Frank Edwin

Experience from a single centre concerning the surgical spectrum and outcome of adolescents and adults with congenitally malformed hearts in West Africa.

BACKGROUND This study was undertaken to review the spectrum and surgical outcome of adolescents and adults with congenitally malformed hearts from January, 1993 to December, 2008. The lack of data on this emerging problem from the West African sub-region prompted this report. PATIENTS AND METHOD This retrospective study is based on 135 adolescents and adults with congenitally malformed hearts. A review of their case notes and operative records was carried out and results analysed. RESULTS Selected patients made up 23% of all congenital cardiac surgeries performed at our institution in the same period. A total of 23 patients (17%) were non-Ghanaian West Africans. There was a female preponderance of 53.3%. The ages ranged from 16 to 70 years (mean 28.6 plus or minus 10.3 years). The mean follow-up was 7.5 plus or minus 4.4 years. Patients were functionally classified (New York Heart Association) as class I (23%), II (58%), and III (19%). In 14 (10.4%) patients, the defects were discovered incidentally. Ventricular septal defects, oval fossa type atrial septal defects, Fallots tetralogy, and patent arterial duct together accounted for 77.8% of the cases. Surgical correction was undertaken in 117 (86.7%) patients; the remainder had palliative procedures. There were six (4.3%) reoperations. The functional class improved to class I or II in 95% of patients within the first postoperative year. The overall hospital mortality was 3% with two late deaths (1.5%). CONCLUSION The study demonstrates the feasibility of surgery for adolescents and adults with congenitally malformed hearts in the sub-region with a good outcome. Majority (77.8%) of patients present with less complex lesions.

Management of intrathoracic oesophageal perforation: analysis of 16 cases.

Intrathoracic oesophageal perforation remains a life-threatening lesion that requires early diagnosis and the appropriate intervention in order to reduce morbidity and mortality. Management depends largely on the cause of the perforation, the integrity of the oesophagus and the time lapse between the perforation and the commencement of treatment. Our aim was to evaluate the management options that were employed in the treatment of patients with oesophageal perforation and the outcome. The records of 16 patients (11 males and 5 females) who had been operated on from 1994–2009 were retrospectively reviewed. Their ages ranged between 2–66 years (mean 36.4). Malignant oesophageal perforations were excluded from the study. The aetiology was iatrogenic in 10 (62.5%), foreign bodies five (31.2%) and spontaneous one (6.2%). Six patients (37.5%) presented within 24 h of their injury and 10 (62.5%) presented after 24 h. Thoracotomy and intrathoracic primary repair was possible in five (31.2%) cases. Oesophagectomy, cervical oesophagostomy and feeding gastrostomy were carried out in 11 (68.8%). Oesophageal substitution was by colon, routed retrosternally. One patient (6.2%) died after oesophagectomy from overwhelming sepsis. Oesophageal perforation is a life-threatening condition. Early diagnosis and the institution of prompt and appropriate treatment ensure good outcome.

Outcome of left heart mechanical valve replacement in West African children - A 15-year retrospective study

BackgroundThe West African sub-region has poor health infrastructure. Mechanical valve replacement in children from such regions raises important postoperative concerns; among these, valve-related morbidity and complications of lifelong anticoagulation are foremost. Little is known about the long-term outcome of mechanical valve replacement in West Africa. We sought to determine the outcome of mechanical valve replacement of the left heart in children from this sub-region.MethodWe conducted a retrospective review of all consecutive left heart valve replacements in children (< 18 years old) from January 1993 - December 2008. The study end-points were mortality, valve-related morbidity, and reoperation.ResultsOne hundred and fourteen patients underwent mitral valve replacement (MVR), aortic valve replacement (AVR) or mitral and aortic valve replacements (MAVR). Their ages ranged from 6-18 years (13.3 ± 3.1 years). All patients were in NYHA class III or IV. Median follow up was 9.1 years. MVR was performed in 91 (79.8%) patients, AVR in 13 (11.4%) and MAVR in 10 (8.8%) patients. Tricuspid valve repair was performed concomitantly in 45 (39.5%) patients.There were 6 (5.3%) early deaths and 6 (5.3%) late deaths. Preoperative left ventricular dysfunction (ejection fraction < 45%) was the most important factor contributing to both early and late mortality. Actuarial survival at 1 and 15 years were 98.1% and 94.0% respectively. Prosthetic valve thrombosis occurred in 5 patients at 0.56% per patient-year. There was 1(0.9%) each of major bleeding event and prosthetic valve endocarditis. Two reoperations were performed at 0.22% per patient-year. Actuarial freedom from reoperation was 99.1% at 1 and 10 years, and 85.1% at 15 years.ConclusionMechanical valve replacement in West African children has excellent outcomes in terms of mortality, valve-related events, and reoperation rate. Preoperative left ventricular dysfunction is the primary determinant of mortality within the first 2 years of valve replacement. The risk of valve-related complications is acceptably low. Anticoagulation is well tolerated with a very low risk of bleeding even in this socioeconomic setting.

Late primary arterial switch for transposition of the great arteries with intact ventricular septum in an african population.

The arterial switch operation (ASO) is the optimal management of transposition of the great arteries with intact ventricular septum (TGA-IVS) within the first 3 weeks of life; beyond this age optimal treatment is debatable. The authors adopted a strategy of primary ASO for TGA-IVS in the first 10 weeks of life regardless of left ventricular (LV) status. This report reviews the early outcomes with this management approach. Between August 2006 and December 2009, 22 patients with TGA-IVS underwent the primary ASO. Sixteen of them were less than 21 days old (early switch group) and 6 were between 31 and 66 days old (late switch group). A review of their hospital records was performed to determine outcomes in the 2 groups. Operative variables and postoperative outcomes were recorded. There was 1 hospital death in the early switch group (6.3%) but none in the late group (0%). Temporary mechanical circulatory support was required in 1 patient (6.3%) in the early switch group and in 2 of the 6 (33.3%) in the late switch group. One late death of undetermined cause occurred in the late switch group 8 weeks after discharge. No significant difference could be demonstrated between the 2 groups in terms of operative variables and the measured postoperative outcomes. It is concluded that the age limit for the primary ASO can be extended to at least 10 weeks; temporary mechanical circulatory support may be required as a rescue.

The spectrum of double-outlet right atrium including hearts with three atrioventricular valves

Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.

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.

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.

Divided Left Atrium (Cor Triatriatum) in the Setting of Common Atrium

Cor triatriatum is commonly used to describe the congenital malformation in which an anomalous fibromuscular shelf divides the left atrium. We report the successful surgical repair of an extremely rare combination of divided left atrium, virtual absence of the interatrial septum, and anomalous right pulmonary venous connection in a 7 year-old girl. Preoperative transthoracic echocardiography could not detect the dividing left atrial shelf. In this report, we point out the limitations of transthoracic echocardiography in the diagnosis of atypical cases of so-called cor triatriatum and highlight errors in the scientific literature resulting from the variance in terminology ascribed to congenital division of the left atrium.

Status and Challenges of Care in Africa for Adults With Congenital Heart Defects.

The 54 countries in Africa have an estimated total annual congenital heart defect (CHD) birth prevalence of 300,486 cases. More than half (51.4%) of the continental birth prevalence occurs in only seven countries. Congenital heart disease remains primarily a pediatric health issue in Africa because of the deficient health-care systems: the adults with CHD made up just 10% of patients with CHD in Ghana, and 13.7% of patients with CHD presenting for surgery in Mozambique. With Africa’s population projected to double in the next 35 years, the already deficient health systems for CHD care will suffer unbearable strain unless determined and courageous action is undertaken by the African leaders.

The paediatric cardiac centre for Africa--proceedings of the March 2012 symposium.

The Pediatric Cardiac Centre for Africa (PCCA) was opened by national patron Mr Nelson Mandela on November 7, 2003. In 2008, the Centre’s international pediatric cardiac symposium was introduced as a learning forum for pediatric cardiac surgeons and cardiologists in the continent. The symposium has consistently grown in attendance and attracted distinguished leaders in the field. The 2012 symposium featured Dr. Thomas Spray of Children’s Hospital of Philadelphia, Dr. David Barron of Birmingham Children’s Hospital, and Dr. John Brown of Indiana University School of Medicine as guest speakers. Experience of the Fontan procedure, the small aortic root, hypoplastic left heart syndrome, right ventricular outflow tract reconstruction, transposition of the great arteries, and interrupted aortic arch were the highlights of the symposium. In the “African Corner,” centers in South Africa, Ghana, and Angola presented work done from across the African continent.