Although Norwood's first stage palliative procedure has been adopted by most proponents of reconstructive surgery for hypoplastic left heart syndrome, several technical modifications have recently been introduced. Some are intended primarily to minimize the adverse effects of cardiopulmonary bypass, hypothermic circulatory arrest, or both. Some are intended to simplify the technical performance of the initial palliative procedure. As the number of patients surviving staged reconstructive surgery for hypoplastic left heart syndrome grows steadily, there is a shift in emphasis toward investigation of methods to minimize morbidity. Specifically, the quality of life after staged reconstructive surgery may be affected by the potential for neurologic injury associated with bypass, deep hypothermia, and circulatory arrest. The pathology of cerebral injury includes ischemic cerebral necrosis, periventricular leukomalacia, necrosis of the brain stem, and intracranial hemorrhage. Neurologic injury associated with cardiac surgery during infancy is undoubtedly multi-factorial, and as yet is poorly and incompletely understood. Several surgical teams, nonetheless, have revised the technical aspects of the palliative operations with a goal of minimizing the duration of hypothermic circulatory arrest.

Introduction and Background: About 1% of live-born children have congenital malformations of the heart. The aim of our study was to investigate the incidence of such defects in children born in Iceland during a period of 10 years, extending from 1990 through 1999. Materials and Methods: Information about the patients was obtained from medical records from two hospitals that cover the whole country, a private clinic of pediatric cardiologists, an echocardiography database, autopsy reports, and death certificates. We investigated the distribution of specific malformations, the age at diagnosis, the symptoms leading to the diagnosis, the source of referral, and treatment and quality of life. Results: Between 1990 and 1999, there were 44,013 live births in Iceland, of which 740 patients were diagnosed with congenital cardiac malformations, accounting for 1.7% of the live-born children. The distribution was made up of 338 patients with ventricular septal defect (45.7%), 90 with atrial septal defect (12.2%), 85 with patency of the arterial duct (11.5%), 48 with pulmonary valvar stenosis (6.5%), 38 with a bicuspid aortic valve (5.1%), 28 with aortic coarctation (3.8%), 22 with tetralogy of Fallot (3.0%), 14 with transposed great arteries (1.9%), 11 with aortic stenosis (1.5%), 10 with atrioventricular septal defect and common atrioventricular orifice (1.4%), 9 with mitral valvar regurgitation (1.2%), 7 with sub-aortic stenosis (0.9%), and 5 with hypoplasia of the left heart (0.7%). Extracardiac anomalies were seen in 89 patients (12.0%). Chromosomal defects were seen in 36 patients, of whom 28 had Down's syndrome. Discussion: The annual incidence of diagnosis of patients with congenital cardiac malformations increased during the period of study. This was noted for minor defects, but the incidence of the major anomalies did not alter. Our observed yearly incidence, at 1.7%, was higher than noted in a previous study covering the years 1985 through 1989, and is also higher than in other population-based studies. The most likely explanation is the fact that access to pediatric cardiologists in Iceland is very good. Diagnosis, registration, and follow-up are conducted by only a few cardiologists, and take place at a single center for pediatric cardiology.

Positional cyanosis is an uncommon finding in young patients. We report three infants who presented with positional cyanosis due to a pedunculated tumour in the right heart. Arterial desaturation was the result of right-to-left shunting at the level of the oval foramen caused by obstruction and/or insufficiency of the tricuspid valve. The obstruction at the level of the tricuspid valve was variable because of the pedunculated nature of the tumours, which gave them considerable mobility. Hence, the degree of right-to-left shunting was dependent on the position of the patient. In all the patients, surgical resection of the tumours resolved the cyanosis.

Objectives: We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998. Background: Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair. Methods: We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports. Results: The mean initial Z-score for the diameter of the tricuspid valve was −0.99 ± 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up. Conclusions: The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.

Due to underlying cardiovascular anatomy and size, epicardial pacing may be the preferred method of pacing in small children. To assess long-term safety, we reviewed all epicardial pacemakers implanted in children between 1971 and 2001. We found that 122 patients, with a median age of 5.4 years, had a total of 181 pacemakers and 260 electrodes implanted over a total follow-up of 789 patient-years. Of the total, 12 patients died after the first implantation, with one death attributable to dysfunction of the pacemaker. Reintervention was required in 75 patients after 5.0 ± 3.2 years, due to depletion of the battery in 45 patients (60%), fracture or dysfunction of electrodes in 27 patients (36%), and infection in 3 patients (4%). In univariate analyses, risk factors for reintervention were an approach via a median sternotomy, with a relative risk of 2.3 (p = 0.0087), and an indication for pacing other than atrioventricular block, with a relative risk of 1.7 (p = 0.0314). In multivariate analyses, the approach via the median sternotomy independently predicted the need for reintervention, with a relative risk of 2.1, and 95% confidence intervals from 1.1 to 4.1 (p = 0.0256). The longevity of the second pacemaker and/or its electrode, assessed in 26 patients, was 3.7 ± 2.6 years, not shorter than the first implantation (p = 0.4037). We conclude that epicardial pacing is a reliable means of achieving permanent pacing in children, with low morbidity and mortality. A substantial proportion, nonetheless, requires reintervention within five years, warranting meticulous follow-up.

The term “functionally single ventricle” includes a variety of congenital cardiac anomalies where there is only one ventricle pumping blood to the systemic and pulmonary circulations. The physiology in this arrangement is a considerable challenge for the cardiac specialist, because the complexity encountered in patients with these lesions necessitates particularly specialized medical and surgical management. Patients with such functionally univentricular physiology often respond to common interventions, such as supplemental oxygen, mechanical ventilation, and vasoactive drugs, differently than patients with conventional circulations.1 Furthermore, these patients tend to be encountered more frequently by pediatricians and cardiologists because they undergo multiple operations, may be more adversely affected by intercurrent illnesses, or have chronic cardiac problems requiring frequent attention. A thorough understanding of the complexities of the physiology encountered is thus imperative for the pediatric cardiologist. In this review, we will address important physiologic and anatomic principles that influence care of neonates with functionally univentricular hearts. Although the anatomy and physiology of each reconstructive stage of palliation are unique, we will focus upon the pre- and post-operative physiology as encountered in the neonate.

the bidirectional cavopulmonary anastomosis is commonly used in the palliation of patients with a functionally univentricular physiology. the management of alternative sources of flow of blood to the lungs, as well as the magnitude of acceptable accessory blood flow at the time of surgery, nonetheless, remains controversial. these issues are particularly significant when a cavopulmonary anastomosis is performed in infants who may become candidates for a fontan procedure. indeed, a long-standing volume overload, which is invariably associated with the maintenance of accessory sources of pulmonary blood flow, may result in systemic ventricular dysfunction. these observations prompted us to review the influence of antegrade pulmonary blood flow in the management of infants undergoing a cavopulmonary anastomosis.

Objective: To explore whether prenatal diagnosis of congenital heart disease is associated with lower levels of parental distress and greater satisfaction with decisions about cardiothoracic surgery when compared to postnatal diagnosis. Methodology: A combined quantitative–qualitative design was used. Participants included the parents of 31 neonates (30 mothers and 22 fathers) admitted to the cardiac intensive care unit between 1 November 2001 and 1 May 2002 for repair of congenital cardiac malformations. Participants completed self-report measures of anxiety, optimism, and life events pre-operatively, and semi-structured qualitative interviews assessing satisfaction with decision-making within 1 week of the operation. Results: At the time of surgery, mothers of neonates receiving the diagnosis prenatally did not differ from mothers of neonates receiving the diagnosis postnatally on measures of anxiety, optimism, and life events. Fathers of neonates receiving the diagnosis prenatally, however, reported more optimism, lower state and trait anxiety, and fewer negative life events than fathers of neonates receiving the diagnosis postnatally. When we analyzed the interviews, we found that, regardless of the timing of the diagnosis, parents felt as though they made a genuine choice for their baby to have surgery. Conclusions: In this pilot study, fathers who learned prenatally that their child had a congenital cardiac malformation were less distressed than those who discovered this fact only postnatally. From the parental perspective, nonetheless, distress and urgency do not impair their ability to make decisions about neonatal cardiac surgery.

A 5-week-old child presented with a cardiac arrest secondary to myocardial ischaemia. Echocardiography demonstrated a single coronary artery arising from the right sinus of Valsalva. The coronary artery branched into left and right arteries, with the left artery then coursing anomalously in the tissue plane between the aortic root and the subpulmonary infundibulum. Compression of the left coronary artery caused severe myocardial ischaemia that resolved following construction of a bypass graft using the left internal thoracic artery. Stenosis at the anastomosis between the graft and the coronary artery was successfully treated by coronary angioplasty 2 years later.

So-called ‘absent pulmonary valve syndrome’ is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.

Patients with congenitally corrected transposition are at risk of right ventricular dysfunction and failure. With this in mind, we examined 13 patients with congenitally corrected transposition, 7 not having undergone surgery, and 6 after physiological repair, comparing them with 6 healthy subjects matched for age and sex, using cardiac magnetic resonance imaging, at rest and during dobutamine stress, in order to determine regional and global right ventricular response to stress.

At rest, the patients had significantly decreased overall wall motion compared to their healthy peers (7.2 ± 0.5, versus 9.8 ± 0.4 mm). During infusion of dobutamine, overall wall motion increased to 12.8 ± 0.4 mm in the healthy subjects, versus 8.8 ± 1.0 mm in patients. At the regional level, significant differences in mural motion were found between patients and controls in the anterior (9.5 ± 1.1, versus 13.2 ± 0.6 mm), posterior (10.2 ± 1.6, versus 13.2 ± 0.8 mm), and septal segments (5.0 ± 0.8, versus 11.2 ± 0.6 mm).

At rest, overall mural thickening in patients was similar to that of controls, but significantly less in patients during stress. During dobutamine stress, patients showed significantly less regional wall thickening than controls, particularly in the septal (2.7 ± 0.6, versus 6.0 ± 0.4 mm, respectively) and in the anterior segments (4.2 ± 0.6, versus 7.8 ± 0.6 mm, respectively). Right ventricular ejection fraction strongly correlated with mural motion and thickening, both at rest and during stress.

Abnormal regional function in the systemic morphologically right ventricle may occur in patients with congenitally corrected transposition, which strongly correlates with right ventricular ejection fraction. Our findings support the hypothesis that, in patients with congenitally corrected transposition, ischemia of the right ventricular myocardium contributes to the development of right ventricular dysfunction.

We inserted covered Cheatham-Platinum stents in 4 patients, ranging in age from 12 to 19 years, who weighed between 45 and 94 kg. All the patients had aortic coarctation, with surgical repair having been attempted previously in one, and with balloon dilation having been performed as the primary treatment in two, resulting in formation of aneurysms. The fourth patient had not received any treatment. The gradients were reduced from 10 to 40 mmHg before insertion of the stent to 0 to 5 mmHg after stenting. No complications were encountered. All the patients are well at an interval of 3 to 14 months after stenting.

We aimed to assess the right atrial performance in patients after surgical correction of tetralogy of Fallot, and to clarify the relationship between the pump function of the right atrium and right ventricular systolic function.

We included in the study 50 asymptomatic patients following corrective surgery of tetralogy of Fallot, comparing them to 30 normal subjects. Right atrial areas were measured by echocardiography, and the active fractional area of emptying was expressed, in percentages, as the area measured at the onset of atrial contraction, minus the minimal area, divided by the area at the onset of atrial contraction. We used this value to assess the atrial pump function. Right atrial peak strain rates were measured by tissue Doppler imaging. Compared to controls, patients with tetralogy of Fallot had a significantly reduced right atrial active fractional area of emptying (p = 0.005), along with a reduced peak late diastolic strain rate (p = 0.002). Among 20 patients who underwent magnetic resonance tomographic examination, a relatively higher right atrial peak late diastolic strain rate was shown in patients with a right ventricular ejection fraction of less than 50% (p = 0.021).

Right atrial performance is reduced in patients after surgical correction of tetralogy of Fallot. When facing right ventricular systolic dysfunction, nonetheless, the right atrial pump function may be relatively enhanced. Tissue Doppler derived strain rate can provide quantitative analysis of regional right atrial performance.

the techniques of computational fluid dynamics are among the most powerful tools available to engineers dealing with the motion of fluids and the exchange of mass, momentum, and energy. they have recently been shown to have an increasing number of applications to the human cardiovascular system, including the fluid dynamics of surgical reconstruction of congenitally malformed parts of the cardiovascular system. in vitro models are the alternative laboratory tools with which to study fluid dynamics. the advantages of computational fluid dynamics over the in vitro models are the easy quantification of haemodynamic variables, such as rates of flow, pressure, and distribution of shear stress, and changes in geometric and fluid dynamics parameters. furthermore, using computational fluid dynamics allows the development of three-dimensional models to reproduce both the complex anatomy of the investigated region and the details of the surgical reconstruction, especially with the recent developments in magnetic resonance imaging. on the basis of the results, it is possible quantitatively to evaluate the surgical correction. this technology, which benefits greatly from the continuous improvement in hardware and software, enables cardiovascular experts and bioengineers to look at the fluid dynamics of various cardiovascular regions with increasing sophistication.

We carried out a detailed clinical, epidemiological, and echocardiographic study in 41 patients ≤14 years of age who were admitted in a public hospital in Salvador, Brazil, with severe rheumatic heart disease.

Mitral insufficiency was severe in 90%, and moderate in 10%, of the patients. A posteriorly directed jet was seen in 93% of the patients. We identified three mechanisms producing the regurgitation: prolapse of the aortic leaflet of the mitral valve in 13 (32%) patients, rupture of tendinous cords in 14 (34%), and a retracted, non-coapting mural leaflet in 14 (34%). The mean ages, with standard deviations, for these three groups were 7.0 (1.6) years, 7.9 (2.2) years, and 10.5 (2.4) years, respectively (p < 0.001). Rheumatic activity was diagnosed in 58.5% of them. Evidence of previous rheumatic fever was present in 54% of patients with prolapse, in all patients with rupture, and in 93% of those with non-coapting leaflets (p = 0.002).

Prolapse of the aortic leaflet, rupture of tendinous cords, and a retracted, non-coapting mural leaflet are the mechanisms responsible for mitral valvar insufficiency in children and adolescents with severe rheumatic heart disease. Prolapse seems to be an early phenomenon in the natural history of rheumatic heart disease, while rupture and non-coaption of the leaflets were associated with older age and signs of chronic rheumatic disease.

Noncompaction of the ventricular myocardium is increasingly recognized as an important cause of cardiomyopathy. Its echocardiographic definition, however, is not yet clearly refined, and differentiation from other conditions with hypertrabeculation can be difficult. We report a prospective short-term follow-up of 15 children with noncompaction, excluding those with associated complex congenital cardiac disease.

The clinical presentation and outcome were variable, with 2 patients being asymptomatic. For 5 patients, presentation was with cardiac failure due to depressed myocardial function. The function deteriorated in two, remained the same in two, and improved in the other patient. Cardiac failure due to mitral regurgitation was the mode of presentation in 2 patients with preserved myocardial function, one of whom needed replacement of the mitral valve. In 6 patients (40%), symptoms of cardiac failure were due to noncomplex congenital cardiac disease. All of them had ventricular septal defects. In addition, two had cleft mitral valves, and one had a large persistently patent arterial duct. The diagnosis of noncompaction was initially missed on more than one echocardiographic study in one-third of our patients. We conclude that noncompaction is under-diagnosed, and is not as rare as is thought. In children, it is often associated with other cardiac lesions that can cause cardiac failure in the presence of preserved myocardial function.

Objectives: To determine the long-term outcomes and risk factors for, reintervention after balloon dilation of congenital aortic stenosis in children aged 6 months or older. Background: Although balloon dilation of congenital aortic stenosis has become a primary therapeutic strategy, few data are available regarding long-term outcomes. Methods: We carried out a retrospective review of 87 children who had undergone balloon dilation of the aortic valve at median age of 6.9 years. Results: The procedure was completed in 98% of the children, with an average reduction in the gradient across the valve of 64 ± 28%, and without mortality. Of the children, 76 had been followed for a mean of 6.3 ± 4.2 years. Reintervention on the aortic valve was required in 32 children, with 12 undergoing reintervention within 6 months, with 1 death. Another patient had died over the period of follow-up due to a non-cardiac event. Estimated freedom from reintervention was 86% at 1 year, 67% at 5 years, and 46% at 12 years. Parametric modeling of the hazard function showed a brief early phase of increased risk, superimposed on an ongoing constant risk. The only incremental risk factor for the early phase was a residual gradient immediately subsequent to the procedure greater than 30 mmHg. Incremental risk factors for the constant phase included the presence of symmetric valvar opening, and greater than moderate regurgitation immediately after dilation. Conclusion: Long-term survival was excellent, albeit that the need for further reintervention was high due to the palliative nature of the procedure.

Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The mean period of follow-up for the 41 living patients was 16.5 years. An arrhythmia of variable severity appeared during the evolution of 27 patients (66%). At present, only 7 cases are in the functional class III or IV of the grading system of the New York Heart Association, but 25 patients (61%) needed some medical treatment, meanly for arrhythmic events. Thus, fetal and neonatal presentation of Ebstein's malformation is associated with a poor outcome. Moreover, the echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality. Arrhythmia and need of medical treatment are common in older children and adults. Survival after surgical treatment is associated with a good outcome.

Surgical treatment of hypoplastic left heart syndrome has generated substantial interest and attention amongst cardiac surgeons since the initial reports from Norwood and his colleagues in 1980.1,2 Initial efforts at most programmes were to create reproducible results, and mortality rates remained high at several institutions throughout the 1980s and 1990s. A recent multi-institutional review demonstrates that the hospital mortality still remains high in numerous centers at the current time.3 Nevertheless, several advances over recent years have led to improved outcomes, and in the best centers, hospital survival now approaches 90%. Survival in successful centers is claimed to relate to the ability of the team to help the patient balance the systemic and pulmonary flows of blood. This ability to balance flow has been enhanced, over recent years, by numerous contributions, including decreasing the size of shunt ordinarily used,4 the use of alpha blockade,5 the rapid deployment of extracorporeal membrane oxygenation,6–8 and various forms of ventilatory manipulation.

We present a 15-year-old white male who presented with an acute myocardial infarction secondary to embolisation of tissue from a left atrial myxoma. He successfully underwent resection of the myxoma, and embolectomy of the fragments of tumour lodged in the coronary artery.