bluuebunny/crossref_metadata_2025_split huggingface dataset:
Contrast echocardiography for diagnosis of pulmonary arteriovenous fistulas late after construction of a Glenn anastomosis
Authors: Sagin-Saylam Gül , Somerville Jane 
Published: August 2008

AbstractTo demonstrate the use of transthoracic contrast echocardiography in the detection of pulmonary arteriovenous fistulas in patients with a previously constructed anastomosis between the superior caval vein and the right pulmonary artery (Glenn shunt), and to examine their prevalence in this special population, we evaluated prospectively all patients followed up in the Grown-Up Congenital Heart Unit subsequent to construction of a classical or bi-directional Glenn shunt. We studied 12 patients, aged from 21 to 38 (mean 28 ± 4.8) years who had had a previous cavopulmonary shunt in place for a period of 4 to 33 years (mean 24±9 years). All were examined with cross-sectional contrast echocardiography, 11 patients had cardiac catheterisation and angiography, and 6 patients had magnetic resonance imaging. Systemic arterial oxygen saturations at rest, and during exercise using the modified Bruce protocol, were measured in all patients. Contrast echocardiography showed evidence of pulmonary arteriovenous fistulas in 7 of the 12 patients, with appearance of echo contrast in the left atrium 1–8 seconds after peripheral venous injection in the arm. Simultaneous appearance of microbubbles in the right atrium revealed a residual communication between the superior caval vein and the right atrium in 2 patients, and presence of collaterals between the superior and inferior caval veins in one. Cardiac catheterisation and angiography showed obvious fistulas in 4 patients, and revealed suggestive findings in 2. In patients deemed to have pulmonary arteriovenous fistulas on contrast echocardiography, arterial oxygen saturations at rest (51–94%, mean 75±15.3%) and on exercise (23–91%, mean 53±24.2%) were significantly lower compared to patients judged to be without fistulas (p<0.005). Pulmonary hypertension in the contralateral lung was more common in patients with fistulas (mean left pulmonary arterial pressure 22–110 mm Hg, p=0.014). In patients with cavopulmonary anastomoses, pulmonary arteriovenous fistulas occur frequently in the long term (10–33, mean 25.7±8 years), and are associated with worsening systemic arterial desaturation. Contrast echocardiography should be included in the regular evaluation of these patients as a simple and sensitive technique for the detection of pulmonary arteriovenous fistulas, particularly with the devel opment of increasing cyanosis.
Criss-cross heart with supero-inferior ventricles: successful surgical correction of associated defects
Authors: Kaushal Sunil K. , Shrivastava Savitri , Iyer Krishna S. 
Published: August 2008

SummaryWe present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.
Noninvasive diagnosis of coronary arterial fistulas using multiplanar cine magnetic resonance imaging
Authors: Tsubata Shin-ichi , Ichida Fukiko , Miyawaki Toshio 
Published: August 2008

AbstractIn three children with coronary arterial fistulas, two extending from the right coronary artery to the right atrium and one from the left circumflex coronary artery to the right ventricle, multiplanar cine magnetic resonance imaging was able accurately to demonstrate dilation of the involved coronary artery, the tortuous nature of the dilated fistulas, blood flow within the fistulas, and luminal narrowing. The technique provides a new adjunct for management of patients, especially young children, with coronary arterial fistulas.
Repair of divided left atrium associated with unroofed coronary sinus
Authors: van Son Jacques A.M. , Walther Thomas , Mohr Friedrich W. 
Published: August 2008

AbstractIn a young infant with divided left atrium, unroofed coronary sinus syndrome, and persistent left superior caval vein, the pulmonary venous blood was successfully routed, after atrial septectomy, to the mitral valve by constructing a baffle using the partially detached diaphragm which had initially separated the pulmonary venous and vestibular components of the left atrium. The baffle was augmented with a patch of untreated autologous pericardium.
Right ventricular biopsy findings of hypertrophic cardiomyopathy in a patient with Noonan syndrome
Authors: Skinker Donna M. , Cottrill Carol M. , O'Connor William N. 
Published: August 2008

SummaryWe report right ventricular ultrastructure in a 20-year-old patient with Noonan's Syndrome. Initial left ventricular and septal myocardial hypertrophy, observed at 2 months without obstruction, progressed to biventricular involvement over 18 years. Histopathologic evidence of cardiomyocytic disarray, with ultrastructural evidence of myofibrillar disarray, confirmed typical biopsy findings of hypertrophic cardiomyopathy on the right ventricular side of the septum.
Surgical ablation of a cardiac rhabdomyoma in an infant with tuberous sclerosis
Authors: Henglein Dagmar , Guirgis Nabil-Maurice , Bloch Gérard 
Published: August 2008

SummaryCardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.
Anomalous origin of the right coronary artery from the pulmonary trunk
Authors: Pinto Fátima Ferreira , Kaku Sashicanta , Ramos José Sousa 
Published: August 2008

SummaryWe report a case of anomalous origin of the coronary artery from the pulmonary trunk. The lesion was discovered in an eleven-month-old asymptomatic boy because of a continuous murmur.
An unusual anomalous course of a coronary artery from the pulmonary trunk, coexisting with congenital mitral stenosis and aortic coarctation
Authors: Ho Siew Yen , Gatzoulis Michael A. , Sheppard Mary 
Published: August 2008

AbstractAnomalous origin of a coronary artery is occasionally associated with other congenital heart malformations. Presence of the anomalous artery can then complicate surgical repair. We report an unusually complex arrangement in a patient who died 16 hours following complete surgical repair despite appropriate management.
A complex variant of tricuspid atresia: survival to 60 years without surgery
Authors: Gerlis L M , Mayet J , Somerville J 
Published: August 2008

SummaryWe report a case of tricuspid atresia in which the symptoms underwent spontaneous improvement during childhood, and did not return until the age of 56 after which the patient went into progressive congestive failure and atrial fibrillation. He died at the age of 60, without ever having surgical intervention. Autopsy confirmed the presence of tricuspid atresia due to absence of the right atrioventricular connection, and also revealed partial left juxtaposition of the atrial appendages, an atrial septal defect in the oval fossa, rudimentary right ventricle, double outlet left ventricle with anterior aorta (anatomically corrected malposition), subvalvar and valvar pulmonary stenosis, dilation of the pulmonary arteries, patent arterial duct and major systemic to pulmonary collateral arteries.It was considered that the development of the systemic to pulmonary arterial anastomoses probably accounted for the initial remission of symptoms, whilst the obstruction of the pulmonary outflow tract prevented the development of any significant degree of pulmonary hypertension in the presence of double outlet left ventricle.
Implantation of endovascular stents for hypoplasia of the transverse aortic arch
Authors: Pihkala Jaana , Pedra Carlos A. C. , Nykanen David , Benson Lee N. 
Published: August 2008

AbstractHypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.
Cyanotic nephropathy and use of non-ionic contrast agents during cardiac catherization in patients with cyanotic congenital heart disease
Authors: Dittrich Sven , Kurschat Katrin , Dähnert Ingo , Vogel Michael , Müller Christian , Lange Peter E. 
Published: August 2008

AbstractBackgroundChronic cyanosis with its associated rheologic changes is a known risk factor for glomerular nephropathy. Therefore, contrast-induced nephrotoxicity should be an important consideration for angiographers comparable to diabetics. On the other hand, progressions in diagnostic and interventional techniques have led to expanded indications and a more widespread use of x-ray contrast agents. The aim of this study was to investigate the risk of contrast-induced nephropathy in the small group of patients with cyanotic heart disease prior to surgical repair.MethodsWe investigated 23 cyanotic patients with an oxygen saturation of 82 (50–92)%, age 25 (5–63) years, and 13 control subjects with atrial septal defect, age 37 (20–66) years. Blood viscosity was measured before and after cardiac catherization. Renal damage was evaluatated by selective analysis of urinary proteins and enzymes.ResultsBefore cardiac catheterization, 48% of the cyanotic patients had a moderate glomerulopathy. Cardiac catherization was performed with 3.0 (1.2 – 6.8) mls/kg non ionic contrast medium. Only one of the 23 patients (4.3%) with normal urinary analysis before cardiac catheterization showed renal damage, which involved tubular and glomerular function. Elevated blood viscosity in cyanotic patients was slightly reduced by the contrast. None of the acyanotic controls had contrast-induced nephropathy.ConclusionsThe use of non-ionic contrast medium does not worsen cyanotic glomerulopathy. This finding may be due to the reduction of blood viscosity by the application of the contrast medium. The finding of contrast-induced nephropathy in one patient underlines the importance of monitoring renal function after cardiac catheterization.
Coronary arterial fistulas in childhood
Authors: Wong Kin-tak , Menahem Samuel 
Published: August 2008

AbstractWe reviewed 16 patients with coronary arterial fistulas seen between 1976 and 1997, and aged 2 days to 16 years, with a median age of 3.2 years. Only four patients were symptomatic: two had heart failure, one had exertional dyspnoea, and one infective endocarditis. The fistulas originated from the right coronary artery in seven patients, from the left coronary artery in seven, from both coronary arteries in one patient, while the origin was not clearly defined in the final patient. Associated cardiac anomalies were discovered in six patients, with three of the fistulas being diagnosed at the same presentation. Cross-sectional echocardiography had revealed a dilated coronary artery in 7 out of 11 subjects. The ratio of pulmonary to systemic flows ranged between 0.9 to 3.0, with a median of 1.5. Ten patients were referred for corrective surgery without any mortality. Trans-catheter closure was successfully undertaken in one patient, while spontaneous closure of the fistula was noted in two patients. We conclude that coronary arterial fistulas, although rare and potentially serious, are generally treatable.
Myocardial perfusion scanning in patients considered for late arterial switch
Authors: McLeod Karen A , De Giovanni Joseph , Silove Eric , Alton Helen , Poyner Robert , Brawn William 
Published: August 2008

AbstractBackgroundOur aims were to evaluate left ventricular uptake of radionuclide in patients with Mustard's or Senning's procedure, comparing them with patients who had undergone banding of the pulmonary trunk and conversion to the arterial switch.MethodsTechnetium perfusion scans were performed on 27 patients (25 male), aged from 10 to 28 years with a mean of 17.8 years and a standard deviation of 5.8 years, who had undergone Mustard's or Senning's procedure for correction of complete transposition. Of the 27 patients, six had been accepted for staged conversion to an arterial switch. At the time of the study, two of the six patients had undergone completion to the switch and four had undergone banding of the pulmonary trunk with two then proceeding to the arterial switch. Cardiac catheterisation to measure left ventricular pressure was performed in all six patients and scores for left ventricular uptake of isotope were compared with echocardiographic index of the thickness of the left ventricular posterior wall and measure ments of left ventricular pressure.ResultsUptake of isotope by the left ventricle was generally poor, but was higher in patients following banding and conversion to the arterial switch, as well as in two patients with native obstruction of the left ventricular outflow tract, and one other who subsequently was found to have pulmonary venous obstruction. There was a positive correlation between the thickness of the left posterior wall in diastole and left ventricular uptake of isotope (r = 0.74, p< 0.05). There also a positive correlation between left ventricular pressure and uptake of the isotope (r = 0.68, p<0.05).ConclusionsUptake of radionuclide by the left ventricle after Mustard's or Senning's procedure for complete transposition appears to reflect ventricular pressure and myocardial mass. A prospective study would be required to determine the predictive ability of such scans regarding the ultimate outcome of conversion to arterial switch, but our initial findings suggest that the technique provides an additional non-invasive method of monitoring left ventricular response to pulmonary arterial banding.
Neonatal intrapericardial teratomas: clinical and surgical considerations
Authors: Pratt Jerry W. , Cohen Daniel M. , Mutabagani Khaled H. , Davis J. Terrance , Wheller John J. 
Published: August 2008

AbstractIntrapericardial teratomas are rare primary cardiac tumors of infancy and childhood. We describe three neonates with intrapericardial teratomas diagnosed during fetal life and treated after birth. Clinical and anatomic considerations suggest that cardiopulmonary bypass provides for safe tumor dissection and complete excision of the tumor, thereby decreasing the risk of recurrence.
A unique case of ventricular isomerism?
Authors: Rinne Katy , Smith Audrey , Hot Siew Yen 
Published: August 2008

AbstractIsomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.
Coarctation of the aorta in dizygotic twins
Authors: Caldas Monica , Dhillon Rami 
Published: August 2008

AbstractThe incidence of congenital heart disease is higher in monozygotic than dizygotic twins, with a higher concordance rate. Although coarctation of the aorta has previously been reported in monozygotic twins, to the best of our knowledge it has not been described in dizygotic twins. We report here such a concurrence in dizygotic twins conceived by in-vitro fertilisation. The finding provides support for both genetic and environmental factors in the aetiology of congenital heart disease. Furthermore, it highlights our lack of data regarding the outcome of in-vitro fertilisation.
Sudden cardiac death in infancy due to histiocytoid cardiomyopathy
Authors: Grech Victor , Ellul Bridget , Montalto Simon Attard 
Published: August 2008

AbstractDetailed post-mortem is crucial in infants who die suddenly and without a known cause. We report a rare case of histiocytoid cardiomyopathy with endocardial fibroelastosis, the second case in the world literature. The infant presented with sudden death, but the cardiac histological appearance was initially believed to be caused by Pompes disease.
Fetal diagnosis of common arterial trunk with interrupted aorticarch using color Power Doppler angiography
Authors: Yasukochi Satoshi , Satomi Gengi 
Published: August 2008

AbstractWe successfully visualized the brachiocephalic arteries and aortic arch in a fetus seen at 19 weeks of gestation with a common arterial trunk and interrupted aortic arch by means of color power Doppler angiography, a new diagnostic development of color Doppler echocardiography. Power Doppler imaging is more sensitive to the state of low flow in fetal vessels, thus providing better visualization of fetal vascular structures from an early gestational stage.
Early onset of progressive subaortic stenosis after complete repair of tetralogy of Fallot
Authors: Grech Victor , Mifsud Anton 
Published: August 2008

AbstractTetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.
A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum
Authors: Freedom Robert M. , Yoo Shi-Joon , Javois Alexander 
Published: August 2008

AbstractA patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.
Anterograde double-balloon valvoplasty for treatment of severe valvar aortic stenosis in a preterm baby weighing 1400 grams
Authors: Peuster Matthias , Paul Thomas , Hausdorf Gerd 
Published: August 2008

AbstractWe describe our treatment of a premature baby born weighing 1400 g with severe aortic stenosis, with a gradient of 80 mmHg across the valve. Efforts to advance a 6 mm angioplasty catheter into the stenotic aortic valve via the left ventricle failed. Anterograde angioplasty, instead, was performed using two 4 mm coronary angioplasty catheters. Six months subsequent to the intervention, the pressure gradient measured 25 mmHg, and there was no hemodynamically significant aortic insufficiency.
Tetralogy of Fallot associated with scimitar syndrome
Authors: Azhari Nawal , Al-Fadley Fadel , Bulbul Ziad R. 
Published: August 2008

AbstractWe present a case of tetralogy of Fallot associated with Scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported.
New insight into left ventricular function in tricuspid atresia after total cavopulmonary connection: a three-dimensional echocardiographic study
Authors: Acar Philippe , Saliba Zakhia , Sidi Daniel , Kachaner Jean 
Published: August 2008

AbstractBackgroundIn patients with tricuspid atresia palliated by construction of a total cavopulmonary connection, both pulmonary and systemic circulations depend on the performance of the dominant left ventricle. When estimating the volume of such ventricles using cross-sectional echocardiography, it is necessary to make assumptions concerning the geometry of the ventricular shape. This is avoided by three-dimensional echocardiography, which provides direct volumetric data. Our purpose was to apply this new method to quantify left ventricular volumes and function in patients with tricuspid atresia after construction of a total cavopulmonary connection.MethodsWe studied ten patients (median age: 8 years) with tricuspid atresia who had undergone a total cavopulmonary connection, comparing them with 10 normal children matched for age, sex and size. The three-dimensional echocardiography was performed with electrocardio graphic and respiratory gating. A new transthoracic integrated probe designed for small children was used with a rotational scanning increment of 3°. The 60 slices obtained from the ventricular cavity were stored and formatted in a commercial system (TomTec®). End-diastolic and end-systolic volumes, stroke volume and ejection fraction were calculated after manual tracing of the endocardial surfaces. The volumes were indexed to the body surface area.ResultsAs seen in the reconstructions, the dominant left ventricle in tricuspid atresia had a spherical shape, whereas the normal left ventricle is oblong. The left ventricular volumes and function in tricuspid atresia were 54±4 ml/m2(end-diastolic volume), 28±3 ml/m2(end-systolic volume), 26±7 ml/m2(stroke volume) and 48±6% (ejection fraction). These volumes were not different from those obtained in the controls (p = NS). The left ventricular stroke volume and ejection fraction in 10 patients with tricuspid atresia were lower than those calculated for the controls (p < 0.05).ConclusionsThree-dimensional echocardiography provides a quantitative insight into the pathophysiologic function of the dominant left ventricle in tricuspid atresia after construction of a total cavopulmonary connection.