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

Regression of Cardiac Rhabdomyomas Producing a Severe Aortic Stenosis: Case Report and Discussion of the Literature

We describe a case of a voluminous rhabdomyoma (R) detected by fetal echocardiography at 32 weeks’ gestation (w.g.) obstructing the left ventricular inflow and aortic outflow tract, with a moderate aortic gradient at birth, not needing immediate surgery. At follow-up, the mass progressively regressed, leaving the aortic valve partly damaged, with a gradient that increased to a maximum of 100 mmHg at 9 years. The girl was then operated on successfully by a plasty of the aortic valve. The literature regarding R is discussed

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

Datasheet2_Cervical aortic arch in the pediatric population: a meta-analysis of individual patient's data.docx

BackgroundThis is the first meta-analysis to analyze all reports of published pediatric cases of cervical aortic arch (CAA) by highlighting the clinical characteristics and treatment outcomes using the reported individual data of the patients. The aim of the study is to investigate the clinical features and surgical outcomes of such a rare disease in the pediatric population.MethodsA comprehensive search was conducted in various academic databases, including PubMed, ScienceDirect, SciELO, DOAJ, and Cochrane Library, until June 2022 for case reports describing the presence of cervical aortic arch in the pediatric age. Case reports and series were included if the following criteria were met: (1) description of the cervical aortic arch; (2) patient of pediatric age; and (3) published in the English language. All other types of publications that lacked patient-specific information were excluded from the analysis. This systematic review was conducted in accordance with the PRISMA guidelines. The primary outcome measure of the analysis was early and late mortality.ResultsThe literature search identified 2,272 potentially eligible articles, 72 of which met our inclusion criteria with 96 patients including the author's institutional case. At a median of 365 (90–730) days, the overall cohort registered a 7.3% (7/96) mortality rate. In the subset of patients who underwent surgery, the mortality rate was also 7.3% (4/55), and the mortality rate following surgery to treat only CAA was 2.4% (1/42). Dyspnea was identified as an independent determinant of mortality by employing the univariable Firth bias-reduced logistic regression method.ConclusionCervical aortic arch is a rare congenital heart disease that poses treatment challenges due to the high anatomical variability, diverse clinical presentations, and the presence of other concomitant diseases. The surgical treatment appears to be a safe and effective approach for resolving the symptoms, although it needs to be tailored individually for each patient.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.</p

Preliminary Results of Cryoablation for Surgical Treatment of Arrhythmias in Adults With Congenital Heart Disease

Background: Arrhythmias in adult congenital heart disease (ACHD) are responsible for the majority of hospital admissions and 20-25% of late deaths. Since need for further cardiac operations is frequent in ACHD, concomitant arrhythmia surgery represents a strategic treatment modality.Material and Methods: A two-center retrospective study was undertaken on cryoablation of supraventricular arrhythmias in 25 conescutive ACHD patients (16/9, M/F, median age 38.5 years, IQR 38-60) operated between 01/2017 and 12/2020. Nineteen (76%) had undergone one or more previous cardiac operations and 8 (32%) one or more trans-catheter ablations. Indications included Fontan conversion in seven patients, septal defect repair in 6, pulmonary valve replacement in 10 and tricuspid surgery in 2. Open-heart cryoablation included: 4 cavotricuspid isthmus ablations, 19 right-sided Maze for atrial tachycardia/flutter, and 2 Cox-Maze III for atrial fibrillation.Results: There were 2 (8%) hospital deaths, unrelated to cryoablation, due to low cardiac output syndrome. There were no intraoperative complications related to cryoablation. Seven (28%) patients required pace-maker implantation due to post-operative atrioventricular block. All patients were discharged on oral antiarrhythmic and anticoagulantion for 6 months. After a median follow-up of 14 months (IQR 7-27) no late mortality was observed. At follow-up, 16/23 (69%) patients are in stable sinus rhythm, 12 without anti-arrhythmic therapy. Two (8.6%) patients had relapse of arrhythmia. Freedom from arrhythmia was 90.9% and cumulative risk of recurrence was 9.6%.Conclusions: Intraoperative cryoablation is safe and effective procedure. Surgical treatment of arrhythmias should always be considered in ACHD, whenever further open-heart repair is needed

Datasheet1_Cervical aortic arch in the pediatric population: a meta-analysis of individual patient's data.docx

BackgroundThis is the first meta-analysis to analyze all reports of published pediatric cases of cervical aortic arch (CAA) by highlighting the clinical characteristics and treatment outcomes using the reported individual data of the patients. The aim of the study is to investigate the clinical features and surgical outcomes of such a rare disease in the pediatric population.MethodsA comprehensive search was conducted in various academic databases, including PubMed, ScienceDirect, SciELO, DOAJ, and Cochrane Library, until June 2022 for case reports describing the presence of cervical aortic arch in the pediatric age. Case reports and series were included if the following criteria were met: (1) description of the cervical aortic arch; (2) patient of pediatric age; and (3) published in the English language. All other types of publications that lacked patient-specific information were excluded from the analysis. This systematic review was conducted in accordance with the PRISMA guidelines. The primary outcome measure of the analysis was early and late mortality.ResultsThe literature search identified 2,272 potentially eligible articles, 72 of which met our inclusion criteria with 96 patients including the author's institutional case. At a median of 365 (90–730) days, the overall cohort registered a 7.3% (7/96) mortality rate. In the subset of patients who underwent surgery, the mortality rate was also 7.3% (4/55), and the mortality rate following surgery to treat only CAA was 2.4% (1/42). Dyspnea was identified as an independent determinant of mortality by employing the univariable Firth bias-reduced logistic regression method.ConclusionCervical aortic arch is a rare congenital heart disease that poses treatment challenges due to the high anatomical variability, diverse clinical presentations, and the presence of other concomitant diseases. The surgical treatment appears to be a safe and effective approach for resolving the symptoms, although it needs to be tailored individually for each patient.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.</p