bluuebunny/crossref_metadata_2025 huggingface dataset:
Non‐steroidal anti‐inflammatory drugs and cardiac failure: meta‐analyses of observational studies and randomised controlled trials
Authors: Scott Paul A , Kingsley Gabrielle H. , Scott David L
Published: August 2008

Aims:

To determine the risks of cardiac failure with non‐steroidal anti‐inflammatory drugs (NSAIDs) and the specific risks with Cox‐2 specific NSAIDs (COXIBs).

Methods:

We performed meta‐analyses examining the risks of developing cardiac failure in observational studies and in randomised controlled trials (RCTs) involving patients with arthritis and non‐rheumatic disorders. Electronic databases and published bibliographies were systematically searched (1997–2008).

Results:

Five case—control studies (4657 patients, 45,862 controls) showed a non‐significant association between NSAIDs and cardiac failure in a random effect model (odds ratio (OR) 1.36; 95% CI 0.99–1.85). Two cohort studies (27,418 patient years, 55,367 control years) showed a significant risk of cardiac failure with NSAIDs (relative risk 1.97; 95% CI 1.73–2.25). Six placebo‐controlled trials (naproxen, rofecoxib and celecoxib) in non‐rheumatic diseases (15,750 patients) showed more cardiac failure with NSAIDs (Peto OR 2.31; 95% CI 1.34, 4.00). Six RCTs comparing conventional NSAIDs and COXIBs in arthritis (62,653 patients) showed no difference in cardiac failure risk (Peto OR 1.14; 95% CI 0.85–1.53).

Conclusion:

Observational studies and RCTs all show that NSAIDs increase the risk of cardiac failure. Overall risks are relatively small and are similar with conventional NSAIDs and COXIBs. Pre‐existing cardiac failure increases risk.
State of the art: Using natriuretic peptide levels in clinical practice
Authors: Maisel Alan , Mueller Christian , Adams Kirkwood , Anker Stefan D. , Aspromonte Nadia , Cleland John G.F. , Cohen‐Solal Alain , Dahlstrom Ulf , DeMaria Anthony , Di Somma Salvatore , Filippatos Gerasimos S. , Fonarow Gregg C. , Jourdain Patrick , Komajda Michel , Liu Peter P. , McDonagh Theresa , McDonald Kenneth , Mebazaa Alexandre , Nieminen Markku S. , Peacock W. Frank , Tubaro Marco , Valle Roberto , Vanderhyden Marc , Yancy Clyde W. , Zannad Faiez , Braunwald Eugene
Published: August 2008

Natriuretic peptide (NP) levels (B‐type natriuretic peptide (BNP) and N‐terminal proBNP) are now widely used in clinical practice and cardiovascular research throughout the world and have been incorporated into most national and international cardiovascular guidelines for heart failure. The role of NP levels in state‐of‐the‐art clinical practice is evolving rapidly. This paper reviews and highlights ten key messages to clinicians:

NP levels are quantitative plasma biomarkers of heart failure (HF).

NP levels are accurate in the diagnosis of HF.

NP levels may help risk stratify emergency department (ED) patients with regard to the need for hospital admission or direct ED discharge.

NP levels help improve patient management and reduce total treatment costs in patients with acute dyspnoea.

NP levels at the time of admission are powerful predictors of outcome in predicting death and re‐hospitalisation in HF patients.

NP levels at discharge aid in risk stratification of the HF patient.

NP‐guided therapy may improve morbidity and/or mortality in chronic HF.

The combination of NP levels together with symptoms, signs and weight gain assists in the assessment of clinical decompensation in HF.

NP levels can accelerate accurate diagnosis of heart failure presenting in primary care.

NP levels may be helpful to screen for asymptomatic left ventricular dysfunction in high‐risk patients.

Published by Elsevier B.V. on behalf of European Society of Cardiology.
Altered glial cell line-derived neurotrophic factor (GDNF) concentrations in the brain of patients with depressive disorder: A comparative post-mortem study
Authors: Michel Tanja M. , Frangou Sophia , Camara Sibylle , Thiemeyer Dorothea , Jecel Julia , Tatschner Thomas , Zoechling Robert , Grünblatt Edna
Published: August 2008

Introduction

A growing body of evidence suggests that the glial cell line-derived neurotrophic factor (GDNF) is involved in the aetiopathology of mood disorders. GDNF is a neurotrophic factor from the transforming growth factor-β-family, playing a role in cell development and function in the limbic system. This is the first study to examine GDNF concentration in different brain regions of patients with depressive disorder (DD).

Material and Methods

We used sandwich-ELISA-technique to ascertain GDNF concentration and Lowry assay for overall protein levels in post-mortem brain tissue of 7 patients with recurrent depressive disorder and 14 individuals without any neurological or psychiatric diagnoses. We included cortical regions as well as limbic area's (hippocampus, entorhinal cortex) basal ganglia (putamen, caudate nucleus), thalamus and cingulated gyrus.

Results

We found a significant increase in GDNF concentration in the parietal cortex of patients with DD compared to the control group. In other regions the trend of an increased GDNF concentration did not reach statistical difference.

Discussion

This proof of concept study supports previous findings of an alteration of the GDNF in patients with depressive disorder. However, for the first time a significant increase of GDNF in a cortical brain area was found in DD.
Prevalence of Bartonella species antibodies and Bartonella species DNA in the blood of cats with and without fever
Authors: Lappin Michael R. , Breitschwerdt Edward , Brewer Melissa , Hawley Jennifer , Hegarty Barbara , Radecki Steven
Published: August 2008

The purpose of this study was to determine whether there are associations between Bartonella species antibody (enzyme-linked immunosorbent assay (ELISA) and Western blot (WB)) and polymerase chain reaction assay results in cats with and without fever. Afebrile control cats (39/93; 42.0%) were more likely to have Bartonella species antibodies than cats with fever (29/93; 31.2%). The difference in prevalence of Bartonella species deoxyribonucleic acid (DNA) in blood of cats with fever (14/81; 17.3%) as compared to afebrile control cats (6/81; 7.4%) approached statistical significance ( P=0.0571). Bartonella species ELISA or WB results frequently did not correlate to the presence or absence of Bartonella species DNA in blood. The results of this study indicate that in cats, Bartonella species antibody tests cannot predict whether fever is due to Bartonella species infection and should not be used to determine the Bartonella species infection status.
Virtual Patient Training to Improve Reproductive Health Care for Women With Intellectual Disabilities
Authors: Boyd Sara E. , Sanders Carla L. , Kleinert Harold L. , Huff Marlene B. , Lock Sharon , Johnson Stephanie , Clevenger Kim , Bush Nathania A. , Van Dyke Eileen , Clark Tara L.
Published: August 2008

A multimedia virtual patient module, involving the case of a young woman with mild intellectual disabilities with a complaint of diffuse abdominal pain, was developed as a clinical training tool for students in health care professions. Primary objectives following use of the module included improved knowledge and reduced perception of difficulty in treating women's health patients with intellectual disabilities. The module was developed using an iterative, collaborative process of a core development team that included medical professionals, multimedia specialists, the parent of a child with intellectual disability, and a disability advocate. Over the course of the module, students were required to identify appropriate and effective clinician–patient interactions in addition to relevant medical and developmental concerns for this patient population. Pilot data from a sample of nursing, physician assistant, and medical students suggest that the module is an effective tool for both improving students' knowledge and reducing their perception of difficulty in providing care to women's health patients with intellectual disabilities.
The surgical repair for complete transposition
Authors: Kirklin John W.
Published: August 2008

Although uncertainty exists, in some situations, as to the optimal type of operation and a true cure may not always be possible, the outlook for patients with all types of complete transposition is poor without surgical treatment. With surgery, and in spite of current uncertainties as to the optimal type of repair, most patients can be greatly benefited, and many given a long and happy life. Even more should be possible in the future.
The diagnostic evaluation of patients with complete transposition
Authors: Rigby Michael L. , Chan Kit-Yee
Published: August 2008

The vast majority of patients with complete transposition present during either the neonatal period or early infancy. Almost always the diagnosis and the categorization of associated lesions can be established by cross-sectional echocardiography. Doppler techniques usually provide enough information about flows to allow palliative or radical surgery to be undertaken without formal cardiac catheterization, because balloon atrial septostomy is carried out with echocardiographic imaging, often on the intensive care unit. Angiography is occasionally performed to visualize the pulmonary arteries or the aorta, and, if there is suspicion of a significant elevation of the pulmonary vascular resistance, it must be measured at cardiac catheterization. Neither cross-sectional echocardiography nor aortography is entirely reliable in identifying those patterns of the coronary arteries which are a contraindication to the arterial switch operation. There is, as yet, no evidence that either magnetic resonance imaging or transesophageal echocardiography are superior to more conventional methods of preoperative diagnosis in complete transposition and it seems unlikely that they will be required.
Morphologic aspects of complete transposition
Authors: Anderson Robert H. , Henry G. William , Becker Anton E.
Published: August 2008

Complete transposition is a morphologic entity which exists by virtue of a concordant atrioventricular connection co-existing with a discordant connection at the ventriculoarterial level. If diagnosed in this manner, all variants can be described simply and unambiguously in terms of associated malformations along with variations in relationships and infundibular morphology.
Coronary arterial anatomy in regard to the arterial switch procedure
Authors: Kurosawa Hiromi , Imai Yasuharu , Kawada Masaaki
Published: August 2008

Since the success of the arterial switch procedure is dependent on the successful translocation of the coronary arteries, a thorough understanding of the anatomic variations of the coronary arteries is essential. We reviewed the anatomy of the coronary arteries encountered in 140 consecutive cases undergoing the arterial switch procedure for complete transposition at the Heart Institute of Japan. The anatomic variations, described using the Shaher and Puddu classification, varied significantly, requiring alteration in the surgical technique.
Réparation à l'Etage Ventriculaire — The REV procedure: Technique and clinical results
Authors: Lecompte Yves
Published: August 2008

From November 1982 to July 1989, fifty patients were treated by the REV procedure for complete transposition associated with ventricular septal defect and obstruction of the pulmonary outflow tract. The age at operation ranged from 4 months to 15 years (mean 3.8 years). The technique, based on the resection of the outlet (infundibular) septum, is described in detail. Operative mortality was 18%, essentially related to technical errors or faulty indications at the beginning of our experience. There was no late death. Six patients needed reoperation for pulmonary stenosis (2 cases) or a residual defect, in some cases associated with tricuspid regurgitation. All survivors were in excellent clinical condition. The REV procedure has fewer limitations and better results than the classical approach using Rastelli's procedure.
Modification of the Mustard operation for surgical treatment of complete transposition by creating a confluence of the caval veins
Authors: Oelert Hellmut
Published: August 2008

In this review, I describe our modification of the Mustard operation for hemodynamic correction of complete transposition which, in principle, consists of intraatrial construction of a caval venous confluence. A new design of the intraatrial baffle has been developed using Gore-Tex. Fewer complications have been encountered with this method when compared to other techniques of atrial redirection. The Mustard procedure, therefore, will retain a future role when anatomic abnormalities, or other circumstances, preclude anatomic correction at the arterial level.
The Senning operation for complete transposition: mid-term physiologic, electrophysiologic, and functional results
Authors: Merrill Walter H. , Stewart James R. , Hammon John W. , Johns James A. , Bender Harvey W.
Published: August 2008

We report analysis of surgical results in 110 consecutive infants and children who underwent atrial repair of simple complete transposition using the Senning operation between February 1978 and May 1990. Mean age at operation was 5.4 months ± 6.1 (range 1 week to 4 years); 75 were less than 6 months old. There were 72 males and 38 females. Operative mortality rate was 5.5%, with one late death. Average follow-up is 48.1 months with 44 followed greater than 3 years, and 27 greater than 5 years. Postoperative cardiac catheterization was performed in 48 patients. Right ventricular ejection fraction averaged 0.52 ± 0.08 and was normal in 28 patients. Response of right ventricular ejection fraction to afrerload stress was abnormal in 12 of 14 patients tested. Right ventricular ejection fraction increased normally during exercise in 6 patients, but was abnormal in 15. Mild tricuspid regurgitation was noted in 12 patients. Mild obstruction of the superior caval vein was noted in 4 patients. Baffle leak requiring reoperation occurred in one patient. Fifty-seven of 90 patients are in sinus rhythm by latest electrocardiogram. Postoperative electrophysiologic studies were performed in 34 patients, and Holter monitoring in 25. Significant arrhythmia occurred in 26 patients: 6 patients required pacemakers for slow junctional rhythm or complete heart block; 20 additional patients have a junctional rhythm. Six patients have delayed sinus nodal recovery time. At last follow-up, 88 children (98%) are in New York Heart Association functional Class I, and 2 (2%) are in Class II. The Senning operation for compete transposition can be accomplished with a low operative and late mortality. Serious baffle complications requiring reoperation are rare. Surviving patients are clinically well, but arrhythmias and depressed right ventricular function may limit their long-term functional status.
Functional assessment of the heart after corrective surgery for complete transposition
Authors: Redington Andrew N.
Published: August 2008

The justification for the introduction of the arterial switch procedure was based, primarily, on concern regarding the long-term ability of the right ventricle to perform as the systemic pumping chamber. In this article, the functional performance of both the systemic and pulmonary ventricles after atrial redirection procedures and the arterial switch operation will be discussed.
Arrhythmias after surgery for complete transposition: Do they matter?
Authors: Deanfield John E. , Cullen Seamus , Gewillig Marc
Published: August 2008

Concern about long-term complications after intraatrial repair of complete transposition has been used as an argument in favor of “anatomic” repair by the arterial switch operation. Late arrhythmias, including loss of sinus rhythm and the development of supraventricular tachycardias, particularly atrial flutter, are widely reported after intraatrial repair. Despite modifications of technique, the electrophysiologic substrate for arrhythmia results from the extensive atrial surgery required. Arrhythmias occur, even in the “modern surgical era” after both Mustard and Senning operations, are progressive, and appear to be inevitable. The circulation after an intraatrial repair is more vulnerable to the effects of excessive tachycardia, and this may place the patient at risk from sudden cardiac death. Current attempts at individual stratification of risk are disappointing using even aggressive electrophysiologic approaches, and a combined assessment involving hemodynamics is likely to be necessary. The electrophysiologic and arrhythmic consequences of the arterial switch operation have been less extensively researched but, as might be expected, are quite different from those seen after intraatrial repair. The atrial activation sequence is relatively undisturbed, and sinus nodal dysfunction and supraventricular arrhythmia are uncommon. Ventricular extrasystoles are the arrhythmia most consistently found during the short follow-up currently available. In the longer term, myocardial ischemia, hemodynamic disturbances and autonomic imbalance may predispose to late arrhythmia. Current evidence would suggest that the lack of clinically significant arrhythmia and the restoration of the left ventricle to the systemic circulation are significant advantages of the arterial switch operation over intraatrial repair procedures.
Results of the arterial switch procedure for complete transposition with an intact ventricular septum
Authors: Mee Roger B. B.
Published: August 2008

With the general acceptance that the arterial switch operation represents the surgical treatment of choice for complex complete transposition, an increasing number of centers are leaning towards favoring this approach for simple transposition as well. As the arterial switch procedure is a more difficult surgical procedure than the Mustard or Senning operation, the learning curve has been longer and more hazardous. Centers with large referral bases have, understandably, enjoyed a more concentrated exposure to the arterial switch operation, and are now achieving early rates of mortality as low as those quoted for atrial repairs.
Update on the rapid two-stage arterial switch procedure
Authors: Jonas Richard A.
Published: August 2008

In 1989, we reported the results of surgical treatment in 11 patients with complete transposition and an intact ventricular septum or restrictive ventricular septal defect who had undergone a two-stage arterial switch procedure beyond the neonatal period with a short period (median 9 days) between banding of the pulmonary trunk and repair. The most common indication for the procedure was referral to our center after the neonatal period from a geographically distant center where the arterial switch procedure was not available. Other indications were multi-organ failure in the neonatal period which contraindicated corrective surgery at that time, as well as prematurity or late development of right ventricular dysfunction, which contraindicated correction at the atrial level planned at the referral center. In this report, we describe our continuing experience.
Anatomic correction of complete transposition with ventricular septal defect in neonates: experience with 42 consecutive cases
Authors: Planché Claude , Serraf Alain , Lacour-Gayet François , Bruniaux Jacqueline , Bouchart François
Published: August 2008

Perhaps paradoxically, it was in the form of complete transposition with a ventricular septal defect rather than an intact ventricular septum that the arterial switch operation was first successfully applied. This was, in part, because of the poor results of the physiologic repair in the presence of a ventricular septal defect, but more because the left ventricle, in the presence of the septal deficiency, is immediately suitable for supporting postoperatively the increased workload imposed on the systemic circulation. Spurred by this success, the procedure was then extended to neonates born with complete transposition and an intact ventricular septum and is currently employed with good results in this group.
Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum
Authors: Anderson Robert H. , Anderson Christine , Zuberbuhler James R.
Published: August 2008

Atresia of the outflow tract of the right ventricle in the presence of an intact ventricular septum poses major problems for surgical management. In the light of known problems, 43 autopsied hearts with this congenital abnormality were evaluated to note the arrangement of the myocardium and the cavity of the right ventricle, the state of the tricuspid valve, the precise substrate for pulmonary atresia, and the presence offistulous communications between the ventricular cavity and the coronary arteries. The specimens could be divided into two groups. The first group (38 hearts) had hypoplasia of the cavity of the right ventricle with mural hypertrophy while the second group (5 hearts) had a dilated right ventricular cavity with thinning of the wall. The larger group could be divided into two subgroups on the basis of the substrate for pulmonary atresia. Twenty hearts had muscular atresia of the right ventricular outflow tract and 18 had a potentially patent outflow tract blocked by an imperforate pulmonary valve. A comparison of these two subgroups revealed that mural hypertrophy and cavitary hypoplasia were more severe in hearts with muscular atresia. The tricuspid valve was hypoplastic and its leaflets were not dysplastic in this group but the leaflets were dysplastic in hearts with valvar pulmonary atresia. Fistulous communications between the ventricular cavity and the coronary arteries were only seen in the cases with muscular atresia. In this series, recognition of muscular pulmonary atresia would have identified those patients with the worst surgical prognosis.
The changes in right ventricular diastolic indices in babies with pulmonary atresia and intact ventricular septum undergoing corrective surgery: a pulsed Doppler echocardiographic study
Authors: Leung Maurice P. , Lo Peter T. S. , Lo Roxy N. S. , Cheung Henry , Mok Che-Keung
Published: August 2008

We used pulsed Doppler echocardiography to study the right ventricular diastolic function of29 normal babies and 12 neonates with pulmonary atresia and intact ventricular septum. Eleven patients underwent staged operations of closed pulmonaryvalvotomy followed by either rightventricular outflow reconstruction (n=5) or balloon angioplasty of the pulmonary valve (n=3). In normal babies, the Doppler wave form showed dominant early (E) and separate late active (A) waves of activity, when the heart rate was slow (<100/min). The two waves gradually merged with increasing heart rate, to form a monophasic active wave. Prior to any intervention, all except one of our patients had only a monophasic active right ventricular filling wave over the entire range of heart rates recorded. To quantitate these differences in ventricular filling, we derived 4 diastolic indices from the ratio of: the peak velocity of the early versus the late active wave (EIA); the integral with time of these waves (E/Aarea); the time of diastolic filling relative to the cardiac cycle (TIRR); and the peak diastolic filling velocity relative to the mean filling velocity over the cardiac cycle (Velocity Index). Profiles of the indices against heart rate for both normal controls and patients indicated that only the index of the time of diastolic filling (T/RR) and the Velocity Index were appropriate for our serial comparisons. Thus, for neonates with pulmonary atresia, the index of the time (TIRR) was significantly lower (0.29±0.03 vs 0.43±0.04, p<0.01) and the Velocity Index higher (5.98±0.79 vs 3.98 ±0.31, p<0.001) than those of normal. After valvotomy, but with a poor surgical result, babies continued to have a predominantly monophasic right ventricular filling pattern without significant improvement (p>0.05) of the index of time (T/RR=0.29±0.05) or velocity (5.88±1.17). Babies who underwent a second stage procedure and achieved a final good result had predominantly biphasic right ventricular diastolic filling waves with significant progression (p<0.001) in the index of time (T/RR=0.42±0.03) and velocity (4.09±0.49).
Exercise in congenital heart disease
Authors: Cullen Seamus , Celermajer David S. , Deanfield John E.
Published: August 2008

Exercise is of both physical and psychological benefit in children with congenital heart disease, leading to improved cardiovascular fitness and better quality of life. In such children, however, capacity for exercise is often impaired, and there is a risk of exercise-induced mortality or morbidity. We have reviewed the abnormal cardiovascular response to exercise in children with congenital heart defects and postoperative residua, and the role of exercise testing and training in their diagnosis and treatment. Some patients should be excluded absolutely from all but mild regular exercise because of known high risk; for example, those with severe aortic or subaortic stenosis. Other patients should be encouraged to participate in sports without any limitation; for example, those with small left-to-right shunts or mild valvar regurgitation. In many patients strict recommendations cannot be made, and one must consider the individual, the lesion and its hemodynamic implications, and the type and level of exercise contemplated. Children with congenital heart disease should be encouraged to participate in exercise and recreational sport within the limits provided by their cardiovascular defect. Understanding the pathophysiology of these defects, and knowledge of the risk of exercise in certain conditions, will allow the physician to make sensible recommendations for participation in exercise by individual patients.
Hypercyanotic spells caused by antegrade catheterization of the pulmonary arteries in tetralogy of Fallot: fact or fiction?
Authors: Murdoch Ian A. , Qureshi Shakeel A. , Anjos Rue Dos , Parsons Jonathan M. , Baker Edward J. , Tynan Michael J.
Published: August 2008

Between January 1985 and March 1990, 66 children with the tetralogy of Fallot underwent 85 cardiac catheterization procedures. The mean age at first procedure was 2.5 years (range 0.1–;14.4 years) and the mean weight was 10.4 kg ( range 2.4–36.0 kg). Diagnostic cardiac catheterization was performed in 60 procedures and balloon dilatation in 25. Hypercyanotic spells had occurred prior to 24 (28%) of the procedures (all the patients being on propranolol) and a systemic-to-pulmonary arterial shunt had been constructed before 28 (33%) procedures. Of the procedures, 54 (64%) were performed under local and 31(36%) under general anesthesia. The pulmonary trunk was entered antegradely in 52 procedures, retrogradely through a shunt in 6 and not entered in 27. Balloon dilatation was performed under general anesthesia on 25 occasions. No procedure was abandoned because of a cyanotic spell. Nine (11%) spells occurred during 86 procedures, one of the procedures being postponed because ofa spell occurring after premedication, the procedure and not, therefore, continuing to catheterization. Five spells occurred before the catheter was positioned in the heart, 2 spells occurred during catheterization. Of the spells, eight occurred during procedures in children who had not had previous shunts. Antegrade entry into the pulmonary trunk in the group with shunts was associated with no spells compared with 6/38 (18%) in the group not having undergone surgery (p<0.l). In the group not undergoing surgery, when the pulmonary trunk was not entered, 1 (5%) spell occurred during 19 procedures compared with 6/38 (18%) when the pulmonary trunk was entered (p<0.4). The only clinically important factor which significantly influenced the incidence of spells was the use of general anesthesia, which was associated with 6/31 (19%) spells compared with local anesthesia which was associated with 2/54 (4%) spells (p<0.026).
The technique of fetal echocardiography, with its indications and results in a selected population
Authors: Azcárate Maria J. Maitre , Jiménez Manuel Quero
Published: August 2008

One of the most important contributions of echocardiography to the diagnosis of congenital cardiac malformations is represented by its use in prenatal evaluation. In the present work we discuss the analysis of echocardiographic findings in a preliminary study of the hearts of fetuses in 273 pregnant women. The cases have been divided into three groups. The first group of 169 women was made up of those without a known factor conferring high risk on the pregnancy. The second group of 18 women was comprised of those of suspicions of either arrhythmias or congenitally malformed hearts. The final group of 86 women, consisted of those having had previous children with congenital heart disease. The incidence of pathology in those without risk was 0.5%. In those referred because of suspicion of disease, the incidence was 16.6%, while in those with previous history of children with heart disease, the incidence was 5.8%. Our findings proved the utility of the echocardiogram for the diagnosis of heart malformations prior to birth and confirmed the increased incidence of malformations in fetuses in mothers who had previously produced children with congenital heart disease. The high incidence of cardiac abnormalities in the group referred on the basis of an earlier scan is due to the filter represented by routine echocardiography performed by the obstetricians.
Cyanosis following a modified Fontan procedure secondary to anomalous inferior systemic venous return
Authors: Day Ronald W. , Harake Bilal , Laks Hillel
Published: August 2008

A persistent subcardinal anastomosis was identified as a source of right-to-left shunting in a patient with excessive cyanosis following a modified Fontan procedure. Anomalies of inferior systemic venous return must be defined when a Fontan procedure is performed by direct caval venous anastomosis to the pulmonary arteries, and hepatic venous drainage is left to mix with pulmonary venous return.
Double outlet left atrium with co-existing double inlet left ventricle and discordant atrioventricular connections
Authors: Gnanapragasam James P. , Houston Allan B. , Anderson Robert H.
Published: August 2008

We describe an unusual heart in which double outlet from the left atrium resulted in atrioventricular connections to a dominant left ventricle and to a left-sided rudimentary right ventricle, while the right atrium also connected to the left ventricle. This produced, in effect, double inlet left ventricle in association with discordant atrioventricular connections. The diagnosis was made by echocardiography and confirmed at post mortem examination.
Ventricular arrhythmias after repair of congenital heart disease: Who needs treatment?
Authors: Garson Arthur
Published: August 2008

Sudden death occurs in patients after repair of congenital heart disease. In those with tetralogy of Fallot, or a similar lesion, ventricular tachycardia has been hypothesized as the major arrhythmic mechanism for sudden death. It would be desirable to identify individuals at risk for sudden death, to determine which arrhythmia would be likely to cause sudden death, and to treat those individuals with an appropriate antiarrhythmic to prevent sudden death. For the last 10 years, physicians have been treating patients with antiarrhythmic drugs, based on a number of criteria, the most common of which is the presence of premature ventricular contractions.1,2 The practice has recently been called into question by the CAST trial. It is the purpose of this paper to review the evidence that repair causes ventricular arrhythmias, that ventricular arrhythmias cause sudden death, and that ventricular arrhythmias should be treated prophylactically.
Kawasaki disease
Authors: Kawasaki Tomisaku
Published: August 2008

In this review, I have outlined the clinical picture, epidemiology, pathology, etiology, and current treatment and management of Kawasaki disease. The disease in question has unique features which cannot be classified into any of the categories of conventional pediatric diseases, and thus the elucidation of the etiology and mode of onset may well raise many new medical problems. It is hoped that young practitioners of clinical and fundamental medicine will be able to resolve the problems relating to the disease which remain as quickly as possible.
Immune abnormalities in Kawasaki disease: prognostic implications and insight into pathogenesis
Authors: Barron Karyl S.
Published: August 2008

There are a number of abnormalities of the immune system seen in children with Kawasaki disease that are widely integrated and lead eventually to the inflammatory response seen clinically as fever, rash, arthritis, coronary arteritis, etc. The initiating event remains a mystery, however the cascade of immune responses involves both T and B lymphocytes, lymphokine production, and cell adhesion. Until the etiologic agent(s)/ of Kawasaki disease is identified, therapeutic intervention must be directed at controlling the effects of the abnormal immunoregulation.
Pathological observations concerning the cardiovascular lesions in Kawasaki disease
Authors: Naoe Shiro , Shibuya Kazutoshi , Takahashi Kei , Wakayama Megumi , Masuda Hirotake , Tanaka Moboru
Published: August 2008

In about 1967, a new clinical entity in infants and children was described in Japan. It was an acute febrile illness associated with engorgement of the conjunctivas, labial erythema, and swelling of the deep cervical lymph nodes without suppuration. This disease is usually called the acute febrile mucocutaneous lymph node syndrome or, more simply, Kawasaki disease.
The role of cross-sectional echocardiography in Kawasaki disease
Authors: Ettedgui José A. , Neches William H. , Pahl Elfriede
Published: August 2008

Cross-sectional echocardiography is an essential tool in the evaluation ofchildren with Kawasaki disease, both in the acute and chronic stages. In the acute phase of the illness, it is valuable for diagnosis and management of pancarditis and for the long-term monitoring of pericardial effusions, left ventricular function, and the rare cases of chronic valvar dysfunction. When coronary arterial abnormalities are detected, echocardiography can serially evaluate long-term treatment with drugs which prevent the aggregation of platelets and monitor the resolution of coronary aneurysms. The value of cross-sectional echocardiography, nonetheless, is very limited in the detection of coronary arterial stenosis. Coronary arteriography is still important for the diagnosis of obstructive lesions in the coronary arteries and should be used in conjunction with cross-sectional echocardiography for the appropriate long- term management of children with Kawasaki disease at high risk of developing coronary arterial stenosis. Perhaps, in the future, high resolution transesophageal echocardiography will allow clear delineation of coronary arterial anatomy and specifically stenosis, but its role in the evaluation and management of children with Kawasaki disease remains to be explored.
Visualization of the coronary arterial lesions in Kawasaki disease by coronary angiography
Authors: Suzuki Atsuko , Kamiya Tetsuro
Published: August 2008

Formation of coronary aneurysms is often seen on the patients in the acute phase of Kawasaki disease by echocardiography, and after the convalescent phase, some patients show disappearance of aneurysms but some of them show combining stenotic lesions at inlets and/or outlets of aneurysms on coronary arteriography. According to follow-up studies of coronary arterial lesions, the degree of stenosis quite frequently increases and progresses over a period up to 10 years or more after the onset of the disease. The severe stenosis appears the most frequently at the proximal portion of the left anterior descending artery and secondly at the main trunk of the main coronary artery. They die suddenly or suffer from severe myocardial infarction unless they are treated with aortocoronary bypass surgery. However, it is rare to have symptoms of ischemic heart disease even in patients with severe obstructive lesions. In fact, the sensitivity of exercise testing in diagnosis of myocardial ischemia is very low. Dipyridamole thallium myocardial imaging is reasonably useful, but dipyridamole can he dangerous when it is given without the precise information of the patient's stenosis. Coronary arteriography is, therefore, essential not only to determine the appropriate treatment of the c ronary arterial lesions but also for follow-up. According to the histopathologic study, even the patients in whom coronary arterial lesions were not visualized on coronary arteriography often show some intimal proliferation. These patients may well present a problem in the future due to the sequels of angiitis. Because of this, continuous regular follow-up should be necessary, even in patients with no evidence of coronary arterial lesion.
Controversies in the medical management of Kawasaki disease
Authors: Pahl Elfriede , Neches William H. , Ettedgui José A.
Published: August 2008

Summary Mucocutaneous lymph node syndrome, now called Kawasaki disease, affects children of all ages and races. The cause of this systemic vasculitis is unknown, thus the diagnosis is based solely on clinical criteria. Coronary arteritis and the formation of coronary arterial aneurysms are the most serious early cardiac complications, while long term sequels, such as coronary stenosis and myocardial infarction, may occur. Current medical therapy is aimed solely at preventing or decreasing the occurrence of these cardiovascular complications in patients with this disease. Past treatment included antibiotics, steroids, and nonsteroidal anti-inflammatory agents. Aspirin remains the most widely used drug, although the use of intravenous gammaglobulin has also become common. When used in combination, these two agents may reduce the incidence of coronary arterial aneurysms. The optimal dosage and duration of treatment has not yet been determined.
Surgical management for cardiovascular lesions in Kawasaki disease
Authors: Kitamura Soichiro
Published: August 2008

Summary Myocardial revascularization is now an accepted therapeutic modality for severe coronary arterial obstructive disease produced by Kawasaki disease which is amenable to usual medical treatment. The mortality rate of myocardial infarction is surprisingly high in this setting, and surgery may be able to prevent many of these deaths. This article focuses on current issues in surgical treatment of ischemic heart disease in children secondary to Kawasaki disease. Coronary arterial obstructive disease is, apparently, a leading problem followed by valvar and myocardial involvements. Introduction of bypass grafting using the internal thoracic artery in children was a major recent advance, because this graft has an excellent capability to adapt to the increments of increased flow and growth of the patient. The saphenous vein is less satisfactory when used as a graft in terms of its long-term patency, particularly in young children less than 8 years old. Although longer follow-up is certainly required, the current results of surgery have been excellent in improving myocardial perfusion, in ameliorating symptoms, and improving the quality of life.
Therapy of acute Kawasaki disease
Authors: Newburger Jane W.
Published: August 2008

Kawasaki disease is an acute vasculitis of unknown etiology that occurs predominantly in infancy and early childhood. It is characterize by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy.1,2Coronary arterial aneurysms, or ectasia, develop in approximately 15 to 25% of children with the disease, and may lead to myocardial infarction, sudden death, or chronic coronary arterial insufficiency.2–4
The hemodynamic significance of patency of the arterial duct in ventilated preterm babies with and without treatment with surfactant: a prospective Doppler echocardiographic study
Authors: Oberhoffer Renate , Gortner Ludwig , Lang Dieter
Published: August 2008

Sixteen very premature babies, born prior to term at gestational ages from 26–30 weeks, who required ventilation, were randomly assigned to groups, which did or did not receive treatment with surfactant. They were then studied prospectively with respect to the hemodynamic significance of the patency of the arterial duct. The velocity of end-diastolic flow in the celiac trunk was measured by Doppler echocardiography. Reduced values were assumed to represent the effect ofa large duct. The volume of systemic and pulmonary flows were estimated by means of Doppler echocardiographic studies at the tricuspid and mitral valvar orifices. Patency of the duct was diagnosed in all patients. Time to subsequent closure and the incidence of necrotizing enterocolitis and intracranial hemorrhage did not differ between the groups. The size of the shunt across the duct was measured as approximately 19% in patients with and without treatment. Velocity of end-diastolic flow, however, and the volumes of systemic flow, were markedly decreased in both, indicating the presence of an arterial duct ofhemodynamic significance. In contrast, in a control group of 20 normal newborns, end-diastolic flow velocities and systemic flows were normal when an arterial duct was diagnosed as patent. These results suggest that treatment with surfactant does not alter shunting across the arterial duct, but that gestational age may be the main factor influencing the hemodynamic significance of the duct. Due to the serious reduction of volumes of systemic flow in preterni babies with a patent duct, early closure should be considered in this population.
Noninvasive assessment of myocardial mechanics—a review of analysis of stress-shortening and stress-velocity
Authors: Colan Steven D.
Published: August 2008

The development of newer, load-independent indices of contractility has not substantially reduced general clinical reliance on ejection fraction and shortening fraction to detect abnormalities of contractility, in spite of common understanding of the preload and afterload dependence of percent fiber shortening. The more widespread application of sensitive indices of contractility has been impeded in part by complex methods of acquisition and analysis of data as well as uncertainty concerning the clinical importance of the additional derived information. Substantial recent experience with analysis of stress-shortening and stress-velocity, nonetheless, demonstrates that physiologically meaningful indices of afterload, preload, and contractility can be obtained noninvasively without hemodynamic interventions. There is extensive theoretical and experimental basis for these methods, and the limitations are similar to other global indices of myocardial mechanics. The superiority of methods which allow distinction between contractile abnormalities and abnormal load are particularly important when altered ventricular loading conditions are a prominent feature of the disease state. Several clinical situations have been identified for which analysis of stress-shortening and stress-velocity demonstrates that assessment by fiber shortening alone has resulted in misrepresentation of myocardial status. The clinical utility of assessment of ventricular function is considerably enhanced when the relative contribution of load and contractile performance is determined.
Effect of enalapril on infants and children with congestive heart failure
Authors: Seguchi Masashi , Nakazawa Makoto , Momma Kazuo
Published: August 2008

The effect of enalapril, an inhibitor of angiotensin converting enzyme, was studied in 35 infants and children with congestive heart failure associated either with residual mitral or aortic regurgitation following intracardiac repair (24 patients) or with dilated cardiomyopathy (11 patients). Enalapril, at an average dose of 0.24 ±0.10 mg/kg, reduced the concentration of angiotensin II in the serum from 115 ± 67 pg/ml to 60 + 30 pg/ml and increased plasma renin activity from 25 + 24 ng/ml/hr to 45 ± 37 ng/ml/hr. There was a significant decrease in hepatomegaly, cardiothoracic ratio, heart rate and blood pressure in both groups of patients. Left ventricular end-diastolic dimension as evaluated by cross-sectional echocardiography, also decreased, whereas fractional shortening and systolic time intervals improved in both groups of patients. Adverse effects were noted in only one infant with postoperative mitral regurgitation who developed renal failure with oliguria, increase of blood urea nitrogen and serum creatinine. Renal function improved dramatically after discontinuation of enalapril. Hyperkalemia (>5mEq/ 1) was observed in four patients, three of whom were receiving spironolactone. Our results showed that enalapril is effective in improving hemodynamics in the relatively short-term treatment of infants and children with congestive heart failure due to postoperative volume overload or dilated cardiomyopathy. These conditions are poorly controlled with conventional medical therapy.
A serial Doppler echocardigraphic study of early diastolic right ventricular events in full term neonates
Authors: Areias Jose C. , Scott William A. , Meyer Robin , Goldberg Stanley J.
Published: August 2008

Our purpose was to study serially, measurements which relate to right ventricular events, using echocardiography and Doppler in full term neonates for the first three weeks after birth. Echocardiographic and pulsed Doppler studies were performed for 21 newborns during the first 36 hours of life, and subsequent serial studies were performed in the second (n=21) and third (n=14) weeks of life. Evaluation included measurement of right isovolumic relaxation time and peak E and A velocities across the tricuspid valve. Pulmonary velocities were studied for evidence of patency of the arterial duct, and to determine acceleration in the pulmonary trunk. Mean right isovolumic relaxation time decreased significantly from 68±9 ms to 39 ± 14 ms (p<0.001) between the first and second week. Mean tricuspid peak E increased significantly between the second (48 ± 7 cm/s) and the third week of life (62 ± 8 cm/s; p<0.001). Mean peak A wave velocity did not change significantly during the time of study. The pulmonary arterial acceleration time increased from the initial measurement (68 ± 10 ms) to the second week (85 ± 9 ms; p<0.001). Changes in right isovolumic relaxation time were significantly related to the changes in the pulmonary arterial acceleration time (r=0.63, p=0.0001), suggesting a significant influence of afterload on diastolic events. These data demonstrate the normal progression of some measures of early right ventricular diastolic function.
Repair in infancy of tetralogy of Fallot with absence of the leaflets of the pulmonary valve (absent pulmonary valve syndrome) using a valved pulmonary artery homograft
Authors: Danilowicz Delores , Presti Salvatore , Colvin Stephen B. , Doyle Eugenie F.
Published: August 2008

Two infants, aged eight days and four months, with tetralogy of Fallot and absence of the leaflets of the pulmonary valve, presented with acute respiratory distress and congestive heart failure. Primary repair was performed using a cryopreserved pulmonary arterial homograft. Both infants survived and are doing well three and two years later. A review of the surgical procedures attempted in this symptomatic group of patients is presented together with a discussion of the pathophysiology of this defect.