Showing papers in "Cardiology in The Young in 2000"
TL;DR: It is recommended that systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease is recommended, due to decreased withdrawal and somatic complaints.
Abstract: In order to test the effect of systematic supervised physical training, we divided a total of 129 children and adolescents with congenital heart disease into a group undergoing intervention and a control group. All patients underwent exercise tests, measurements of physical activity, and a survey of psychosocial factors. An improvement in uptake of peak level of oxygen was observed after intervention. There was also an improvement in physical activity in both groups measured by a monitor, although this was significant only in those with intervention. The psychosocial scales measured by the Child Behavior Checklist showed a decrease in internalizing scores for those subjected to intervention. This was decreased due to decreased withdrawal and somatic complaints. In conclusion, we recommend systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease.
134 citations
TL;DR: One of the reasons for such major differences could be the time lag between the data collected in the 1940's and those collected recently.
Abstract: MAJOR FLUCTUATIONS HAVE BEEN REPORTED concerning the prevalence of congenital heart malformations at birth. Figures quoted range from 2.10 per 1,000 live births in New England and 2.17 per 1,000 found in Toronto,\" (Table 1) to 12.3 per 1,000 live births found by Manetti et al in Florence. '~(Table 2) One of the reasons for such major differences could be the time lag between the data collected in the 1940's and those collected recently. The oldest surveys, which all
119 citations
TL;DR: In a population of adolescents with trivial to moderate congenital cardiac malformations, beliefs in self-efficacy were the most influential factors in determining whether or not adolescents will engage in sports or other physical activities.
Abstract: Our purpose was to examine the cognitive processes that influence involvement in physical activity among 100 adolescents, 55 boys and 45 girls, ranging in age from 12 to 18 years, with trivial, mild, or moderate forms of congenital cardiac disease. We hypothesized, first, that the severity of the congenital cardiac malformation itself has an indirect effect on self-efficacy regarding physical activity, and that the relationship between the two is mediated by the recommendations of the cardiologist and the attitude of the mother. Second, we argued that self-efficacy serves as a mediating variable between the recommendations of the cardiologist and the attitude of the mother, on the one hand, and involvement in physical activity, on the other. The results confirmed both hypotheses. In a population of adolescents with trivial to moderate congenital cardiac malformations, beliefs in self-efficacy, rather than severity of the disease, were the most influential factors in determining whether or not adolescents will engage in sports or other physical activities. We also demonstrated the importance of the role played by the recommendations of the cardiologist in determining both the attitudes of the mother and the belief in self-efficacy of the adolescents.
103 citations
TL;DR: In the authors' experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.
Abstract: Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (-0.91 +/- 0.99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were -0.67 +/- 1.1 and -0.89 +/- 1.2 respectively. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a mean time from the Fontan operation of 4.4 +/- 1.4 years, the mean Z score for height was -1.15 +/- 1.2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.
82 citations
TL;DR: It is concluded that the CardioSEAL and Starflex double umbrella devices produce excellent results when used to close defects of small to moderate size, or else complications ensure, when attempts are made to close very large defects.
Abstract: In this review, we describe the experience from 13 European centres using the CardioSEAL and Starflex double umbrella devices to close interatrial communications within the oval fossa (so-called 'secundum' defects). Between October 1996 and April 1999, the procedure was attempted in 334 patients with a mean age of 12 years and a mean weight of 44kg. The mean measured stretched diameter of the defect was 15 mm. In the overall group, the defect was solitary in 245 patients (73%), multiple in 21 (6%), associated with an aneurysm of the flap valve in 15 (5 %), was represented by patency of the oval foramen in 44 (13%), and was a fenestration in a Fontan repair in 9 (3%). In all patients, the devices were inserted under general anesthesia, using fluoroscopic and transesophageal echocardiographic control. Implantation was achieved in 325 (97,3%). The device embolized within either a few minutes or a few hours in 13 patients (4%). Of these, uncomplicated surgical repair was undertaken in 10, while the device was retrieved in 3 using catheters and a second device was successfully implanted. Residual shunting was detected immediately after the procedure in 41% of the patients, with the incidence decreasing to 31% at discharge, 24% at 1 month, 21% at 6 months, and 20.5% at one year. During the period of follow-up, elective surgical repair became necessary in two patients, due to malposition of the device in one, and late embolization in the other. Fractures of arms were seen in 6.1 %, most commonly with the largest devices. All those with fractured arms of the device were asymptomatic, and no clinical complications related to the fractures were observed. There were no arrhythmias, endocarditis, valvar distortion, thromboembolic events, or other complications. After one year of follow-up, clinical success, defined as complete closure of the defect or presence of only a trivial leak, had been obtained in 92.5% of the patients. We conclude, therefore, that these devices produce excellent results when used to close defects of small to moderate size. Results are less than optimal, or else complications ensure, when attempts are made to close very large defects.
76 citations
TL;DR: The Fontan procedure, as performed by Fontan and Baudet, is now entering its 4th decade, and the indications for this operation have been widely extended, as have the criterions for consideration for atrial separation and construction of an atriopulmonary connection.
Abstract: THE FONTAN PROCEDURE, AS CONCEIVED AND performed by Fontan and Baudet, is now entering its 4th decade. While initially applied to patients with tricuspid atresia, the indications for this operation have been widely extended, as have the criterions for consideration for atrial separation and construction of an atriopulmonary connection. From the original operation as performed by Fontan, there has also been considerable evolution in surgical technique.Much of the interest in modifying the original operation was based on the observations of de Leval, who suggested that a classical atriopulmonary connection with its dilated right atrium was not energy-efficient. This observation led to the concept of the lateral tunnel, and to total cavopulmonary connections, both considered to be more energy-efficient. More recently, the concept has been extended with the so-called extracardiac Fontan procedure, an operation that can be performed in some patients without cardiopulmonary bypass." Other maneuvers, considered by some contentious, have been introduced in order to reduce mortality at the time of the Fontan procedure." These include atrial fenestration at the time of the Fontan procedure, with later closure either using a surgical snare, or any of a variety of devices introduced on catheters."'" Staging with a bidirectional cavopulmonary shunt, or by the so-called hemi-Fontan procedure as popularized by Norwood, with correction of associated
70 citations
TL;DR: Symptomatic patients less than 2 years of age can undergo successful closure of an atrial septal defect using the Amplatzer device, but the rates of success are less, and procedure time longer, than in older children or adults.
Abstract: Aims To assess results of closure of atrial septal defects within the oval fossa by devices delivered by catheterisation in symptomatic infants and children under 2 years of age. Methods and results The Amplatzer septal occluder was used. Results, and complications of transcatheter device treatment in patients aged below 2 years were compared to previous results from our institution. Patients We attempted closure in 12 consecutive patients below the age of 2 years who presented with an atrial septal defect between May 1997 and 1999. Symptoms were failure to thrive in 6, frequent chest infections in 5, and the need for treatment of heart failure in the other. All were thought to have a defect suitable for interventional closure. The atrial defects were seen in isolation in 10 children, but 2 had associated pulmonary stenosis which had been treated by balloon dilation prior to placement of the Amplatzer occluder. Results The Amplatzer septal occluder was implanted at a mean age of 1.4 +/- 0.4, with a range from 0.8 to 1.8 years. Ratios of pulmonary-to-systemic flow had been 2.1 +/- 0.5, with a range from 1.6 and 3.2, and the defect was measured at 12 +/- 4 mms. Fluoroscopy time was 12.8 +/- 10.2 minutes, with a range from 5 to 43 minutes, and the time of the overall procedure was 162 +/- 70 minutes, with a range from 85 to 360 minutes. It proved necessary to remove the device in 2 patients (16%) because of a residual shunt and movement after release. One of these developed transient neurological complications. Both subsequently underwent surgical treatment. Conclusion Symptomatic patients less than 2 years of age can undergo successful closure of an atrial septal defect using the Amplatzer device, but the rates of success are less, and procedure time longer, than in older children or adults.
66 citations
TL;DR: Elevated levels of psychological distress, and less adequate styles of coping, were found in the parents of patients about to undergo cardiac surgery, especially the mothers, when compared to reference groups.
Abstract: Aims We sought to assess the level of psychological distress, and the styles of coping of, parents of children with congenital heart disease. The study was based on questionnaires, which were completed, on average, four weeks, with a range from 0.1 to 22.1 weeks, prior to elective cardiac surgery or elective catheter intervention. Methods We used the General Health Questionnaire, and the Utrecht Coping List, to compare scores from parents of those undergoing surgery, with scores of reference groups, and with scores of the parents of those undergoing intervention. Results Overall, in comparison with our reference groups, the parents of the 75 children un dergoing surgery showed elevated levels of psychological distress, manifested as anxiety, sleeplessness, and social dysfunctioning. They also demonstrated less adequate styles of coping, being, for example, less active in solving problems. With only one exception, no differences were demonstrated in parental reactions to whether cardiac surgery or catheter intervention had been planned. The mothers of the 68 patients who were to undergo cardiac surgery, however, reported greater psychological distress and manifested greater problems with coping than did the fathers. Conclusion Elevated levels of psychological distress, and less adequate styles of coping, were found in the parents of patients about to undergo cardiac surgery, especially the mothers, when compared to reference groups. Future research should investigate whether these difficulties persist, and whether this will influence the emotional development of their children with congenital cardiac malformations.
62 citations
TL;DR: Exercise-induced hypertension, and an elevation in the average systolic 24 hour blood pressures, were observed, but less frequently than elevated baseline values, suggesting that socalled white-coat” hypertension may be present in this population of patients with surgically repaired coarctation.
Abstract: BACKGROUND Despite successful surgical repair of aortic coarctation, life expectancy is reduced, and up to one-third of patients remain or become hypertensive. So as to characterize the responses for blood pressure, we have studied 55 patients with surgically repaired coarctation. Their mean age was 11.3 +/- 5.97 years. We documented maximal uptake of oxygen, anaerobic threshold, plasma renin activity and blood pressures during a Bruce protocol treadmill test. The velocity across the site of repair as imaged by cross-sectional echocardiography was measured before and after exercise. We measured the changes in heart rate and blood pressure subsequent to an infusion of 1 ug per kg of isoprenalin, monitoring blood pressure over 24 hours in all patients. RESULTS When compared with 40 healthy age-matched controls, the patients with coarctation had a normal exercise capacity. Resting systolic blood pressures above the 95th percentile were present in 45% of the patients. Exercise-induced hypertension, and an elevation in the average systolic 24 hour blood pressures, were observed, but less frequently than elevated baseline values, suggesting that so-called white-coat" hypertension may be present in this population. Abnormal reactions and elevation of plasma renin activity were related to a history of paradoxical hypertension at the time of surgery. Attenuation of the circadian rhythm for blood pressure was a frequent finding, and may have implications in the development of long-term damage to end-organs. A high correlation was found between mean systolic blood pressure measured by 24 hour monitoring and left ventricular hypertrophy (r=0.65, p<0.05). CONCLUSIONS Abnormalities in blood pressure occurred independently of significant mechanical obstruction. Despite successful surgical repair, abnormalities in the shape of the aortic arch, reduced sensitivity of baroreceptor reflexes, and neurohumoral factors may all contribute to the development of hypertension.
61 citations
TL;DR: The results indicate that the Computer Science & Application monitor is a valid instrument for assessing physical activity and may be used to validate the levels of physical activity level in children with congenital heart disease after medical and surgical treatment.
Abstract: The aim of the present study was to evaluate different aspects of a device designed to monitor physical activity. Measurements of different axes and placement of the monitor were tested using a treadmill with ranging increments in incline or speed. The monitor was also used to assess the level of physical activity among children and adolescents with congenital heart disease and in healthy controls at the same age. The results indicate that the monitor is a valid and reproducible instrument for measurements of physical activity. The study revealed that the level of activity was higher for healthy boys than healthy girls (p<0.0001). Boys with congenital heart disease also displayed higher values compared to girls with congenital heart disease, although the difference was not significant (p=0.067). Healthy boys revealed a significantly higher level of activity than did boys with congenital heart disease (p=0.003), but no such difference was found in girls (p=0.757). Nor were any differences found between younger and older individuals among patients with congenital heart disease. Young healthy controls, however, showed significantly higher levels of activity than their older counterparts. There were differences in activity monitored during the week, with lower activity in the weekends, but the activity on the same day in different weeks seemed stable. Neither were there any differences between measurements over whole weeks. The results indicate that the Computer Science & Application monitor is a valid instrument for assessing physical activity. The monitor may also be used, therefore, to validate the levels of physical activity level in children with congenital heart disease after medical and surgical treatment.
49 citations
TL;DR: This multi-centric registry shows good results in the intermediate term for treating infants with severe aortic valvar stenosis with balloon valvotomy through a carotid arterial cutdown.
Abstract: OBJECTIVES The purpose of this study was to evaluate the short and intermediate term results of infants who have undergone balloon aortic valvotomy from the carotid arterial approach, and to identify risk factors in those infants who had a poor outcome. METHODS Between 1988 and 1999, balloon aortic valvotomy was attempted at four centres in 95 infants with severe aortic stenosis. Echocardiographic and hemodynamic data, and outcome, were analysed retrospectively. RESULTS Valvotomy was accomplished in 92 of the 95 infants, with a median age of 5 days, a range from 0 to 191 days, and weighing 3.4 kg, with a range from 1.0 to 6.5 kg. Major procedural complications occurred in 10 infants. Post-procedural aortic regurgitation was severe in 5 patients. There were 13 early deaths, and 4 late deaths. The period of mean follow-up has been 2.1 years, with a range from 0 to 9.3 years. The actuarial survival at 3 years was 76 +/- 6%. Further interventions were needed in 19 patients, giving a 3-year freedom from reintervention of 67 +/- 6%. The 51 infants who were duct-dependent were further analyzed, and found to have a higher mortality (38%) compared to those infants not dependent on the arterial duct (5%). Risk factors for a poor outcome in the duct-dependent infants were mitral stenosis (p<0.005), a left ventricle which did not form the cardiac apex (p<0.005), and an aortic valve with a diameter of less than 6 mm (p<0.05). CONCLUSIONS This multi-centric registry shows good results in the intermediate term for treating infants with severe aortic valvar stenosis with balloon valvotomy through a carotid arterial cutdown. Infants dependent on prostaglandin had a worse outcome, especially if they had any of the identified risk factors.
TL;DR: Its unique design, and ease of use, has resulted in its widespread adoption by many entres, and there is, as yet, no long-term followup data available for this life-long implant.
Abstract: The Amplatzer Septal Occluder is made from a Nitinol wire mesh shaped into 2 disks with a connecting waist, which serves to center the device in the defect while occluding it. The Amplatzer device is also available in a configuration with no central waist for use in patients with patent oval foramen, or multiperforated aneurysm of the interatrial septum. For the purposes of this review, we analysed our experience using the Amplatzer device in 150 patients with interatrial communications. Of these, 104 had a defect within the oval fossa, 33 a patent oval foramen, and 13 had undergone fenestration of a Fontan procedure. Of those with defects within the oval fossa, a device was implanted in 100 patients, and 2 of these patients subsequently required surgical intervention, 1 because of migration and the other because of malformation of the device. Of the remaining 98 patients, complete occlusion has been achieved in 90% at 1 year. Any residual leaks are either trivial or small. In those with a patent oval foramen, the septal occluder was used to close 20, whilst the device designed specifically for this purpose was used in 13. On follow-up contrast echocardiography, only 2 patients have a small residual right-to-left shunt. Complete occlusion was achieved for all the Fontan fenestrations, although 1 patient later underwent surgery for baffle dehiscence. Other significant complications occurred in 2 patients who developed deep vein thrombosis, and 3 patients who suffered transient supraventicular arrhythmias. Although the Amplatzer device has been in clinical use for only 3 years, its unique design, and ease of use, has resulted in its widespread adoption by many entres. The results to date are very encouraging, but it must be remembered that there is, as yet, no long-term followup data available for this life-long implant.
TL;DR: Structural anomalies of the arch are relatively common in patients with a cervical aortic arch, and may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the Arch or its embryologic precursors.
Abstract: BACKGROUND: Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases METHODS: Between 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2 In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3 A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2 The descending aorta was circumflex (left-sided) in 3 patients Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1 RESULTS: Repair was successful in all cases, with no perioperative complications At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications Fluorescent in situ hybridization revealed microdeletion of chromosome 22q11 in 1 patient (not performed in the others) CONCLUSIONS: Structural anomalies of the arch are relatively common in patients with a cervical aortic arch Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind
TL;DR: The middle aortic syndrome was present in more than half the authors' patients, and does not necessarily reflect a bias because of cardiologic referral, and is an important requirement for complete evaluation of patients with Williams' syndrome.
Abstract: The middle aortic syndrome, with diffuse narrowing of the thoracic and abdominal aorta, was present in 10 of 18 patients with Williams' syndrome (55%). There were 3 thoracic coarctations, and 2 abdominal coarctations, with gradients greater than 20 mmHg across the zone of narrowing. Seven patients had mild renal arterial stenosis, and 6 had visceral arterial stenoses. Ten were hypertensive. Measured dimensions of the aortic lumen failed to increase with age in 3 males who had serial angiographic studies. One developed mesenteric arterial stenosis, with mild bilateral renal arterial stenoses, between the ages of 9 and 19 years. Aortic intravascular ultrasound performed in 2 patients confirmed abnormally thick vessel walls with small lumens. Diffusely narrowed and thick-walled stiff arteries, lacking elastin, are a feature of Williams' syndrome. The arteriopathy tends to progress with age, and systemic hypertension is common in teenagers and beyond. The middle aortic syndrome was present in more than half our patients, and does not necessarily reflect a bias because of cardiologic referral. Aortography with measurement of aortic diameters and delineation of the visceral branches is an important requirement for complete evaluation of patients with Williams' syndrome.
TL;DR: A previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels is described.
Abstract: We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed echocardiographic and angiographic evaluations were performed in all, and biopsies of the skin in seven. All patients have elongated facies, prominent ears, micrognathia and laxity of their joints. Angiographic pictures showed a varying degree of lengthening and tortuosity of systemic, pulmonary, and coronary arteries. Pulsatile carotid arteries formed cervical masses in 2 patients, and three had severe renal arterial stenoses. All showed varying degrees of branch and peripheral pulmonary arterial stenosis, necessitating placement of stents in six. Biopsy of the skin proved normal in all seven patients studied, thus excluding cutis laxa, Ehlers-Danlos and Marfan syndromes. The constellation of abnormalities suggests a genetic syndrome of connective tissue etiology. Further genetic studies, and gene mapping, are underway.
TL;DR: The majority of children diagnosed by a paediatric cardiologist as having innocent murmurs had the so-called Still's, or musical/vibratory murmur, and none had abnormal investigations, suggesting that such investigations are largely superfluous for the purpose of diagnosis in those patients with innocent murmur.
Abstract: Innocent murmurs are common in childhood. They require accurate diagnosis to avoid unnecessary anxiety and/or restrictions. With this in mind, we studied prospectively children diagnosed by a paediatric cardiologist as having innocent murmurs to review the necessity of investigations in excluding organic abnormalities. We included 63 children in the study. The majority had the so-called Still's, or musical/vibratory murmur. None had abnormal investigations, suggesting that such investigations are largely superfluous for the purpose of diagnosis in those patients with innocent murmurs seen by a paediatric cardiologist. They are often done, nonetheless, as part of the assessment and/or expectations of the parent or the referring physician.
TL;DR: It is suggested that ablation should be avoided before the age of 5 or 6 years, and the procedure should become the first line of treatment for symptomatic patients older than 12 years of age.
Abstract: There is less certainty about the recommendations for radio-frequency ablation as a therapeutic option for Wolff-Parkinson-White syndrome in children as opposed to adults because of the different natural history and the age-related risks of ablation. To help decision-making, we evaluated the long term clinical evolution and electrophysiologic characteristics of pre-excitation in our series of children and young adults. We reviewed the clinical course of 109 patients below the age of 18 years who had been followed up over a period of 9+4 years, with a range from one to 27 years. This corresponded to 986 patient-years. We examined also the electrophysiologic data from 98 of those patients who underwent a transesophageal study. At the discovery of pre-excitation, 59% of patients were asymptomatic, while 29% developed supraventricular tachycardia during follow-up. The peak incidence of the onset of supraventricular tachycardia occurred during infancy. These patients had the highest incidence of subsequent spontaneous disappearance of the tachycardia (53%), such a favourable evolution being encountered in only 12% of patients in whom the abnormal rhythm first appeared after 12 years of age. Chronic medical treatment was required in 47% of patients, and proved completely effective in 45% of cases. The potential to induce atrial fibrillation, and the incidence pre-excitation considered to be high risk, with the shortest pre-excited RR interval equal to or less than 220 msec, was lowest in the group of patients aged less than 6 years of age, and highest in those older than 12 years of age (p <0.001). Pathways producing arrhythmia with high risk were more common in symptomatic (29%) than in asymptomatic patients (7%) (p<0.001). No mortality occurred. On the basis of our findings, we suggest that ablation should be avoided before the age of 5 or 6 years. Thereafter, the procedure should become the first line of treatment for symptomatic patients older than 12 years of age.
TL;DR: It is adavantageous to have a firm grasp of the anatomy of the normal atrial septal structures, this then providing the basis to understand the morphology of the holes which can exist between the chambers, not all of which are true sePTal defects.
Abstract: Increasingly, the interventional cardiologist is seeking to close interatrial communications by inserting devices by means of catheterisation. So as to optimise these procedures, it is advantageous to have a firm grasp of the anatomy of the normal atrial septal structures, this then providing the basis to understand the morphology of the holes which can exist between the chambers, not all of which are true septal defects. A true septal structure can be removed without exiting from the cavities of the heart. It is the flap valve of the oval fossa, along with the anterior rim of the fossa, which fulfill this criterion. The remainder of the extensive rim of the normal fossa is no more than an infolding between the walls of the right and left atria and their venous tributaries, and has different dimensions at various points around the circumference. The so-called muscular atrioventricular "septum" is a sandwich incorporating a layer of epicardial fibro-adipose tissue. True defects of the atrial septum, therefore, exist because of deficiency, perforation, or absence of the flap valve. Most of these defects will prove suitable for interventional closure, but potential caveats include multiple defects, aneurysm of the flap valve, or adjacency of the fossa to the venous orifices. The other interatrial communications, namely the sinus venosus, coronary sinus, and "ostium primum" defects are outside the confines of the oval fossa. Recognition of this feature is the key to their diagnosis, and their differentiation from true atrial septal defects. Of these defects, only the coronary sinus defect is likely to be suitable for device closure, and then only in the very rare circumstances when it is seen in isolation.
TL;DR: An unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant is described, and the infant is asymptomatic 18 months after the second surgery.
Abstract: We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant The infant is asymptomatic 18 months after the second surgery The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combinationThe inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes To our knowledge only 9 cases have been published of such tumor arising within the heart1–6
TL;DR: The experience of postoperative fast-tracking of children undergoing cardiac surgery during a charitable mission in Venezuela shows that early extubation can safely be carried out in well-selected patients after surgery to correct congenital cardiac malformations.
Abstract: In recent years, post-operative intensive care of the child with congenital cardiac disease has placed an emphasis on earlier weaning from mechanical ventilation. We describe our experience of postoperative fast-tracking of children undergoing cardiac surgery during a charitable mission in Venezuela, where resources and equipment were severely limited. During our stay, 11 children, with a median age of 2 years, underwent total correction of tetralogy of Fallot. The median duration of ventilation was 2.5 hours, and all patients were extubated within 12 hours of surgery. Effective analgesia was achieved without the need for continuous intravenous infusions of opiates. This experience shows that early extubation can safely be carried out in well-selected patients after surgery to correct congenital cardiac malformations. This allows faster throughput of patients, and helps provide an efficient and cost-effective service.
TL;DR: The present recommendations should be considered as consensus statements, and as describing accepted practice, which could be used as a basis for ensuring and improving the quality of future care.
Abstract: The aims of these recommendations are to improve the outcome for patients after, and to provide acceptable standards of practice of, therapeutic cardiac catheterisation performed to treat congenital cardiac disease. The scope of the recommendations includes all interventional procedures, recognising that for some congenital malformations, surgical treatment is equally as effective as, or occasionally preferable to, interventional treatment. The limitations of the recommendations are that, at present, no data are available which compare the results of interventional treatment with surgery, and certainly none which evaluate the numbers and types of procedures that need to be performed for the maintenance of skills. Thus, there is a recognised need to collect comprehensive data with which these recommendations could be reviewed in the future, and re-written as evidence-based guidelines. Such a review will have to take into account the methods of collection of data, their effectiveness, and the latest developments in technology. The present recommendations should, therefore, be considered as consensus statements, and as describing accepted practice, which could be used as a basis for ensuring and improving the quality of future care.
TL;DR: It is concluded that coronary arterial fistulas, although rare and potentially serious, are generally treatable.
Abstract: We reviewed 16 patients with coronary arterial fistulas seen between 1976 and 1997, and aged 2 days to 16 years, with a median age of 3.2 years. Only four patients were symptomatic: two had heart failure, one had exertional dyspnoea, and one infective endocarditis. The fistulas originated from the right coronary artery in seven patients, from the left coronary artery in seven, from both coronary arteries in one patient, while the origin was not clearly defined in the final patient. Associated cardiac anomalies were discovered in six patients, with three of the fistulas being diagnosed at the same presentation. Cross-sectional echocardiography had revealed a dilated coronary artery in 7 out of 11 subjects. The ratio of pulmonary to systemic flows ranged between 0.9 to 3.0, with a median of 1.5. Ten patients were referred for corrective surgery without any mortality. Trans-catheter closure was successfully undertaken in one patient, while spontaneous closure of the fistula was noted in two patients. We conclude that coronary arterial fistulas, although rare and potentially serious, are generally treatable.
TL;DR: At moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure.
Abstract: The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than -2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.
TL;DR: Severe mechanical haemolysis occurred in an 11-month-old boy after implantation of the new Amplatzer duct occluder and temporary balloon occlusion of the aortic ampulla was performed 4 days after the initial procedure, leading to prompt abolition of the ha Hemolysis.
Abstract: Severe mechanical haemolysis occurred in an 11-month-old boy after implantation of the new Amplatzer duct occluder. Temporary balloon occlusion of the aortic ampulla was performed 4 days after the initial procedure leading to prompt abolition of the haemolysis.
TL;DR: While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.
Abstract: OBJECTIVE To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies. BACKGROUND Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival. METHODS We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study. RESULTS We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure. CONCLUSIONS Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.
TL;DR: The data support the concept that anthracycline-induced cardiotoxicity is not clearly dose dependent, and myocardial high-energy phosphate metabolism may be impaired even in the absence of cardiomyopathy.
Abstract: ObjectivesWe aimed to investigate whether changes in high-energy phosphate metabolism after treatment of children and young adults with anthracycline can be demonstrated non-invasively by 31P magnetic resonance spectroscopyBackgroundAbnormal myocardial energy metabolism has been suggested as a mechanism for anthracycline-induced cardiotoxicity Deterioration in such has been shown in animal studies by resonance spectroscopyMethodsWe studied 62 patients, with a mean age of 135 ±5 years,37±43 years after a cumulative anthracycline dose of 270±137 mg/m2 Normal echocardiographic findings had been elicited in 54 patients The control group consisted of 28 healthy subjects aged 20±7 years Resonance spectrums of the anterior left ventricular myocardium were obtained at 15 Tesla using an image-selected in vivo spectroscopy localization techniqueResultsThe ratio of phosphocreatine to adenosine triphosphate after blood correction was 109±043 for the patients, and 136±036 (mean±SD)for controls (p = 0005), with a significantly reducedmean ratio even in the subgroup of patients with normal echocardiographic results ( l11 ± 0 44 versus136±036, p=001) The ratio did not correlate with the cumulative dose of anthracycline The ratio of phosphodiester to adenosine triphosphate was similar in patients and controls (090±056 versus 088±062)ConclusionsIn patients treated with anthracyclines in childhood, myocardial high-energy phosphate metabolism may be impaired even in the absence of cardiomyopathy Our data support the concept that anthracycline-induced cardiotoxicity is not clearly dose dependent
TL;DR: Implantation of a balloon-expandable stent was used successfully in 4 children with hypoplasia of the transverse aortic arch occurring after repair of coarctation, leading to both anatomical and physiological relief of obstruction.
Abstract: Hypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.
TL;DR: This investigation requires a multiplane transesophageal echocardiographic probe, since only oblique planes will demonstrate the entrance of the systemic and pulmonary veins and their relationship to the defect.
Abstract: Closure of so-called "secundum" atrial septal defects with a device inserted on a catheter necessitates precise delineation of their morphology. Echocardiography is the diagnostic method of choice to demonstrate this morphology, and to differentiate such defects located within the oval fossa from the other variants producing an interatrial communication. Precordial echocardiography usually allows selection of cases likely to be suitable for closure in this fashion. This selection is based on the localisation and the size of the deficiency in the oval fossa, the length of the interatrial septum, and the adequacy of the infolded rims surrounding the defect. Suitability for closure is reevaluated by transesophageal echocardiography: either as a separate investigation or at the start of the interventional catheterisation. This investigation requires a multiplane transesophageal echocardiographic probe, since only oblique planes will demonstrate the entrance of the systemic and pulmonary veins and their relationship to the defect. Transesophageal echocardiography sen es as an important monitoring tool during the interventional procedure. As such, it is a necessary adjunct to fluoroscopy. The stretched diameter of the defect measured with a balloon is the main determinant of the choice of the type and size of the device. This diameter can be measured fluoroscopically, as well as on echo. Colorflow mapping serves to rule out residual shunting during the occlusion of the defect with the balloon. During deployment of the device, constant echocardiographic visualisation of the device and its position relative to the atrial septum facilitates proper placement. Such constant visualisation can only be provided by repeated quick acquisitions of multiple planes. Once the device is released, the investigator should continue to record the position of the device, and assess the potential for residual shunting. Most of the devices show some subtle change in position during the first 20 minutes after implantation.
TL;DR: Physical growth improved significantly following the surgical procedures, and the concentrations of the growth factor measured in the serum were not different from levels in healthy children, suggesting normal nutritional status in both palliative situations.
Abstract: INTRODUCTION The total cavopulmonary connection, and the bidirectional Glenn anastomosis, are widely used for palliation of patients with complex functionally univentricular hearts. Little attention has been paid to the potential for postoperative growth in children after these operations, which are now performed at increasingly younger age. MATERIAL AND RESULTS Physical growth, and levels of insulin-like growth factor I in the serum, were measured in 20 patients, aged 11.5 +/- 5.6 years, 2 (0.5-6) years after a total cavopulmonary connection in 12, or a Glenn anastomosis in 8. All patients were in functional class I or II of the categorisation of the New York Heart Association, with excellent haemodynamic and angiographic findings. None of the patients had clinical signs of protein losing enteropathy. Controls included 33 healthy children, aged 11.5 +/- 2.7 years. Preoperatively, the mean Z-scores for weight and height were negative, -1.1 +/- 0.8 and -0.5 +/- 1.5. At follow-up, both parameters had improved significantly by 1.1 +/- 0.9 and 0.8 +/- 1.2 percentiles, and Z-scores were comparable between the two groups (p=0.81 for weight and p=0.88 for height). No correlations were found between haemodynamics and the improvement in growth noted during follow-up. Increases equal to, or greater than 2 standard deviations for weight and height were seen only in children undergoing surgery before the age of 5 years. A significant correlation between age at operation and improvement in growth, however, could not be found. Levels of growth factor measured in the serum were not statistically different from levels in healthy children for either group of patients (p=0.07 for girls and p=0.37 for boys). CONCLUSION Physical growth improved significantly following the surgical procedures. The concentrations of the growth factor measured in the serum were not different from levels in healthy children, suggesting normal nutritional status in both palliative situations.
TL;DR: Balloon angioplasty failed to reduce the pressure gradient across the coarctation, so a stent was implanted retrogradely as an emergency procedure and end-to-end anastomosis created without complications at seven months of age.
Abstract: We describe a neonate with critical coarctation of the aorta. Since treatment with Prostaglandin E1 had failed to reopen the arterial duct, and surgery was deemed to be associated with an unacceptably high risk in this unstable neonate, it was decided to perform balloon dilation of the coarctation as a palliative procedure to stabilize the patient. Balloon angioplasty failed to reduce the pressure gradient across the coarctation, so a stent was implanted retrogradely as an emergency procedure. Subsequently, the stented segment was resected surgically and end-to-end anastomosis created without complications at seven months of age.