bluuebunny/crossref_metadata_2025 huggingface dataset:
Divided right atrium (‘cor triatriatum dexter’) with azygous drainage of the superior caval vein
Authors: Mühler Eberhard G. , Franke Andreas , Messmer Bruno J.
Published: August 2008

We describe the symptoms, the techniques for imaging and the surgical therapy in a patient with divided right atrium (‘cor triatriatum dexter’) combined with atresia of the superior caval vein. In addition to peripheral venous congestion and cyanosis, macrocephaly was the most prominent sign, the high pressure in the superior caval vein disturbing the circulation of cerebrospinal fluid. Transesophageal echocardiography was the optimal technique for imaging the dividing shelf in the right atrium. Cardiac catheterization after surgical excision of the shelf and reconstruction of the junction between the superior caval vein and the right atrium excluded any residual gradient or shunt.
Benign congenital cardiac tumor associated with a ‘malignant’ cardiac malformation
Authors: Davis Andrew M. , Silver Meredith M. , Freedom Robert M.
Published: August 2008

We describe a multicystic tumor of the atrial septum associated with a fatal cardiac malformation. The tumor contained bronchogenic cysts and two types of vascular hamartomas. A complex form of double outlet right ventricle was present. We speculate that this tumor formed at a critical time in cardiac embryogenesis and contributed to the abnormal formation of the heart.
Endovascular stenting of the left pulmonary artery using the Strecker stent
Authors: van Oort Anton , Tanke Ronald , van de Kaa Christine
Published: August 2008

An intravascular stent was successfully implanted in a 15-year-old gid with stenosis of the left pulmonary artery subsequent to a modified Fontan correction. Complaints of tiredness, edema, ascites and enlargement of the left breast disappeared. Unfortunately, she died of a rhythm disturbance unrelated to the stent. Autopsy revealed excellent endothelialization of the stent without any thrombosis and with patency of the orifice of the branch to the upper lobe of the lung.
Combined construction of bidirectional cavopulmonary and aortopulmonary shunts as an intermediate stage in the repair of an 18-year-old high-risk Fontan candidate
Authors: Yamagishi Masaaki , Kurosawa Hiromi , Hashimoto Kazuhiro
Published: August 2008

A combined procedure of a bilateral bidirectional cavopulmonary shunt and a central aortopulmonaiy shunt was performed on an 18-year-old boy with pulmonary atresia. Arterial oxygen saturation was elevated after the combined shunt, whereas the ventricular stroke work was reduced. Such a combined shunt is a useful alternative prior procedure which does not produce ventricular overload in older or higher-risk candidates for the Fontan procedure.
Inflammatory lung disease in a patient with tricuspid atresia palliated by a Glenn anastomosis
Authors: Thorne Sara , Somerville Jane
Published: August 2008

A 37-year-old man with tricuspid atresia, in whom a chronic right apical aspergilloma had stimulated formation of extensive aortopulmonary collateral circulation, suffered reversal of flow within his long-standing fistulous Glenn anastomosis. By reversing the flow through the pulmonary arteriovenous fistula and raising oxygen saturation in the right atrium, the acquired aortopulmonary collateral circulation prevented the increase in cyanosis which usually occurs when fistulous changes develop late after the Glenn operation.
Percutaneous retrieval of a broken pacemaker fragment from the superior caval vein in a child
Authors: Karaaslan Sevim , Çeliker Alpay , Günal Nazlihan
Published: August 2008

The increase in use of endovascular devices in the treatment and diagnosis of malformations of the cardiovascular system has created a need for another device suitable for retrieval of dislodged catheters or fragments in children. We describe the use of a snare made of nickel-titanium (nitinol) cable for retrieval of a broken pacemaker electrode lodged in the superior caval vein.
Anomalous muscle bundle in the left ventricle causing subaortic obstruction
Authors: Kothari Shyam S. , Iyer Krishna S.
Published: August 2008

We report a 14-year-old boy in whom an anomalous muscle bundle in the left ventricle caused subaortic obstruction. It was diagnosed by echocardiography and resected at operation. The anomalous muscle most likely represents the hypertrophied anterolateral muscle bundle.
Cardiopulmonary reponse to exercise after the Fontan Operation—a cross-sectional and longitudinal evaluation
Authors: Mertens Luc , Rogers Ralph , Reybrouck Tony , Dumoulin Monique , Vanhees Luc , Gewillig Marc
Published: August 2008

The purpose of this study was to assess cardiorespiratory responses to submaximal exercise in patients with univentricular atrioventricular connection after the Fontan operation, and to evaluate whether changes occur during medium-term follow-up. Eighteen patients (age 12.1±5.5 years) underwent graded exercise test on a treadmill 2.3±1.4 year after the Fontan repair. Ventilatory gases were measured using breath-by-breath analysis. Results were compared to gender/age-matched controls. Twelve patients (age 14.2±5.4 years) were reevaluated 2.4±2.1 years after the first test. Aerobic exercise performance was subnormal in all patients during the first test. At the lowest level of exercise, the ventilatory threshold was already surpassed in 6/18 patients, while it was reduced in all other patients (p<0.001). All patients were in stable sinus rhythm throughout the test. Heart rate at all exercise levels was ±10% below normal (p<0.05). The respiratory frequency was increased at all exercise levels (p<0.001). The ventilatory equivalent for oxygen was increased (p<0.001), and the end-tidal tension of carbon dioxide was decreased (p<0.001). The ratio of physiological dead space/tidal volume was increased in all patients (p<0.001), while the normal decrease of this ratio during exercise was not observed. Upon reevaluation heart rate, respiratory rate, oxygen uptake, venti latory equivalent for oxygen, end-tidal carbon dioxide tension and physiological dead space did not change signifi cantly. Only a slight further decrease in ventilatory threshold was observed. Aerobic performance after the Fontan procedure ranges widely from just above resting metabolic rate to the lower limit of normal. Dyspnea during exercise is exacerbated by a decreased ventilatory threshold, increased physiological dead space, and decreased respir-atory efficiency. Cardiorespiratory response to exercise, nonetheless, remains relatively stable during medium-term follow-up.
Evaluation of coronary arterial patterns in complete transposition by laid-back aortography
Authors: Yoo Shi-Joon , Burrows Patricia E. , Moes C. A. Frederic , MacDonald Cathy , Williams William G. , Houde Christine , McCrindle Brian W. , Freedom Robert M. , Benson Lee
Published: August 2008

For the visualization of the coronary arterial patterns in complete transposition (concordant atrioventricular and discordant ventriculoarterial connections), balloon occlusion aortography has been performed in right and left anterior oblique or in frontal and lateral views. These views, however, are often unsatisfactory because of super-imposition of the aortic sinuses. The so-called “laid-back” view is a newly developed angiographic projection in which the aortic sinuses and the intervening commissures are imaged as if they were seen from below and the front. We performed such laid-back aortography in 36 consecutive patients with an echocardiographic diagnosis of complete transposition. In 23 of 36 patients, we obtained additional aortograms in right and left anterior oblique views. For the evaluation of the diagnostic value of the laid-back view, as compared with that of the combined right and left anterior oblique views, both sets of aortograms were reviewed independently by the five reviewers who were asked to make a diagnosis and to specify their levels of confidence (“definite,” “probable,” and “possible”) for their diagnosis. Error rates were significantly lower for the laid-back aortograms than for the combined right and left aortograms (4 and 11%, respectively). An increased level of confidence was significantly associated with the laid-back aortograms. We illustrate the anatomy of various coronary arterial patterns seen by laid-back aortography.
Arrhythmias after surgical correction of complete transposition—follow-up into adolescence
Authors: Hutter Paul A. , Baars Menno W. , den Boer Karin T. , van den Haak Ronald F. F. , Harinck Eric , Kingma J. Herre , Benatar Avram A. , Meijboom Erik Jan
Published: August 2008

The long-term natural progression of cardiac rhythm and the incidence of serious arrhythmias in relation to previous procedures and associated heart defects were studied in a group of 76 patients after an arterial switch operation for complete transposition and compared to a group of 24 patients who had undergone intraatrial corrections (Mustard or Senning operation). Standard and 24-hour ambulatory electrocardiograms were reviewed. Mean follow-up was 7.9 years (range 2-16) after an arterial switch and 15.2 years (range 6-26) following the Mustard or Senning procedures. One patient died after an arterial switch from pulmonary hypertension (age 9.9 years), and three patients died suddenly, presumably from arrhythmias, following atrial redirection procedures (ages 13, 18 and 20 years). Symptomatic arrhythmias were not seen after the arterial switch operation. Three patients required pacemaker implantation after atrial redirection, and a further two required medication to control tachyarrhythmias. Survival analysis of sequential electrocardiograms showed a mean maintenance of sinus rhythm during 12.9 years (95% confidence interval 11.4-14.5) after the arterial switch and 9.0 years (7.3-10.7) after atrial procedures (p=0.003). Development of heart rate was significantly different (p=0.001), with higher rates in adolescents after an arterial switch. Twenty-four-hour recordings were abnormal in five of 72 patients following arterial correction, disclosing excessive ventricular extrasystoles in four (three monomorphic, one polymorphic) and a wandering pacemaker in one. After atrial procedures, 11 of 19 were abnormal (p<0.001), with sinus or atrial bradycardia in eight, atrial flutter in two, and monomorphic ventricular tachycardias in one. Abnormal findings on either 12-lead or 24-hour electrocardiograms were seen in 22% of patients following an arterial switch procedure and 83% of patients undergoing atrial redirection (p<0.001). Sinus rhythm, therefore, is preserved longer and arrhythmias are less frequent and less severe after the arterial switch operation than after the Mustard or Senning operations.
Etiology and danger of pericardial effusion in infants and children
Authors: Zreik Hani , Li Jennifer , Garson Arthur T.
Published: August 2008

Pericardial effusion requires attention because of its underlying pathology and its potential for producing hemodynamic compromise. It was our hypothesis that the etiology of and danger from large pericardial effusions are different at different ages. We reviewed the clinical data on 44 consecutive patients seen between 1986 and 1993. All (age range 0-19 years) had a large pericardial effusion identified by echocardiography, and 38 had fluid available for analysis, pericardiocentesis in these cases being undertaken on the basis of the clinical decision of the attending physician. History, physical examination, chest x-ray, electrocardiogram, and echocardiographic Doppler findings were evaluated retrospectively. Of the patients, 17 presented with tamponade. Of the 14 patients aged less than one year, 12 had tamponade (85%), whereas in the 30 patients older than one year, only five (16%) had tamponade. Surgical drainage was needed in seven patients. Typical physical and electrocardiographic findings (distant heart sounds, pericardial friction rub, decreased QRS voltage, and ST-T wave abnormalities) were present in less than half the patients. Etiologies of the effusion included infection in 10 (four less than one year old, and six greater than one year of age). Among these, two unusual pathogens were detected: Mycobacterium avium-intracellulaire and Candida tropicalis. In eight patients, the effusion was due to trauma (six less than one year old, and two older than one year), and in 11 it was due to post-pericardiotomy syndrome (two less than one year of age and nine older than one year). Malignancy was the cause in six (all older than one year of age), radiation in two, and collagen vascular disease, drug induced, and uremia in one each, all older than one year. Only three patients had idiopathic effusions (6%). These data suggest that, unlike adults, an etiologycan be found in most children with large pericardial effusions. Those less than one year of age are much more likely to present in cardiac tamponade.
False aneurysm of the pulmonary trunk complicating complete transposition and presenting as pyrexia of unknown origin
Authors: Gerlis Leon M. , Hirsch Rafael , Somerville Jane
Published: August 2008

A patient with complete transposition and a ventricular septal defect was clinically improved by an atrial septectomy at nine years but continued sepsis in the thoracotomy scar resulted in blood-stream infection with Staphylococcus aureus at the age of 24 years. A deep nylon suture was found and removed at surgical exploration. Five months later she again became febrile and had a vasculitic skin rash which responded to antibiotic therapy. At that time she was cyanosed and had a restricted exercise tolerance and was found to have aneurysmal dilatation of the pulmonary trunk. A year later she had acute chest pain with increased breathlessness, fever, hepatomegaly and pulmonary regurgitation. The fever did not respond to antibiotic therapy and she died suddenly following removal of a Hickman line. At postmortem examination, there was found to be a false aneurysm in the mediastinum due to rupture of the aneurysmal pulmonary trunk. No evidence of infection was found and it was considered that the low grade pyrexia had been due to the large hematoma within the false aneurysm.
Large blood cyst causing severe left ventricular obstruction in a fetus
Authors: Cook Andrew C. , Fagg Nuala L. K. , Sharland Gurleen K.
Published: August 2008

A case of left ventricular obstruction is reported in a fetus of 21 weeks gestation. Obstruction to the left ventricular inflow and outflow tracts was documented by prenatal echocardiography and, at autopsy, the cause was found to be a large blood cyst. It is likely that the associated hypoplasia of the left heart was a result of reduced blood flow beyond this obstructive lesion.
Supravalvar stenosing tricuspid ring in congenitally corrected transposition
Authors: Gladman Gordon , Casey Frank , Adatia Ian
Published: August 2008

We report an unusual case with discordant atrioventricular and ventriculoarterial connections. In addition to the commonly associated intracardiac anomalies ofventricular septal defect and subpulmonary tissue tags, a supravalvar ring was present above the left-sided, morphologically tricuspid valve. This case highlights the value of color flow Doppler echocardiography in making the diagnosis, particularly as the supravalvar ring was seen poorly angiographically.
Basilar arterial migraine—a substrate for non-hypotensive syncope
Authors: Moran Adrian M. , Arnold Lucy W. , Saul J. Philip
Published: August 2008

Syncope occurring simultaneously with documented normal blood pressures has been termed “psychogenic” in some patients. Two cases of basilar arterial migraine are presented, both with a diagnosis of syncope and documented normal blood pressures during the syncopal episodes. These findings highlight that syncope seen in the setting of basilar migraine is a variant from the typical form of neurally mediated syncope or cardiac arrhythmia. There is a need to consider the diagnosis of migraine when normal blood pressures and heart rates are recorded during such syncopal episodes.
Cervical aortic arch with anomalous origin of the left subclavian artery from Kommerell's diverticulum
Authors: Norgård Gunnar , Gerlis Leon , Tulloh Robert
Published: August 2008

We report here a patient with right cervical arch and anomalous origin of the left subclavian artery from the diverticulum of Kommerell. This uncommon abnormality can give rise to symptoms due to compression of the trachea and esophagus. Unlike right aortic arch with mirror image pattern of branching, however, this abnormality is seldom associated with intracardiac defects. Most patients are asymptomatic, and surgery is indicated only if severe symptoms are present.
Left-sided hepatic vein connected to the coronary sinus
Authors: Király László , Deanfield John E. , de Leval Marc R.
Published: August 2008

A left-sided hepatic vein connected to the coronary sinus is reported in a case of a 22-month-old boy with isomerism of the left atrial appendages, complete atrioventricular septal defect and azygous continuation of the inferior caval vein. The diagnosis of the anomalous hepatic vein was made intraoperatively and successful biventricular repair has been accomplished. To the best of our knowledge, this is the first communication on this peculiar entity diagnosed during life, notwithstanding Nabarro's description of a similar autopsy finding in 1903. Aspects of the development of this rare entity are discussed.
The bidirectional Glenn and Fontan procedures—integrated management of the patient with a functionally single ventricle
Authors: Mainwaring Richard D. , Lamberti John J. , Moore John W.
Published: August 2008

The Fontan procedure provides a means of separating the systemic and pulmonary circulations in patients with congenital heart defects characterized by a functionally single ventricle. The procedure has undergone numerous changes since its introduction 25 years ago. Technical modifications have now increased its applicability to virtually all forms of univentricular atrioventricular connection, as well as many hearts with biventricular connections. More recently, philosophical changes have been introduced to address increased rates of morbidity and mortality observed in certain subsets of patients. These changes appear to have improved short- and midterm results. The purpose of this review is to offer our strategy for management of such patients. We will discuss the interrelationship between the bidirectional Glenn and Fontan procedures. We will also focus on a number of observations concerning the possible role of accessory pulmonary blood flow, aortopulmonary collateral arteries, and endocrinologic changes.
Maximal exercise test in long-term follow-up after atrial redirection for complete transposition in a population-based cohort
Authors: Gilljam Thomas , Eriksson Bengt O.
Published: August 2008

In western Sweden, less than one-third of children born with complete transposition between 1964 and 1983 are long-time survivors. During bicycle ergometry, using a steady-state protocol with analyses of the expiratory gases and lactate, we investigated the exercise capacity in survivors of atrial redirection. There were 16 patients who had a Mustard procedure, now aged 16.9±3.6 years (12.0–22.0) and 15 patients who underwent a Senning correction aged 9.4±1.6 years (7.2–12.1). Nineteen percent of Mustard cases and 53% of Senning cases had minor or no sequels. Moderate sequels were found in 44% of post-Mustard cases, and 40% of post-Senning cases, including baffle complications, moderate systemic ventricular dysfunction, sinus node dysfunction and tracheal stenosis. Severe problems were encountered in 37% of post-Mustard cases and 7% of post-Senning cases, including pulmonary hypertension, pulmonary venous obstruction, atrioventricular block and hemiparesis. The maximal exercise capacity was low (p<0.0001); 71% had an oxygen uptake below −2 SD of predicted values, with severely impaired oxygen uptake in all except one of the cases with severe sequels, but no significant difference was found between patients having mild and moderate sequels. The maximally achieved heart rate was low (p<0.0001); 71% had values below −2 SD of predicted, including all cases with sinus nodal dysfunction, as well as 40% of cases without it. The maximal oxygen pulse was low (p<0.01), below −2 SD in 36%, but it was less affected than other exercise variables in 68% of the patients. Our study, therefore, presents exercise data in an epidemiologically-based population, relates the data to postoperative sequels and discusses some possible adaptive mechanisms.
Prevalence of valvar regurgitation by color Doppler echocardiography in neonates with normal hearts
Authors: Du Zhong-Dong , Roguin Nathan , Barak Mila , Milgram Elliot
Published: August 2008

Valvar regurgitation has been commonly found by echocardiography in adults and older children with normal hearts, but there is limited knowledge in neonates. To evaluate the prevalence of valvar regurgitation and analyze risk factors, echocardiography was performed on 975 consecutive neonates (1–7 days of age) with normal hearts. Regurgitation was detected and graded with pulsed, continuous-wave and color Doppler imaging techniques. Thirty-five cases with valvar regurgitation were followed for one month. The results showed that 369 (37.8%) neonates had evidence of regurgitation in one or more valves. Tricuspid regurgitation was most commonly found and was detected in 345 (35.4%) neonates, mitral regurgitation in 51 (5.2%), pulmonic regurgitation in 25 (2.6%), and aortic regurgitation in eight (0.8%). Most of them were trivial or mild and involved just one valve. Moderate or severe regurgitation was detected only in the tricuspid valve (3%) and the mitral valve (0.1 %). Five (0.5%) neonates with severe tricuspid regurgitation had heart murmurs, and two (0.2%) of them presented with cardiorespiratory distress. The risk factors for tricuspid and mitral regurgitation included lower chronologic age, gestational age and whether the neonates had a patent arterial duct; whereas those for tricuspid regurgitation also related to lower Apgar scores, birth weight and whether the neonates had a patent oval foramen. About 90% of the tricuspid regurgitation disappeared and the remaining 10% decreased in one month. We concluded that trivial or mild valvar regurgitation is commonly found in neonates with normal hearts. Moderate or severe regurgitation is relatively rare and occurs only in the tricuspid and mitral valves with a few cases causing clinical attention.
Transfusion associated graft-versus-host disease in DiGeorge syndrome—index case report with survey of screening procedures and use of irradiated blood components
Authors: Franklin Rodney C. G. , Onuzo Obed , Miller Paul A. , Goldblatt David , Cummins David
Published: August 2008

Most patients with DiGeorge syndrome require surgery to their heart defect during infancy and are at risk of developing transfusion-associated graft-versus-host disease if non-irradiated blood is used perioperatively. Because of its wide clinical variability, DiGeorge syndrome may not be recognized before surgery and thus a high level of vigilance is required. An infant is described who developed fatal graft-versus-host disease after receiving non-irradiated blood components during surgery for classical tetralogy of Fallot; the postoperative clinical course and autopsy findings were consistent with underlying DiGeorge syndrome. An informal survey of the 18 pediatric cardiology units in the United Kingdom and the Republic of Ireland showed no consensus as to which patients should be screened for DiGeorge syndrome, which screening test to use and to whom irradiated blood components should or should not be given to prevent transfusion associated graft-versus-host disease. Assessment of T-cell numbers appears to be the preferred screening method to determine those who might benefit from receiving such therapy.
Progressive pulmonary hypertension in cyanotic congenital heart disease with severe pulmonary stenosis
Authors: Cheung Yiu-Fai , Leung Maurice P. , Wang E. P.
Published: August 2008

We report the progressive development of pulmonary hypertension despite the presence of severe pulmonary stenosis in three patients with cyanotic congenital heart disease. The associated intracardiac lesions were complete transposition with a ventricular septal defect, double outlet right ventricle, and a heart with univentricular atrioventricular connection, respectively. Serial measurements on hemodynamics and histological findings documented the progression of pulmonary hypertension and pulmonary vascular diseases. The underlying etiology remains speculative. In view of the possibility of development of pulmonary hypertension despite a ‘protected’ pulmonary vasculature, corrective surgery should be contemplated as soon as technically feasible.
Tetralogy of Fallot in a neonate following intrauterine exposure to valproic acid
Authors: Stewart Wendy A. , Giuffre R. Michael
Published: August 2008

Valproic acid is a known teratogen, giving rise to a typical craniofacial appearance, the fetal valproate syndrome. Congenital heart malformations in this syndrome have most commonly been ventricular septal defects. We report a child with familial craniosynostosis, and tetralogy of Fallot, following intrauterine exposure to valproic acid.
Response of fetal tachycardia to transplacental procainamide therapy
Authors: Triedman John K. , Walsh Edward P. , Saul J. Philip
Published: August 2008

We describe two fetuses presenting with hydrops and tachycardia who were treated by prolonged dosage with procainamide. Initial therapy with digoxin in both had been unsuccessful in controlling the rapid heart rate. The first child was delivered after five weeks of transplacental therapy, and demonstrated Wolff-Parkinson-White syndrome on the second day which was controlled with flecainide. The second fetus required three and a half weeks of intrauterine treatment. Wolff-Parkinson-White syndrome became manifest four days after premature labor at 33 weeks gestation. No tachycardia occurred postnatally and the infant has been well during follow-up. The implications of treatment are discussed.
Isolation of the left brachiocephalic artery associated with right aortic arch and left-sided arterial duct
Authors: Kaku Sashicanta , Pinto Fatima , Lima Manuela
Published: August 2008

We report a case of right aortic arch and isolation of the left brachiocephalic artery. Stenosis of the left pulmonary artery, ventricular septal defect and left superior caval vein draining to the right atrium via the coronary sinus were also present in a 13-month-old asymptomatic girl with Down's syndrome. To our knowledge, this association of lesions has not previously been described.
Preferential flow to the right pulmonary artery after an early arterial switch procedure
Authors: Fletcher Scott E. , Martin Ameeta B. , Cheatham John P.
Published: August 2008

Despite early arterial switch repair of complete transposition of the great vessels, significant physiologic aberrations may still occur. Two cases of vascular disease in the left pulmonary arterial system are described and potential mechanisms are discussed. We conclude a small number of children with simple transpostion may develop asymmetric patterns of pulmonary blood flow without evidence of anatomic narrowing of either arterial vessel.
The mendelian basis of congenital heart defects
Authors: Dallapiccola Bruno , Marino Bruno , Digilio Maria Cristina , Mingarelli Rita , Novelli Giuseppe , Giannotti Aldo
Published: August 2008

The revolution in molecular genetics is contributing to the understanding of normal and abnormal cardiovascular development and morphogenesis. Recent investigations have shown that a growing number of congenital heart malformations is due to single gene defects. The combined contribution of clinical and molecular studies is providing the chromosomal map of the genes related to these isolated cardiac defects, and to syndromes characteristically associated with specific cardiac malformations. These advances are relevant to clinical practice, since the accumulated knowledge can improve the quality of management of affected patients.
Epidemiology of congenital heart disease in Merseyside—1979 to 1988
Authors: Jackson Mark , Walsh Kevin P. , Peart Ian , Arnold Robert
Published: August 2008

All infants and children born alive with documented congenital heart disease (excluding those weighing less than 2500 g at birth with a diagnosis of patent arterial duct) within the county of Merseyside between 1979 and 1988 were identified from the combined records of the Liverpool Congenital Malformations Registry and the Cardiac Unit, Royal Liverpool Children‘s Hospital Alder Hey, Liverpool. In total, 1543 cases were disclosed from a live-birth population of 203,880 providing a prevalence rate at birth of 7.57 (95% confidence interval: 7.20–7.96) cases per 1000 livebirths. The overall crude death rate was 18.5% (95% confidence interval: 16.4–20.3). An additional 240 (15.6% [95% confidence interval: 13.8–17.5%]) cases of heart disease not identified by the registry were disclosed from the records of the cardiac unit, demonstrating the unreliability of rates published by monitoring bodies. The annual prevalence rate at birth varied from 6.3 to 9.0 per 1000 livebirths, and seemed to be associated with the impact of new imaging technology. Regional variation was marked (5.3 to 8.9 per 1000 livebirths) and unaccountable in terms of potential morphogenetic mechanism, suggesting inconsistencies in referral of cases. Exclusion of regions of poor ascertainment in the calculation of a revised figure for the prevalence rate at birth suggests that as many as 89 in every 10,000 infants born alive may have structural cardiac anomalies. In conclusion, annual and regional variation of ascertainment in registry studies remains the enemy of an accurate description of the true prevalence rate at birth for congenital heart disease.
Descriptive epidemiology of membranous and muscular ventricular septal defects in the Baltimore-Washington Infant Study
Authors: Lewis David A. , Loffredo Christopher A. , Correa-Villaseñor Adolfo , Wilson P. David , Martin Gerard R.
Published: August 2008

Although ventricular septal defect is the most common form of congenital heart disease, its epidemiology is not completely understood. The Baltimore-Washington Infant Study, a case-control study of congenital cardiovascular malformations, enrolled 4,390 case infants during the study period 1981–1989. Ventricular septal defect, the most common malformation, was present in 1,465 (33%) of affected infants. The purpose of this report was to describe the epidemiological characteristics of membranous and muscular ventricular septal defects, the most common types of ventricular septal defects. Membranous ventricular septal defects (n=895) and muscular ventricular septal defects (n=429) accounted for 90% of all ventricular defects. During the study, prevalence increased from 0.9 to 2.1 /1000 live births. Associated non-cardiac defects, such as chromosomal defects, genetic syndromes, and defects of other organs were more common among infants with ventricular septal defect than among control infants, and more common among infants with membranous ventricular defect (20.8%) than among those with muscular defects (7.5%). Infants with ventricular septal defects and non-cardiac defects had increased mortality in comparison to infants with isolated ventricular septal defects or controls. Epidemiological information derived from population-based studies provide valuable data for counseling families and may allow for examination of etiologic factors.
Pattern of symptomatic congenital heart disease among oriental neonates—a decade's experience
Authors: Leung Maurice P. , Yung T. C. , Ng Y. K. , Wong K. Y. , Lee S. L. , Mok C. K. , Lee Jan , Chiu Clement
Published: August 2008

Between 1981 and 1990, 765 symptomatic neonates with major congenital heart malformations were admitted into the Grantham Hospital. This represented an incidence of 10 per 10,000 live births for Hong Kong. The figure was comparable to those reported for Caucasians. Among the 744 Oriental neonates, obstruction of the pulmonary outflow tract occurred most frequently (281, 37.8%), followed by left ventricular outflow tract obstruction (169, 22.7%), left-to-right shunting (115, 15.5%), complete transposition (92, 12.4%), common mixing situations (62,8.3%), and miscellaneous causes (25, 3.3%). When compared with the available reports from the West, Chinese neonates had a high preference for pulmonary outflow tract obstruction (p<0.005), especially the anomaly of pulmonary atresia and intact ventricular septum. This correlated well with cyanosis as the commonest neonatal presentation (64%). Contrary to previous reports that aortic coarctation was rare among Orientals, this abnormality was observed frequently in our study. The rare occurrence of critical aortic valvar stenosis among Chinese, however, was supported by our present analysis. Other lesions, such as left-to-right shunting and complete transposition, showed no significant racial difference in the frequency of occurrence. Such knowledge concerning the pattern of congenital heart disease amongst Oriental neonates can facilitate early diagnosis and timely referral of babies to the appropriate center for management.
Effect of adenosine on atrioventricular conduction in children and young patients with supraventricular tachycardia
Authors: Dorostkar Parvin C. , Dick Macdonald , Serwer Gerald A. , LeRoy Sarah , Armstrong Brian
Published: August 2008

Adenosine, when given as an intravenous bolus, has been shown to produce atrioventricular nodal block in humans. To examine the effect of adenosine on conduction across both accessory pathways and the atrioventricular node in children, we reviewed our experience with adenosine administered during both atrial and ventricular pacing in 42 patients with atrioventricular resting tachycardia and in eight patients with atrioventricular nodal reentry tachycardia. Adenosine was administered as a mean bolus of 195 μg/kg/dose during both atrial and ventricular pacing, examining antegrade and retrograde conduction before and after radiofrequency ablation. In those patients with persistent or intermittent pre-excitation, anomalous ventricular activation was either unchanged (n=8) or increased (n=11). Retrograde conduction (either through the accessory pathway alone in three, or across both the accessory pathway and the atrioventricular node in 19) persisted in 92% of the 24 patients studied. Adenosine produced either first or third degree antegrade heart block in all patients studied without pre-excitation (those with either dual atrioventricular nodal pathways or concealed accessory pathways). Adenosine produced retrograde block in all of the eight patients with dual atrioventricular nodal pathways. In contrast, retrograde conduction persisted in 82% (14/17) of patients with concealed accessory pathways (p=0.001). When used to examine retrograde conduction, adenosine was a sensitive (82%) and highly specific (producing retrograde atrioventricular block in all patients with dual atrioventricular nodal pathways) predictor of tachycardia supported by a concealed accessory pathway. Adenosine yielded a sensitivity and specificity of 96% and a positive predictive value of 99.5% for the success of ablation of accessory pathways. These data indicate that the pattern of adenosine-induced changes in either antegrade or retrograde atrioventricular conduction, or conduction in both directions, in young patients with supraventricular tachycardia is related to the mechanism of the tachycardia. Adenosine, therefore, is a useful adjunct in the electrophysiologic evaluation of supraventricular tachycardia in children.
Outcome of, and risk factors for, second degree atrioventricular block in children
Authors: Villain Elizabeth , Bonnet Damien , Trigo Conceicão , Iserin Laurence , Sidi Daniel , Kachaner Jean
Published: August 2008

Second degree atrioventricular block is uncommon in children. In order to evaluate its outcome, and to find early prognostic factors, we reviewed the history and evolution of 21 children with this arrhythmia discovered on their surface electrocardiogram. Twenty-four-hour monitoring displayed variations in the conduction patterns in almost all children, from long PR interval to complete heart block alternating in the same patient. At follow-up (nine months to 19 years), 13 children (62%) had received implantation of a pacemaker because of progression either to complete atrioventricular block or to severe ventricular bradycardia. Atrioventricular conduction improved on exercise in 11 children, but this did not predict a favorable outcome since four of them required pacing. In contrast, deterioration during sinus acceleration in four patients predicted further aggravation. A supra-Hisian location of the block did not protect against the occurrence of syncope in the two patients who underwent His recordings. Finally, the outcome was not linked to age at diagnosis. Second degree atrioventricular block, therefore, should be considered a serious disease because of its trend to worsen towards complete block and/or severe complications. Close follow-up, including repeated 24-hour monitoring, is the most effective way to unmask such a progression and to avoid its clinical consequences.
Alternative uses of the Rashkind umbrella device in congenital and post-surgical cardiovascular lesions—early results and impact on clinical course
Authors: Tabatabaei Hussein , Nykanen David G. , Williams William G. , Freedom Robert M. , Benson Lee N.
Published: August 2008

Implantation of the Rashkind double umbrella device for closure of the persistently patent arterial duct is now routine in many units. The use of this device in non-ductal positions, however, has been reported in only a limited number of patients, and its impact on the subsequent clinical course has not been established. A retrospective review of all such occlusions, therefore, was undertaken to address the early results and impact on clinical management. From 9/1988 through 2/1994, implantation of a double umbrella device was attempted in 21 patients (eight female, 13 male) with cardiovascular communications other than a persistent arterial duct excluding the intentional fenestrations made in the modified Fontan procedure. Median age at implantation was 2.6 years (three months-12.4 years) and weight was 11.6 kg (4.1–45) kg. Lesions included four ventricular septal defects, an aortopulmonary window, an aortopulmonary collateral artery, an atrial septal defect, four Gore-tex® shunts, and a residual persistent communication between the superior caval vein and the right atrium following either a bidirectional cavopulmonary anastomosis, or a classical Glenn operation. Eight patients underwent occlusion of unintentional residual right-to-left (six) or left-to-right (two) communications following the Fontan procedure. Implantation was accomplished in all without mortality, utilizing either 12 mm (13) or 17 mm (eight) devices. Surgical removal of the device was required in one patient four weeks subsequent to implantation. At latest follow-up (1–66 months, median four months), color-flow Doppler studies revealed complete occlusion in 17 (77%) patients. Implantation of the double umbrella in sites other than the duct is, therefore, feasible and simplified technically with a modification of the delivery system. Such novel applications of transcatheter intervention using double umbrella devices may avoid or complement subsequent surgery.
Percutaneous closure of small patent arterial ducts using occluding spring coils and a snare
Authors: Zeevi Benjamin , Berant Michael , Bar-Mor Galit , Blieden Leonard C.
Published: August 2008

In recent years, the percutaneous closure of small and medium-sized patent arterial ducts has been achieved using occluding spring coils. We describe our experience in 33 patients with this technique using a snare to facilitate the procedure. All patients had a clinically apparent patent arterial duct and underwent an attempt at transcatheter closure at a mean age of 5.6 years. In one patient, the duct was a residual lesion following surgical ligation, and in three was residual following attempted closure with a Rashkind double-umbrella. The mean narrowest diameter of the ducts was 1.9 mm. The coil embolized in two of the first four patients, and subsequent to that experience we used a snare to improve delivery. Of the 33 patients, implantation was successful in 32 (97%) using one [29 patients] or two [two patients] coils and in one by a combination of a double-umbrella device and an occluding spring coil. The mean fluoroscopic screening time for the whole group was 26.5 minutes, this time decreasing to 18.5 minutes in the last 26 patients [p <0.05]. Color-Doppler echocardiogram performed the morning after placement of the coils has shown residual leaks in nine of 32 patients [28%]. At a mean follow-up of 7.6 months, repeated imaging has shown residual leaks in four of these patients [12.5%]. No patient had a residual continuous murmur immediately following occlusion. Based on our results, we conclude that occlusion of small to medium-sized ducts using coils appears to be effective. The use of a snare to hold and manipulate the coil as it is delivered improves both control of the coil and the accuracy of its placement.
Transcatheter occlusion of arterial duct with new detachable coils
Authors: Hazama Kyousuke , Nakanishi Toshio , Tsuji Tohru , Kinugawa Yoshikazu , Matsuoka Suguru , Mori Kazuhiro , Saitou Akihiro , Tomita Hitoshi , Momma Kazuo
Published: August 2008

Percutaneous transcatheter embolization of the arterial duct, using new detachable coils, was performed in 19 patients aged from two to 16 years. The minimal ductal diameter ranged from 0.5 to 2.7 mm. We used a 5 mm diameter coil, 8 cm in length and/or an 8 mm diameter coil, which was 10 cm in length. The coil was connected to a delivery wire with a screw system, and could be detached by rotation of the delivery wire. The coil was straightened by inserting a mandril, with loops then being formed by withdrawal of the mandril. In all patients, the coil was advanced retrogradely and either one or two coils were successfully implanted. Implantation was performed in four patients without the catheter entering the pulmonary trunk across the arterial duct. The ductal ampulla was either absent or very small in five patients, so we implanted a coil which had been cut short. Angiography revealed no residual shunting in 14 patients (74%) and trace residual shunting in five patients. Migration of the coils to the pulmonary arteries, or to a systemic artery, did not occur in any patient. Follow-up using Doppler color flow imaging showed complete closure of the arterial duct in all patients (100%), and no turbulence either in the left pulmonary artery or in the descending aorta in any patient. The use of the new detachable coil may improve the accuracy of placement of the device and reduce the incidence of its migration.
Life-threatening cardiac hypertrophy associated with dexamethasone treatment of bronchopulmonary dysplasia
Authors: Gallaher Keith J. , Strickland Braxton , Pickett Kimberly , Carter Jan M.
Published: August 2008

We report the case of an infant who developed severe cardiac compromise due to hypertrophic obstructive cardiomyopathy that developed while he was being treated with dexamethasone for bronchopulmonary dysplasia. This case is illustrative, as previous reports have described steroid-induced hypertrophic obstructive cardiomyopathy as being transient and benign. In this infant, the hypertrophic obstructive cardiomyopathy, waxed, waned, waxed, and waned again during two courses of dexamethasone, thus strengthening a causal link with steroid therapy. A high index of suspicion of hypertrophic obstructive cardiomyopathy as the etiology of hypoperfusion in steroid-treated infants with bronchopulmonary dysplasia is important. Serial echocardiographic monitoring of these infants is helpful in identifying cardiac compromise.
Ebstein's malformation with covered defect of the ventricular septum
Authors: Franke Andreas , Mühler Eberhard G. , Hügel Werner
Published: August 2008

The case of a 27-year-old male with Ebstein's malformation combined with a ventricular septal defect is reported. On its right ventricular side the defect was covered by the dysplastic septal tricuspid leaflet, creating a small chamber in continuity with the left ventricle, but without shunt into the remaining components of the right ventricle. The ventricular septal defect and a patent oval foramen were closed operatively after a cerebral embolic event to eliminate probable sources of thromboemboli preventing the inherent risks of life-long anticoagulation for a young patient. Intraoperative morphology confirmed the preoperative findings.
Absence of the left ventricular myocardium in a newborn
Authors: Lindinger Angelika , Masur Yvonne , Limbach Hans-Gerhard
Published: August 2008

Absence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.
Propafenone associated granulocytopenia in a newborn
Authors: Paul Thomas , Luhmer Ingrid , Reiter Alfred
Published: August 2008

Progressive granulocytopenia (neutrophil count <1000/ml) developed in a newborn during treatment with propafenone (200 mg/m2) for recurrent paroxysmal supraventricular tachycardia. The total white blood cell count, however, remained within normal limits. The neutrophil count increased gradually after propafenone had been stopped. The time course of treatment and the development of granulocytopenia make it very likely, but not certain, that propafenone was the responsible agent. Pediatricians, and especially pediatric cardiologists, should be aware of this rare adverse effect when they consider treatment with propafenone in children with cardiac arrhythmias. Analysis of the white cell blood count, including a differential count, is recommended in all children in order to assess the true incidence of this adverse effect.
Hypoplastic left heart syndrome: multiprofessional follow-up in the mid-term following palliative procedures
Authors: Hagemo Petter S. , Rasmussen Magnhild , Bryhn Grete , Vandvik Inger Helena
Published: August 2008

Of the 12 children who represent the complete cohort of Norwegian patients surviving the complete palliative operative programme for hypoplastic left heart syndrome, 10 were studied by a pediatric cardiologist, a pediatric neurologist, a neuropsychologist and a child psychiatrist. We found significant morbidity. Hemodynamic function was relatively uncompromised in eight of the patients, but one had significant cyanosis due to use of an operative technique subsequently abandoned and one had obstruction of the flow of blood from the left to the right atrium. Five children had disturbances of rhythm, with one requiring insertion of a pacemaker. One child had epilepsy. Delayed motor development, most often to mild degree, was found in seven children, while six had attention problems. Mental retardation was found in two children and autistic traits in one. Despite these various problems, the parents were seemingly satisfied with their own lives, and the quality of life of their children.