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

Predictors of unfavourable early outcome following Fontan completion

Background Although the magnificent improvement in Fontan operation results in the last two decades, there are still some concerns regarding the unfavourable early outcomes that may follow Fontan completion. Methods From 2003 to 2016, 79 Patients underwent Fontan operation at IRCCS Policlinico San Donato. Unfavourable early outcome was defined by the presence of one or more of these occurrences: prolonged hospital stay >25 days, Prolonged pleural effusion 6514 days and Prolonged inotropic support 6572\u202fh. Univariable and multivariable analyses were performed to detect the risk factors associated with early unfavourable outcome after Fontan completion. Results Prolonged hospital stay >25 days was found in 24.05% of patients and its associated significant risk factors were low preoperative O2 saturation (p 0.007), Fontan fenestration (p 0.009) and plasma transfusion (p 0.030). Prolonged pleural effusion 6514 days was found in 24.05% and no significant risk factors were detected. Prolonged inotropic support 6572\u202fh was found in 35.44% and significant risk factors were prolonged cardiopulmonary bypass time (P 0.003), fenestration (P 0.023), plasma transfusion (P 0.028) and non staged Fontan (P 0.039). In multivariable analysis of combined unfavourable outcome, significant risk factors were fenestration (P 0.030) with some trends towards low preoperative O2 saturation (P 0.056). Conclusion Unfavourable early outcome can occur following Fontan completion with associated prolonged hospital stay. Risk factors include low preoperative O2 saturation, prolonged cardiopulmonary bypass time, Fontan fenestration, Plasma transfusion and non staged Fontan

Potential therapeutic effects of natural heme oxygenase-1 inducers in cardiovascular diseases.

Significance: Many physiological effects of natural antioxidants, their extracts or their major active components, have been reported in recent decades. Most of these compounds are characterized by a phenolic structure, similar to that of α-tocopherol, and present antioxidant properties that have been demonstrated both in vitro and in vivo. Polyphenols may increase the capacity of endogenous antioxidant defenses and modulate the cellular redox state. Such effects may have wide-ranging consequences for cellular growth and differentiation. Critical Issues: The majority of in vitro and in vivo studies conducted so far have attributed the protective effect of bioactive polyphenols to their chemical reactivity toward free radicals and their capacity to prevent the oxidation of important intracellular components. One possible protective molecular mechanism of polyphenols is nuclear factor erythroid 2-related factor (Nrf2) activation, which in turn regulates a number of detoxification enzymes. Recent Advances: Among the latter, the heme oxygenase-1 (HO-1) pathway is likely to contribute to the established and powerful antioxidant/anti-inflammatory properties of polyphenols. In this context, it is interesting to note that induction of HO-1 expression by means of natural compounds contributes to prevention of cardiovascular diseases in various experimental models. Future Directions: The focus of this review is on the role of natural HO-1 inducers as a potential therapeutic strategy to protect the cardiovascular system against various stressors in several pathological conditions

Lifetime costs and outcomes of repair of tetralogy of fallot compared to natural progression of the disease: great ormond street hospital cohort.

Tetralogy of Fallot is a congenital heart disease that requires surgical repair without which survival through childhood is extremely rare. The aim of this paper is to use data from the mandatory follow-up of patients with Tetralogy of Fallot to model the health-related costs and outcomes over the first 55-years of life

Right Ventricle Has Normal Myofilament Function But Shows Perturbations in the Expression of Extracellular Matrix Genes in Patients With Tetralogy of Fallot Undergoing Pulmonary Valve Replacement

BACKGROUND: Patients with repair of tetralogy of Fallot (rToF) who are approaching adulthood often exhibit pulmonary valve regurgitation, leading to right ventricle (RV) dilatation and dysfunction. The regurgitation can be corrected by pulmonary valve replacement (PVR), but the optimal surgical timing remains under debate, mainly because of the poorly understood nature of RV remodeling in patients with rToF. The goal of this study was to probe for pathologic molecular, cellular, and tissue changes in the myocardium of patients with rToF at the time of PVR. METHODS AND RESULTS: We measured contractile function of permeabilized myocytes, collagen content of tissue samples, and the expression of mRNA and selected proteins in RV tissue samples from patients with rToF undergoing PVR for severe pulmonary valve regurgitation. The data were compared with nondiseased RV tissue from unused donor hearts. Contractile performance and passive stiffness of the myofilaments in permeabilized myocytes were similar in rToF‐PVR and RV donor samples, as was collagen content and cross‐linking. The patients with rToF undergoing PVR had enhanced mRNA expression of genes associated with connective tissue diseases and tissue remodeling, including the small leucine‐rich proteoglycans ASPN (asporin), LUM (lumican), and OGN (osteoglycin), although their protein levels were not significantly increased. CONCLUSIONS: RV myofilaments from patients with rToF undergoing PVR showed no functional impairment, but the changes in extracellular matrix gene expression may indicate the early stages of remodeling. Our study found no evidence of major damage at the cellular and tissue levels in the RV of patients with rToF who underwent PVR according to current clinical criteria

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

Variations in right ventricular outflow tract morphology following repair of congenital heart disease: Implications for percutaneous pulmonary valve implantation

Objective: Our aim was to identify sub-groups of right ventricular outflow tract morphology that would be suitable for percutaneous pulmonary valve implantation and to document their prevalence in our patient population. Materials and Methods: Eighty-three consecutive patients with right ventricular outflow tract dysfunction (5-41 years, 76% tetralogy of Fallot) referred to our center for cardiovascular magnetic resonance were studied. A morphological classification was created according to visual assessment of three-dimensional reconstructions and detailed measurement. Diagnosis, right ventricular outflow tract type, surgical history and treatment outcomes were documented. Results: Right ventricular outflow tract morphology was heterogeneous; nevertheless, 5 patterns were visually identified. Type I, a pyramidal morphology, was most prevalent (49%) and related to the presence of a transannular patch. Other types (II-V) were seen more commonly in patients with conduits. Two patients had unclassifiable morphology. Ninety-five percent of patients were assigned to the correct morphological classification by visual assessment alone. Percutaneous pulmonary valve implantation was performed successfully in 10 patients with Type II-V morphology and in 1 patient with unclassifiable morphology. Percutaneous implantation was not performed in patients with Type I morphology. Only right ventricular outflow tract diameters 50% of outflow tract morphologies may be suitable for this approach, in particular with the development of new devices appropriate for larger outflow

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