bluuebunny/crossref_metadata_2025 huggingface dataset:
Repair of tetralogy of Fallot with pulmonary atresia in early infancy
Authors: Uemura Hideki , Yagihara Toshikatsu , Kawashima Yasunaru
Published: August 2008

Believing early repair to offer major benefits, we have repaired tetralogy of Fallot with pulmonary atresia in five infants aged less than six months. The ventricular septal defect was closed via a right atriotomy. The right ventriculotomy was 30±3% of the right ventricular length. The posterior wall of the right ventricular outflow tract was created by anastomosing directly the pulmonary trunk to the right ventriculotomy, or either by interposition of the left atrial appendage or an autologous pericardia! flap. The pathway was then roofed over with an equine pericardia! patch. All patients survived and are now doing well from 18 to 41 (31±11) months after the repair, although one patient required reoperation for relief of stenosis at the site of an anastomosis between the pulmonary arteries and the interposed left appendage. In the other patients, postoperative sequential echocardiography has shown no obstruction in the right ventricular outflow tract, nor significant pulmonary or tricuspid regurgitation. We conclude that primary repair in early infancy is an excellent option for surgical treatment of tetralogy of Fallot with pulmonary atresia.
Stent characteristics for preservation of patency of the arterial duct—experimental evaluation
Authors: Rosenthal Eric , Qureshi Shakeel A. , Tabatabaie A. Hussein , Persaud Deo , Kakadekar Ashok P. , Baker Edward J. , Tynan Michael
Published: August 2008

In neonates with duct-dependent congenital heart defects, a reliable non-pharmacological, non-surgical method of maintaining flow through the arterial duct may simplify the management. Stent implantation into the arterial duct has been proposed as such a method. In order to evaluate the technical difficulties which may accompany clinical application, stent implantation into the arterial duct was attempted in 32 newborn lambs aged two to nine days (mean 4.2±1.4) weighing 2.4–7.3 kg (mean 4.6±1.0). Four different types of stent were implanted and the ease of implantation and outcomes were assessed. Stent implantation was technically successful in 28 of the 32 lambs (88%). Of these, 11 lambs died shortly after stent implantation with patent ducts, five lambs had early occlusion of the duct and 12 lambs were entered into a long-term study. The Gianturco-Roubin stent was too rigid and too long to enable successful implantation. The Palmaz-Schatz stent was poorly radio-opaque and was difficult to place accurately. When the aortic orifice was not completely covered, the duct occluded within a few days of implantation. The articulation gap also allowed duct tissue to prolapse into the middle of the stent. The Medinvent and Tower stents were simpler to position due to their superior radio-opacity. The Tower stent had an additional advantage in being a single strand stent which could be easily retrieved if misplaced.
Prenatal diagnosis of tricuspid valvar dysplasia and regurgitation—always a poor outcome?
Authors: Chiappa Enrico , Gaglioti Piero , Todros Tullia
Published: August 2008

The anatomic and pathophysiologic features of tricuspid valvar dysplasia are discussed in the light of two cases observed during fetal life which progressed with an unexpectedly favorable course. Some fetal echocardiographic features are proposed to explain this outcome. First, absence of associated cardiovascular abnormalities; second, clear separation between well represented right ventricular walls and the tricuspid valvar leaflets; third, normal proximal insertion of the tricuspid leaflets and, fourth, normal dimensions of the pulmonary trunk. We recommend karyotyping because of the possible association of this malformation with an aneuploid karyotype. We also describe transitory functional atresia of the pulmonary valve at birth, as far as we know for the first time, in a neonate that had normal forward flow of blood as seen on Doppler sampling in fetal life.
Right superior caval vein draining into the left atrium—diagnosis by color flow mapping
Authors: Alday Luis E. , Maisuls Hector , De Rossi Roberto
Published: August 2008

We report two female patients, one aged four years and the other a newborn, referred for evaluation of cyanosis with otherwise normal cardiovascular findings, who proved to have the right superior caval vein draining into the morphologically left atrium. In both patients, the diagnosis was made by color flow mapping. The older child underwent catheterization and subsequent successful surgical correction. A right superior caval vein draining into the left atrium, although very rare, should always be considered a diagnostic possibility in the presence of cyanosis and normal clinical findings. Color flow mapping is an excellent method with which to make the diagnosis.
Atresia of a solitary pulmonary vein
Authors: Douglas Yvonne L. , van der Werf Tjip S. , Bink-Boelkens Margreet Th.E. , Nikkels Peter G.J. , Ebels Tjark
Published: August 2008

We describe two patients, aged seven and 21 years, with unilateral atresia of a solitary pulmonary vein. Until now, to the best or our knowledge, this condition has been reported only twice. Although symptoms, diagnostic measures and treatment have been described in detail, clinical diagnosis is still difficult.
Right atrial aneurysm
Authors: Kim Yong jin , Kim Hyunjo , Choi Jung Yun
Published: August 2008

Aneurysmal dilation of the right atrium is described in a four-year-old boy who presented with cardiomegaly and symptoms of congestive heart failure. Echocardiography revealed the aneurysmal dilation of the right atrium, but a definite diagnosis was obtained only after surgical exploration. This had caused compression of the other cardiac chambers resulting in congestive heart failure. Surgical resection was successful and the patient was discharged on the eleventh postoperative day with good results.
Cerebral infarction after Fontan procedure for tricuspid atresia
Authors: Rosti Luca , Cerini Emma , Frigiola Alessandro
Published: August 2008

Cerebrovascular accidents are a rare but feared complication of open heart surgery. We report a child with tricuspid atresia who suffered cerebral infarction after a Fontan procedure and discuss possible risk factors.
The evolution of a hypoplastic left ventricle in an infant with an atrioventricular septal defect detected antenatally
Authors: Yates Robert William Michael , Sharland Gurleen Kaur , Qureshi Shakeel Ahmed
Published: August 2008

Complex congenital heart defects are accurately detectable antenatally using fetal echocardiography. In continuing pregnancies with fetal cardiac abnormalities, regular follow-up with advancing gestation is essential. This report documents the changes that occurred in the fetal cardiac anatomy in a fetus detected during antenatal scanning to have an atrioventricular septal defect with isomerism of the left atrial appendages.
Successful neonatal balloon aortic valvoplasty following prenatal diagnosis of critical aortic stenosis
Authors: Yasukochi Satoshi , Satomi Gengi , Harada Yorikazu
Published: August 2008

We report a successful neonatal balloon aortic valvoplasty after prenatal diagnosis of critical aortic stenosis with severe left ventricular dysfunction. The infant was delivered by cesarean section at 34 weeks of gestation and had balloon valvoplasty via the right common carotid artery 67 minutes after birth. Left ventricular contraction improved immediately after the procedure. Scheduled balloon aortic valvoplasty immediately after birth subsequent to prenatal diagnosis can improve the outcome of critical aortic stenosis.
Interruption of the aortic arch with patency of the arterial duct, discrete subaortic stenosis, restrictive ventricular septal defect and severe pulmonary hypertension—survival to adulthood and successful correction by balloon dilation and surgery
Authors: Pinto Robin Joseph , Sharma Satyavan , Shah Nemish
Published: August 2008

We report a case of interruption of the aortic arch associated with patency of the arterial duct, restrictive ventricular septal defect, obstruction of the left ventricular outflow due to a discrete subaortic shelf and severe pulmonary arterial hypertension. The patient survived to adulthood, when a complete angiographic diagnosis was made. The subaortic obstruction was treated by percutaneous balloon dilation performed through a right brachial arteriotomy. Correction of the interrupted arch was carried out surgically using a woven Dacron graft from the aortic arch to the descending thoracic aorta associated with ligation of the duct. Hemodynamic studies after one year revealed a marked decrease in pulmonary vascular resistance and a sustained reduction in the gradient across the left ventricular outflow tract. The case is of interest in the light of the unusual survival and the success of staged management of this complex situation.
Resource Based Relative Value Scale for children—comparison of pediatric and adult cardiology work values
Authors: Garson Arthur , Wolk Michael J. , Morrin Sandra B. , Gold William , Dickstein Michael , Dobson Allen
Published: August 2008

Current procedural terminology codes generally do not distinguish between patients based on age or etiology of disease. As standardized payment schedules based upon such codes are being developed, the precise definition of a “typical” patient for each code becomes increasingly important. Since small size, young age, or presence of complex congenital disease could potentially render the work value of a physician's service different between children and adults, a study was jointly undertaken by the American College of Cardiology and the Cardiology Section of the American Academy of Pediatrics to compare relative work for the most commonly used Current Procedural Terminology codes in pediatric cardiology. A Technical Advisory Panel chose codes for evaluation and wrote clinical descriptors for each service. A separate rating panel of eight pediatric cardiologists (academic and private practice) and three adult cardiologists participated in a mail survey assigning work values to each service relative to current Health Care Financing Administration adult relative value units. The Panel then met and employed a modified Delphi process to arrive at consensus values. Of the 20 codes rated, only five were determined to be similar to adults. The pediatric median values were an average of 44% higher than the adult values (general cardiology codes 8% higher, echocardiography 90%, catheterization 49%, electrophysiology 35%). Thus, work values for cardiology were found to be different between children and adults in 75% of services assessed. The magnitude of these differences should be confirmed in a broader study. The Current Procedural Terminology Committee of the American Medical Association should consider, at a minimum, creating modifiers by specific age for certain procedures. Further study of the quantitative difference between services performed in adults and children is warranted.
Use of pulmonary arterial unifocalization into cryopreserved homograft pulmonary arterial bifurcation grafts to facilitate complete correction of pulmonary atresia with ventricular septal defect, nonconfluent (absent) central pulmonary arteries and multiple major aortopulmonary collateral arteries
Authors: Hopkins Richard A. , Imperato Deborah A. , Cockerham John T. , Shapiro Stephen R.
Published: August 2008

Three children with pulmonary atresia and ventricular septal defect with absent central pulmonary arteries underwent successful complete correction following preliminary staging procedures for unifocalization and deletion of multiple major aortopulmonary collateral arteries. The unifocalization procedures were accomplished using valveless pulmonary arterial cryopreserved allografts. This technique was found to facilitate and simplify the final repair. All patients survived the complete correction. The patients have been followed for 1½-4 years following the final surgery. One child died two years after repair from pneumonia complicated by asthma. One patient appears to have progression of pulmonary vascular disease despite the complete repair, and one patient continues to do well both symptomatically and hemodynamically. The results suggest that this method of unifocalization solves many of the problems inherent to more classical techniques, but the overall prognosis of patients in this diagnostic entity has yet to be completely defined.
Replacement of the mitral valve in children—an analysis of 130 cases
Authors: Abid Fekria , Fekih Manoubia , Khayati Adel , Drissa Habiba , Fehri Wafa , Abid Abdelfettah
Published: August 2008

Replacement of the mitral valve was performed in 130 children less than 15 years of age—115 for rheumatic and 15 for congenital disease. Early mortality was 5.4%. Of 123 survivors, 112 were followed postoperatively for a mean period of nine years, with 80 of them on anticoagulants. Thromboembolism is the major late problem, with 25 thromboembolic accidents noted in 25 patients (22.3%). Anticoagulation was ineffective or lacking in 72% of them. Bacterial endocarditis was noted in five cases (4.4%) and a prosthetic leak in three (2.6%). Particular problems are observed in this group of young patients. First, recurrence of rheumatic fever in nine patients with an increasing severity of aortic valvar incompetence; second, with growth the prosthesis can become small as observed in six of our patients. A reoperation was needed in 15 patients, with one requiring two reoperations. Late mortality was 21%. Optimal timing for replacement of the valve is crucial. Appropriate anticoagulation and correct prophylaxis of rheumatic fever and bacterial endocarditis is mandatory. Conservative surgery is certainly preferable to valvar replacement, but if replacement proves necessary, large prostheses should be used.
Long-term left atrioventricular valvar function following surgical repair of atrioventricular septal defect
Authors: Han Ling , Kang Soon Ung , Park Sang C. , Ettedgui Jose A. , Neches William H.
Published: August 2008

Long-term left atrioventricular valvar function was evaluated in 95 of 110 survivors following surgical repair of atrioventricular septal defect between 1975 and 1984. A common or complete form was present in 40 and a partitioned or partial form in 55 patients. The patients have been followed for three to 13 years with a mean of 8.3 years. Pulmonary arterial banding was performed in 17 patients with a common atrioventricular valve prior to complete repair. The left atrioventricular valvar regurgitation was evaluated by clinical examination, Doppler and/or angiography. Three patients required valvar replacement postoperatively. Previous pulmonary arterial banding, pulmonary hypertension or pulmonary-to-systemic flow ratio did not affect the incidence or severity of left atrioventricular valvar regurgitation postoperatively. In this series left atrioventricular valvar regurgitation increased in the early postoperative period but rarely progressed at late follow-up.
Balloon dilation of stenosed systemic venous pathways following the Mustard operation
Authors: Sreeram Narayanswami , Dikkala Venkateswararao , Arnold Robert
Published: August 2008

Stenoses of the systemic venous pathways are a potentially dangerous complication after the Mustard operation. The acute and intermediate-term efficacy of balloon dilation of these stenoses has not previously been studied in a large group of patients. A retrospective analysis of clinical and angiographic data, with prospective clinical and echocardiographic follow-up, was performed in a consecutive group of 25 patients (17 male, 8 female) who underwent balloon dilation of the superior or inferior caval venous pathways. For 17 dilations of stenoses in the superior pathway, the mean pressure gradient decreased from 9.1±5.7 to 3.4±2.3 mm Hg (p<0.01), while the diameter of the stenosis increased from 5.9±2.9 to 11.2±3.0 mm (p<0.01). For 25 dilations of the inferior pathway, the mean gradient decreased from 4.3±1.8 to 1.4±1.1 mm Hg (p<0.01), while the diameter of the stenosis increased from 7.3±1.5 to 10.6±2.3 mm (p<0.01). Over a median follow-up of 3.5 years, 16 patients have been without symptoms or signs suggestive of recurrence of stenoses. In the remaining nine patients, 11 further procedures were performed at a median of 3.1 years after initial dilation. In five patients who had previously undergone dilation of the superior (3), inferior (1) or both (1) pathways, further dilations of the same pathway(s) and/or stent implantations were performed. Three patients, including one in whom superior caval venous stenosis had recurred, had developed a new stenosis of a previously undilated pathway, while in two patients no further dilations were required. Balloon dilation offers an alternative to reoperation for stenoses of the systemic venous pathways after the Mustard operation, and appears to provide satisfactory intermediate-term palliation. Recurrences of stenoses in previously dilated pathways, and new stenoses in previously unobstructed pathways, are commonly seen at follow-up. Alternative measures, such as placement of stents, need to be considered in the long-term management of these patients.
Visceroatrial heterotaxy syndrome induced by maternal hyperthermia in the rat
Authors: Morishima Masae , Miyagawa-Tomita Sachiko , Yasui Hiroshi , Ando Masahiko , Nakazawa Makoto , Takao Atsuyoshi
Published: August 2008

Maternal hyperthermia induces malformations in human and laboratory animals, but there are only a few reports of cardiovascular anomalies induced by maternal hyperthermia. Pregnant Wistar Imamichi rats were exposed to hyperthermia on day nine of gestation by immersion in a hot-water bath (43.0–43.5 °C) twice a day. Their fetuses were examined on day 18 after fixing with 2.5% glutaraldehyde solution. According to the average body temperature of the dam, the fetuses were divided into five groups, each group increasing by 0.1 °C from 42.5 °C to 42.9 °C. The incidence of cardiovascular anomalies was increased from 24 to 100% with the rise in body temperature of the dams. In the groups with average temperatures of 42.8 and 42.9 °C, most of the fetuses (70–80% of cases with anomalies) showed visceroatrial heterotaxy associated with severe cardiovascular anomalies, including common atrioventricular orifices with double outlet right ventricle or discordant ventriculo-arterial connections. The visceral structures of those fetuses showed a tendency to left isomerism, such as bilateral left atrial appendages, single lobation of the lung on both sides, a symmetrical liver with interruption of the posterior caval vein, hypoplasia of the spleen, and malrotation of the intestinal loop. Day nine of gestation in the rat may be a critical period for determining left-right sidedness of the viscera. The results indicate that the determination of morphological left-sidedness of visceral structures is related in some way with maternal hyperthermia.
Morphometric study of the oval fossa in fetal and neonatal hearts
Authors: Alvarez Luis , Aránega Antonia , Marchal Juan A. , Hernández María J. , Fernández Juan E. , González Amanda R.
Published: August 2008

Microdissection of the oval fossa from its right and left atrial aspects showed that the rim of the fossa was formed from an infolding of the atrial wall, which began between the base of the superior caval vein and the insertion of the right pulmonary vein to the left atrium. Because the rim varied in shape from circular to oval, we used morphometric methods to obtain patterns of normality of the diameters of the oval fossa, oval foramen, and the area of right and left atrial aspects of the septum. These values were found to correlate with log fetal and neonatal weight. Due to technical advances in prenatal diagnosis, these structures can now be examined in fetal life. Therefore, our morphometric data, when appropriately interpreted, have immediate clinical and surgical applications in the treatment of fetal and neonatal cardiovascular disorders.
The degree of scooping of the interventricular septum influences electrocardiographic findings in atrioventricular septal defect
Authors: Suzuki Kiyoshi , Mimori Shigekazu , Murakami Yasuo , Mori Katsuhiko , Tatsuno Katsuhiko , Kikuchi Toshio , Takahashi Yukihiro , Ohtaki Eiji
Published: August 2008

The aim of this study was to clarify those morphological features and hemodynamic stress factors which influence the electrocardiographic findings in atrioventricular septal defect. In 64 patients with the incomplete form of atrioventricular septal defect (separate valvar orifices) with usual atrial arrangement, the length from the left ventricular apex to the aortic valve (outlet dimension), to the so-called cleft (scoop dimension), and to the lowest point of the left atrioventricular valve (inlet dimension) were measured by biplanar cineangiography. The size of the ostium primum defect, the ventricular pressure ratio, and the pulmonary/systemic flow ratio were also evaluated. The results showed that the lower the scoop/outlet ratio (r=0.60, p<0.001), or the higher the ventricular pressure ratio (r= −0.55, p<0.001), the more deviated to the left and superior was the mean frontal QRS axis. These factors also had significant correlation with the QRS patterns in the Frank vectorcardiogram. We conclude that the scoop/outlet ratio, supposedly reflecting the degree of scooping of the interventricular septum, is one of the most significant factors which influence the electrocardiographic findings in atrioventricular septal defect.
Closure of the ventricular component of an atrioventricular septal defect during fetal life
Authors: Sharland Gurleen Kaur , Qureshi Shakeel Ahmed
Published: August 2008

A case of a complete atrioventricular septal defect was detected in a 20-week gestation fetus, in whom the ventricular component of the defect was subsequently noted to close spontaneously with advancing gestational age. The antenatal and postnatal findings are discussed.
Congenital anomaly of the right coronary leaflet complicated by infective endocarditis
Authors: Benatar Avram , Bennink Ger , van de Wal Henry
Published: August 2008

A congenital defect of the right coronary leaflet (fenestration) is an unusually rare cause for aortic incompetence in childhood. We report an eight-year-old child with such an anomaly who presented with infective endocarditis of the left ventricle. The importance of excision of the vegetation prior to the occurrence of life-threatening embolic complications is discussed.
Development of right ventricular outflow tract obstruction after double-lung transplantation for primary pulmonary hypertension
Authors: Saylam Gül Sagin , Somerville Jane
Published: August 2008

We present a patient with primary pulmonary hypertension who had unusually high pulmonary arterial pressure prior to double-lung transplantation. Obstruction of the right ventricular outflow tract developed after transplantation and progressed over the subsequent two years.
Aortic atresia with normal left ventricle and intact ventricular septum—a major form of subaortic stenosis complicating an atrioventricular septal defect with intact septal structures
Authors: Houyel Lucile , Zupana Véronique , Roset Françoise
Published: August 2008

We report an unusual form of aortic atresia with a normal sized left ventricle, intact ventricular septum and isolated cleft of the left atrioventricular valve, with abnormal attachments creating both subaortic atresia and massive regurgitation across the left atrioventricular valve. This combination, never described before as far as we are aware, can be considered as an extreme form of subaortic stenosis complicating a very particular type of atrioventricular septal defect.
Tetralogy of Fallot with pulmonary atresia associated with aortopulmonary window and major aortopulmonary collaterals
Authors: Bhagwat A. R. , Pinto R. J. , Sharma Satyavan
Published: August 2008

We report a patient with tetralogy of Fallot and pulmonary atresia in whom the predominant extracardiac supply of arterial blood was via an aortopulmonary window. Major aortopulmonary collateral arteries originating from the descending thoracic aorta provided an additional source of pulmonary blood flow. The patient underwent successful correction and the postoperative recovery was uneventful.
Surgical treatment of Ebstein's malformation—the ‘Hôpital Broussais’ experience
Authors: Chauvaud Sylvain M. , Mihaileanu Serban A. , Gaer Julian A.R. , Carpentier Alain C.
Published: August 2008

Ebstein's malformation is usually treated by replacing the tricuspid valve, albeit with controversial results. The “Carpentier technique,” introduced in 1980, offers both a new classification and new surgical methods for treatment of this lesion. The classification is based on the severity of the displacement of the septal and posteroinferior leaflets, with four grades of increasing severity. The surgical techniques involve mobilization of the anterosuperior leaflet, reduction of the diameter of the annulus, and longitudinal plication of the atrialized part of the right ventricle. Following this approach, 64 patients underwent consecutive surgical evaluation, with only one valvar replacement. The mean age was 26±15 years. The hospital mortality was nine percent. The mean follow-up is now 5.18±3.25 years. The actuarial survival rate was 87% at one year and 82% at five and 10 years. Of the patients, 90% were in postoperative functional class I or II. Control echocardiography revealed either no insufficiency, or minimal regurgitation in 80%. A second operation was needed in six patients because of right ventricular failure (two patients) or recurrence of valvar insufficiency (four patients). Sinus rhythm was present in 44 (88%) of the surviving patients. We conclude that repair of the malformed tricuspid valve, associated with longitudinal plication of the right ventricle, is safe and efficient. The prognosis depends on right ventricular contractility, which should be evaluated carefully before and after the operative procedure.
Evidence for time-dependent autonomic cardiac reinnervation in children subsequent to transplantation of the heart
Authors: Kreuder Joachim , Netz Heinrich , Paul Thomas , Müller Andreas , Bauer Jürgen , Repp Reinald
Published: August 2008

Autonomic denervation has been assumed to persist after orthotopic heart transplantation. Analyzing spontaneous and induced variations of heart rate, the status of autonomic cardiac innervation was investigated in six children 19–37 months after cardiac transplantation. The age at the time of transplantation varied from three weeks to 15.4 years. Heart rate variability was assessed on 24-hour Holter recordings by calculating time-domain indices (standard deviation of all RR intervals; standard deviation of the mean RR intervals from successive five-minute periods; mean of the standard deviations of RR intervals from successive five-minute periods; proportion of adjacent RR intervals > 50 msec different; square root of mean square successive differences in RR intervals) and frequency-domain variables (low frequency power, high frequency power and total spectrum power). Sinus node recovery time, sinoatrial conduction time and post-pacing cycle lengths were examined at different rates of endocardial atrial pacing. After the first year subsequent to transplantation, standard deviation of the mean RR intervals reached the normal range in four patients, whereas the other time-domain variables became normal in two patients. Both patients displayed normal or near-normal power spectrums of heart rate with normal day-to-night variations. For the remaining patients, spectrums of heart rate failed to show the main frequency peaks. Only the patient with normal variability in heart rate exhibited a decrease in corrected sinus node recovery time at higher stimulation rates, shortening of the first recovery cycle below the pre-pacing level, and a rapid decline of the accelerated post-pacing heart rate as described in innervated hearts. These results suggest the evolution of time-dependent efferent autonomic reinnervation after cardiac transplantation in children.
Repaired aortic coarctation in adults—magnetic resonance imaging with velocity mapping shows distortions of anatomy and flow
Authors: Kilner Philip J. , Shinohara Tokuko , Sampson Cynthia , Mohiaddin Raad H. , Firmin David N. , Somerville Jane
Published: August 2008

Magnetic resonance velocity mapping of intraaortic flow was performed prospectively in adolescents and adults after coarctation repair. The aims were to assess the feasibility and clinical usefulness of the technique in this patient group, and to study flow velocity distributions in repaired regions. Twenty consecutive patients attending for follow-up after repair of aortic coarctation, aged 15–39, mean 25 years, were studied using a 0.5 tesla Picker magnetic resonance machine. Spin echo and cine imaging with phase velocity mapping, echo time 3.6 ms, were used to study anatomy and flow in the repaired region. Transcutaneous ultrasonic examination, with continuous wave Doppler velocity measurement was performed independently on the same day. Velocity maps, acquired successfully in all patients, showed asymmetry and nonhomogeneity of flow in relation to anatomical distortions of repaired regions. Magnetic resonance and Doppler measurements of peak velocity compared as follows: n=20, range 1.2–3.9 m/sec, mean 2.33 m/sec, mean of differences (Doppler-MR) 0.22 m/sec, standard deviation of differences ±0.27 m/sec. Localized velocity peaks adjacent to wall deformations were identified by magnetic resonance in five patients without significant restenosis. Magnetic resonance imaging with velocity mapping proved reliable and informative in follow-up assessment in adolescents and adults after surgical repair of aortic coarctation. There was satisfactory agreement between magnetic resonance and Doppler measurements of peak velocity. Velocity maps showed that localized velocity peaks may occur in limited parts of the stream adjacent to distorted aortic boundaries without stenosis. This could be a cause of overestimation of pressure gradients from peak velocity data, a possibility which requires further investigation.
Irregular heart rate in the fetus—not always benign
Authors: Simpson John M. , Yates Robert W. , Sharland Gurleen K.
Published: August 2008

One hundred-ninety-four fetuses with irregular heart rates were seen over a five-year period at a tertiary center for fetal cardiology. The median gestation at referral was 31 weeks, with a range from 19 to 41 weeks. Of these fetuses, 157 had extrasystoles of either atrial or ventricular origin. Blocked atrial ectopic beats had led to slow ventricular rates (80–110 beats per minute) in 37 fetuses. The structure of the heart was normal in all except two fetuses. Postnatal outcome was known for 165 of the fetuses. Of these, 157 (95%) had an uneventful antenatal and postnatal course. Tachyarrhythmias developed in eight fetuses (5%) in either the prenatal (n=4) or postnatal (n=4) period. Five of 37 fetuses with blocked atrial ectopic beats (13%) developed a tachyarrhythmia. No fetus developed hydrops, and all infants survived. All cases had required treatment with antiarrhythmic drugs. The occurrence of an irregular heart rhythm in the fetus, therefore, is not always benign. Fetuses with blocked atrial ectopic beats require particularly close monitoring.
Serial echocardiographic Doppler evaluation of diastolic function in the normal human fetus
Authors: Weber Howard S.
Published: August 2008

The assessment of diastolic function in the developing fetus is difficult because of constantly changing conditions of loading. Previous reports suggesting improved ventricular compliance with fetal development are limited by the Doppler indices utilized. Using load-dependent and independent variables, we examined and compared serially right and left ventricular diastolic function in 11 normal fetuses (44 echocardiographic studies) at 22±1.0 weeks; 30±1.0 weeks; 36±1.0 weeks of gestation and 64±25 hours postnatally. Load-dependent indices included early to late time velocity integral ratios and percentage of early filling to total diastolic filling. The load-independent index was the peak filling rate normalized to the total time velocity integral. Both right and left ventricular time velocity integral ratios were similar initially, and shifted from late to early diastole with increasing gestational age (greater ratios). The shift in left ventricular ratios was greater, and increased earlier in fetal development. The right ventricular normalized peak filling rate decreased with increasing gestational age, while the left ventricular normalized peak filling rate remained constant, but was greater than the right ventricular rate postnatally. Both right and left ventricular filling shifts from late “active” to early “passive” diastole during fetal development. Left ventricular ratios are greater and increase earlier in fetal development, likely influenced by changes in preload. The right and left ventricular normalized peak filling rates were similar prenatally, indicating similar diastolic filling properties, but disparate postnatally, consistent with improved left ventricular relaxation immediately preceding or at the time of onset of transitional circulation.
Relationship between scooping of the ventricular septum, morphology of the inferior bridging leaflet and electrocardiographic findings in atrioventricular septal defect with common valvar orifice
Authors: Suzuki Kiyoshi , Tatsuno Katsuhiko , Mimori Shigekazu , Murakami Yasuo , Mori Katsuhiko , Takahashi Yukihiro , Kikuchi Toshio
Published: August 2008

We described previously that the degree of deficiency of the ventricular septum (scooping) affects electrocardiographic findings in atrioventricular septal defect with separate right and left atrioventricular orifices. The aim of this study was to clarify the clinicomorphological relationships of the degree of scooping, the precise morphology of the atrioventricular valve, and electrocardiographic findings in hearts having a common atrioventricular orifice. In 63 patients with usual atrial arrangement, the outlet, scoop and inlet dimensions of the ventricular septum were measured by biplanar cineangiography and were compared with the body surface area. The scoop/outlet ratio and inlet/outlet ratio were also compared with the body surface area, atrioventricular valvar morphology, electrocardiographic findings, and severity of regurgitation of the atrioventricular valve. The results showed that the three individual dimensions of the septum correlated significantly with the body surface area, even though the scoop/outlet ratio and inlet/outlet ratio did not. A correlation was found between the scoop/outlet ratio and the degree of left axis deviation on the electrocardiogram (r=0.74, p<0.001). In 16 patients with division of the inferior bridging leaflet, there was a lower scoop/outlet ratio than in the remaining 37 with an undivided bridging leaflet (p<0.01). Of 24 patients with counterclockwise rotation in the horizontal loop of the vectorcardiogram, 22 had an undivided inferior bridging leaflet (p<0.01). The morphology of the superior bridging leaflet, in contrast, was not associated with the scoop/outlet ratio, and neither was the severity of valvar regurgitation. We conclude that the degree of scooping of the ventricular septum is related not only to electrocardiographic findings, but also to the morphology of the inferior bridging leaflet in atrioventricular septal defect with common atrioventricular orifice.
Diagnostic findings and outcome in children with primary restrictive cardiomyopathy
Authors: Neudorf Ulrich , Bolte Angelika , Lang Dieter , Hentrich Frank , Schmaltz Achim A.
Published: August 2008

Restrictive cardiomyopathy is a rare disease in children and adolescents. We have now encountered eight such cases in infants and children aged between nine months and 14 years. The reasons for admission to hospital were not very specific and included recurrent bronchitis, thoracic deformity, pathologic findings on routine electro-cardiography, or signs of cardiac failure. Despite two cases with atrial flutter, the characteristic electrocardiographic sign was a P-wave in lead II of about 0.6 to 1.5 m V. Echocardiographically, the leading feature was enlargement of the atriums, seen in cross-sectional and M-mode tracings. Diameters and functional parameters for the left ventricle, and the EF-slope of the mitral valve were normal. In four patients examined specifically for diis aspect, a typical E/A-ratio was found. Cardiac catheterization was performed in all patients. Left atrial or pulmonary capillary wedge pressures were all abnormal, and pulmonary arterial pressures ranged from 30 to 60 mm Hg. Calculated pulmonary resistances were 3 to 12 units per meter squared. End-diastolic left ventricular pressures were elevated, while comparable right-sided pressures were normal. Percutaneous endomyocardial biopsy was done in six patients and performed as an open biopsy in two. Only unspecified signs, such as myocytic hypertrophy or interstitial fibrosis, were found. At present, five of the children have died within two years of diagnosis. One boy has undergone cardiac transplantation success-fully. The two youngest children are still alive. As there is such a poor prognosis, and no alternative specific therapy is known, cardiac transplantation must be discussed with the family at an early stage, emphasizing its consequences.
The clinical impact of percutaneous balloon expandable endovascular stents in the management of early postoperative vascular obstruction
Authors: Hatai Yoshiho , Nykanen David G. , Williams William G. , Freedom Robert M. , Benson Lee N.
Published: August 2008

To assess the clinical impact of percutaneously implanted balloon expandable endovascular stents on patient management, we reviewed 20 children aged 14 days to 4.8 years (median 1.3 years) with residual vascular obstructions in the immediate postoperative period. Patients included 11 with pulmonary arterial stenosis, five with stenosis of venous pathways after a modified Fontan procedure or bidirectional cavopulmonary anastomosis, and four with a restrictive modified Blalock-Taussig shunt. Placement of the stent was optimal in 18 of 20 patients (24 of 26 Palmaz or Palmaz-Schatz implants). In two patients, rupture of the balloon resulted in malposition of the stent. Reoperation was avoided and symptomatic improvement was noted in 11 of 20 patients (55%), while procedural complications occurred in eight patients. Seven of 10 patients presenting with a low cardiac output syndrome died despite relief of the obstructions. This early experience supports consideration of the application of these devices in the management of significant vascular obstructive lesions in the immediate postoperative period, thus avoiding early reoperation in this profoundly compromised population.
Arterial morbidity following interventional balloon dilation procedures
Authors: Barlow Amanda , Menahem Samuel , Wilkinson James L.
Published: August 2008

Interventional catheterization procedures performed via the arterial route are well recognized therapies which are being employed with increasing frequency. This review summarizes our experience with local arterial complications of such procedures performed on 44 patients in our institution since 1985. A total of 51 balloon dilations were performed. All patients were heparinized during the procedure. Twenty-one procedures (41%) were followed by clinical evidence of reduced arterial circulation to the affected leg more than four hours after the procedure. Continuing infusion of heparin was used in 17 patients, with return of the pulse concerned in six, while two further patients developed evidence of arterial obstruction after an interval (having initially been found to have ‘satisfactory’ pulses at the end of the procedure). The final infant, whose femoral artery had been torn and could not be repaired, was observed without continuing heparinization. Streptokinase and/or tissue plasminogen activator was employed in eight patients, with resultant success in four. The remaining three, and three who failed thrombolytic therapy, underwent embolectomy. The incidence of arterial damage was related to the size of the catheter (assessed by the size of sheath required to introduce the collapsed balloon, indexed to body surface area) and to low weight or young age at the time of the procedure. No relationship could be established with other variables, including operator, type of catheter, or year or duration of procedure. At long-term follow-up, 11 (22%) patients still have absent or reduced pulses in the leg concerned. Arterial damage following interventional catheterization procedures remains a frequent occurrence despite increasing experience and technical improvements.
Cardiac catheterization in children on extracorporeal membrane oxygenation
Authors: Ettedgui José A. , Fricker F. Jay , Park Sang C. , Fischer Donald R. , Siewers Ralph D. , del Nido Pedro J.
Published: August 2008

There are limited reports in the literature that address the unique issues related to cardiac catheterization on extracorporeal membrane oxygenation. Since 1990, cardiac catheterization has been performed on nine patients on extracorporeal membrane oxygenation. The median age was 10 months (range four days to 18 years) and the median weight was 4 kg (range 2.3–73.8 kg). The indications for catheterization were to relieve pulmonary edema in five patients with severe left ventricular dysfunction, and in one instance each, evaluation of probable anomalous left coronary artery, tetralogy of Fallot associated with a diaphragmatic hernia, possible pulmonary venous abnormalities, and postoperative evaluation after an arterial switch. Venous access was obtained through a preexisting femoral venous line in six patients, and through a cutdown of the saphenous or femoral vein in the other three. Interventional catheterization procedures were performed in six of the nine patients in this group. These consisted of blade atrial septostomy followed by balloon septostomy or dilation of the atrial septum in four patients, balloon septostomy alone in one, and placement of a catheter from the left atrium to the circuit used for extracorporeal oxygenation in another. A transseptal puncture was performed in five of them. Adequate decompression of the left atrium with relief of pulmonary edema was achieved in five patients. Six of the nine children in this group died, although there were no complications or deaths related to the catheterization. Children in cardiopulmonary failure on extracorporeal support represent a population at high risk. Diagnostic cardiac catheterization and atrial septostomy to decompress the left heart, when indicated, can be performed safely in such patients.
Postoperative complications after the Mustard operation for complete transposition—a qualitative evaluation with magnetic resonance imaging
Authors: Kaemmerer Harald , Theissen Peter , Schirg Eckart , Kaulitz Renate , Schicha Harald , Kallfelz Hans-Carlo
Published: August 2008

Long-term follow-up studies after intraatrial repair for complete transposition have shown postoperative morphologic and hemodynamic abnormalities in many patients which often require reevaluation and therapeutic intervention. The purpose of this study was to assess, using magnetic resonance imaging, the presence and extent of late postoperative complications in a large cohort of 44 patients undergoing repair with the Mustard procedure. Of these 34 had an intact ventricular septum with or without obstruction of the left ventricular outflow tract, while 10 had an associated ventricular septal defect. Transverse spin-echo and gradient-echo images were acquired of the entire heart from the diaphragm to the bifurcation of the pulmonary trunk. Additional oblique images were acquired for better visualization of venous connections. Abnormal findings were diagnosed by visual inspection of spin-echo and gradient-echo images, and diagnoses were compared to previous findings at cardiac catheterization. On magnetic resonance imaging, a baffle leak was seen in eight patients, two had pulmonary venous obstruction, four had obstruction at the caval veins, 23 had obstruction of the left ventricular outflow tract and 18 had tricuspid regurgitation. Cardiac catheterization showed a leak across the baffle in 16, pulmonary venous obstruction in two, obstruction at the caval veins in four, obstruction of the left ventricular outflow tract in nine, and tricuspid regurgitation in nine. We conclude that combined spin-echo and gradient-echo magnetic resonance imaging provides extensive noninvasive assessment of postoperative sequels and residuals in patients after the Mustard procedure for complete transposition.
Perfusion defects in patients with complete transposition after the Mustard or Senning operation
Authors: Lubiszewska Barbara , Gosiewska-Marcinkowska Elzbieta , Teresinska Anna , Rozanski Jacek , Rydlewska-Sadowska Wanda , Ruzyllo Witold
Published: August 2008

Atrial correction for complete transposition involves a potential risk of later development of right ventricular dysfunction. To determine the reasons contributing to such systemic ventricular failure, radioisotope first-pass and SPECT perfusion studies at rest after one dose of Technetium-99m MIBI were performed in 26 asymptomatic patients, 5.9±2.7 years after a Mustard or Senning repair. Mean age at operation was 4.3±4.3 years. All patients were followed with 24-hour Holter monitoring and echocardiography. Normal perfusion was observed in 11 patients with a mean age at operation of 2.25±1.8 years and mean right ventricular ejection fraction of 49.0±8.7%. Perfusion defects were found in 15 patients with a mean age at operation 5.8±4.8 years (p<0.05) and mean right ventricular ejection fraction of 43.2±9.3% (NS). In the group of older patients, 10 had extensive perfusion defects with significantly lower right ventricular ejection fraction (39.7±9.3%, p<0.05) and even older mean age at the surgery (7.1±5.6 years p<0.02). Mild or moderate tricuspid regurgitation was found significantly more frequently in those patients with perfusion defects (73%) than in the group of younger patients (36.3%), p<0.05. There was no difference in the frequency and type of arrhythmias, nor in the extent of follow-up, between the two groups. We conclude that, in patients undergoing surgery at an older age, it is more common to find perfusion defects and, often, lower right ventricular ejection fraction. Perfusion abnormalities found in such asymptomatic patients may be a result of several factors: impaired right ventricular function as a consequence of pressure and volume overload, enhanced myocardial fibrosis, or long-lasting preoperative hypoxemia and tricuspid valvar dysfunction secondary to ventricular dilation. We suggest that the perfusion defects observed in this study may be a better indicator of systemic ventricular impairment.
Lower mid-line skin incision and minimal sternotomy—a more cosmetic challenge for pediatric cardiac surgery
Authors: Komai Hiroyoshi , Naito Yasuaki , Fujiwara Keiichi , Takagaki Yusaku , Nishimura Yoshiharu , Kawasaki Sadao , Nakamura Takako
Published: August 2008

We performed ten operations in children using a lower mid-line incision and limited minimal sternotomy. The skin incision is made from the level of nipples to the lower end of the xiphoid process, and only the body of the sternum is cut vertically. There was neither operative mortality nor complications. The upper limit of the scar was, at the most, 1 cm beyond the level of the nipples, and was difficult to observe in typical open-necked shirts. We believe our method is suitable in fulfilling the cosmetic needs for eligible children.