bluuebunny/crossref_metadata_2025 huggingface dataset:
Supraventricular escape rhythms during transient episodes of bradycardia in preterm infants
Authors: Andriessen Peter , Koolen André M. P. , Bastin Frank H. , Lafeber Harrie N. , Meijler Frits L.
Published: August 2006

Objective: To evaluate the origin of transient episodes of sinus bradycardia, atrial escape rhythm, and atrioventricular nodal escape rhythm in preterm infants. Material and methods: The study was observational, and was carried out in a third level neonatal intensive care unit. We studied 19 spontaneously breathing infants born healthy but prior to term, the examinations being carried out between the ages of 3 and 28 days. The mean gestational age was 29.2 ± 1.9 weeks, and the mean birth weight was 1154 ± 264 g. Transient episodes of bradycardia were defined as a decline in heart rate equal to or greater than 25% from baseline, lasting for at least 3 successive RR-intervals. To discriminate between different types of escape mechanisms, we used the P wave and the P axis of the electrocardiogram. Sinus bradycardia was diagnosed when the P axis was from +0 to +90 degrees; atrial escape rhythm when it was from +91 to +359 degrees, and atrioventricular nodal escape rhythm when the P wave was absent, hidden, or followed the QRS complex. Results: The mean P axis was +50 ± 11 degrees. We observed 60 transient episodes of bradycardia in 11 of the 19 infants. Of these, 34 (57%) were classified as sinus bradycardia, and 13 (22%) as atrial escapes. Atrioventricular nodal escapes developed during 6 episodes (10%), while 7 episodes (11%) could not be classified. Conclusions: Atrial excitation as evidenced by the P axis during sinus rhythm is similar in very preterm infants to that seen in children and adults. Escape rhythms with different origins occur during transient episodes of bradycardia in healthy preterm infants. In at least one third, the episodes are due to atrial or atrioventricul ar nodal escape.
Development of the human pulmonary vein and its incorporation in the morphologically left atrium
Authors: Webb Sandra , Kanani Mazyar , Anderson Robert H. , Richardson Michael K. , Brown Nigel A.
Published: August 2006

Objective: Using a newly acquired archive of previously prepared material, we sought to re-examine the origin of the pulmonary vein in the human heart, aiming to determine whether it originates from the systemic venous sinus (“sinus venosus”), or appears as a new structure draining to the left atrium. In addition, we examined the temporal sequence of incorporation of the initially solitary pulmonary vein to the stage at which four venous orifices opened to the left atrium. Methods: We studied 26 normal human embryos, ranging from 3.8 mm to 112 mm crown-rump length, and representing the period from the 12th Carnegie stage to 15 weeks of gestation. Results: The pulmonary vein canalised as a solitary vessel within the mediastinal tissues so as to connect the intraparenchymal pulmonary venous networks to the heart, using the regressing dorsal mesocardium as its portal of cardiac entry. The vein was always distinct from the tributaries of the embryonic systemic venous sinus. The orifice of the solitary vein became committed to the left atrium by growth of the vestibular spine. During development, a marked disparity was seen between the temporal and morphological patterns of incorporation of the left-sided and right-sided veins into the left atrium. The pattern of the primary bifurcation was asymmetrical, a much longer tributary being formed on the left than on the right. Contact between the atrial wall and the venous tributary on the left initially produced a shelf, which became effaced with incorporation of the two left-sided veins into the atrium. Conclusions: The initial process of formation of the human pulmonary vein is very similar to that seen in animal models. The walls of the initially solitary vein in humans become incorporated by a morphologically asymmetric process so that four pulmonary veins eventually drain independently into the left atrium. Failure of incorporation on the left side may provide the substrate for congenital division of the left atrium.
Cardiac complications in children following infection with varicella zoster virus
Authors: Abrams Dominic , Derrick Graham , Penny Daniel J. , Shinebourne Elliot A. , Redington Andrew N.
Published: August 2006

Infection with varicella zoster virus, leading to chicken pox in susceptible hosts, is usually a benign self-limiting disease conferring immunity in those affected. Cardiac complications are rare, but when present may lead to severe morbidity or mortality.

We have recently encountered three children, all of whom developed significant cardiac complications secondary to infection with varicella. Myocarditis has long been associated with such infection. The pathological mechanism is presumed similar to other cardiotropic viruses, where both direct cytopathic and secondary auto-immune effects contribute to myocardial cellular destruction and ventricular dysfunction. Complications include arrhythmias and progression to dilated cardiomyopathy.

Pericarditis, and secondary pericardial effusion, related to infection with the virus is most commonly associated with secondary bacterial infiltration. Both cardiac tamponade and chronic pericardial constriction may result.

Endocarditis complicating varicella has only been described in the last fifteen years, and is associated with the emergence of virulent strains of both streptococcus and staphylococcus, the two organisms most commonly associated with endocarditis. The exact mechanism by which varicella causes secondary bacterial endocarditis remains unclear.

Whilst cardiac complications of infection with the varicella zoster virus are rare, the resulting complications are potentially life threatening. Evidence of varicella-induced carditis must be aggressively pursued in any child with signs of acute cardiac decompensation in whom chicken pox is confirmed or suspected.
Atrioventricular block in a new born with acquired long QT syndrome
Authors: Phillips John R. , Case Christopher L. , Gillette Paul C.
Published: August 2006

We report a case of 2:1 atrioventricular block associated with acquired long QT syndrome. A newborn presented to our neonatal intensive care unit with intermittent bradycardia due to 2:1 atrioventricular block. Initial evaluation showed QT prolongation and significant electrolytic abnormalities. After correction of the electrolytic imbalance, the QT interval normalized and atrioventricular block resolved. Compared to congenital long QT syndrome with 2:1 atrioventricular block, acquired long QT syndrome with comparable atrioventricular block has a benign prognosis, provided treatment is initiated quickly.
Use of terbutaline in the treatment of complete heart block in the fetus
Authors: Robinson Blair V. , Ettedgui José A. , Sherman Frederick S.
Published: August 2006

Between 1989 and 2000, 21 fetuses were diagnosed with complete atrioventricular block. Seven women with fetal ventricular rates of less than 60 were given oral terbutaline, and 6 of these had an initial increase in the fetal ventricular rate. Four fetuses (57%) maintained an increased average rate of 60 beats per minute and survived. Two fetuses returned to rates below 55 and died. The final fetus, with hypertrophic cardiomyopathy, was unresponsive. Terbutaline, therefore, is initially effective in raising the fetal ventricular rate, but this effect may be transient.
The spectrum of subdivided left atrium: diagnosis, treatment and outcome in eight patients
Authors: Knirsch Walter , Lewin Martin A. G. , Rein Joachim G. , Uhlemann Frank
Published: August 2006

From January 1996 to April 2001, we treated eight patients with subdivided left atrium. Their ages at diagnosis ranged from newborn to 6.4 years. Prominent clinical symptoms were failure to thrive, respiratory symptoms, demand for oxygen, and congestive cardiac failure. Diagnosis was made by transthoracic echocardiography in all cases. Cardiac catheterization was necessary only in those patients who had associated cardiac anomalies, or suspected signs of pulmonary hypertension. In 7 patients, surgery was performed immediately after diagnosis, but one preterm infant died before operation due to neonatal sepsis and respiratory distress syndrome. In the postoperative period, one patient developed a severe capillary leak syndrome, and died due to irreversible congestive cardiac failure. The other 6 patients have all been followed up, with good results in the short- and intermediate-term at a mean of 34.3 ± 20.2 months. The infants were thriving, had a reduction in the frequency of infections of the respiratory tract, no significant arrhythmias, and showed early recovery from pulmonary hypertension and right ventricular hypertrophy.
Cleft of the mitral valve in patients with Down's syndrome
Authors: Fraisse Alain , Massih Tony Abdel , Bonnet Damien , Sidi Daniel , Kachaner Jean
Published: August 2006

Differentiation between a cleft of the mitral valve and the cleft of the left side of an atrioventricular septal defect – a lesion commonly found in patients with Down's syndrome – is surgically important since the distribution of the conduction tissue varies between the 2 lesions. We sought to determine if cleft of the mitral valve occurs also in patients with Down's syndrome. We studied 5 patients with Down's syndrome and cleft of the mitral valve followed in our institution. Echocardiography showed in all 5 patients a cleft dividing the anterior (aortic) leaflet of mitral valve with normal papillary muscle position, mural leaflet size, and ratio of the inlet/outlet dimension of the left ventricle. Associated cardiac lesions were present in all 5 patients: perimembranous ventricular septal defect in 3, ostium secundum atrial septal defect in 2 and patent ductus arteriosus in 2 patients. During the 5.6 years (0.2–11) of the follow-up period, surgical repair of the cleft was never indicated since the mitral regurgitation through the cleft remained mild or absent in all the patients. Two patients underwent closure of a ventricular septal defect, with atrial septal defect closure in one and ductal ligation in 2. One patient died suddenly at home, without evidence of a cardiac cause. In conclusion, a cleft of the mitral valve has important developmental and morphologic differences with atrioventricular septal defect and may occur in patients with Down's syndrome. If surgical repair of the cleft or of associated cardiac lesion is indicated, it is necessary to distinguish it from atrioventricular septal defect where the conduction axis is displaced posteriorly and may be exposed during surgery.
The T wave as a marker of dispersion of ventricular repolarization in premature infants before and while on treatment with the IKr channel blocker cisapride
Authors: Benatar Avram , Cools Filip , Decraene Tine , Bougatef Adel , Vandenplas Yvan
Published: August 2006

Aims: Measurement of electrocardiographic intervals to assess dispersion in ventricular repolarization may be helpful in the assessment of the risk of ventricular arrhythmia. We measured QTc, QT dispersion, and T wave intervals in premature infants before and while on treatment with the IKr blocker cisapride as markers for dispersion in ventricular repolarization. Methods and Results: We enrolled 15 non-ventilated premature infants with a mean gestational age of 30.5 weeks, ranging from 26.5 to 33.5 weeks, and mean postnatal age of 24 days, with a range from 5 to 51 days. A digital 12 lead electrocardiogram was recorded prior to and 3 days after administering cisapride at a dose of 0.8mg/kg/day. Serum electrolytes were simultaneously measured. Electrocardiographic measurements before and after included: QT, QTc Bazett, QT dispersion, R–R, T wave interval peak to end, T wave interval peak to end/onset Q to T wave peak, T wave axis, T wave maximum voltage and QRS–T angle. A paired t test and analysis of variance was used to compare the variables before and during treatment. The QTc, T wave interval peak to end and the ratio T wave interval peak to end/onset Q to T peak increased significantly following treatment with cisapride. Results expressed as before and during treatment were for QTc: 429 (65) ms versus 454 (29) ms p < 0.02; for T wave interval peak to end: 65 (11) ms versus 103 (24) p < 0.01, for the ratio T wave interval peak to end/onset Q to T peak: 0.32 (0.06) versus 0.55 (0.16) p < 0.001. Treatment with the IKr blocker did not significantly alter the QT dispersion, T wave voltage, angle or QRS–T angle. Conclusion: The interval from the peak to the end of the T wave and the ratio of this value to the onset Q to T peak interval, represents regional dispersion of repolarization across the ventricular wall. This is a potentially useful clinical index in the assessment of arrhythmic risk in premature infants being treated by blockade of the IKr channels.
Coil occlusion of the small patent arterial duct without arterial access
Authors: Anil Sivadasan Radha , Sivakumar Kothandam , Krishna Kumar R.
Published: August 2006

Background: Arterial access is traditionally considered mandatory during coil occlusion of the patent arterial duct. Arterial access necessitates heparinization and carries the risk of femoral artery occlusion in small children. Methods and Results: Between July 1999 and May 2001, we attempted coil occlusion of patent arterial ducts in 104 patients without arterial access. The patients were aged from 3 months to 14 years. The median age was 2 years. They weighed 3–35 kg. The median weight was 9.8 kg. The duct diameter at pulmonary artery insertion was 1.8–3.5 mm. The patients were selected on basis of echocardiographic evaluation of duct diameter at pulmonary artery insertion and morphology of the ampulla. Doppler color flow imaging was used in the catheterization laboratory to confirm duct closure. Arterial access was required in 21 patients. The reasons included accidental puncture in 5 patients, failure to obtain venous access in 1 patient, aortic embolization in 3 patients, poor echo images in 2 patients, requirement for additional coils in 8 patients and, failure to cross the duct from pulmonary artery in 2 patients. The fluoroscopic time ranged from 2.2 to 20 min with a mean of 5.3 ± 3.8 min. Immediate closure was achieved in 98 patients and this included 79 of the 83 patients in whom arterial access was avoided. Color Doppler 3–24 h later showed residual flow in 2 patients. Four patients had new-onset left pulmonary artery turbulence with peak gradients below 5 mm of mercury. Coil embolization occurred in 6 patients and all coils were retrieved. Three-month follow up information was available for 78 patients. Small residual ductal leaks were seen in 4 patients, 2 of whom had leaks at 24 h. Two patients had recanalized their ducts. Conclusion: It is feasible to occlude small patent arterial ducts with coils using venous access alone in carefully selected patients with excellent immediate and short-term results.
Congenital atresia of the orifice of the left coronary artery
Authors: Gerlis Leon M. , Magee Alan G. , Sheppard Mary N.
Published: August 2006

A 3-month-old infant developed signs of cardiac failure, which was initially attributed to cardiomyopathy. At 8 months, further investigations showed evidence of myocardial ischaemia with reversal of the flow of blood in the left coronary artery, which received no demonstrable inflow from the aorta. An anomalous connection of this artery with the pulmonary trunk was diagnosed but, at surgery, it was found that the arterial orifice was completely atretic, although the main stem was of normal size. A left internal thoracic arterial graft to the anterior descending coronary artery was performed, but he died on the third day after the operation. Postmortem examination showed a small dimple within the aorta at the site of the orificial atresia, extensive myocardial infarction, and two zones of myocardial bridging of the anterior descending coronary artery. We discuss the relationship of coronary orificial atresia with single coronary artery. Although they are related, they typically have different and contrasting clinical presentations. The possible role of the myocardial bridging is also considered.
Exertional pulmonary edema revealing anomalous origin of the left coronary artery from the right coronary aortic sinus
Authors: Massih Tony Abdel , Clur Sally-Ann B. , Bonhoeffer Philipp
Published: August 2006

We report a 12-year-old child with anomalous origin of the left coronary artery from the right coronary aortic sinus, the artery taking a proximal intramural course. The anomalous artery was reimplanted into the left coronary aortic sinus. Postoperative stenosis was successfully treated with percutaneous transluminal angioplasty and implantation of a stent.
“Masked” pulmonary venous obstruction in patients with isomerism of the right atrial appendages: an overstated association
Authors: Mahnke C. Becket , Sandor George G. , Boyle Gerard J. , Webber Steven A.
Published: August 2006

Isomerism of the right atrial appendages is associated with anomalies of pulmonary venous return, which may be obstructive. The associated pulmonary arterial obstruction, however, has been reported to “mask” the pulmonary venous obstruction, with resultant pulmonary edema following augmentation of the flow of blood to the lungs. We postulated that the frequency of “masked” pulmonary venous obstruction has been overreported in the literature. To ascertain the true situation, we evaluated the frequency of “masked” pulmonary venous obstruction in a large, unselected, group of patients with isomerism of the right atrial appendages. We evaluated the clinical, anatomic, and radiographic data of 65 newborns seen with this syndrome since 1970. Development of pulmonary venous obstruction, both before and after systemic-to-pulmonary shunting and/or infusion of prostaglandin E1, was determined and correlated to clinical and radiographic findings. Of the 65 patients, 19 (29%) were ultimately diagnosed with pulmonary venous obstruction. The pulmonary veins themselves connected in infradiaphragmatic fashion in 10 patients, supracardiac in 3, to the atriums directly in 1, and in mixed fashion in the other 5. Pulmonary venous obstruction was readily apparent in 15 of the 19, as demonstrated by pulmonary edema on initial chest radiography. The remaining four cases ultimately diagnosed with pulmonary venous obstruction received augmentation of pulmonary blood flow with resultant pulmonary edema. Of these four severely cyanosed patients, pulmonary vascular markings on the initial chest radiograph had been normal in one but increased in three. Due to the presence of these clinical markers, they do not truly represent “masked” pulmonary venous obstruction. Except for two patients with minimally obstructed pulmonary arterial blood flow, 44 patients without pulmonary venous obstruction had normal or decreased pulmonary vascular markings at presentation. Of these 44, 14 received infusions of prostaglandin E1, with none developing pulmonary edema. We conclude that pulmonary venous obstruction is usually readily apparent at time of presentation in patients with right isomerism, and that “masked” pulmonary venous is a very rare event which has been over-emphasized in the literature. Careful evaluation of clinical and radiographic findings at time of presentation can correctly identify pulmonary venous obstruction in such patients.
Mitral valvar anomalies and discrete subaortic stenosis
Authors: Cohen Laurence , Bennani Raja , Hulin Sylvie , Malergue Marie-Christine , Yemets Ilya , Kalangos Afksendiyos , Murrith Nicolas , Ouaknine Ruth , Lecompte Yves
Published: August 2006

On the basis of our clinical experience, we hypothesized that the role of mitral valvar anomalies in the development and recurrence of discrete subaortic stenosis might be underestimated. From January 1994 to October 2000, the anatomy of the mitral valve and its relationship to the other components of the left ventricular outflow tract were studied by echocardiography in a series of 73 consecutive patients referred to our institution for surgical correction of discrete subaortic stenosis. In all patients for whom it was considered advisable, surgical correction of the mitral anomaly was performed, together with resection of the fibro-muscular subaortic stenosis. One or more mitral valvar anomalies were found in 35 patients (48%). They could be grouped into five categories: insertion of a papillary muscle into the aortic leaflet, insertion of a papillary muscle into the ventricular wall, “muscularization” of the subaortic portion of the aortic leaflet, anomalous insertion of the valvar tissue into the ventricular wall, and accessory valvar tissue. In all cases with anomalous mitral valvar anatomy, surgical correction was feasible. It consisted of transection of the anomalous papillary muscle or its attachment, resection of accessory valvar tissue, and/or patch enlargement of the aortic leaflet. The incidence of mitral valvar anomalies associated with subaortic stenosis is probably underestimated. Our data suggest that they should be systematically searched for during the evaluation of all cases of subaortic stenosis. Their surgical correction is generally feasible, and might improve the mid and long term results.
Fatal aortoesophageal fistula in two cases of tight vascular ring
Authors: Angelini Annalisa , Dimopoulos Konstantinos , Frescura Carla , Gamba Piergiorgio , Piccolo Roberto Lo , Stellin Giovanni , Thiene Gaetano
Published: August 2006

Vascular rings are rare vascular congenital anomalies causing oesophageal and tracheal compression. An aortoesophageal fistula is a devastating, in part iatrogenic, complication of vascular rings. It is seen with increasing frequency, and can be misleading, since differential diagnosis with other causes of haematemesis and melaena is often difficult, especially in infants. We report two infants with aortoesophageal fistulas secondary to double aortic arches forming a vascular ring. In both, the diagnosis was missed, and massive haemorrhage led to death. In both cases, the fissuration on the oesophageal and aortic sides of the fistula had sharp edges, highly suggestive of an iatrogenic laceration caused by manipulation of nasogastric tubes. The key for the diagnosis of vascular rings is, therefore, clinical suspicion and awareness of this condition. Prompt identification in infants with stridor, wheezing, or respiratory distress can prevent prolonged intubation, thus avoiding the formation of an aortoesophageal fistula and hopefully preventing a fatal outcome.
Formation of pannus on prosthetic valves in a child with pseudoxanthoma elasticum
Authors: Radford Dorothy J. , Pohlner Peter G.
Published: August 2006

A child with pseudoxanthoma elasticum had mitral valvar prolapse with severe regurgitation necessitating replacement with a prosthetic valve. Extensive formation of pannus caused obstruction of two mechanical valves, one after twenty months and the second after three years. Histology of the pannus was similar to the endocardial lesions that are considered unique to pseudoxanthoma elasticum.
Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta
Authors: Jung Mi-Jin , Yoo Shi-Joon
Published: August 2006

We report a case of anomalous origin of the right pulmonary artery from the ascending aorta that was diagnosed by fetal ultrasound at 21 weeks of gestation. The clue to the diagnosis was present in the three-vessel view, this being one of the views that we use for fetal cardiac screening. The anomaly was corrected surgically at 11 days of age. We discuss the importance of prenatal diagnosis in the management of this rare anomaly.
Echocardiographic diagnosis of partial obstruction of Blalock-Taussig shunts
Authors: Juraszek Amy L. , Atz Andrew M. , Shirali Girish S.
Published: August 2006

While echocardiography has previously been used to differentiate between patency and complete occlusion of aortopulmonary shunts, the echocardiographic diagnosis of partial obstruction of a shunt has not been reported. We describe a unique color flow Doppler finding that quickly and accurately diagnosed three cases of partial distal obstruction of aortopulmonary shunts.
Successful closure of the arterial duct in the setting of rubella syndrome
Authors: Gorenflo Matthias , Ulmer Herbert E. , Brockmeier Konrad
Published: August 2006

A 9-year-old boy, with significant left-to-right shunting across a large duct in the context of rubella syndrome, was tested during catheterization to establish the feasability of occluding the duct with a device. The testing, including temporary closure of the duct and monitoring of pulmonary vascular reactivity to vasodilative substances, lead to the decision to implant an Amplatzer occluder. Sixteen months later, there was no residual shunting across the duct, and pulmonary arterial pressures had normalised. It remains unclear why the patient had not developed irreversible pulmonary vascular disease.
Physiological impact and transcatheter treatment of the persisting left superior caval vein
Authors: Trivedi Kalyani R. , Freedom Robert M. , Yoo Shi-Joon , McCrindle Brian W. , Benson Lee N.
Published: August 2006

A left superior caval vein frequently occurs in the malformed, as well as in the structurally normal, heart. Its physiological impact varies, and is determined by its connections and whether there are associated cardiac lesions. In this review, we describe 3 patients with such a vein, 1 without other lesions and 2 with the anomalous venous channel as a component of a complex cardiac malformation. In all cases, transcatheter techniques were used to treat the physiological dysfunction caused by the presence of the vein. The connections of the vein, and the complexity of the associated cardiac lesions, determine the options for treatment. We define the role of surgical as opposed to transcatheter intervention, and discuss strategies for transcatheter treatment.
Use of electrophysiologic testing to assess risk in children with Wolff-Parkinson-White syndrome
Authors: Dubin Anne M. , Collins Kathryn K. , Chiesa Nancy , Hanisch Debra , Van Hare George F.
Published: August 2006

In adults with Wolff-Parkinson-White syndrome, the likelihood of sudden death can be estimated based upon the presence or absence of symptoms. Comparable data in children do not exist. To date, therefore, invasive risk stratification has been used to guide management regarding radiofrequency ablation in symptomatic children. As the safety of electrophysiology study and radiofrequency ablation in children has improved, asymptomatic patients have been referred more commonly for invasive risk stratification. We sought to compare the findings from electrophysiologic studies in symptomatic children with Wolff-Parkinson-White syndrome to the findings in asymptomatic children with Wolff-Parkinson-White pattern on their electrocardiogram. Thus, we compared the findings from electrophysiologic studies carried out in patients seen at Stanford University and University of California, San Francisco, from April 1999 to February 2001 with a preexcitation pattern on their electrocardiogram. The patients were divided into three groups: 23 asymptomatic patients studied for risk stratification, 19 patients who presented with syncope, and 77 patients presenting with documented supraventricular tachycardia. Data were collected for commonly accepted invasive criterions for stratification of risk: an effective refractory period of the accessory pathway less than 270 ms, multiple pathways, septal location of pathway, and inducibility of supraventricular tachycardia. Groups were compared by chisquare. No differences were found between the groups for any of the variables. These data suggest that risk factors for sudden death, developed in studies of adult patients, are not clearly applicable to children. Further studies are needed better to define the indications for study and ablation in children.
Corrosive behaviour of Amplatzer® devices in experimental and biological environments
Authors: Kong Huafu , Wilkinson James L. , Coe James Y. , Gu Xiaoping , Urness Myra , Kim Tae-Hoon , Bass John L.
Published: August 2006

Purpose: Nitinol, a nickel-titanium alloy, is a valuable material in the construction of interventional endoluminal devices because of its biocompatibility, super elasticity, high resiliency and shape memory. The possibility of nickel toxicity has been raised with devices constructed of Nitinol. Our investigation examines the long-term corrosive behavior of this alloy in experimental and biological environments. Methods: We performed three levels of study. Microscopic examination was made of 64 devices of various sizes, randomly selected from 240 Amplatzer® Septal Occluders that had been exposed to saline solution at 37°C for fourteen months. All samples were studied by electron microscopy ranging from 50 to 5000 times magnification. We also studied microscopically 3 Amplatzer® devices explanted 18–36 months after implantation in dogs, and 2 Amplatzer Septal Occluders removed from patients 18 months (cardiac transplant) and 19 months (died of causes unrelated to device placement) after implantation, which were examined grossly and by electron microscopy up to 5000 times magnification. We then measured the levels of nickel in the blood using inductive plasma mass spectroscopy in 19 patients with implanted Amplatzer® devices, making measurements before and 6 months after implantation. Results: Electron microscopy showed an intact titanium oxide layer with no evidence of corrosion in vitro and in vivo. One explanted device in direct contact with the platinum leads of a pacemaker for eighteen months showed minor pitting of the titanium oxide layer believed to be galvanic in nature. No wire fractures were found in vitro after cycle testing with 400 million cycles, nor in devices taken from the animals and humans. Biochemical studies showed no significant elevation of levels of nickel levels after implantation. Conclusion: Nitinol wire of Amplatzer® septal occlusion devices is resistant to corrosion when exposed to physiologic saline solution, and in experimental animals as well as humans. A device in contact with a platinum pacemaker electrode developed minimal pitting of the titanium oxide layer, believed to be galvanic in nature and of no structural or clinical significance. There is no increase of concentrations of nickel in the blood of patients who have received Amplatzer® nitinol devices. These favorable testing results reveal that nickel-titanium is an inert, corrosion resistant alloy.
Disease-related difficulties and satisfaction with level of knowledge in adults with mild or complex congenital heart disease
Authors: Kamphuis Mascha , Verloove-Vanhorick S. Pauline , Vogels Ton , Ottenkamp Jaap , Vliegen Hubert W.
Published: August 2006

Objectives: To evaluate difficulties in daily life, and satisfaction with level of knowledge about their disease, in patients with congenital cardiac disease in order to improve counselling. Methods: A self-administered questionnaire was completed by 80 patients with mild, and 76 with complex, congenital cardiac disease. They were aged from 17 to 32 years. Results: Even patients with only mild malformations experienced difficulties related to their disease, but being found in only 11%, these were significantly less than those uncovered in 87% of those with complex disease (p < 0.001). Those patients with complex malformations frequently felt restricted in choices because of their disease in areas such as sport (59%), employment (51%), and education (34%). Other difficulties reported were: paying a higher premium for life insurance (29%), having to give up on a sport (28%), and being excluded from a job (18%). Depending on the item, between one-fifth and two-thirds of participants reported gaps in knowledge, most frequently for “causes of congenital cardiac disease”, “future consequences”, and “family planning”. For 53% of those with mild anomalies, and 93% of severely affected patients, the cardiologist is the most important source of information. Conclusions: A minority of adults with mild, and a majority of those with complex congenital cardiac disease report difficulties in daily life. A substantial number of these patients feel that they have an inadequate level of knowledge about their disease. Our results suggest the need for a specific programme of counselling.
Supravalvar aortic stenosis with supravalvar pulmonary stenosis and peripheral vascular stenoses
Authors: Spaetgens Renée L. , Duncan Walter J. , Taylor Glenn P.
Published: August 2006

A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Followup angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. Angioplasty has only partially relieved these obstructions. The pathology and possible etiology are reviewed.
Haemoptysis as a late complication of a Mustard operation treated by balloon dilation of a superior caval venous obstruction
Authors: Berg Ansgar , Norgård Gunnar , Greve Gottfried
Published: August 2006

Haemoptysis was the presenting symptom in a 27-year-old male. He had undergone a Mustard operation for connection of complete transposition at the age of 2 years. For 6 months prior to admission, he had complained of dyspnoea without chestpain, and swelling of the fingers during hard physical work. Chest radiography and computer tomographic scans showed normal features of the pulmonary parenchyma, and no sign of cardiomegaly or vascular stasis. Fiberoptic bronchoscopy demonstrated a blood clot in the upper right bronchus, without any associated abnormalities of the bronchial tree. Doppler echocardiography showed obstruction of the superior caval vein, which was verified by cardiac catheterization. Balloon dilation at the site of obstruction increased the diameter of the vein from 0.5 to 1.7 cm, and the mean pressure in the superior caval vein was reduced significantly from 18 to 10 mmHg. The haemoptysis did not recur, and no complaints of dyspnoea or swelling of fingers during physical activity was reported 2 years later. Transthoracic echocardiography undertaken at this time revealed no obstruction of the superior caval vein. We conclude that hemoptysis is a rare complication of increased venous pressure in the upper body of patients with superior caval venous obstruction, which can be treated by balloon dilation or stenting.
Atresia of the orifice of the coronary sinus after surgery
Authors: Tateno Shigeru , Niwa Koichiro , Terai Masaru
Published: August 2006

We describe a patient with a coronary arteriovenous fistula, atresia of the orifice of the coronary sinus, and persistence of the left superior caval vein. Depression of her ST segments was revealed by exercise electrocardiography long after the initial surgical reconstruction of the coronary sinus.
Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life
Authors: Sandor George G. S. , Cook Andrew C. , Sharland Gurleen K. , Yen Ho S. , Potts James E. , Anderson Robert H.
Published: August 2006

Objectives: To establish the prevalence of coronary arterial abnormalities in mid-trimester fetuses with pulmonary atresia with intact ventricular septum, and whether their presence correlates with right ventricular morphology. Background: The presence of coronary arterial fistulas significantly alters the surgical options and prognosis for patients with pulmonary atresia with intact ventricular septum. The lesion can reliably be diagnosed using fetal echocardiography, and further definition of the prognosis is important for counselling parents. Methods: We examined the hearts of 39 pathological specimens diagnosed during fetal life, 3 of whom died postnatally. Coronary arterial abnormalities were defined as non-connection of the left or right coronary arteries to the aorta, ostial stenosis, marked tortuosity, dilation, thickening or abnormal myocardial branching. Mild tortuosity, or myocardial bridging, were considered normal. We measured the dimensions of the tricuspid valve along with the inlet and outlet portions of the ventricles. Ebstein's malformation, tricuspid valvar dysplasia, and the presence or absence of the infundibulum, were especially noted. We examined also 12 normal hearts as controls. Results: Coronary arterial abnormalities were found in 14/39 (36%). The dimensions of the right ventricle and tricuspid valves, and the gestational ages of the fetuses, were compared for these 14 with the 25 having no abnormalities using independent t-tests. The gestational ages were similar, 21.9 vs 21.1 weeks. The mean dimensions of the tricuspid valve, median z-scores, and right ventricle were smaller, 2.9 vs 7.2 mm; p < 0.002; −4.46 vs 0.23; p < 0.03; and 6.9 vs 13.7 mm; p < 0.002, for those with coronary arterial abnormalities. Ebstein's malformation, or dysplasia of the tricuspid valve, was present in 4 of 14 with, vs 15 of 25 without, coronary arterial abnormalities. A patent infundibulum was noted in 34 of 39 specimens. Conclusions: Mid-trimester fetuses with pulmonary atresia with intact ventricular septum already exhibit coronary arterial abnormalities, with a prevalence of 36%. The presence of a patent infundibulum confirms that atresia of the pulmonary valve is an acquired process. Coronary arterial abnormalities are seen in 50% of those with hypoplastic right ventricles, but less frequently in the presence of well developed ventricles. This is important information for those involved in counselling parents.
Polymorphisms in cytokine genes do not predict progression to end-stage heart failure in children
Authors: Webber Steven A. , Boyle Gerard J. , Gribar Steven , Law Yuk , Bowman Pamela , Miller Susan A. , Awad Mohammed R. , Ahmed Mamun , Martell Joan , Zeevi Adriana
Published: August 2006

A number of cytokines have been implicated in the pathophysiology of congestive heart failure. Genetic polymorphisms of several cytokine genes are known to result in altered gene expression, enabling us to characterize patients as "high" or "low" producers of specific cytokines. We speculate that the cytokine genotypes for a population of children who underwent heart transplantation for end-stage ventricular failure due to cardiomyopathy or congenital heart disease would be enriched for "high producers" of pro-inflammatory cytokines and "low producers" of anti-inflammatory cytokines. Methods: Cytokine genotyping was performed for the following cytokines on 94 transplanted children using polymerase chain reaction-sequence specific technique: tumor necrosis factor-α (−308), interleukin 10 (−1082, −819, −592), interleukin 6 (−174), transforming growth factor-β1(codons 10 & 25), and interferon-γ (+874). Patients with ventricular failure after transplantation for dilated cardiomyopathy, numbering 37, or for congenital heart disease, numbering 34, were compared to 15 children transplanted for structural disease, such as hypoplastic left heart syndrome, without ventricular failure, and to data from healthy children. An additional 8 children with restrictive or hypertrophic cardiomyopathy were also studied. Results: No differences in genotypic distribution were seen between the groups, and all patients were comparable to genotypic distributions as assessed from published normal data. Conclusion: No evidence is found to support the hypothesis that these polymorphisms for cytokine genes influence progression to end-stage heart failure in children undergoing transplantation because of cardiomyopathy or congenital heart disease.
Percutaneous transcatheter occlusion of the patent arterial duct using the pfm DuctOcclud Coil® via a trans-aortic and trans-pulmonary approach
Authors: Friedberg Mark , Khoury Asaad , Schwartz Yitzhak , Braver Yulia , Lorber Avraham
Published: August 2006

Occlusion using coils is now the treatment of choice for closure of the patent arterial duct. The DuctOcclud (pfm AG, Cologne, Germany) device is a relatively new retrievable coil for such trans-catheter closure. This study expands on previously reported experience with this device, summarizes the advantages of the device, and compares trans-pulmonary and trans-aortic delivery in 47 patients. There were 27 females. The mean, and median, ages were 4.6, and 2.85 years, respectively. The youngest patient was aged 9 months, weighing 7 kg. A trans-aortic delivery was used in 41 cases, and a trans-pulmonary approach in 6 cases. Of the 47 procedures, 45 (96%) were successful at the first attempt. The other two patients were treated successfully at the second attempt, giving a 100% rate of success. The technical characteristics of the coil allowed for its repeated maneuvering until an optimal position was obtained prior to release. Closure was confirmed by lack of ductal flow by echo-doppler on follow- up echocardiography. No short- or long-term complications of the procedure were noted in any of the patients. We conclude that the DuctOcclud device is an effective and safe method for closure of the small-tomoderately patent arterial duct. In a large proportion of patients, trans-aortic delivery is the preferred approach.
Age-related aspects of balloon angioplasty for postsurgical aortic recoarctation
Authors: Agnoletti Gabriella , Bonhoeffer Philipp , Borghi Adele , Boudjemline Younes , Abdel-Massih Tony , Bonnet Damien , Sidi Daniel
Published: August 2006

Balloon angioplasty is now the elective technique for relief of aortic recoarctation, since it has low mortality, low morbidity, and good results at follow-up. Some concern exists concerning the possible increased risk in young children. To investigate such age-related aspects, we examined 58 children undergoing consecutive balloon angioplasty for postsurgical recoarctation. Of the children, 30 were younger and 28 older than 1 year. Recoarctation was more frequent with ventricular septal defect or other cardiac anomalies (p < 0.001). Systolic hypertension was present in 70% of children aged less than 1 year, but in only 32% of those older than 1 year (p < 0.001). The initial gradient was inversely related with the age at dilation (r = −0.28, p < 0.05), and correlated with systolic blood pressure (r = 0.81, p < 0.01). The procedure was successful in 87% of children older than, and 86% of those under 1 year. Age was not related with residual gradient, nor with the percentage increase of diameter of the site of stenosis. After balloon angioplasty, systolic hypertension was still present in 37% of children younger than 1 year, and in 25% of those older than 1 year (p < 0.05). Three complications occurred in children younger than 1 year, and 6 in those older (p < 0.05). Our results show, first, that recoarctation occurs earlier in the setting of complex disease, second that children suffering recoarctation at a younger age are more likely to be hypertensive, either before or after balloon angioplasty, third, that balloon angioplasty has the same rate of success in children below or above the age of one year, although the procedure still carries a not negligible risk. Finally, the procedure does not carry a higher risk for those children below the age of 1 year.
Rapidly progressive idiopathic dilation of the right atrium in infancy associated with dynamic obstruction of the airways
Authors: Divekar Abhay , Soni Reeni , Ross David
Published: August 2006

We present a young infant with rapidly progressive idiopathic dilation of the right atrium, complicated by symptomatic and dynamic obstruction of the airways secondary to proximal obstruction which was reversed subsequent to surgical reduction of the right atrium. We discuss the surgical implications of the distorted cardiac anatomy, along with post-operative complications, which included refractory arrhythmias.
Radiofrequency ablation of a left-sided atrioventricular pathway in a patient with Marfan syndrome
Authors: Yetman Anji T. , Temple Joel , Erickson Christopher C.
Published: August 2006

Supraventricular tachycardia is a documented feature of Marfan syndrome. The safety and efficacy of radiofrequency ablation in this population of patients, however, has not been reported. We report on the successful use of radiofrequency ablation utilizing a trans-septal approach for the treatment of supraventricular tachycardia produced by an accessory muscular atrioventricular connection in a patient with Marfan syndrome.
Remodelling after surgical repair of atrial septal defects within the oval fossa
Authors: Attenhofer Jost Christine H. , Oechslin Erwin , Seifert Burkhardt , Maly Friedrich , Fatio Renata , Turina Juraj , Jenni Rolf
Published: August 2006

In a retrospective study, we analysed the data from 101 adults with echocardiographic follow-up after surgical repair of defects within the oval fossa at a mean age of 35 ± 17 years; 56% of the cohort being above the age of 30 years. Mean age at follow-up was 44 ± 18 years, and length of follow-up was up to 40 years (11 ± 12 years). At follow-up, atrial fibrillation or flutter was present in one quarter. Dilation of the right atrium, found in 64%, of the left atrium, found in 44%, and of the right ventricle, found in 29%, were also frequent, as well as pulmonary arterial hypertension, which was found in 30%. Diminished right ventricular ejection fraction, in contrast, was very rare, found only in 1%, and abnormal left ventricular ejection fraction was not encountered. By multivariate analysis, predictors for right or left atrial, or right ventricular, dilation were age at follow-up, degree of tricuspid regurgitation, pulmonary hypertension, and/or atrial fibrillation. In a subset of 21 patients in sinus rhythm, we correlated prospectively the diastolic and systolic function of both ventricles with levels of brain natriuretic peptide, comparing values to those of 20 age-matched controls with a mean age of 46 ± 14 years. Levels of brain natriuretic peptide were significantly higher in patients than in controls (p = 0.006), and correlated significantly with diastolic dysfunction (p = 0.007) and left atrial size (p < 0.0001). In the long-term follow-up after surgical repair of defect within the oval fossa, therefore, complete normalization of heart size and function is rare. Despite preserved systolic function, persistent diastolic dysfunction is common and is associated with elevated levels of brain natriuretic peptide, which may explain the late occurrence of atrial arrhythmias.