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

Controlling medicine prices in Sudan: the challenge of the recently established Medicines Regulatory Authority

This study evaluated the capacity of the medicine pricing mechanism of the National Medicines and Poisons Board [NMPB], the medicine regulatory agency of Sudan, in controlling prices of imported medicines. The study was conducted between July and September 2008 and data on the 50 most sold medicines in 2007 were obtained from the records of the regulatory authority and pharmacies in Khartoum; 135 products were included. The cost and freight [CandF] prices of 23% of the selected medicines approved by NMPB were over 10 times the International Reference Price; 71% of these items were generics. The wholesale price of only 40% of the studied medicines was less than that approved by NMPB, while the retail price of 47% of medicines was less than that approved by the authority. The retail price of 11 of 12 originator medicines was equal to or more than their prices published in the British National Formulary. The price of the medicines distributed by Central Medical Supplies was about 2-fold their C and F price. The current pricing system is of limited benefit in controlling medicine prices in Sudan

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

Antimalarial prescribing and dispensing practices in health centres of Khartoum state, 2003-04

This indicator-based study assessed the antimalarial drug prescribing and dispensing practices of health care providers in health centres of Khartoum state, and compared these with national guidelines for malaria treatment. A total of 720 patients and their prescriptions in 24 health centres were included. Prescribers adhered to national treatment guidelines for only 278 [38.6%] of patients. Although all were treated for malaria, only 77.6% patients had fever or history of fever and only 64.6% had fever and positive blood films. More than 90% of prescriptions prescribed antimalarial drugs by generic names but dosage forms were correctly written in only 23.5%. There was a high rate of prescribing antimalarial injections. Only half the patients had adequate knowledge of their treatment

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

Hospital Maternal Mortality. Analysis of cause and contributing factors

This is a retrospective hospital based study of 72 maternal deaths reported at Kassala New Hospital [Eastern Sudan] in the period from 1/1/1997 to 31/12/1998. The objective of this study is to analyze and determine the cause and contributing factors of these deaths. 41.7% of these were due to direct obstetrical causes. The hospital maternal mortality ratio was found to be 2351 per 100,000 live birth. Complicated malaria [30.2%] and severe anaemia [20.8%] were the leading causes. Puerperal sepsis [11.1%]was the leading cause of direct obstetrical deaths followed by haemorrhage [9.7%] and obstructed labour [8.3%]. Delay in seeking care was found in 87.5% of cases, failure to refer cases by health visitors, midwives and doctors in 64.7% and delay in receiving optimum care in index hospital in 11.1%