Extrathoracic heart in northern Cameroon: a case report

Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. The prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity. Ectopia cordis is frequently associated with other congenital defects involving multiple organ systems. We report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon

Extrathoracic heart in northern Cameroon: a case report

Tantchou et al report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon. Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. The prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity. Ectopia cordis is frequently associated with other congenital defects involving multiple organ systems. We report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon

Occurrence, aetiology and challenges in the management of congestive heart failure in sub-Saharan Africa: experience of the Cardiac Centre in Shisong, Cameroon

Introduction: The aim of the study was to investigate the occurrence, the aetiology and the management of congestive heart failure in the cardiac centre of the St. Elizabeth catholic general hospital Shisong in Cameroon. Methods: Between November 2002 and November 2008, a population of 8121 patients was consulted in the referral cardiac centre of St. Elizabeth Catholic General Hospital. Of these patients, 462 were diagnosed with congestive heart failure according to the modified Framingham criteria for the diagnosis of heart failure. Complementary investigations used to confirm and establish the aetiology of the disease were the chest X-ray, electrocardiography, bi-dimensional Doppler echocardiography. Results: The results showed that the occurrence of congestive heart failure in our centre was 5.7%. Congestive heart failure was diagnosed in 198 females and 264 males, aged between 8 and 86 years old (42.5±18 years old). Post rheumatic valvulopathies (14.6%) and congenital heart diseases (1.9%) were the first aetiologic factor of congestive heart failure in the young, meanwhile cardiomyopathies (8.3%) in elderly followed by hypertensive cardiomyopathy (4.4%). Congestive heart failure was also seen in adults with congenital heart diseases in 0.01%. In this zone of Cameroon, we discovered that HIV cardiomyopathy (1.6%) and Cor pulmonale (8%) were represented, aetiological factors not mentioned in previous studies conducted in urban areas of Cameroon. The mean duration of hospital stay for the compensation treatment was thirteen days, ranging between 7 and 21 days), the mortality being 9.2%. All the medications recommended for the treatment of congestive heart failure are available in our centre but many patients are not compliant to the therapy or cannot afford them. Financial limitation is causing the exacerbation of the disease and premature death. Conclusion: Our data show a high incidence of congestive heart failure mainly due to post rheumatic valvulopathies in young patients in our centre. National program to fight against rheumatic fever and complications are of great urgency in our country. The compensation treatment of congestive heart failure is challenging in our milieu, characterized by poor compliance and financial limitations.Key words: Congestive heart failure, cardiac centre Shisong, valvulopathies, cardiomyopathy, Cameroon, Hypertension, heart failur

The Shisong Cardiac Center in Cameroon: An Example of a Long-Term Collaboration/Cooperation Toward Autonomy

Congenital heart diseases (CHD) are present in nearly 1% of live births; according to WHO, there are 1. 5 million newborns affected by CHD per year and more than 4 million children waiting for cardiac surgery treatment worldwide. The majority of these children (~90%) could be treated, saved and subsequently have a good quality of life but unfortunately, in developing countries with a suboptimal care or no access to care, they are destined to die. Cameroon, one of the 40 poorest countries in the world, is a typical example of this dramatic scenario and this is why we started a collaboration project with a local religious partner (Tertiary Sisters of Saint Francis) in 2001 with the aim of establishing the first cardiac surgery center in this country. There are various well-known organizational models to start a cooperation project in pediatric cardiac surgery in a developing country. In our case, the project included a long-term collaboration with a stable local partner, a big financial investment and a long period of development (10 years or more). It is probably the most difficult model but it is the only one with the greatest guarantee of success in terms of sustainability and autonomy. The aim of this study is to analyze the constructive and problematic aspects of the 17-year collaboration in this project, and to assess possible solutions regarding its critical issues. Although much has been done during this 17-year we are aware that there is still a lot that needs to be done

Transcatheter closure of atrial septal defect in young children Results and follow-up

AbstractObjectivesThis study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure of secundum atrial septal defect (ASD) in young children.BackgroundResults of ASD transcatheter closure in adults are widely reported but there are no large published series concerning young children.MethodsBetween December 1996 and February 2002, 48 of 553 patients percutaneously treated at our institution were children age ≤5 years. Indications for closure were: elective closure in 32 patients; frequent respiratory infections in 8; failure to thrive in 2; liver transplantation in 5; and a fenestrated Fontan in 1. The procedure was carried out under general anesthesia with fluoroscopy and transesophageal control. Two different devices were used: 1) the CardioSEAL/StarFLEX (CS/SF) and 2) the Amplatzer septal occluder (ASO). Basal physical examinations and echocardiograms were performed prior to the procedure and at follow-ups (1, 6, and 12 months, and yearly thereafter).ResultsThe mean age at closure was 3.6 ± 1.3 years. A CS/SF was used in 10 subjects; an ASO was used in 38 patients. No deaths or immediate major complications occurred. The total occlusion rate was 87% at procedure, rising to 94% at discharge. The mean follow-up was 18 ± 14 months. No midterm major or minor complications occurred. The occlusion rate rose to 100% at 12 months of follow-up. Symptomatic patients improved significantly.ConclusionsIn the current era and in experienced hands, ASD closure can be performed safely and successfully, even in very young children

3D printing is a transformative technology in congenital heart disease

Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease. Three-dimensional printing is an emerging technology that is fundamentally affecting patient care, research, trainee education, and interactions among medical teams, patients, and caregivers. This paper first reviews key clinical cases where the technology has affected patient care. It then discusses 3-dimensional printing in trainee education. Thereafter, the role of this technology in communication with multidisciplinary teams, patients, and caregivers is described. Finally, the paper reviews translational technologies on the horizon that promise to take this nascent field even further

NEU3 sialidase role in activating HIF-1α in response to chronic hypoxia in cyanotic congenital heart patients

Background Hypoxia is a common feature of many congenital heart defects (CHDs) and significantly contributes to their pathophysiology. Thus, understanding the mechanism underlying cell response to hypoxia is vital for the development of novel therapeutic strategies. Certainly, the hypoxia inducible factor (HIF) has been extensively investigated and it is now recognized as the master regulator of cell defense machinery counteracting hypoxic stress. Along this line, we recently discovered and reported a novel mechanism of HIF activation, which is mediated by sialidase NEU3. Thus, aim of this study was to test whether NEU3 played any role in the cardiac cell response to chronic hypoxia in congenital cyanotic patients. Methods Right atrial appendage biopsies were obtained from pediatric patients with cyanotic/non-cyanotic CHDs and processed to obtain mRNA and proteins. Real-Time PCR and Western Blot were performed to analyze HIF-1\uce\ub1 and its downstream targets expression, NEU3 expression, and the NEU3 mediated effects on the EGFR signaling cascade. Results Cyanotic patients showed increased levels of HIF-1\uce\ub1, NEU3, EGFR and their downstream targets, as compared to acyanotic controls. The same patients were also characterized by increased phosphorylation of the EGFR signaling cascade proteins. Moreover, we found that HIF-1\uce\ub1 expression levels positively correlated with those recorded for NEU3 in both cyanotic and control patients. Conclusions Sialidase NEU3 plays a central role in activating cell response to chronic hypoxia inducing the up-regulation of HIF-1\uce\ub1, and this represent a possible novel tool to treat several CHD pathologies