bluuebunny/crossref_metadata_2025_split huggingface dataset:
Compression of the pulmonary veins by the descending aorta in patients corrected surgically by the Fontan procedure
Authors: Kawahira Youichi , Kadoba Keishi , Matsuda Hikaru 
Published: August 2008

AbstractWe describe 4 patients in whom the pulmonary veins were compressed between the descend ing aorta and the atrial part of the heart in consequence of an abnormal distribution of the pulmonary blood flow after the Fontan procedure.
Repair of the mitral valve because of pure rheumatic mitral valvar incompetence in the young
Authors: Al Jubair Khalid A. , Jaralla Abdullah , Fadala Mohsen , Bukhari Emad , Al Faraidi Yahya , Al Qethami Huwaida , Al Fagih Mohamed R. 
Published: August 2008

AbstractBetween January 1985 and April 1994, 42 children aged between 7 and 14 years (mean 11.3 years) underwent repair of severely incompetent rheumatic mitral valves with no accompanying mitral stenosis. In 19 patients the tricuspid valve was severely incompetent, while 8 patients had severe aortic incompetence. Overall, the repair incorporated shortening of elongated tendinous cords and insertion of a Duran or Carpentier prosthetic ring. The repair was the sole procedure in 15 patients, whilst 19 patients also had a De Vega tricuspid valvar annuloplasty and 8 had repair or replacement of the aortic valve. There were no hospital deaths. Postoperative transthoracic echocardiographic studies revealed trivial residual mitral valvar regurgitation in 6 patients, mild regurgitation in 18, moderate in 15, and regurgitation severe enough to warrant replacement of the mitral valve in 3 patients. The mean follow-up period was 37 months (maximum 120 months, minimum 1 month). 0139 patients followed-up, 28 attended for more than 5 years. Of these, 7 underwent replacement of the mitral valve for severe regurgitation within 4 years of the repair. Severe mitral regurgitation in 3 patients was controlled by medical therapy. One was eventu ally lost to follow-up, and one patient died of causes unrelated to surgery. The remaining 16 patients had absent to moderate mitral valvar regurgitation. These results contrast with 10 repairs of congenital mitral incompetence, where no patients required re-operation in the immediate 5 years period of follow-up. Every effort should be made in children with rheumatic mitral incompetence to preserve the natural valve by con servative repair, despite the fact that repair of the incompetent rheumatic mitral valve is not so durable as repair of congenitally incompetent valves.
Changes in patterns of left ventricular diastolic filling revealed by Doppler echocardiography in infants with ventricular septal defect
Authors: Ito Tadahiko , Harada Kenji , Tamura Masamichi , Takada Goro 
Published: August 2008

AbstractTo evaluate left ventricular diastolic filling in infants with ventricular septal defect, which has yet to be documented, we measured various Doppler echocardiographic indexes from transmitral flow in the following groups: 10 infants with ventricular septal defect without pulmonary hypertension; 10 infants with ventricular septal defect with pulmonary hypertension; and 9 normal infants to serve as controls. The peak A, total velocity time integral, E area, and A area in patients without pulmonary hypertension were all significantly larger than those in controls. The peak ratio E/A, and 1/3 filling fraction, in patients without pulmonary hypertension were significantly lower than in controls. The peak A, A area, and deceleration time in patients with pulmonary hypertension were significantly larger than in patients without pulmonary hypertension and controls. The peak E/A, area E/A, and 1/3 filling fraction in patients with pulmonary hypertension were significantly lower than in those without pulmonary hypertension and controls. The index of left ventricular mass, as well as the index of end-diastolic left ventricular wall thickness, correlated strongly with peak A, A area, and deceleration time. The ratio between the systolic pulmonary and systemic pressures correlated strongly with peak A, A area, peak E/A, area EtA, and 1/3 filling fraction.These results demonstrated that the patterns of left ventricular filling in infants with ventricular septal defect were different from those in normal infants, and suggested that the abnormal patterns may indicate the insufficiency of adaptation of left ventricle (increase of left ventricular compliance) for volume overload in the presence of a ventricular septal defect..
Sternal defects associated with congenital pericardial and cardiac defects
Authors: Cottrill Carol M. , Tamaren Janet , Hall Brian 
Published: August 2008

AbstractWe describe three cases of sternal defects of varying severity associated with other congenital anomalies. In the most severe case, both anterior and posterior defects were seen, with near-absence of the sternum and pericardium continuous with a large omphalocele. This resulted in external location of organs usually confined within the thoracic and abdominal cavities. A ventricular septal defect was present, and the arterial duct was absent. The course of the ascending aorta was anomalous. The baby had anencephaly and rachischisis. In the intermediate case, a proximal sternal cleft was associated with shortening of the ster num, and absence of the manubrium. Anterior pericardial and diaphragmatic defects were seen, while a scalp defect and an encephalocele were present on the posterior aspect of the head. This baby had tricuspid atresia. The qremaining case had only an anterior defect with a shortened sternum. A supra-umbilical omphalocele contained a left ventricular diverticulum without interposing pericardium or diaphragm. Ventricular and atrial septal defects were present. The first two cases can be considered as representing fail ure of development of both an anterior and a posterior midline field. The third case, much milder than the other two, represents failure of development of an anterior field.
Repair of tetralogy of Fallot associated with atrioventricular septal defect
Authors: Tláskal Tomás̆ , Huc̆ín Bohumil , Kostelka Martin , Chaloupecký Václav , Marek Jan , Tax Petr , Janouàek Jan , Kuèera Vladimír , Hruda Jaroslav , Reich Oleg , S̆kovránek Jan 
Published: August 2008

AbstractTetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiogra phy alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restroration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2–12.5 years after surgery (median 4.9 years, mean 5.9 ± 3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.
Thiamine deficiency mimicking acute rejection following cardiac transplantation
Authors: Gennery Andrew R. , Bartlett Kim , Hasan Asif 
Published: August 2008

AbstractWe describe a recipient of an orthotopic cardiac transplant who developed severe ventricular dysfunction following an episode of sepsis. He had normal cardiac biopsies and responded to treatment with thiamine. Causes other than rejection should be considered in patients who have received a cardiac transplant and who present in cardiac failure.
Intracardiac thrombus associated with resistance to activated protein C
Authors: Kohlhase Bernd , Kececioglu Deniz , Nowak-Göttl Ulrike 
Published: August 2008

SummaryFormation of thrombus in the heart is a rare event in children. Genetic risk factors determined for thrombophilia with additional exogenous risk factors may, none the less, promote cardiac thrombosis. We report on a boy without predisposing cardiac disease, heterozygous for resistance to activated protein C, who developed multiple thrombuses attached to the mitral valve which led to recurrent arterial throm boembolism. In addition this patient showed enhanced levels of lipoprotein (a).
Fetal ventricular tachycardia in long QT syndrome
Authors: Yamada Miho , Nakazawa Makoto , Momma Kazuo 
Published: August 2008

AbstractWe report a case of a fetus presenting intermittent ventricular tachycardia during midgestation, whose mother had long QT syndrome. The arrhythmia disappeared spontaneously, and the electrocardiogram of the baby after birth showed prolonged QTc. As far as we are aware, this is the first case in which ventricular tachycardia was documented during fetal life in a baby with long QT syndrome.
Surgical treatment for aortic regurgitation and left ventricular dysfunction caused by Kawasaki disease
Authors: Takahashi Toshiki , Taniguchi Kazuhiro , Matsuda Hikaru 
Published: August 2008

SummaryA 10-year-old girl required replacement of the aortic valve because of massive regurgitation caused by Kawasaki disease at the age of 3 months. Postoperatively, left ventricular function and mass improved remarkably, with reduction of both hypertrophy and interstitial fibrosis. During a follow-up period of 10 years, she has been free from any cardiac event.
Unusual presentation during childhood of left ventricular myxoma
Authors: Kapoor A. , Radhakrishnan S. , Sinha N. 
Published: August 2008

AbstractAmongst all primary cardiac tumors, myxomas are the commonest, and their commonest site of origin is the left atrium. Myxomas originating in the left ventricle are rare. When seen, they usually present with a history of systemic embolisation and/or syncopal episodes, with constitutional symptoms being absent. We report here a child with left ventricular myxoma who presented with a prolonged febrile illness.
Holt-Oram syndrome and multiple ventricular septal defects: an association suggesting a possible genetic marker?
Authors: Bennhagen Rolf G. , Menahem Samuel 
Published: August 2008

AbstractA family is described where the father has the many skeletal, but none of the cardiac abnormalities associated with the Holt-Oram syndrome. His two daughters have similar skeletal anomalies, but with identical cardiac lesions, as does another patient, raising the possibility of an associated genetic marker.
Left isomerism with tetralogy of Fallot and anomalous systemic and pulmonary venous connections
Authors: Da Cruz E. , Milella L. , Corno A. 
Published: August 2008

AbstractThe association of left isomerism with interruption and azygos continuation of the inferior caval vein, anomalous connection of the right pulmonary veins to the right atrium, an interatrial commu nication of sinus venosus type, and tetralogy of Fallot with right aortic arch and anomalous coronary artery has been observed in a 6-month-old patient. As far as we are aware, the association of these congenital car diac malformations has not previously been reported in patients with isomeric left atrial appendages.
Surgical ablation of a cardiac rhabdomyoma in an infant with tuberous sclerosis
Authors: Henglein Dagmar , Guirgis Nabil-Maurice , Bloch Gérard 
Published: August 2008

AbstractCardiac 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.
Rheumatic fever
Authors: Cleonice de Carvalho Coelho Mota  , Meira Zilda Maria Alves 
Published: August 2008

AbstractIn recent years, rheumatic fever has been declining in the industrialized countries, and has became a rare disease. In developing areas, nonetheless, the sequels of its cardiac involvement have important implications from the stance of public health, and this disease is still the main cause of heart disease in children and young adults. From a historical perspective, the long-term prevention and the control of socioeconomic problems have been proven to be effective, and it is these measures which have contributed to the decline of the disease in developed countries. But, due to its present high prevalence around the world, and the potential for his resurgence, this disease remains an unsolved problem. Furthermore, the complete understanding of its pathophysiology remains a challenge. In this presentation, we will discuss our experience with epidemiological, clinical and prophylatic aspects of this enigmatic disease, and the strategies we have developed to study and control heumatic fever in Minas Gerais, Brazil.
Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death
Authors: Thierrien J. , Gerlis L. M. , Kilner Philip , Somerville Jane 
Published: August 2008

AbstractA patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistu-lous communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.
Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease
Authors: Elizari Amalia , Somerville Jane 
Published: August 2008

AbstractA clinical study on the outcomes of Glenn anastomoses performed since 1987 in eight consecutive patients aged ≥16 years, and in two performed earlier, showed poor results. One badly selected patient died early as a consequence of high venous pressure, while a further seven had early complications. Seven of eight hospital survivors were followed for 1–10 (median 4.2) years with two deaths (1 and 4 years later). Of the remaining five patients, two improved temporarily, but increased arterial oxygen saturation was not maintained after 6 months. The two patients who had undergone a Glenn anastomosis 10 and 34 years earlier were shown to have pulmonary arteriovenous fistulas. The Glenn anastomosis in these older patients is associated with high rates of complication and appears not to give adequate palliation, particularly when it is the only source of pulmonary blood supply. In the adult, the Glenn anastomosis can be used as a staging procedure for Fontan-type surgery, but must be combined with another source of pulmonary arterial supply. Any adult having a Glenn anastomosis, particularly without another source of pulmonary arterial supply, should be warned of the possibility of worsening of cyanosis and symptoms. The second stage of the procedure may need to be performed soon after the first should the hypoxia prove intolerable.
Acute complications in the current era of therapeutic cardiac catheterization for congenital heart disease
Authors: Zeevi Benjamin , Berant Michael , Fogelman Rami , Galit Bar-Mor , Blieden Leonard C. 
Published: August 2008

AbstractThe acute complications of therapeutic cardiac catheterization for congenital heart disease as performed currently in a small unit were reviewed. In recent years, there has been a significant increase in the number of lesions thought amenable to catheter therapy. Only a few reports, however, have addressed the overall incidence of acute complications of therapeutic cardiac catheterization, all representing the experience of centres performing moderate-to-large numbers of procedures. A retrospective review was performed of 425 therapeutic catheter procedures performed at our institution between May 1993 and November 1997. Acute complications were retrieved from the database. This included all adverse events that were clinically recognized at the time of or within 2 weeks after the procedure and which, to the best of the authors' clinical judgement, were related to the catheterization and not part of the natural history of the child's illness. All patients were observed overnight following the procedure, and stayed in hospital if a complication developed. There were 49 acute complications (11.5%), of which 43 (10.1%) were deemed minor and 6 (1.4%) were considered major. The rate was low in patients with valvar pulmonary stenosis, including three neonates (3/45, 6.7%), for those undergoing angioplasty of native co-arctation (1/15, 6.7%) and pulmonary arteries (2/21, 7.4%); and for coil embolization of systemic to pulmonary collateral arteries (1/16, 6.3%). The rate was high in patients with valvar aortic stenosis, including 12 neonates (9/37, 24.3%), and for angioplasty of re-coarctation (4/23, 21.7%). There were more overall complications in neonates (25.6%) than in older patients (10.1%) (p< 0.01). Two patients died (0.5%), but no patient required emergency surgical intervention. In spite of the introduction of many new therapeutic modalities with greater intrinsic risk, and the fact that patients with more complex lesions and who are more acutely ill are being treated, the overall rate of complications remains relatively low. This probably reflects improvements in pericatheterization medical management, in selection of patients, in procedural techniques, and in the experience of operators.
Tachycardias in children originating in the right ventricular outflow tract: lack of clinical features predicting the presence and severity of the histopathological substrate
Authors: Drago Fabrizio , Mazza Andrea , Gagliardi Maria Giulia , Bevilacqua Maurizio , Renzi Paolo Di , Calzolari Armando , Francalanci Paola , Boldrini Renata , Bosman Cesare , Liso Gaetano Di , Ragonese Pietro 
Published: August 2008

AbstractThe aim was to determine whether the clinical features of tachycardias originating from the right ventricular outflow tract in children with an apparently normal heart could predict the presence and the severity of the histopathological substrate. Thirteen children (median age 6 years; range 6 months–12 years) with tachycardia originating from the right ventricular outflow tract of apparently normal hearts, were assessed by echocardiography, heart catheterization with angiography, endomyocardial biopsy (13 patients) and magnetic resonance imaging (MRJ) (nine patients). Tachycardia was symptomatic in six and sustained in nine. Endomyocardial biopsy and MRI revealed acute myocarditis in five patients (38%), fatty infiltration of the right ventricle in two (15%), and minor histologic abnormalities in three (23%). Myocarditis was diagnosed in three of nine patients with sustained ventricular tachycardia, as opposed to two of four with non-sustained tachycardia (p = NS); in three of six symptomatic versus two of seven asymptomatic patients (p = NS); and in two of eight patients in whom ventricular tachycardia was induced during exercise testing as opposed to one of three in which it was not inducible (p = NS). A histopathological substrate was found in six of nine patients with sustained ventricular tachycardia, and in all four with non-sustained tachycardia (p = NS); in five of six patients with symptoms versus five of seven asymptomatic patients (p = NS); and in five of eight with inducible ventricular tachycardia during exercise testing versus all three in whom it was not inducible (p = NS). The mean rate of tachycardia was 184 ± 39 beats min−1 in patients with myocarditis, as opposed to 171 ± 48 in patients without myocarditis (p = NS); and 163 ± 33 in patients with a histopathological substrate compared with 210 ± 65 in patients without a histopathological substrate (p = NS). It is concluded that a histopathological substrate is present in the greater majority of children affected by the so-called right ventricular outflow tract tachycardia, but that the clinical features of the tachycardia do not predict the presence and the severity of this histopathological substrate.
Sternal wound and mediastinal infections in infants with congenital heart disease
Authors: Kearns Barbara , Sabella Camille , Mee Roger B. B. , Moodie Douglas S. , Goldfarb Johanna 
Published: August 2008

AbstractThe objective was to describe the epidemiologic, clinical, bacteriologic and therapeutic features of seven infants who developed sternal wound and mediastinal infections following palliation and/or repair procedures for congenital heart disease. A retrospective chart review was used. All infants with sternal wound and mediastinal infections were < 30 days of age at the initial operative procedure. Six of the infants had hypoplastic left heart syndrome, and one had complete transposition. Two infants required delayed closure of their chest wound. Three infants had superficial sternal infections and presented at a mean of 12 days postoperatively. Four infants had infection of the deep mediastinal structures: they were all asymptomatic and had purulent collections in their mediastinum at their second palliative operation, which was performed at a mean of 120 days after the initial surgery.Staphylococcus aureus, or coagulase-negativeStaphylococcus, was isolated from the wound and/or blood of six infants. All infants with mediastinal infections were managed with operative debridement. Infants with superficial infections underwent local debridement. All infants received long-term intravenous antibiotics. Mediastinal infections in infants undergoing palliative staged procedures for congenital heart lesions may be chronic and indolent, resulting in delayed repair of congenital heart lesions.