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

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

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

Circulating S100B and Adiponectin in Children Who Underwent Open Heart Surgery and Cardiopulmonary Bypass

Background. S100B protein, previously proposed as a consolidated marker of brain damage in congenital heart disease (CHD) newborns who underwent cardiac surgery and cardiopulmonary bypass (CPB), has been progressively abandoned due to S100B CNS extra-source such as adipose tissue. The present study investigated CHD newborns, if adipose tissue contributes significantly to S100B serum levels. Methods. We conducted a prospective study in 26 CHD infants, without preexisting neurological disorders, who underwent cardiac surgery and CPB in whom blood samples for S100B and adiponectin (ADN) measurement were drawn at five perioperative time-points. Results. S100B showed a significant increase from hospital admission up to 24 h after procedure reaching its maximum peak (P0.05) have been found all along perioperative monitoring. ADN/S100B ratio pattern was identical to S100B alone with the higher peak at the end of CPB and remained higher up to 24 h from surgery. Conclusions. The present study provides evidence that, in CHD infants, S100B protein is not affected by an extra-source adipose tissue release as suggested by no changes in circulating ADN concentrations

The first coronary by-pass grafting surgery done in western and central Africa

Africa bears a significant proportion of the global burden of chronic diseases, along with poor countries of Asia and Latin America. The World Health Organisation projects that over the next ten years the Continent will experience the largest increase in death rates from cardiovascular disease, cancer, respiratory disease and diabetes. Probably for the first time in Western and Central Africa, the very first coronary artery bypass surgery grafting was performed in the Cardiac Centre of Shisong in Cameroon.Key words: Coronary artery bypass grafting, cardiac centre, Cameroo

Life experiences and coping strategies in adults with congenital heart disease

Many adults with congenital heart disease (ACHD) have to face considerable psychosocial difficulties. The aim of this study was to explore the life experiences of ACHD patients, from when they become aware of having a condition, till after the open heart surgery they underwent. The study was conducted with the use of unstructured, in-depth interviews, performed on 11 patients (age ranging: 20 - 56 y) after they recovered from open heart surgery and a focus group, which included 16 participants (age ranging: 22 - 46 y). Both the interviews and the focus group were recorded, transcribed and analyzed according to Grounded Theory procedures. Our findings show that the condition of diversity is the core of the emotional experiences connected to ACHD. Feeling different and being perceived as being different are clearly interlinked and coping strategies adopted resulted as being influenced by this perception. This study also clearly outlines the importance of having an adequate perception of one's condition and the link between maladaptive coping strategies and an incorrect perception of one's heart condition. Results are discussed in order to promote psychosocial interventions within and outside of the hospital setting in order to improve the patients' emotional wellbeing

Functional tricuspid valve regurgitation in adults with congenital heart disease: an emerging problem.

At present, limited data are available regarding functional tricuspid regurgitation (TR) in adults with congenital heart disease (ACHD). Functional TR is the consequence of right ventricular (RV) dilation or dysfunction that, in these patients, is typically associated with chronic RV volume overloading. The study aim was to resolve this problem by evaluating results obtained from patients after surgical treatment of ACHD. METHODS: A total of 65 patients (mean age 46 years) with ACHD underwent an operation to correct functional TR during elective cardiac surgery between January 2000 and December 2008 at the authors' institution. The preoperative median NYHA functional class was 3, and the median TR grade was 3. Surgical treatment of the primary cardiac lesion included atrial septal defect (ASD) closure in 40 patients and pulmonary valve implantation in 25. Functional TR was treated by annuloplasty (n = 48), rigid tricuspid valve ring (n = 14), or tricuspid valve replacement (n = 3). RESULTS: There were no in-hospital deaths. The median TR grade at discharge was 0. During a mean follow up period of 63 months (range: 12-96 months) there was one delayed death that was not cardiac-related. Both, the NYHA functional class (p = 0.001) and TR grade (p = 0.001) were significantly improved among survivors. One patient (1.5%) had tricuspid valve replacement at five years after annuloplasty. No significant differences regarding annuloplasty versus rigid tricuspid valve ring were noted during the follow up period. CONCLUSION: Functional TR is an emerging, though as yet still underestimated, problem in ACHD. It is a consequence of RV dilation/dysfunction that, in these patients, is typically related to chronic RV volume overloading. Surgery should be considered to resolve this problem, and can be performed at low risk and with good mid-term results