bluuebunny/crossref_metadata_2025 huggingface dataset:
Isolated origin of the left subclavian artery from the left pulmonary artery
Authors: Russell Jennifer L , Smallhorn Jeffrey F. , Black Michael D. , Hornberger Lisa K.
Published: August 2008

We describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.
Left ventricular function in adolescents and adults with restrictive ventricular septal defect and moderate left-to-right shunting
Authors: Magee Alan G. , Fenn Lynn , Vellekoop Joric , Godman Michael J.
Published: August 2008

Background

The long-term haemodynamic effects of a restrictive ventricular septal defect permitting moderate left-to-right shunting are not known.

Patients and methods

Echocardiographic measure ments of left heart dimensions and function were compared between a group of 9 older children and adults (median age 21 years, range 12–24.5 years) having restrictive ventricular septal defects (median Qp/Qs 1.7, range 1.4–2.1) and a group of 10 age matched controls.

Results

Left ventricular mass indexed to body surface area was significantly greater in subjects than in controls (102±29 vs. 75±13 g/m2, p=0.02), although there was no significant difference in the ratio of mass to volume. There were no significant differ ences between indexes of left ventricular systolic function. Ratios of peak early to late diastolic ventricular filling were lower in those with septal defects (1.5±0.3 vs. 1.8±0.3, p=0.013), but there were no differences in other indexes of diastolic function.

Conclusions

Resting left ventricular function does not appear to have deteriorated by early adult life in patients with restrictive ventricular septal defects and moderate volume loading. This would support a continued conservative approach to management in these patients.
Neonatal pulmonary hypertension during extracorporeal membrane oxygenation
Authors: Tanke Ronald B. , Daniëls Otto , van Lier Henk J. , van Heyst Arno F. , Festen Cees
Published: August 2008

Objectives

This prospective study was designed to monitor severe pulmonary hypertension during extra corporeal membrane oxygenation using echo Doppler variables.

Background

All neonates treated with extracorporeal membrane oxygenation also have severe pulmonary hypertension. A study which monitors the reaction of the pre-existing pulmonary hypertension during extracorporeal oxygenation by frequent sampling of those variables related to pulmonary pressure is still lacking. Such a study is necessary to analyze the complex haemodynamic changes in patients undergoing extracorporeal membrane oxygenation.

Method

In 29 neonates, we estimated pulmonary arterial pressure using peakflow velocity of regurgitation across the tricuspid- and pulmonary valve, peakflow velocity of shunting across persistent arterial ductus, and systolic time intervals of the right ventricle. Correlation between the several estimations of pulmonary arterial pressure were analysed with the Spearman correlation coefficient.

Results

Systolic pulmonary arterial pressure measured by the velocity of tricuspid regurgitation illustrated severe pulmonary hypertension prior to extra corporeal membrane oxygenation (mean 63 mmHg, sd 20). Similar levels for the systolic pulmonary arterial pressure could be derived (mean 73 mmHg, sd 17) from ductal shunting. A fair correlation of 0.76 (p< 0.002) could be demonstrated. Pulmonary hypertension responded well and quickly to treatment by extra corporeal membrane oxygenation, with reductions within 24 hours to mean systolic levels of 35 mmHg, sd 23. This very early reaction has not previously been demonstrated and could be of importance in defining parameters for weaning from cardiopulmonary bypass. Diastolic pulmonary arterial pressure was investigated because of its relation to vascular resistance. It proved more difficult to measure because of the low incidence of pulmonary regurgitation. Derived diastolic pressures did not show any good correlations.

Conclusion

Pulmonary hypertension is well documentated prior to extra corporeal membrane oxygenation and respons very quickly to the institution of treatment. Ultra sound techniques are indicated at the bedsite, and prove useful in monitoring pulmonary blood pressure during the procedure.
Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.
Authors: Gerlis L. M. , Durà-Vilà G. , Ho S. Y.
Published: August 2008

We present two cases in which there was isomeric arrangement oi the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided. In the other case, there was left isomeric broncho-pulmonary morphology, and both liver and stomach were right-sided. These unusual combinations show the need for full description of the morphology of other organs, and the venoatrial connections, in cases with isomeric arrangement of the atrial appendages. They also demonstrate that connection of the inferior caval vein cannot be taken as a reliable marker of an atrium having a morphologically right appendage.
Anomalous origin of the main stem of the left coronary artery from the non-facing sinus of valsalva associated with sudden death in a young athlete
Authors: Hamamichi Yuji , Okada Eikichi , Ichida Fukiko
Published: August 2008

A young female athlete is described with anomalous origin of the main stem of the left coronary artery from the non-facing sinus of Valsalva who sustained myocardial infarction and died suddenly after physical exertion. Autopsy findings illustrated the mechanistic importance of acute angle take-off of the left main coronary artery and a slit-like orifice, which was likely compressed and obstructed by acute expansion of the aortic wall. This rare type of coronary anomaly has been regarded as having little clinical significance, but it can lead to sudden cardiac death under physical exertion.
Dependence on a respiratory ventilator due to an atrial septal defect
Authors: Motz Reinald , Grässl Gerhard , Trawöger Rudolf
Published: August 2008

An atrial septal defect is commonly thought of as a benign cardiac lesion especially in infancy. The haemodynamic consequences for the lungs, nonetheless, can be comparable to that produced by patency of the arterial duct. In a preterm boy, this lesion led to the development of broncho-pulmonary dysplasia, and dependence on a respiratory ventilator. He could be extubated shortly after surgical closure of the septal defect.
Aortic vasculopathy with aneurysm: a rare cause of fetal hydrops
Authors: Nissen Teo , Silverman Norman H. , Ursell Philip C.
Published: August 2008

An hydropic fetus seen at 28-weeks gestation had a saccular aortic aneurysm in the descending thoracic aorta. Histology disclosed marked fibrointimal hyperplasia, thrombus, and attenuation of the tunica media. The remainder of the descending thoracic aorta showed fibrointimal hyperplasia. We speculate that the narrowed lumen and rigid aortic wall resulting from this vasculopathy provided an increased afterload leading to cardiac failure.
Intermittent complete closure of the arterial duct
Authors: Chessa Massimo , Vettukattil Joseph J , Stümper Oliver
Published: August 2008

Intermittent functional closure of a patent duct has been reported both clinically and echocardio-graphically. We describe the case of a 6-year-old with intermittent complete closure of a patent duct occurring during attempted transcatheter closure. Recurrence of clinical and echocardiographic signs prompted restudy. A patent duct of moderate size was demonstrated and successfully occluded by coil embolization. The interventional aspects of this unusual condition are discussed.
Fetal hydrops due to a tachyarrhythmia progressing to organic pulmonary stenosis
Authors: Fesslova V. , Villa L. , Nicolini U.
Published: August 2008

We discovered supraventricular tachycardia with advanced hydrops in the setting of normal cardiac anatomy at 26 weeks of gestation which resolved successfully following administration of digoxin and flecainide to the mother. A female baby was born after a premature rupture of the membranes at 30.6 weeks. The neonate was in sinus rhythm, showed a progressive regression of right ventricular insufficiency, but developed signs of acquired pulmonary valvar and subvalvar stenosis at 2 months of age.
Diagnosis and natural history of isolated congenital pulmonary regurgitation in fetal life
Authors: Mori Kazuhiro , Hayabuchi Yasunobu , Kuroda Yasuhiro
Published: August 2008

We describe a rare instance of isolated pulmonary regurgitation caused by a dysplastic pulmonary valve which was detected prenatally. Fetal echocardiography demonstrated severe pulmonary regurgitation, and progressive cardiomegaly because of right ventricular volume overload. After birth, conservative therapy was successful in alleviating the pulmonary vascular resistance, and the pulmonary regurgitation gradually decreased.
Pulmonary and systemic arteriovenous fistulas in patients with left isomerism
Authors: Kawata Hiroaki , Kishimoto Hidefumi , Ikawa Seiichiro , Ueno Takayoshi , Nakajima Tohru , Kayatani Futoshi , Inamura Noboru , Nakada Takeshi
Published: August 2008

Abstract Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.
Early clinical experience with use of the ‘Amplatzer Septal Occluder’ device for atrial septal defect
Authors: Wilkinson James L , Goh Tiow Hoe
Published: August 2008

Device closure of oval fossa atrial scptal defects with the Amplatzer Septal Occluder was performed in 26 patients ranging in age from 0.89 to 60.44 years. In eight additional patients no device implant was performed because of the presence of multiple defects or because the defect was of a size unsuitable for closure with the devices currently available. The strectched diameter of the defects that were closed ranged from 4 to 23 mm (mean 14±5.4 mm) and device sizes ranged from 4 to 24 mm. Two devices were unstable, of which one embolized to the right atrium after release. Both devices were retrived at the same procedure. One of these parients subsequently underwent a successful device closure of his defect using a larger (24-mm) device. Three patients had multiple defects, which were successfully closed with a single device. At 1-month follow-up 23/26 (88%) and at 3-month follow-up 22/24 (92%) patients had complete closure of their defects, while two had residual shunts. One further patient who had complete closure of his defect at 1-month post-implant had his device removed and his atrial septal defect patched surgically 8 weeks after device closure. This was done as a result of the development of a vegetation affecting the device after an episode of septicaemia, which was not relate to the cardiac problems. There was no procedure-related morbidty or martality and all patients remain well at the present time.
Growing up with congenital heart disease: the dilemmas of adolescents and young adults
Authors: Tong Elizabeth M. , Sparacino Patricia S.A. , Messias DeAnne K.H. , Foote Dru , Chesla Catherine A. , Gilliss Cartherine L.
Published: August 2008

Abstact

Advances in diagnosis, medical management and surgical intervention have improved the longevity and quality of life for children with congenital heart disease. Despite this, research studies specifically examining the psychosocial concerns of adolescents and young adults with congenital heart disease are few. To explore the subjective experiences and dilemmas of this population during the transition from adolescence to young adulthood, we interviewed, using a semi-structured protocol, a convenience sample of nine adolescents and young adults. Using analytic procedures inherent in Grounded Theory methodology, seven themes were identified: the dilemma of normality; dilemmas in disclosure; dilemmas in strategies for management of illness; the challenge of social integration versus social isolation; the challenge of dependence versus independence; the challenge of uncertainty; and strategies for coping. An understanding of these experiences by health professionals can be beneficial in helping this clinical population as they grow up and face the challenges of an uncertain, yet promising, future.
Verapamil therapy in infants with hypertrophic cardiomyopathy
Authors: Moran Adrian M. , Colan Steven D.
Published: August 2008

We sought to evaluate the saftey and efficacy of acure and chronic treatment with verapamil infanta with hypertrophic cardimyopathy. Prior studies have shown an improvement in adluts with hypertrophiuc cardiomyophathy who were treated with verapamil. Acutely, it reduced the degree of left ventricular outflow tract abstruction. Chronic therapy was associated with an improvement in sysmptoms ans incresed long-term survial. To date, no data are available on the efficacy of this drug in infants with hypertrophic cardiomyopathy. We evaluated prospectively the safety and efficacy of verapamil in infants. The acute and chronic effects of verapamil on infants with an echocardiographic diagnosis of hypertrophic cardiomyopathy were evaluated at a single institution between 1980 and 1994, with long-term follow-up available until 1996. Acute effects of an intravenous bolus of 0.1 mg/kg and infusion at 0.007 mg/kg/min were evaluated, where possible, in the cardiac catheterization laboratory. Oral verapamil at 3–5 mg/kg/day was started after catheterization. Follow-up included serial clinical, echocardiographic and Holter monitoring. A total of 22 patients were studied, 17 having a presumed diagnosis of primary hypertrophic cardiomyopathy including three with Noonan's syndrome. Acute infusion of the drug was well tolerated by all, without adverse electrophysiological effects. Haemodynamic effects were consistent with a negative inotropic action with significant falls in cardiac index (4.6±1.2 to 4.1±0.9 l/min/m2), systolic blood pressure (88±16 to 82±14 mmHg) and gradient across the left ventricular outflow tract (nine patients 48.2±30.4 to 28.4±24.1 mmHg). End-diastolic pressure was unchanged (14.0±6.8 to 13.9±4.7 mmHg). Three patients with primary hypertrophic cardiomyopathy died (two while being treated). In the group with primary hypertrophic cardiomyopathy continuing with long-term treatment, follow-up revealed regression in two, progression in three (two died) and stability in 10. For those treated, there was a trend towards improvement in clinical status. Verapamil is well tolerated acutely in infants with hypertrophic cardiomyopathy. The outcome was considerably better in these patients compared with prior reports, though careful long-term assessment is needed.
Epidemiology and diagnosis of ventricular septal defect in Malta
Authors: Grech Victor
Published: August 2008

Malta is a small island with minimal changes in its population, making it an ideal location for epidemiological and historical studies dealing with congenital heart malformations. Ventricular septal defect was studied retrospectively from 1930 to 1994. A sharp and significant decline in age at diagnosis was found, predating echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence of this lesion as seen at birth, particularly of minor defects. The prevalence of ventricular septal defect from 1990 to 1994 was 3.85/1000 live births, with 3.O3/1OOO not needing surgery and 0.83/1000 requiring operative intervention. Half the defects closed spontaneously. The majority of ventricular septal defects overall, and those spontaneously closing, were muscular defects. The overall prevalence at birth was significantly higher than that reported in recent studies using similar methodologies, implying that the reported rate is more likely to be the true prevalence at birth of clinically detectable defects.
History, diagnosis, surgery and epidemiology of pulmonary stenosis in Malta
Authors: Grech Victor
Published: August 2008

The Maltese populartion constitutes an ideal location for epidemiological and historical studies dealing with congenital hearr malformations. Pulmonary stenosis was studied retrospectively from 1943 to 1994. A sharp, significant decline in age at diagnosis was found, which predates the introduction of echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence at birth of this lesion, patticularly of pulmonary stenosis not requiring intervention. The prevalence at birth of pulmonary stenosis from 1990 to 1994 was 1.65/1000 live births (95% CL: 1.21–2.24), with 1.11/1000 mild lesions (95% CI: 0.76–1.62) and 0.54/1000 lesions requiring intervention (95% CI: 0.31–0.92). The prevalence at birth overal was significantly higher than that reported in recent studies with similar methodologies due to the higher pickup of milder variants of pulmonary stenosis by echocardiography.Significantly more pulmonary more pulmonary stenosis was found in females than in males.
Rare forms of isolation of the subclavian artery: echocardiographic diagnosis and surgical considerations
Authors: McElhinney Doff B. , Silverman Norman H. , Brook Michael M. , Reddy V. Mohan , Hanley Frank L.
Published: August 2008

Isolation of the subclavian artery is an unusual anomaly in which the subclavian artery arises not from the aortic arch but from a pulmonary artery via an arterial duct. Such isolation most often occurs with a right aortic arch, and in lesions frequently associated with a right arc, such as tetralogy of Fallot. Since1994, we have undertaken surgery in four young infants with isolated subclavian arteries and unusual associated anomalies, including one with atrioventricular septal defect and common valvar orifice, two with interruption of a left aortic arch and one with interruption of a right aortic arch. In both patients with interrupted left arch, the isolated subclavian artery was diagnosed preperatively by echocardiography. We emphasize the significant issues.
Predictors of developmental outcomes in children with complete transposition
Authors: Gomelsky Alex , Holden E. Wayne , Ellerbeck Kathy A. , Brenner Joel I.
Published: August 2008

Cognitive, functional, educational achievement and behavioural measures were employed to assess neurobehavioral status in 57 of 60 participants who were initially enrolled in the Baltimore–Washington Infant Study, and who survived surgical correction of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). Charts were reviewed to investigate the relationship between birth variables, surgical strategy and developmental outcomes. Higher preoperative weight was associated with better outcomes on the Stanford–Biner Short-term Memory subtest, while lower preoperative oxygen tension was associated with better outcomes on the Abstract/Visual Reasoning subtest and a test of Visual–Motor Integration. Longer total bypass time was associated with poor outcomes on the Short-term Memory subtests. Higher average flow rates during cooling and rewarming were associated with higher scores in the test of short term memory but poorer outcomes on a test for visual motor integration. Longer cooling times were associated with higher scores on the test for Visual–Motor Integration. Patients suffering seizures scored lower on the Stanford–Biner Composite, as well as in their tests of achievement. The data indicate that non-verbal. skills may be particularly sensitive to variations in surgical strategies employed to correct complete transposition. Overt neurological events, such as seizures, were related to global deficits in intellectual functioning. Prospective studies evaluating systemic variations in surgical procedures and attempts to prevent and manage perioperative neurological events are important for further investigation of neurodevelopmental outcomes in children surviving surgical correction.
Possible sources of right-to-left shunting in patients following a total cavopulmonary connection
Authors: Buheitel Gernot , Hofbeck Michael , Tenbrink Ursula , Leipold Georg , Emde Jürgen v.d. , Singer Helmut
Published: August 2008

Despite a good haemodynamic result, many children have amildly decreased arterial oxygen saturation following a total cavopulmonary connection. Our study was performed to determine possible mechanisms of right-to-left shunting in these patients. We performed elective cardiac catheterization in 19 children at a mean interval of 3.6 years following a total cavopulmonary connection. The intrapulmonary right-to-left shunt, the intracardiac right-to-left shunt and the total right-to-left shunt were calculated under mechanical ventilation with 100% oxygen. The intrapulmonary right-to-left shunt was 10.8±3.5% of the pulmonary blood flow, and the total right-to left shunt accounted for 18.9±5.2% of the systemic blood flow. The intracardiac right-to-left shunt in patients with no relevant venovenous collaterals or leaks in the atrial tunnel was calculated at 6.4±3.0% of the systemic blood flow, while the intracardiac right-to-left shunt in patients with relevant collaterials or leaks accounted for 13.0±5.9% of the systemic blood flow. Since intrapulmonary arteriovenous fistulas were not demonstrated angiographically in any of our patients, the intrapulmonary right-to-left shunt is probably due to low ratios of perfusion to ventilation in some pulmonary segments. The intracafdiac right-to-left shunt was due to leaks across the interatrial baffle, collaterals between stystemic and pulmonary veins, and to the coronary sinus draining to the pulmonary venous atrium.
New approach in partial cavopulmonary connection
Authors: Paulista Paulo. P. , Santana Maria Virginia. T. , Henriques Neto Antonio. T. M. , Fontes Valmir F.
Published: August 2008

The development of pulmonary atteriovenous fistulas after bidirectional cavopuomonary operat-tions, such as the bidirectional Glenn shunt and Kawashima's procedure, has raised concern. Development of these fistulas, which are more frequent than initially thought, can represnt a limiting factor in the late outcome of these patients and may even limit the indication for these of surgery. Whether the fistulas can be reversed by transforming the surgical procedures has yet to be established. In the hope of avoiding this kind of complication, thought to be caused by the lack of passage of a hypothetical hepatic factor through the pulmonary circulation. wedevelped an inverred type of gidirectional cavopulmonary connection in which the blood coming from the liver perfuses immediately both lungs. This is made possi-ble by shuntiong via an intra-atrial tunnel the blood from the superior caval vein directly to the left atrium, and the blood from the inferior caval vein to the right branch of the pulmonary trunk (keeping its bifurcation intact). We describe findings in two patients undergoing successful surgery with this technique. Serial follow -up with cohtrast echocardiography did not show evidence of arteriovenous pulmonary fistulas.Despite our numbers being small, and the time of follow-up being limited, we believe that it is importantto document these and similar cases
Radiofrequency catheter ablation of multiple haemodynamically unstable ventricular tachycardias in a patient with surgically repaired tetralogy of Fallot
Authors: papagiannis John , Kanter Ronald J , Wharton J.Marcus
Published: August 2008

A patient with repaired tetralogy of Fallot presented with recurrent syncope and had multiple haemodynamically unstable ventricular tachycardias unresponsive to antiarrhythmic medications.Ventricular tachycardias became haemodynamically tolerated with amiodarone, procainamide and dopamine, permitting activation and entrainment mapping. Radiofrequency ablation of three tachycardia circuits was performed. Ventricular tachycardia could not be induced 1 week, and 3 and 9 months later.Radiofrequency ablation is feasible for multiple, haemodynamically unstable ventricular tachycardias in repaired tettralogy of Fallot
Sudden death 10 years after patch aortoplasty for coarctation
Authors: Hauser Michael , pollock James , Wilson Neil
Published: August 2008

A 25-year-old man died 10 years after a Dacron patch was used to repair a coarctation of the aorta. Death was due to rupture of an unrecognized aneurysm at the site of the patch. After the initial operation at the age of 15 years, there had been no signs of residual or recurrent obstruction. He had no evidence of hypertension and was discharged some years later from regular hospital follow-up to the care of his general practitioner. We strongly recommend that patients who have undergone repair of aortic coarctation by patch aortoplasty should have lifelong follow-up in cardiac units with imaging facilities for monitoring aortic dilation. We would now recommend surgical intervention in the presence of progressive aortic dilation.
Serious sequels of Kawasaki disease
Authors: Kim Nam Su , Menahem Samuel
Published: August 2008

A male infant, aged 2 month, with Kawasaki disease had a myocardial infarction despite intravenous infusions of gamma globulin and aspirin at high dosage. He developed progressively a thin walled, dilated aneurysm of the apex of the left ventricle which became lined with thrombus despite treatment with warfarin. Another boy, aged 6 years, was noted on the 10th day of the evolution of Kawasaki disease to have developed a giant aneurysm of the main stem of the left coronary artery. Despite infusion of gamma globulin, the aneurysm remained unaltered and developed a thrombus. The thrombus resolved following treatment with warfarin, though the giant aneurysm has persisted. These two cases illustrate the serious consequences that can follow Kawasaki disease despite management optimal by current standards.
Delayed hemopericardium due to trivial chest trauma
Authors: Çil Ergün , Senkaya Isik , Tarim Ömer
Published: August 2008

We report two cases of hemopericardium occurring in seven-month-old and 12-year-old boys, who had no history of major trauma. The possible cause of the hemopericardium for the infant was falling from a bed which was 75 cm high two weeks prior to the admission. The 12-year-old boy had fallen from a chair and damaged his chest 4 weeks previously. Their coagulation tests were all normal. By means of pericardiotomy, we drained 120 ml and 1200 ml of blood, respectively. The boys have now been well over follow-up periods of 24 and 18 months, respectively
Complete transposition in a chick embryo demonstrated by scanning electron microscopy
Authors: Männer Jörg , seidl Wolfgang , Steding Gerd
Published: August 2008

Chick embryos are frequently used as animal models when researching the developing heart. In the past, every attempt to induce complete transposition (the combination of concordant arrioventricular and discordant ventriculo-arterial connections) failed in chicks, suggesting that it might be impossible to develop a chicken modle for this malformation. We demonstrate, to the best of our knowledge, the first well-documented case of complete transpositon occourrine in the chick
Counselling strategies for parents of infants with congential herat disease
Authors: Menahem Samula
Published: August 2008

Congenital herat disease is a significant cause of morbidity and mortality in the newborn. Its diagnosis may lead to a crisis in the affercted families; there are the percerived implications of having an abnormality of so vital on organ. To that may be added the assumed guilt or blame, grief and at times anger, frequently experienced by parents of abnormal infants. It often befalls the paediatric cardiologist to initiate counselling while providing the expert information concerning the abnormality and its optimum management

Such counselling differs from that needed for minor lesions as compared for more complex abnormalities where a fatal outcome may ensure. While it is important to provide an accurate diagnosis and management plan to the parents, early detailed information is often confusing and may not be assimilated at a time of great stress. The parents seem more concerned as to whether the infant will survive, what the long term outlook will be, whether he or she will attend school, play, work and so on. With the more severe cardiac abnormalities, especially where there is a family history, one need be aware of the often perceived guilt of the parents. At times, it may be necessary to help the parents retain sufficient ‘self-control’, delaying the grieving process to enable them to contribute to the decision making. Where the infant has died, a follow-up appointment can facilitate grieving and help deal with unresolved issues

Through skilled counselling, the cardiologist in addition to his/her diagnostic and management skills, may meaningfully influence the ongoing care of the infant. They may help avoid the development of unrealistic fears or an over-optimistic outlook, thereby fostering the normal development of the child
The Brock Procedure (closed infundibular resection) for Fallot's tetralogy: 43 years later
Authors: Gerlis L. M. , Smith C. E. T. , somerville J
Published: August 2008

Innovative cardiac surgery (‘blind’ or indirect infundibular resecion) for tetralogy of Fallot on a child of 4 years was followse by survial for 43 years without further surgery. The patinent remained well until about one year before death, when he developed clinical features of progressive biventricular failure associated with pulmonary hypertension and incompetence of the pulmonary and tricuspid value. Postmortem examination showed severe damage to one of the leaflets of the pulmonary valve, interpreted as due to indavertent avulsion during the original surgical procedure. A large ventricular septal defect was present but there was no residual subpulmonary infundibula obstruction
Pediatric cardiac surgery in Indonesia
Authors: Asou Toshihide , Rachmat Jusuf
Published: August 2008

Pediatric cardiac surgery in Indonesia first developed thanks to the cooperation of various cardiac centers abroad. The establishment of the ‘Harapan Kita’ National Cardiac Center in 1985 was one of the most important initial steps. Thereafter, the discipline advanced remarkably in terms of the number of the operations performed and the variety of the diseases treated and, as a result, the surgical outcome also improved. Numerous problems remain to be solved. Only 1% of the children with congenital heart disease are today properly treated in Indonesia. Some of the underlying problems responsible for this situation include a shortage of pediatric cardiac professionals, the lack of the information and education on the part of the patients, and a shortage of funding, both privately and publicly. It would thus be welcome for pediatric cardiac surgeons, cardiologists and nurses in Indonesia to learn about congenital heart disease from doctors and nurses in advanced countries in order to improve the outlook at home.
Sterile vegetations in children with severe tetralogy of Fallot
Authors: Cilliers Antoinette M. , Vanderdonck Kathy M. , du Plessis Jan P. , Cronje Stefanus L. , Levin Solomon E.
Published: August 2008

The finding of bland, sterile vegetations in children with severe tetralogy of Fallot is unexpected, and to our knowledge, has not been reported previously. Eight patients diagnosed with tetralogy between January 1993 and July 1997 had sterile vegetations proven by histological and microbiological evaluation, in their right ventricular outflow tracts. Four of these patients were experiencing severe hyper-cyanotic spells, and four had severely reduced effort tolerance at presentation. They all underwent cardiac catheterization and were submitted for surgical repair. At surgery, the vegetations were thought to be causing further narrowing of the already tight fibrotic infundibular stenosis. Two of these patients had evidence of damaged valves, without evidence of active endocarditis. Although initially sterile, these vegetations, may in some instances, become infected.
Anomalous origin of one pulmonary artery from the ascending aorta: 36 years' experience from one centre
Authors: Abu-Sulaiman Riyadh M. , Hashmi Aijaz , McCrindle Brian W. , Williams William G. , Freedom Robert M.
Published: August 2008

Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16).

Conclusion

While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.
Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy
Authors: McElhinney Doff B. , Mohan Reddy V. , Silverman Norman H. , Brook Michael M. , Hanley Frank L.
Published: August 2008

Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpul-monary stenosis. A single patch technique, with closure of the zone of apposition (‘cleft’) in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosu-perior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.
Transcatheter closure of atrial septal defects within the oval fossa: medium-term results in children using the ‘ASDOS’-technique
Authors: Hausdorf Gerd , Schneider Martin , Fink Christoph , Neudorf Ulrich , Fischer Gunther , Tynan Michael , Friedli Beat
Published: August 2008

Objectives

The purpose of this study was to evaluate the safety and efficacy of the ASDOS-tech-nique (Sulzer-Osypka GmbH, Germany) for transcatheter closure of atrial septal defects within the oval fossa.

Background

Although several attempts have been made to occlude defects within the oval fossa by transcatheter techniques, none of these has gained general acceptance.

Methods

Patients with a defect in the oval fossa measuring equal to or less than 20 mm diameter, with a residual septal rim of 5mm or greater, body weight greater than 10 kg, with clinical indications for surgical closure were considered for transcatheter closure. Follow-up investigations were performed at discharge, after 1, 3, 6 and 9 months, as well as after 1 and 2 years.

Results

Of 78 patients considered for closure, a device was inserted in 41 patients (53%), with success being achieved in 40 patients (98%). The ages ranged from 1.1 to 15 years (7.8 ± 1.92 years), the ’stretched’ diameter of the defect from 10 to 20 mm (14.7 ± 2.60 mm), and the diameters of the inserted devices from 25 to 45 mm (33.2 ± 5.43 mm). Transient impairment of atrioventricular conduction occured in 4 patients. During the follow-up of 23.0 ± 5.6 months elective surgical closure of a residual shunt was performed 26 months after insertion of the devcie in one patient. None of the other patients required surgery, hospitalisation or medical treatment, and none is requiring further treatment of the defect within the oval fossa. Fracture of one arm of the device occurred in 4 patients, but the fractured arms are in an unchanged and stable position after a period of at least 19 months.

Conclusions

Our medium-term data show that transcatheter closure in children of defects within the oval fossa can be performed with a high efficacy and safety using the ASDOS-device.
Extracardiac conduit fontan for children with heterotaxy and functionally single ventricle
Authors: Mainwaring Richard D. , Lamberti John J.
Published: August 2008

Children with functionally single ventricle in the setting of visceral heterotaxy (isomerism) may present a surgical challenge at the time of Fontan completion because of anomalies of systemic and pulmonary venous drainage. We have used an extracardiac conduit in this population to direct inferior caval venous blood to the pulmonary arteries.

Over the past five years, nine children with heterotaxy and a functionally single ventricle underwent correction by placement of an extracardiac synthetic (Gore-Tex) conduit. All patients had previously undergone a bidirectional Glenn procedure. Age and weight at the time of insertion of the extracardiac conduit were 26±15 months, and 11±2 kilograms, respectively.

Results

Of the nine children, six had an uneventful recovery. One developed elevated venous pressures and required a ’fenestration procedure’. Two patients developed pleural effusions. Median length of stay in hospital was 10 days. All children are alive and well, with follow-up of 19±16 months. There have been no thromboembolic complications.

Conclusions

The extracardiac conduit has worked well in our experience for the completion of the Fontan circulation in children with functionally single ventricle in the setting of visceral heterotaxy.
Results of 546 Blalock-Taussig shunts performed in 478 patients
Authors: Jubair Khalid A. Al , Fagih Mohamed R. Al , Jarallah Abdullah S. Al , Yousef Saad Al , All Khan M.A. , Ashmeg Abdullah , Faraidi Yahya Al , Sawyer William
Published: August 2008

Between 1983 and 1995, 546 Blalock-Taussig shunt procedures were performed in 472 patients: 128 (23.0%) were classical shunts, 90 of them on the same side as to the aortic arch, and 418 (77.0%) were modified shunts, 182 on the same side of the arch. At the time of surgery, 78 patients were aged below one week, 270 from one week to 12 months, and 198 patients were over one year of age. The mean pre-operative arterial saturation (71.7%±16.5%) was significantly increased to 83%±17.9% imme-diately after the procedure (p=0.017). The overall hospital mortality rate was 2.9% (16/546), with rates of 2.3% (3/128) for the classical, and 3.1% (13/418) for the modified shunts (p= not significant). The rate was significantly higher, however, for classical shunts when the pulmonary arterial diameter was less than 4 mm (15.4% versus zero; p=0.047), though this relationship was reversed for modified shunts (zero versus 3.6%; p=0.338). Early mortality was significantly influenced by the age at surgery, 5/78 (6.4%) in patients aged below 1 week, 3.7% between 1 week and 1 year, and 0.5% over 1 year (p=0.019). Early mortality was also significantly increased in patients weighing 3kg or less, 8/156 (5.1%), versus 3/303 (1.0%), p=0.037. Overall, 51 shunts failed (9–3%), 10 early and 41 late. Early failure was significantly increased in patients weighing 3kg or less, 8/156 (5.1%) versus 3/303 (1.0%), p=0.016. The overall early failure rate was 1.4% (3/215) when heparin was administered intra-operatively and for 48 hours postoperatively, in contrast to an early failure rate of 3.4% (7/203) when heparin was not used (p = 0.294). Overall rates of failure during follow-up were 9.1% (17/188) in heparinized patients versus!3.6% (24/177), (p=0.173) in non-heparinized patients. Failure of classical shunts was 10.2% (13/128), compared with 6.7% (28/418) for modified shunts (p=0.195). Failure was more common overall if the pulmonary arterial diameter was less than 4 mm, 14.7% (9/61), as opposed to 8.7% (26/300) when the diameter was 4 mm or greater, (p=0.144). Administration of aspirin during follow-up after the modified shunt procedure reduced failure from 11 % (18/163) to 6.7% (10/150), p=0.176. Classical or modified Blalock-Taussig shunts, either on the same side or opposite to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. The use of intra- and post-operative heparin appears to reduce the overall rate of failure, and the administration of aspirin during follow-up appears to reduce failure of modified Blalock-Taussig shunts.
Covert fears, anxiety and depression in congenital heart disease
Authors: Gupta S. , Giuffre R.M. , Crawford S. , Waters J.
Published: August 2008

This study compared anxiety, fears, depression and behavioural problems as occurring in children with congenital heart disease, comparing them with samples of normal children. It further considered the influence of maternal anxiety, as well as analyzing a subgroup of children with cyanotic forms of congenital heart disease to determine if they were at higher risk than acyanotic children for the problems identified.

Method

We recruited 40 consecutive children with congenital heart disease without obvious psychoso-cial problems from the Cardiology clinic at the Alberta Children‘s Hospital. Of the 40 children, 39 families consented to have the children participate, of which 24 were cyanotic and 15 acyanotic. Children completed the revised versions of the Fear Survey Scale-Revised and the Child Manifest Anxiety Scale as well as the Child Depression Inventory. Mothers completed the Child Behaviour Checklist, and the State Trait Anxiety Scale.

Results

Children with congenital heart diseases demonstrated more medical fears, and more physiological anxiety, than the normative samples. More specifically, children with cyanotic forms of congenital heart disease demonstrated more fears of the unknown, physiological anxiety, depression, and delinquent behaviors than the acyanotic children with congenital heart disease. Mothers of the children with cyanotic forms of congenital heart disease scored higher on both the state and trait scales, with higher maternal anxiety correlating with higher anxiety, medical fears and behavioral problems in the child.

Conclusion

In a clinical setting, children with congenital heart diseases who do not present with psychological adjustment problems are still at risk for covert physiological anxiety, medical fears, depression and behavioral problems. The children with cyanotic malformations represent a subgroup at higher risk for these problems, which may be further exacerbated by increased maternal anxiety.
Spontaneous closure of muscular ventricular septal defect identified by echocardiography in neonates
Authors: Du Zhong-Dong , Roguin Nathan , Wu Xing-Jian
Published: August 2008

Muscular ventricular septal defects were diagnosed by echocardiography in 97 neonates within 7 days of birth. In 82 of the neonates (84.5%), the defect was solitary, while 15 had multiple defects. The solitary defects was located at mid-septal, apical, anterior and inlet locations in 42 (51.2%), 21 (25.6%), 14 (17.1%) and 5 (6.1%) neonates, respectively. Multiple defects occurred in the apical, anterior and mid-septal areas. The diameter of the solitary defects ranged from 1 to 6 mm (2.3 ± 0.8 mm), while the multiple lesions were 1 to 4 mm in diameter (2.1 2.3 ± 0.8 mm 0.8 mm) in 28 instances in which they could measured. It proved possible to follow 79 of the patients for period of 10 to 13 months. The defects closed spontaneously in 56 (84.8%) of 66 patients with a single defect, and in 7 (53.8%) of 13 of those with multiple defects (P<0.05). For the solitary defects, the position and size were factors determining the likelihood and speed of closure. Defects located at the apical septum, or defects larger than 4 mm in diameter, closed slowly and at a later stage. Echocardiography is an useful technique in establishing of natural history of muscular ventricular septal defects encountered in neonates.