bluuebunny/crossref_metadata_2025_split huggingface dataset:
Assessment of left ventricular mass and volume by cross-sectional echocardiography in newborns and infants with tricuspid atresia prior to surgical intervention
Authors: Vogel Michael , Skovaranek Jan , Bühlmeyer Konrad 
Published: August 2008

SummaryBecause left ventricular mass may be important in judging feasibility of Fontan type of palliation in tricuspid atresia, this study was undertaken to generate data on left ventricular mass, volume and mass to volume ratio in newborns and infants with tricuspid atresia, native pulmonary stenosis and concordant ventriculoarterial connections prior to any surgical palliation to obtain values for “normal” left ventricular dimensions in tricuspid atresia. The left ventricle was evaluated in the apical two and four chamber view. From these two perpendicular imaging planes, we calculated mass as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yields the index: mass to volume. Data from 23 newborns and infants with tricuspid atresia were compared to data from 30 age-matched controls with normal hearts. In both patient and control groups, growth of the left ventricle was not linear but related to the 1.4 (volume) or 1.25 (mass) power of body surface area. The equation best describing relation between left ventricular volume and body surface area in normals is volume = 60.7(body surface area)1.4−0.2 ml; in patients with tricuspid atresia volume calculates as 96.7(body surface area)1.4+0.9 ml. In relation to normal infants, infants with tricuspid atresia had a left ventricular volume of 167 (135–206)% of normal and a left ventricular mass of 163 (132–201)% of normal. Left ventricular mass assessed in normals calculates as mass = 59.9(body surface area)1.25+1.3 grams; in patients with tricuspid atresia it calculates as mass= 117.5 (body surface area)1.25−0.9 grams. The index of left ventricular mass to volume in patients with tricuspid atresia measured 1.32, not significantly different from controls, in whom this index calculates as 1.34.
Ectopic accessory liver masquerading as spleen in a case with isomerism of the right atrial appendages
Authors: Ho Siew Yen , Trotter Simon E. , Redington Andrew N. 
Published: August 2008

SummaryWe report a case of an infant with isomerism of the right atrial appendages in whom a spleen-like nodule was found at post-mortem. The nodule, on histology, was found to be a fragment of liver. The cardiac lesions were typical of those associated with right isomerism, a complex generally described as the “asplenia syndrome.” The presence of either a spleen, or a nodule masquerading as a spleen, does not preclude an isomeric arrangement of the atrial appendages nor its commonly associated constellation of cardiovascular anomalies.
The role of color-flow mapping in confirming the association of divided left atrium with an atrioventricular septal defect
Authors: Cazzaniga Mario , Gómez Manuel Casanova , de Melo Walquiria Domínguez 
Published: August 2008

SummaryWe describe the unusual association of a divided left atrium (cor triatriatum) with an atrioventricular septal defect with separate atrioventricular orifices in an eight-year-old asymptomatic girl. The diagnosis had been made by cross-sectional echocardiography combined with Doppler technology and color-flow mapping. These methods together demonstrate both the anatomic and hemodynamic findings.
Magnetic resonance in the detection and review of aortic aneurysms following surgical repair of aortic coarctation
Authors: Hayes Alison M. , Baker Edwards J. , Murdoch Ian , Rigby Michael L. 
Published: August 2008

SummaryFormation of aortic aneurysms is an important and not infrequent sequel of surgical repair of aortic coarctation. Detection is often haphazard and fatal ruptures are reported. We report a series of cases where resonance imaging identified aneurysmal dilatation of the thoracic aorta after either patch aortoplasry or subclavian flap repair of aortic coarctation. In one, rupture of the aneurysm occurred five months following the initial resonance study. Repeat imaging confirmed the clinical suspicion of rupture and successful surgical resection was subsequently performed. Magnetic resonance is a practical and reproducible imaging technique which is particularly valuable in the identification of abnormalities within the thoracic aorta following surgical aortoplasty or interventional procedures for aortic coarctation. As a method of routine surveillance in these groups of patients, it is superior to any alternative technique.
Non-cardiac cyanosis in left isomerism—report of two cases of multiple pulmonary arteriovenous malformations
Authors: Burch Michael , Iacovides Paris , Habibi Parviz , Celermajer David 
Published: August 2008

SummaryIsomerism of the left atrial appendages is often associated with cyanosis due to intracardiac structural abnormalities such as common atrium and/or anomalous pulmonary or systemic venous connections. We report two cases of children with cyanosis and left isomerism but no other lesions within the heart. In these cases, multiple small pulmonary arteriovenous malformations were found bilaterally, resulting in intrapulmonary right-to-left shunting and systemic desaturation.
Two-stage repair in a patient with “absent pulmonary valve syndrome”
Authors: Tamimi Hussam , Galal Omar , Halees Zohair Al 
Published: August 2008

SummaryA seven-month-old boy with severe respiratory distress because of absence of the leaflets of the pulmonary valve underwent banding of the pulmonary trunk. Postoperative recovery was uneventful with dramatic improvement of his general and hemodynamic status. Repair was performed successfully almost two years later when the child had grown satisfactorily and was in stable condition.
Appearance of a localized bulge in the anterior aspect of the descending aorta after balloon angioplasty of native aortic coarctation—aneurysm or reopening of the arterial duct?
Authors: Galal Omar , Vogel Michael , Bjoernstad Per 
Published: August 2008

SummaryBalloon dilation of native aortic coarctation was performed in a three-month-old boy. Angiography immediately after dilation revealed a localized bulge on the anterior as pect of the descending aorta interpreted initially as an aneurysm. Angiography four months later, however, failed to detect this bulge. We speculate that the early aneurysmal appearance of the anterior wall of the aorta may have been caused by factors other than a tear in the aortic adventitia.
Insertion of a bypass graft from carotid artery to descending aorta in the management of aortic coarctation with severe hypoplasia of the aortic arch—a report of two cases
Authors: Prabhakar Ganga , Kumar Naresh , Halees Zohair Al , Wilson Neil 
Published: August 2008

AbstractRepair of severe hypoplasia of the aortic arch with coarctation must be based on the individual anatomy of the lesion and, where necessary, one should take into consideration associated cardiac abnormalities. We report a surgical technique which was employed when standard procedures for reconstruction of the arch had failed to relieve the obstruction adequately. A conduit from the carotid artery to the descending aorta was used successfully in two patients to abolish residual stenosis.
Malnutrition and severe rheumatic heart disease underscoring failure to thrive in an eleven-year-old boy
Authors: Saraiva Lurildo Ribeiro , Barbalho Tacito , Filho Djair Brindeiro 
Published: August 2008

SummaryWe describe an eleven-year-old boy with severe retardation in physical growth and severe rheumatic valvar disease. At surgery, a sample of the atrial appendage showed Aschoff nodules, although no antecedent of rheumatic fever was recorded. The patient was HLA-DR5 positive. The case emphasizes the strong association of malnutrition with severe rheumatic heart disease in northeast Brazil.
Subpulmonary tag originating from both sides of the ventricular septum in congenitally corrected transposition
Authors: O'Sullivan John J. , Farrell Desmond J. , Hunter Archibald S. 
Published: August 2008

AbstractObstruction to the left ventricular outflow tract is not uncommon in patients with congenitally corrected transposition. A case of congenitally corrected transposition is described with significant subpulmonary obstruction which was due to a subpulmonary tag. The patient required an aortopulmonary shunt. Her clinical course was subsequently significantly influenced by arrhythmias associated with an accessory pathway. She died suddenly from an arrhythmia. Post-mortem examination confirmed a large subpulmonary tag which originated from both sides of the ventricular septum—a previously unreported finding. Careful evaluation of the nature of left ventricular outflow obstruction is important in this condition as it may alter the surgical management.
Totally anomalous pulmonary venous connection through the right lung and via a “Scimitar” vein to the inferior caval vein
Authors: Dua Rakesh , McTigue Christine , Wilkinson James.L 
Published: August 2008

AbstractWe report a case of totally anomalous pulmonary venous connection in which the two pulmonary veins from the left lung joined to form a common vein which then passed across the midline into a hypoplastic right lung and, after receiving small veins from the right lung, passed inferiorly, exiting the lung below the hilum as a “scimitar” vein and terminating in the inferior caval vein. A separate pulmonary vein from the right lung passed inferiorly independently and joined the “scimitar” vein before it entered the inferior caval vein. There was an associated hypoplastic left heart syndrome.
The value of resonance imaging in diagnosis of pulmonary mycotic aneurysm secondary to infective endocarditis
Authors: Zabal Carlos , Ramírez-Marroqín Samuel , Attie Fause 
Published: August 2008

SummaryMycotic aneurysms of the pulmonary trunk are a rare but recognized complication of infective endocarditis. They are usually associated with infected congenital heart lesions, and especially with persistent patency of the arterial duct. This report deals with a patient with a persistent duct and aortic coarctation who developed a mycotic aneurysm of the pulmonary trunk secondary to infective endocarditis. The lesion was evaluated before and after surgery by magnetic resonance imaging.
An angiographic study of coronary arterial disease in children after heart transplantation
Authors: Ladeia Ana , Bidois Jérôme Le , Piéchaud Jean François , Metzger Jean Philippe , Vouhé Pascal , Kachaner Jean 
Published: August 2008

SummaryCoronary angiograms were analyzed in 29 children after heart transplantation. Age at transplantation was one month to 14 years (mean 4.5 years) with follow-up from five months to six years (mean 2.9 years). Immunosuppression was based on cyclosporine and azathioprine; six patients also received maintenance steroids. Coronary angiograms were obtained after giving an intracoronary arterial bolus of nitroglycerin to avoid spasm. Coronary angiograms showed stenoses, luminal irregularities, loss of luminal diameter and obliteration of branches, and one example of myocardial bridging. The review of angiographic recordings in some patients showed a loss of normal motion of coronary arteries which, instead, appeared rigid. No abnormalities were seen in eight patients (28%). Coronary arterial changes were present between six months and four years after transplantation in 15 patients (52%). Isolated loss of normal motion of the coronary arteries was present in six patients (20%). The separate analysis of 15 patients who underwent more than one coronary angiogram showed a progression of lesions in eight cases. Coronary arterial disease is a frequent complication of heart transplantation in children and there is a progression of lesions in a large proportion of patients. Since there is no satisfactory therapy for this complication, heart transplantation should be reserved for children having no other reasonable therapeutic option.
Embryonic ventricular diastolic and systolic pressure-volume relations
Authors: Keller Bradley B. , Tinney Joseph P. , Hu Norman 
Published: August 2008

SummaryThe embryonic heart and vascular bed interact dynamically to support rapid growth of the embryo during cardiovascular development. Pressure-volume relations define ventricular function during alterations in loading conditions. We analyzed these relationships in the embryonic heart in order to define ventricular function and the response of the ventricle and vascular bed to acute changes in preload. We simultaneously measured ventricular pressure and recorded 60 video images per second in n≥6 stage 16, 18 and 21 white Leghorn chick embryos at baseline and during the infusion of 1–2 microliters of physiologic buffer into the venous sinus (sinus venosus). Ventricular tetany was then induced with the topical application of 2 Molar sodium chloride. Video fields were traced for ventricular pressure and epicardial cross-sectional area. Cross-sectional area was converted to volume assuming ellipsoid geometry, and cavity volume was calculated as total volume minus wall volume derived from the tetanized heart. We defined end-diastole at the onset of ventricular contraction and end-systole at maximum pressure/volume ratio. Stroke volume increased linearly with end-diastolic volume. End-diastolic pressure-volume relations were positive and linear, and end-systolic pressure-volume relations were curvilinear downward. Arterial elastance decreased with growth of the embryo and with volume infusion. Pressure-volume loop area, an index of consumption of energy, doubled between the embryonic stages. Thus, embryonic ventricular pressure-volume relations define diastolic and systolic function at rest and in response to altered preload.
Catheter ablation in children with supraventricular tachycardia mediated by accessory pathways—use of radiofrequency current as a first line of therapy
Authors: Hebe Joachim , Schlüter Michael , Kuck Karl-Heinz 
Published: August 2008

AbstractCatheter ablation of an accessory atrioventricular pathway using 500 kHz radiofrequency current was attempted in 53 children and young adolescents less than 16 years of age (mean 9.3±4.1 years) who were referred for treatment of symptomatic supraventricular tachycardia. Thirty children had the Wolff-Parkinson-White syndrome and 23 had tachyarrhythmias related to an accessory pathway conducting only in retrograde fashion. Of the latter, seven were found to have the permanent form of junctional reciprocating tachycardia, which had resulted in depressed left ventricular function in four. Ablation of left-sided accessory pathways was usually attempted utilizing an arterial approach to the annulus of the mitral valve, while the venous route to the atrial aspect of the tricuspid valvar annulus was chosen for right-sided accessory connections. To reduce invasiveness and the duration of the procedure, a technique with a solitary catheter was attempted in 20 children with the Wolff-Parkinson-White syndrome, regardless of the anatomical site of the accessory pathway. Ablation of 54 of 56 accessory connections was achieved in 51 patients (96% success) with a median of six applications of radiofrequency current. The sessions were completed within 3.4±2.2 hours, with a median exposure to radiation of 31.1 minutes. During a 10-month period of follow- up, no patient required antiarrhythmic medication, and 51 children (96%) were completely free of any symptoms related to arrhythmias. Left ventricular function returned to normal in the four children with tachycardia-induced cardiomyopathy. Except for a patient with an arterial thrombotic occlusion, no serious complications were encountered. Catheter ablation using radiofrequency current is a highly effective and safe curative approach for treating young patients with supraventricular tachycardias mediated by accessory pathways. The use of fewer catheters of smaller size is feasible and advisable in this cohort of patients.
An immunohistochemical study of arterial lesions due to pulmonary hypertension in patients with congenital heart defects
Authors: Aiello Vera Demarchi , Lourdes Higuchi Maria de , Lopes Edgard Augusto , Barbosa Lopes Antonio Augusto , Barbero-Marcial Miguel , Ebaid Munir 
Published: August 2008

AbstractIn order to understand some of the cellular mechanisms of interaction in secondary pulmonary vaso-occlusive disease, we studied 21 lung biopsies from patients with different types of congenital cardiac defects. Their ages ranged from four to 248 months (mean 71.5 months; median 41 months). Changes in the cytoskeleton and extracellular matrix were assessed in the arterial wall. Immunostaining was applied to formalin-fixed, paraffin- embedded tissue, using antibodies to muscle-specific actin, vimentin and fibronectin in supra-optimal dilution. The staining for muscle-specific actin in the medial layer revealed a heterogenous pattern, with areas exhibiting low or absent labelling, reflecting a process of dedifferentiation of the smooth muscle cells in those segments. Within intimal proliferative lesions, the expression of muscle-specific actin was variable, being weak in some lesions and strong in those showing concentrically arranged intimal smooth muscle cells, suggesting a reversion of the migrated cells to the contractile phenotype. The endothelial cells of arteries from cases presenting severe qualitative lesions exhibited strong expression of vimentin, reflecting their heightened regenerative activity and/or their necessity to maintain their shape. The expression of fibronectin was greater in the predominantly cellular lesions of the intima when compared to the fibrotic lesions, indicating the role of that matrix glycoprotein in cellular migration and in replicative processes.
Assessment of ventricular septal defect with aortic valvar prolapse by means of echocardiography and angiography
Authors: Wang Jou-Kou , Lue Hung-Chi , Wu Mei-Hwan , Young Ming-Lon , Chiu Ing-Sh , Chang Chung-I , Hou Shou-Hsien , Chu Shu-Hsun , Hung Chi-Ren 
Published: August 2008

SummaryA total of 80 patients, diagnosed by echocardiography as having ventricular septal defect with aortic valvar prolapse, underwent cardiac catheterization and surgery. Echocardiographic and angiographic results were compared with surgical findings. The ventricular septal defects as observed during surgery were found to be doubly committed and subarterial in 49 (61%), muscular outlet in 10 (13%), and perimembranous in 21(26%). The location had been erroneously categorized by echocardiography and angiography in 12 (15%) and in 15 (19%) patients, respectively. Prolapse of the right coronary leaflet of the aortic valve, as documented by echocardiography, was confirmed by angiography in all but two cases. Prolapse of the noncoronary leaflet was detected by both imaging modalities in three patients. Prolapse of the right coronary and noncoronary leaflets was observed at surgery in 49 and three patients, respectively. The mean size of the ventricular septal defect, when measured by echocardiography, was significantly smaller than that found following surgical measurements (3.3±1.3 vs 8.4±3.8 mm, p<0.001). Our study showed that the ventricular septal defect was erroneously classified in the presence of prolapse of the aortic valve in 15% and 19% of our cases by echocardiography and angiography, respectively. The herniated sinus of Valsalva forming the “roof” of the ventricular septal defect probably redirected the jet across the defect to cause the errors in interpretation. Echocardiography, nevertheless, is as reliable as angiography in our hands in the follow-up of patients with ventricular septal defect opening to the outlet of the right ventricle.
Mitral valvar stenosis in children maintained on hemodialysis for chronic renal failure-clinical significance and an attempt at percutaneous balloon mitral valvoplasty
Authors: Nakazawa Makoto , Takeda Yumiko , Satomi Gengi , Matsumoto Yasutoshi , Kawaguchi Hiroshi , Momma Kazuo , Ito Katsumi , Ando Masahiko 
Published: August 2008

AbstractWe describe three children with chronic renal failure who developed mitral obstructive disease over a period when maintained on hemodialysis. The development and progression of the mitral disease was documented by cardiac catheterization in one child and by Doppler echocardiography in the other two children. These three patients had the lowest body weight among the 16 children undergoing chronic dialysis who were examined cardiologically in our hospital. Autopsy in one child who died from heart failure revealed marked thickening of the leaflets and subvalvar apparatus of the mitral valve with severe obliteration of the intercordal spaces. We found no sign of calcification at the mitral annulus nor at the attachments of the leaflets of the aortic valve, and no Ashoff bodies. We attempted percutaneous catheter balloon valvoplasty in one child which permitted successful renal transplantation after failure of an initial transplantation because of severe pulmonary venous congestion. We conclude that mitral obstructive disease could be a complication of long-term hemodialysis for chronic renal failure. Catheter intervention may serve to release the obstruction, although the procedure is not curative.
Spectrum of valvar abnormalities in Noonan's syndrome—a pathologic study
Authors: Sreeram Narayanswami , Kitchiner Denise , Smith Audrey 
Published: August 2008

AbstractFour patients with the phenotypic characteristics of Noonan's syndrome presented in infancy with a suspected cardiac defect. The cardiac segmental connections were normal. All four were diagnosed to have valvar pulmonary stenosis by cross-sectional echocardiography, the valve being typically thickened and dysplastic as expected for the syndrome. In addition, all had hypertrophic cardiomyopathy and evidence for important abnormalities of the other cardiac valves. Two of the four patients died following cardiac surgery. Of the other two, one child died during interventional catheterization and another died while awaiting cardiac surgery. The cardiac abnormalities, confirmed at postmortem, were dysplastic pulmonary valve with stenosis (four patients), dysplastic aortic valve (three patients), supravalvar aortic stenosis (one patient), small and dysplastic tricuspid and mitral valves (four patients), hypertrophic obstructive cardiomyopathy affecting both ventricles, with asymmetric septal hypertrophy (four patients), atrial septal defect within the oval fossa (one patient). In addition to the usually described abnormalities of the pulmonary valve and ventricular myocardium, the patients described showed a wide spectrum of valvar dysplasia which has received little attention in the past. If these patients do indeed have Noonan's syndrome, and the evidence supports this contention, then these findings support the hypothesis of a more generalized disorder in the development of the ventricles and valves in the syndrome, which has a variable expression. They also stress the need to look carefully for abnormalities of valves other than the pulmonary valve in patients who present with symptoms in infanscy.
Solitary arterial trunk
Authors: Penny Daniel J. , Dua Rakesh , Wilkinson James L. 
Published: August 2008

AbstractA heart is described in which there was a solitary arterial trunk that exited from the heart astride a large perimembranous ventricular septal defect, with the pulmonary arterial supply originating entirely from collaterals from the descending aorta. There was no evidence of either hilar pulmonary arteries or an atretic pulmonary trunk. The morphology of the ventricular outlet component, with absence of the outlet septum supports the description of this heart as solitary arterial trunk, rather than tetralogy of Fallot with pulmonary atresia.