Pregnancy in non-palliated functionally single ventricle: challenges of management in resource-poor settings.

Women with complex functionally univentricular hearts rarely survive into adulthood without corrective or palliative surgery. Reports of pregnancy outcome in this group of patients in resource-poor settings are sparse. We report a case of unrepaired pulmonary atresia ventricular septal defect (VSD) with major aorto-pulmonary collateral arteries (MAPCA) who survived into adulthood and was able to complete a successful pregnancy in a resource-poor country.

Conotruncal Heart Defect Repair in Sub-Saharan Africa: Remarkable Outcomes Despite Poor Access to Treatment.

BACKGROUND: The outcome of children born with conotruncal heart defects may serve as an indication of the status of pediatric cardiac care in sub-Saharan Africa (SSA). This study was undertaken to determine the outcome of children born with conotruncal anomalies in SSA, regarding access to treatment and outcomes of surgical intervention. METHODS: From our institution in Ghana, we retrospectively analyzed the outcomes of surgery, in the two-year period from June 2013 to May 2015. The birth prevalence of congenital heart defects (CHDs) in SSA countries was derived by extrapolation using an incidence of 8 per 1,000 live births for CHDs. RESULTS: The birth prevalence of CHDs for the 48 countries in SSA using 2013 country data was 258,875; 10% of these are presumed to be conotruncal anomalies. Six countries (Nigeria, Democratic Republic of the Congo, Ethiopia, Tanzania, Uganda, and Kenya) accounted for 53.5% of the birth prevalence. In Ghana, 20 patients (tetralogy of Fallot [TOF], 17; pulmonary atresia, 3) underwent palliation and 50 (TOF, 36; double-outlet right ventricle, 14) underwent repair. Hospital mortality was 0% for palliation and 4% for repair. Only 6 (0.5%) of the expected 1,234 cases of conotruncal defects underwent palliation or repair within two years of birth. CONCLUSION: Six countries in SSA account for more than 50% of the CHD burden. Access to treatment within two years of birth is probably <1%. The experience from Ghana demonstrates that remarkable surgical outcomes are achievable in low- to middle-income countries of SSA.

Hypothermic cardiopulmonary bypass without exchange transfusion in sickle-cell patients: a matched-pair analysis.

OBJECTIVES: Sickle-cell patients undergo cardiopulmonary bypass (CPB) surgery in our institution without perioperative exchange transfusion. We sought to determine whether this protocol increased mortality or important sickle-cell-related complications. METHODS: We adopted a 1:1 matched-pair case-control methodology to evaluate the safety of our protocol. Sickle-cell patients who underwent CPB between January 1995 and January 2014 were matched with haemoglobin AA (HbAA) controls according to sex, age, weight and type of cardiac procedure. RESULTS: Thirty-three sickle-cell patients (21 HbAS, 7 HbSS and 5 HbSC) underwent CPB surgery using our institutional protocol. Sickle-cell patients and controls were similar according to the matching criteria. Preoperatively, haemoglobin SS (HbSS) and haemoglobin SC (HbSC) patients were anaemic (8.5 ± 1.4 vs 13.5 ± 1.9 g/dl; P <0.01 and 11.0 ± 0.6 vs 12.7 ± 0.9 g/dl; P = 0.01, respectively). Operative procedures included valve repair and replacement (12) as well as repair of congenital cardiac malformations (21). The duration of CPB and lowest CPB temperatures was similar for sickle-cell patients and controls. Systemic hypothermia (23.8-33.5°C), aortic cross-clamping, cold crystalloid antegrade cardioplegia and topical hypothermia were used in sickle-cell patients without complications. There was no acidosis, hypoxia or low cardiac output state. No mortality or important sickle-cell-related complications occurred. Although blood loss was similar between sickle-cell patients and controls, HbSS (unlike HbAS and HbSC) patients required more blood transfusion than controls (30.0 ± 13.3 vs 10.8 ± 14.2 ml/kg; P = 0.02) to counter haemodilution and replace blood loss. In-patient stay was similar for sickle-cell patients and controls. CONCLUSIONS: Perioperative exchange transfusion is not essential for a good outcome in sickle-cell patients undergoing CPB. A simple transfusion regimen to replace blood loss is safe in HbSS patients; blood transfusion requirements for HbSC and HbAS patients undergoing CPB are similar to those of matched HbAA controls. The use of systemic hypothermia during CPB does not increase sickle-cell-related complications. Cold crystalloid cardioplegia and topical hypothermia provide safe myocardial protection without the need for more sophisticated measures.

The changing epidemiology of esophageal cancer in sub-Saharan Africa - the case of Ghana.

INTRODUCTION: Esophageal cancer portends a grim prognosis. Most patients present with incurable disease. Scanty epidemiologic data on the disease has contributed to its low priority on the national. We sought to evaluate the current national trend in the presentation and outcome of esophageal cancer using our institutional experience from 1992 - 2010. METHODS: This is a retrospective study based on 152 patients who were seen in our institution during the study period. The perioperative data of these patients were retrieved and the relevant details recorded. Histopathological reports were available for 75 patients managed over the period. The study setting was The National Cardiothoracic Centre, which serves as the only tertiary referral centre in the country for cardiothoracic problems. RESULTS: There were 122 males and 30 females with a mean age of 57.8 ± 11.7 years. The yearly trend from 1992 to 2010 showed a steady increase in the incidence of esophageal cancer. High alcohol consumption and smoking dominated the history of 82.2% of the patients. Squamous cell carcinoma accounted for 78.7% and adenocarcinoma 21.3%. Distribution of esophageal carcinoma by anatomical location was 84.9% for distal third, 11.8% for middle third and 3.3% for upper third. All patients presented with incurable disease. CONCLUSION: The study shows an increasing incidence of esophageal carcinoma in this country. Alcohol abuse and smoking are major risk factors; squamous cell carcinoma is the dominant histological type in this study.

Colopharyngoplasty for intractable caustic pharyngoesophageal strictures in an indigenous African community--adverse impact of concomitant tracheostomy on outcome.

OBJECTIVES: Surgical management of caustic strictures of the upper digestive tract poses difficult challenges. This is because reconstruction above the cricopharyngeal junction interferes with the mechanisms of swallowing and respiration. This report reviews the outcome of colopharyngeal reconstruction of severe diffuse pharyngoesophageal caustic strictures in an indigenous African community. METHOD: The medical records of patients who underwent colopharyngoplasty from January 2006 to December 2008 were retrospectively reviewed to obtain information on patients' demographics, surgical technique and outcome. RESULTS: In the study period, 20 patients underwent reconstruction for caustic esophageal strictures; in five (three males, two females) colopharyngoplasty was required. Their ages ranged from four to 56 years (mean 25 years). Follow-up ranged from 23 to 94 months (mean 33 months). Colopharyngoplasty using left colon tunneled retrosternally was performed in all patients. Rehabilitative training for deglutition was required for 0.5-5.0 months postoperatively to restore near-normal swallowing in all patients. However, tracheostomy complications caused two deaths (one early, one late) and varicella encephalitis caused another late death. CONCLUSION: In this African community, colopharyngoplasty provided an effective mean of restoration of upper digestive tract continuity in patients with severe caustic pharyngoesophageal strictures. Tracheostomy in this setting portends a significant long-term mortality risk.