bluuebunny/crossref_metadata_2025_split huggingface dataset:
Kawasaki disease
Authors: Kawasaki Tomisaku 
Published: August 2008

SummaryIn 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

SummaryThere 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.
The role of cross-sectional echocardiography in Kawasaki disease
Authors: Ettedgui José A. , Neches William H. , Pahl Elfriede 
Published: August 2008

SummaryCross-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

SummaryFormation 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

SummarySummary 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

SummarySummary 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.
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

SummarySixteen 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

AbstractThe 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

SummaryThe 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

SummaryOur 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

SummaryTwo 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.
Assessment of interventricular septal motion by cross-sectional echocardiography in patients with isolated atrial septal defects within the oval fossa
Authors: Vogel Michael , Schulze Karin , Bühlmeyer Konrad 
Published: August 2008

SummaryTo assess whether paradoxical (systolic anterior) motion of the interventricular septum is a true abnormality of septal contraction caused by right ventricular volume overload, we examined the regional pattern of left ventricular contraction in 20 patients with an atrial septal defect within the oval fossa using a fixed and floating reference system of analysis of wall motion. The patients, with a median age of 6.8 (3.5–13.4) years, had a Qp/Qs ratio of 3.5:1 (1.2:1–8:1). They were studied by cross-sectional echocardiography four days (3–5) before and 14.5 (3–67) days after surgical closure of the atrial septal defect. The postoperative Qp/Qs ratio was 1.1:1 (0.9:1–2:1). Regional wall motion of the left ventricle was analyzed in the parasternal short axis view at the level of the mitral valve and papillary muscles as well as in the apical four-chamber view. The endocardium was digitized manually in end-systolic and end-diastolic frames. The center of mass of this figure was calculated and connected to an outside reference point in the right ventricle. With the floating system, both centers of mass and the reference lines were superimposed, thus correcting for movement of the heart inside the thorax. With the fixed system,both end-diastolic and end-systolic frames were measured separately without correcting for movement of the heart. The left ventricle was divided in eight segments in a clockwise fashion and regional change in area was measured and compared to 40 normal age matched controls. With the floating system, left ventricular regional wall motion was normal in all patients before and after closure of the atrial septal defect and, thus, was not influenced by the change in right ventricular volume load. With the fixed system, both before and after surgical closure of the atrial septal defect, left ventricular regional wall motion was reduced in the two segments representing the basilar and middle portion of the interventricular septum. The floating system of analysis of left ventricular regional wall motion has the ability to correct for movement of the heart, which makes the supposed “abnormal” systolic anterior motion in right ventricular volume overload, a condition prevalent in patients with atrial septal defect, disappear. Thus, we conclude that the so-called paradoxical septal motion in atrial defect is an artifact. This may be caused by an increased motion of the heart but is unrelated to the volume load of the right ventricle.
Interobserver variability of determination of the size of the left-to-right shunt in isolated atrial septal defects within the oval fossa by Doppler and cross-sectional echocardiography
Authors: Vogel Michael , Stern Heiko , Bühlmeyer Konrad 
Published: August 2008

SummaryAlthough noninvasive estimation of the ratio of pulmonary to systemic flow by measuring the cross-sectional areas of the pulmonary and aortic valves and the respective time integrals of the velocity of blood is widely used to determine the amount of shunting in patients with left-to-right shunts, few data exist concerning interobserver variability. We assessed such variability of shunt calculations derived from Doppler echocardiography in 10 children aged six (3.5–11) years with an isolated atrial septal defect within the oval fossa. The ratio of flows was calculated by measuring stroke volumes over the pulmonary trunk and the aorta immediately before and at an average of seven days (5–11) following surgical closure of the atrial septal defect. Two independent observers measured the cross-sectional areas of the aortic and pulmonary orifices and the velocity time integrals and calculated the size of the shunt from these data. All measurements were performed five times, and the mean of these five measurements was calculated and compared for analysis. Before surgery, observer I had measured a ratio of pulmonary to systemic flow of 3.8 (2.1–6.2) and observer II of 3.5 (1.9–5). The mean interobserver difference in terms of the size of the shunt was 1.1 (or 30%). After surgery, observer I measured a ratio of 1.3 (0.8–1.7) and observer II of 1.1 (0.7–1.6). The cross-sectional area of the pulmonary valve was measured as 3.6 (2.6–4.9) cm2 and 3.4 (2.3–5.8) cm2 respectively, with a mean interobserver difference of 0.8 cm2 or 23%. For the velocity time integrals across the pulmonary trunk, the mean interobserver difference was 13% (4–38) before and 16% (0–55) after surgery. The respective variability for the aortic velocity time integrals was 11 % (4–46) before and 9% (0.5–23) after surgery. The variability of measurements of maximal flow velocity across the pulmonary trunk was 15% (4–38) before and 18% (1–77) after surgery. The respective variability for the aortic flow velocity was 11 % (2–28) before and 9% (4–23) after surgery. A paired t-test showed no significant differences for all parameters assessed. The difficulty of reproducing measurements of the pulmonary valve is an important factor affecting interobserver variability. The magnitude of interobserver difference was significantly related to the cross-sectional area of the pulmonary trunk (r=0.84; p<0.02). The interobserver variability may be considerable, and is large enough to influence clinical decisions. Thus, we conclude that a decision regarding need for surgery cannot be based solely on calculation of the shunt derived only from Doppler measurements in patients with isolated atrial septal defects within the oval fossa.
Defective regional myocardial development and vascularization in one variant of tricuspid atresia—clinical and necropsy findings in three cases
Authors: O'Connor William N. , Cottrill Carol M. , Marion Michael T. , Noonan Jacqueline A. 
Published: August 2008

SummaryFive previously reported cases have established the combination of a small right ventricle (sometimes with Uhl's anomaly), imperforate tricuspid valve with fibrotic tensor apparatus, congenital absence of the pulmonary valve, and an intact interventricular septum with muscular subaortic stenosis as a rare variant of tricuspid atresia. In this study of three new autopsy cases, we additionally identified Ebstein's malformation of the imperforate tricuspid valve, partial Uhl's anomaly, regional dysplasia of right ventricular myocardium and thinning of the interventricular septum by intramyocardial sinusoids from the right ventricle. All three new cases had epicardial anomalies of the right coronary artery—a fistula to right ventricle, ostial stenosis and proximal arterial hypoplasia. Sinusoidal connections from the right ventricle to the right coronary artery and to the sclerotic left anterior descending artery were identified in serial sections of the right ventricle and septum. Myofiber disarray, with thick walled intramyocardial arteries and sinusoids from left ventricle, involved the bulging subaortic interventricular septum. These studies are consistent with the hypothesis that defective development of the the right ventricle, along with its blood supply and associated atrioventricular and arterial valves, may underlie this unusual form of congenital heart disease.
Atrioventricular septal defects with severe left ventricular hypoplasia—clinical findings and surgical options
Authors: Troconis Carlos J. , Di Donato Roberto M. , Marino Bruno , Vairo Ugo , Marcelletti Carlo 
Published: August 2008

SummaryRight ventricular dominance was observed in 22 (6.6%) out of 332 patients with atrioventricular septal defects. Their mean age was 2.24 ± 2.73 months. Chromosomal anomalies were seen in only three patients (Down's syndrome in two and trisomy nine in one). Seventeen patients underwent surgical treatment. A pulmonary artery banding was performed in eight of them with one long-term survivor who underwent a successful staged orthoterminal repair. A Norwood-type of palliation was attempted in four without success. Biventricular repair was performed in four additional patients with two mid-term survivors, in one of whom the patch used for closure of the atrial septal defect was fenestrated. One patient underwent successful heart transplantation at seven months of age. Pathophysiological problems and surgical options are discussed.