Robin H. Kinsley

Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta

We report 2 infants with the unusual combination of origin of the left coronary artery from the right pulmonary artery as well as coarctation of the aorta. In the first case congestive cardiac failure persisted despite satisfactory repair of the coarctation. Further investigation revealed the anomalous origin of the left coronary artery. Corrective surgery, 24 days later was unsuccessful. When an identical diagnosis was made in the second child, it was decided, at a single operation, to combine correction of the coarctation together with re-implantation of the anomalous left coronary artery into ascending aorta. This patient has survived, and to our knowledge is the first such case with this outcome. The haemodynamic reasons for this approach are discussed.

Surgical correction for one pulmonary artery arising from ascending aorta - report of five cases☆

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.

Emergency Heart Valve Replacement: An Analysis of 170 Patients

The results of 170 emergency heart valve procedures performed during a 4 1/2-year period were analyzed. Five pathological groups of patients were recognized: those with infective endocarditis (Group 1, 28 patients); acute rheumatic carditis (Group 2, 43 patients); previous valve operation (Group 3, 29 patients); acute-on-chronic cardiac disease (Group 4, 67 patients); and miscellaneous conditions (Group 5, 3 patients). Mitral, aortic, and multiple valve procedures were performed on 58, 65, and 44 patients, respectively. The most common functional lesion was regurgitation. Hospital mortality was highest in Groups 3 (34%) and 4 (31%). By contrast, among the hospital survivors, the highest rate of attrition was in Group 2. Myocardial failure was the predominat cause of death. In view of the hopeless prognosis without operation, the 52% overall 3-year actuarial survival is a gratifying salvage. Unnecessary procrastination can only jeopardize the prospects for surgical cure.

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.

The Third Aldo Castañeda Lecture: The Neglect of Neonatal/Infant Cardiac Disease in Africa—Continental Genocide?

The advances made in pediatric cardiology and cardiac surgery now make it possible for survival into adulthood of the majority of children born with congenitally malformed hearts. On the African continent, unfortunately, this is only a dream as roughly 280,000 neonates born every year on the continent are left untreated, demonstrating the natural history of the congenitally malformed heart by default. Pediatric cardiac surgery is available in very few countries on this continent. This article takes a look at the problem of neonates and infants born with cardiac defects on the continent and attempts an extrapolation of the magnitude of the problem. Using the experience gained at the Walter Sisulu Pediatric Cardiac Center since its inception in 2003, issues of financing indigent patients, training local personnel, and building capacity through infrastructure development and regional cooperation are discussed. The success of the Walter Sisulu model demonstrates the benefits of treatment for the neonates and ...The advances made in pediatric cardiology and cardiac surgery now make it possible for survival into adulthood of the majority of children born with congenitally malformed hearts. On the African continent, unfortunately, this is only a dream as roughly 280,000 neonates born every year on the continent are left untreated, demonstrating the natural history of the congenitally malformed heart by default. Pediatric cardiac surgery is available in very few countries on this continent. This article takes a look at the problem of neonates and infants born with cardiac defects on the continent and attempts an extrapolation of the magnitude of the problem. Using the experience gained at the Walter Sisulu Pediatric Cardiac Center since its inception in 2003, issues of financing indigent patients, training local personnel, and building capacity through infrastructure development and regional cooperation are discussed. The success of the Walter Sisulu model demonstrates the benefits of treatment for the neonates and infants with congenitally malformed heart, on the continent. It is emphasized that African governments and all stakeholders must participate to ensure a good outcome for the African child with congenital cardiac defect.

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.

Prediction of left ventricular assist device implantation after repair of anomalous left coronary artery from the pulmonary artery

OBJECTIVE We sought to determine the value of preoperative left ventricular function and cardiopulmonary bypass parameters in the prediction of left ventricular assist device implantation after repair of anomalous left coronary artery from the pulmonary artery. METHODS Multivariate logistic regression was performed to identify a predictive model for postrepair left ventricular assist device implantation using the records of 27 patients who underwent direct aortic implantation of anomalous left coronary artery from the pulmonary artery from 1994 to 2011. RESULTS Seven patients required left ventricular assist device implantation. Patients in group 1 (n = 20) were successfully weaned from cardiopulmonary bypass. Patients in group 2 (n = 7) required left ventricular assist device as a bridge to recovery. The 2 groups were similar in age, weight, and body surface area. Six of the 7 patients (85.7%) who required left ventricular assist device survived to hospital discharge. Hospital mortality was 3.7%. In the univariate model, fractional shortening, ejection fraction, and aortic crossclamp time were significantly associated with left ventricular assist device implantation (P = .026, .035, .031, respectively). In the multivariate analysis, the aortic crossclamp time was the only significant independent predictor of left ventricular assist device implantation. Aortic crossclamp time and fractional shortening together accounted for 80.9% (P < .001) of the variability in left ventricular assist device implantation and constituted the best predictive model: All patients requiring postrepair left ventricular assist device implantation had a fractional shortening less than 20% and an aortic crossclamp time greater than 56 minutes. CONCLUSIONS The fractional shortening and aortic crossclamp time together predict 80.9% of the variability in postrepair left ventricular assist device implantation after repair of anomalous left coronary artery from the pulmonary artery. When preoperative left ventricular dysfunction is severe (fractional shortening < 20%), an aortic crossclamp time greater than 56 minutes is associated with a substantial risk of left ventricular assist device implantation after repair of anomalous left coronary artery from the pulmonary artery.

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with an emphasis on excellence in teaching, research, and community service.

Paediatric cardiac surgery for a continent – The experience of the Walter Sisulu Paediatric Cardiac Centre for Africa

Very few African countries have the resources to provide optimum paediatric cardiac services to their largely indigent populations. In the current era, in countries with access to modern paediatric cardiac care, mortality for congenital heart disease occurs more often in adulthood than in childhood. This level of care is largely unavailable in Africa. The Walter Sisulu Paediatric Cardiac Centre for Africa was set up in 2003 as a public-private collaborative initiative to extend modern paediatric cardiac care to the continent. Three core functions form the basis of our operations: service delivery, training, and research. This communication reviews our experience with this effort over an eight-year period. We have performed 2 023 procedures on 1 738 patients including a large proportion of neonates and infants with an overall mortality of 7.1%. Our charity arm sponsored 21.5% of these patients. We have encountered problems peculiar to the African context which we discuss. We also describe innovative techniques in management of specific patient populations. Our training efforts yielded two qualified paediatric cardiac surgeons who now work at the centre and two additional surgeons are in training. We have participated in research leading to publication of papers in peer-reviewed journals. In spite of our achievements, we recognise the enormous challenges faced by the continent in terms of paediatric cardiac care. An attempt has been made to quantify the burden of congenital disease in Africa to guide planning and training. We offer recommendations on how to address some of these pressing health issues for children of the continent.