Noncompaction cardiomyopathy: a new mechanism for mitral regurgitation with distinct clinical, echocardiographic features and pathological correlations

Noncompaction cardiomyopathy [NCCM] is a primary, genetic cardiomyopathy with variable clinical manifestations that include mitral regurgitation [MR]. This study comprised patients diagnosed with NCCM and MR in two cardiac centers [King Abdul-Aziz Cardiac Center, Riyadh, Kingdom of Saudi Arabia and Sudan Heart Institute, Khartoum, Sudan], and seen in the period between 2002 and 2013. The study describes follow up, clinical, echocardiographic, and histopathological findings. Nineteen cases [85% females] were identified. Ten percent of the cases had relapses and remissions of heart failure. Echocardiographic features included leaflet retraction in all patients, characteristic malcoaptation, and a zigzag deformity of anterior leaflet in 57% of patients. Ruptured chordae were found in 15% of the patients. One patient had pathological examination of the mitral valve which showed myxomatous degeneration, and sclerotic and calcific changes. We describe and discuss a new mechanism for MR caused by NCCM with identifiable clinical and echocardiographic features, and pathological correlations

Cardiac involvement in Sudanese children with connective tissue disease

Connective tissue diseases [CTD] are associated with a variable range of cardiac abnormalities. We identified all patients with CTD and cardiac involvement seen at Jafar Ibn Ouf Children's Hospital and the Sudan Heart Centre by one cardiologist between Jan 2005 and January 2010. Clinical and echocardiographic evaluations were done and arranged consultation with rheumatologist. Nine patients were identified, three males and six females. Five patients [62%] were initially seen by the cardiologist and CTD was suspected by clinical examination in four patients and in one patient diagnosed by routine screening during workup for pulmonary hypertension. Symptoms were present for one month to 3 years before presentation to the cardiologist. In three patients [30%] the cardiac symptoms preceded the rheumatological symptoms. In one patient, aortic valve replacement for presumed rheumatic aortic regurgitation preceded arthritis by 12 months. Patients with CTD may present first to the cardiologist with findings that mimic common cardiac lesions. All patients with CTD should have routine evaluation by the cardiologist so as to implement optimal early interventions

Diagnosis and management of adults with congenital heart disease: the experience of the Sudan Heart Centre

Adults with congenital heart disease [ACHD] constitute an increasing burden on cardiology service worldwide. All patients with congenital heart disease [CHD] 18 years of age or more seen at the Sudan Heart Centre by a paediatric cardiologist from July 2004-April 2009 were included in this retrospective review. Their clinical and echocardiographic features as well as their outcome were recorded. In the study period 2680 patients with CHD were evaluated at the clinic. One hundred and thirty patients with ACHD [4.8% of patients with CHD] were identified. The age ranged between 18-65 years. Males were 69 [53. 1%] and females 61 [46.9%]. The most common cardiac defects were Tetralogy of Fallot and atrial septal defect [23% each] followed by ventricular septal defect [15%]. 15% of those with large shunts were found to have Eisenmengers' syndrome. Our final diagnosis matched the referral diagnosis in 30% of patients. In 30% the final diagnosis was different and in the other 40% the referral diagnosis was partially matching the final diagnosis. Co-existing conditions included systemic hypertension, diabetes mellitus, cerebrovascular accidents, endocarditis and pregnancy. Surgical/catheter interventions were done in 46 patients [35%]. Catheter treatment included patent ductus arteriosus, atrial and ventricular septal defect closure as well as pulmonary valve balloon dilatation with acceptable results. ACHD is a growing specialty and, early diagnosis and treatment is necessary to prevent irreversible complications. Training of more staff in diagnostic and interventional technologies is warranted

Paediatric cardiology programs in countries with limited resources: how to bridge the gap

Establishing paediatric cardiology service in a country with limited resources like Sudan is a challenging task. A paediatric cardiac team was formed then the services in different disciplines were gradually established. Echocardiography [echo] clinics were founded in tertiary and peripheral hospitals. Cardiac catheterization [cath] was established at the Sudan Heart Centre [SHC] in 2004 and over 400 procedures had been performed including interventional catheterization like pulmonary valve dilatation, patent ductus arteriosus and atrial septal defect device closure. Congenital heart surgery started in 2001, currently 200 cases are done each year including closed procedures as well as open heart procedures for patients weighing more than 8 kg. Cardiology cardiac surgery as well as adult congenital heart disease meetings were held and contributed positively to the services. The cardiology-cardiac surgery scientific club meeting was founded as a forum for academic discussions. A fellowship program was established in 2004 and included seven candidates trained in paediatric cardiology and intensive care. Two training courses had been established: congenital heart disease echo and paediatric electrocardiogram interpretation. Links with regional and international cardiac centres had important roles in consolidating our program

Trans-catheter closure of large secondum atrial septal defects in patients with Ebstein's malformation of the tricuspid valve: a report of two cases

Atrial septal defects are common in patients with Ebstein malformation of the tricuspid valve and may contribute to their hemodynamic disturbances. We report our experience of trans-catheter closure of large atrial septal defects in two patients with Ebstein malformation using Amplatzer in one and Helex septal occluder in second patient with no residual shunts. There was no immediate complication, and 18 months follow up revealed clinically stable patients with normal sinus rhythm and no residual shunt. We can say that trans-catheter closure of large atrial septal defects in Ebstein malformation is feasible with a good short-term out come