation and urgent surgical resection is warranted. Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

Acute pulmonary thromboembolism during mitral valve repair.

Perioperative pulmonary thromboembolism during cardiac surgery is extremely rare. We report a 55-year-old male, who developed acute pulmonary thromboembolism during mitral valve repair. Intra-operative trans oesophageal echocardiography failed to demonstrate thromboembolus in the pulmonary arteries but it showed the secondary signs of acute pulmonary artery obstruction. Thromboembolectomy was done after high index of suspicion on clinical grounds was found and the patient recovered. The diagnostic accuracy and sensitivity of transoesophageal echocardiography in circumstances with altered haemodynamics is questionable and has not been investigated. Therefore, indirect evidence of pulmonary artery obstruction on echocardiography may have a predictive value and failure to demonstrate pulmonary thromboembolism by this tool should not exclude the possibility of it especially when it is highly suspected.

Coronary artery fistula with coronary sinus obstruction and retrograde drainage.

Coronary artery to coronary sinus fistula (CACSF) in association with stenosis of coronary sinus (CS) ostium and retrograde drainage via persistent left superior vena cava (LSVC) is an extremely rare anomaly presenting in the neonatal period. We report herein a 2-day-old boy with similar anomalies, who presented to us with congestive heart failure and underwent surgical repair. Echocardiography including Doppler ultrasound was a non-invasive, quick and accurate tool to diagnose each abnormality satisfactorily. This anomaly has clinical, physiological and surgical implications due to presence of altered flow pattern and variable anatomy. Therefore, such malformations need a careful assessment, preferably by non-invasive diagnostic tools, and appropriate management.

Pulmonary atresia, VSD in association with coronary-pulmonary artery fistula.

Congenital coronary-pulmonary artery fistula is rare in patients with pulmonary atresia and ventricular septal defect. The nomenclature, physiological, clinical, and surgical implications of these fistulas are yet to be defined. We report a one-year-old child with pulmonary atresia, ventricular septal defect, and a right coronary-pulmonary artery fistula who also had a diminutive, disconnected left pulmonary artery in addition to aortopulmonary collaterals. The patient underwent corrective surgery. However, the fate of diminutive pulmonary arteries is unknown. The literature was reviewed to explore the clinical or surgical implications of such fistulas for improved understanding and management in the future.

Atresia of coronary sinus ostium with retrograde drainage via persistent left superior vena cava.

Atesia of the coronary sinus ostium (ACSO) with retrograde drainage of cardiac veins via the left superior vena cava (LSVC) is a very rare abnormality. This condition is usually asymptomatic during life and a majority of the cases were reported as incidental postmortem findings. If there is retrograde venous drainage via persistent LSVC, this communication cannot be ligated irrespective of its size or the presence of a communicating vein because of resultant cardiac congestion and edema. We report herein a 9-month-old Chinese female who underwent repair of a perimembranous ventricular septal defect, patent ductus arteriosus and secundum atrial septal defect. During the operation, ligation of LSVC resulted in myocardial congestion and distension of the heart. The release of ligature decompressed the heart immediately.