bluuebunny/crossref_metadata_2025_split huggingface dataset:
Calcifying myocarditis mimicking an intracardiac tumor on fetal echocardiography
Authors: Oberhoffer Renate , Hillen Uwe , Lang Dieter 
Published: August 2008

SummaryWe report echocardiographic findings of an intracardiac mass in a hydropic fetus who died after four weeks of follow-up. Within that period, the left ventricular myocardium had developed extensive echo-dense patterns suggesting tumorous infiltration. Autopsy revealed extreme calcification of the mitral valve, which had mimicked a tumorous mass on fetal echocardiography. The histological results were consistent with viral myocarditis.
Retrograde flow in the distal aortic arch and proximal descending aorta—a clue to presence of a major cerebral arteriovenous malformation
Authors: Davis Andrew M. , Menahem Samuel 
Published: August 2008

SummaryTwo neonates are described presenting in severe cardiac failure within a few hours of birth. Echocardiography and the demonstration by color-coded Doppler of retrograde diastolic flow in the proximal descending aorta suggested the presence of large cerebral arteriovenous malformations. Such lesions were demonstrated by cranial ultrasound. The early diagnosis permitted early intervention, albeit with less than satisfactory outcome.
Discrete subaortic stenosis—rapid evolution in infancy
Authors: Krishnan Usha , Kitchiner Denise , Sreeram Narayanswami 
Published: August 2008

AbstractDiscrete subaortic stenosis is an acquired lesion that is rare in infancy. We report an infant in whom the lesion developed within four months after cross-sectional echocardiography had revealed an apparently normal outflow tract. Surgical repair was successfully undertaken.
The Fontan operation with a fenestrated patch—rationale and results
Authors: Castañeda Aldo R. , Bridges Nancy D. 
Published: August 2008

AbstractA fenestration in the atrial septum may be temporarily beneficial in high–risk patients undergoing the modified Fontan operation. The venous system is decompressed into the left atrial cavities resulting in an improvement in the systemic output at the expense of a moderate arterial desaturation due to a right–to–left shunt. Two groups of patients undergoing the modified Fontan operation were compared. Those treated with the classic operation (non–fenestrated Fontan group, n=56) had lower prevalence of preoperative risk factors for the Fontan operation than those undergoing the Fontan operation with a fenestrated patch (n=91). Nevertheless, early results of mortality, repair take–down and morbidity (duration of pleuropericardial effusions) were comparable or better in the fenestrated Fontan group. By regression analysis the presence of a fenestrated patch was the major determinant in the reduction in the amount and duration of postoperative effusion. Of the early 73 survivors in the fenestrated Fontan group, fifty tolerated the closure of the fenestration, which occurred either spontaneously (five cases) or was achieved by a clamshell device during cardiac catheterization. Eleven patients failed the first attempt at fenestration closure, but this was successful at the second attempt a few months later. Coil embolization of arterial collaterals to the lung circulation and balloon dilatation of pulmonary artery stenoses were required in some cases. Other patients following the Fontan operation with a fenestrated patch showed an improvement of their ventricular function which was initially impaired. This favorable experience demonstrates the usefulness of the “fenestrated Fontan approach” in patients who are suboptimal candidates for the classic operation, provided that the preoperative risk factors are potentially reversible.
Thrombolysis in the treatment of patients with Kawasaki disease
Authors: Suzuki Atsuko , Tetsuro Kamiya , Ono Yasuo , Kinoshita Yoshihisa 
Published: August 2008

AbstractThrombolysis was used in 13 procedures to treat eight patients with massive thrombosis of coronary aneurysms due to Kawasaki disease. The patients have now been followed for periods ranging from 2.5 years to 7.4 years subsequent to treatment. The procedures included intracoronary or intravenous infusion of urokinase and intravenous administration of tissue plasminogen activator. Thallium myocardial imaging, coronary arteriography and echocardiography were used for evaluation of the efficacy of the therapy and for follow-up studies. The latest time of treatment was 17 months after the onset of the disease. No new formation of massive thrombus was observed in any of the aneurysms. In five patients, eight of 13 procedures were judged to be successful. In the four patients with symptoms of myocardial ischemia, thrombolysis was undertaken immediately after the onset of symptoms. In the other patient, thrombolysis was attempted 36 days after the onset and produced remarkable improvement in the degree of ischemia. In the other four patients with no symptoms of ischemia, the therapy was undertaken when thrombus was detected in a coronary aneurysm. Aortocoronary bypass surgery was undertaken subsequent to the thrombolytic therapy in three patients, but proved satisfactory in only one patient. At follow-up, five patients have shown improvement in the degree of myocardial ischemia after treatment. Our results suggest that careful observation is needed to detect formations of thrombus in large coronary arteries for at least 1½ years after the onset of Kawasaki disease. Even if repeated thrombolytic therapy is required, it seems to be effective in checking the progression of ischemic heart disease.
Clinicopathological correlation—pulmonary atresia with imperforate tricuspid valve and aneurysmal dilatation of ascending aorta
Authors: Hirsch Rafael , Gerlis Leon , Somerville Jane 
Published: August 2008

AbstractA patient with the rare combination of an unusual form of pulmonary atresia with intact ventricular septum and a Marfan-like disease is presented. She was extremely cyanosed in childhood but improved after construction of a left peripheral shunt at the age of nine years. She deteriorated gradually after the onset of atrial fibrillation at the age of 33, the subsequent course being complicated by a stroke and, finally, by intractable heart failure and death at the age of 39.
Ultrafiltration and pediatric cardiopulmonary bypass
Authors: Naik Surendra K. , Elliott Martin J. 
Published: August 2008

The technique of modified ultrafiltration is a more efficient application of the concept of ultrafiltration during cardiopulmonary bypass. It has been shown to be superior to the conventional method of ultrafiltration.The method can save considerable quantities of donor blood by returning not only the red cells, but also the white cells, platelets, and clotting factors back to the patient, components which otherwise would be discarded. The elevation of the hematocrit made possible by this method after bypass will permit an “acceptable” hematocrit to be achieved when temperatures become normal after ultrafiltration. The return of platelets and clotting factors along with a high hematocrit will contribute towards significantly minimizing the postoperative loss of blood, and, consequently, the amount of blood requiring to be transfused. The possibility of elevation of the hematocrit allows for a lower hematocrit during bypass. This can achieve a considerable saving of donor blood, particularly in small children who invariably need blood added to the prime, making bloodless pediatric surgery a real possibility. The technique also minimizes the rise in total water in the body after cardiopulmonary bypass in children and promises favorably to alter the course of leaking capillaries as seen frequently in neonates and infants. It has been shown to improve hemodynamics at a crucial time when the patient has been weaned from cardiopulmonary bypass and needs optimum conditions for recovery. From the point of view of research, ultrafiltration offers a window to look at the inflammatory process induced by cardiopulmonary bypass.It promises to be a valuable technique to investigate the elimination and possibly quantification of toxic metabolites produced during bypass. It also allows evaluation and assessment of different protocols for bypass, involving varying rates of flow and temperature, for their response to the production of these toxic metabolites, thus offering the potential to achieve a model of cardiopulmonary bypass which is as physiological as possible. With advances in the systems of perfusion, aided by computers, it should be possible to control the rate of ultrafiltration and precisely regulate the transfusion of the venous reservoir fluid with the aid of feedback loops from the venous and arterial pressures in the heart. This could make ultrafiltration an integral part of routine cardiopulmonary bypass.
Cerebral cellular response to profound hypothermia
Authors: Watanabe Takao , Washio Masahiko 
Published: August 2008

AbstractIn our first two experiments, we examined brain tissue pH and tensions of oxygen and carbon dioxide in dogs core cooled to 20°C. So as to evaluate the effects of 60 minutes of circulatory arrest, 120 minutes of low-flow perfusion (25 mI/kg/mm), and 120 minutes of moderate-flow perfusion (50 mi/kg/mm), all conducted with and without pulsatile assistance. We further determined the effects of blood gas strategy on the same variables with 60 minutes of circulatory arrest. In a third experiment, we directly observed microcirculation at the surface of the brain during profoundly hypothermic perfusion. In the fourth experiment, we measured cerebral blood flow, oxygen consumption, and excessive production of lactate. Profound anoxia occurred within 20 minutes of circulatory arrest, causing severe and progressive acidosis in the brain tissues along with hypercapnia. The inhalation of 5% or 7% carbon dioxide during core cooling made the brain unacceptably acidotic. The brain acidosis was mild with low flow perfusion, and slight with moderate-flow perfusion. Pulsatile assistance improved acidosis in the brain tissues at all rates of flow. It also improved the microcirculation, the patent number of arterioles and stabilized flow in bridging veins. Cerebral metabolism became aerobic without alterations in cerebral consumption of oxygen during low-flow perfusion. We recommend flow rates above 25% of normal, alpha-stat strategy, and pulsatile assistance for better protection of the brain during profound hypothermia.
Neonatal and infant physiology—impact of cardiopulmonary bypass in the developing patient
Authors: Auten Richard Lambert 
Published: August 2008

AbstractCardiopulmonary bypass has been extended to the very young patient undergoing operative correction of congenital heart defects. Growth and development of the central nervous, cardiovascular, pulmonary, and renal systems place significant metabolic and nutritional demands on cellular growth and repair. Immature homeostatic regulation and cellular function require modification of the approaches to preservation of organs and cardiovascular support used in older children and adults undergoing open-heart surgery. Aspects of newborn and infant physiology relevant to cardiopulmonary bypass and postoperative care are reviewed. Current approaches and future strategies designed to address the needs of the developing patient who requires cardiopulmonary bypass are discussed.
Abnormal histology of the aortic arch in coarctation of the aorta
Authors: Wolterbeek Derk W. , Kappetein Arie P. , Gittenberger–de Groot Adriana C. 
Published: August 2008

SummaryWe examined the number of elastic lamellae in the wall of the proximal aortic arch, aortic isthmus and descending aorta in patients with coarctation of the aorta. In the proximal aortic arch, the number of elastic lamellae was significantly lower in patients with coarctation compared to those with normal hearts without aortic anomalies and those with intracardiac defects but without aortic anomalies. The isthmus also showed a significantly lower number of elastic lamellae in the presence of preductal coarctation. In the descending aorta, the number of elastic lamellae was not significantly different between the different groups. There is doubt about the etiology of coarctation. Recent investigations showed that cells from the cardiac neural crest contribute to the formation of the arch arteries and the media of the arch. A developmental error of the neural crest might be responsible for the abnormal mural structures found in patients with aortic coarctation.
Diagnostic utility of color flow Doppler in extracardiac causes of congestive heart failure in infants
Authors: Reed Carl M. , Boulden Thomas F. , Becker Judith A. , Tonkin Ina L. , Alpert Bruce S. , DiSessa Thomas G. 
Published: August 2008

SummarySystemic vascular malformations are a rare cause of congestive heart failure in the infant and newborn infant. Clinical diagnosis is often problematic. Accordingly, this study presents the clinical, radiographic, ultrasound imaging, pulsed and color Doppler features of systemic vascular malformations in six infants. In all cases, color Doppler provided vital information regarding size, location, source of feeding arteries and venous drainage essential for appropriate management of these patients. Color flow Doppler ultrasonography is an accurate and sensitive modality for detecting and evaluating vascular malformations.
The pathological diagnosis of pulmonary atresia with abnormal ventriculoarterial connections in the fetus
Authors: Jeffrey Lona Jane Mary , Ho Siew Yen , Moncrieff Deborah Peta Murray , Ross Leslie David , Pearce John Malcolm 
Published: August 2008

AbstractPulmonary atresia with abnormal ventriculoarterial connections in the presence of an intact ventricular septum is rare even in postnatal life. Two cases are described in which this combination of lesions was diagnosed in previable fetuses at autopsy. Both pregnancies were terminated for gross structural abnormalities discovered on antenatal ultrasound scans. In the first case, the pulmonary atresia was associated with intact ventricular septum, a double outlet left ventricle and hypoplasia of the tricuspid valve, right ventricle and arterial duct, a combination of lesions that would appear to be unique. In the second case, there was complete transposition and a hypoplastic left ventricle. The severity of the cardiac lesions in both fetuses was such that it is unlikely that either would have survived to term. In the first case no other malformation was present, but in the second there was also a large encephalocoele secondary to early amnion rupture, and a single umbilical artery. The association of the cardiac lesion with early amnion rupture in this second fetus would appear to be a chance event.
‘Malignant’ congenital cardiovascular disease in twins with William's syndrome
Authors: Davis Andrew M. , Burn John , Karl Tom R. 
Published: August 2008

SummaryTwins of like sex and concordant for William's syndrome are presented. The first twin presented late with aortic arch atresia and has subsequently developed hypertension associated with renal arterial stenosis. The second twin died with myocardial ischemia late after surgery for supravalvar aortic and pulmonary stenosis.
Persistent fifth aortic arch in association with interruption of the aorta proximal to the brachiocephalic artery
Authors: Boothroyd Anne E. , Smith Audrey , Peart Ian 
Published: August 2008

SummaryWe describe a male infant with the combination ofan aortic interruption proximal to the brachiocephalic artery and a persistent fifth aortic arch providing a pulmonary-to-systemic shunt. As far as we know, interruption of the aorta at this site has not previously been reported. Documented cases of a persistent fifth aortic arch itself are few, although failure of recognition may account for its apparent rarity.
Unsuccessful two-stage correction despite appropriate surgery in a case with disconnection (so-called absence) of the right pulmonaty artery
Authors: Gildein Hans P. , von Bernuth Götz , Mocellin Rolf 
Published: August 2008

SummaryA right-sided modified Blalock-Taussig anastomosis was created using a 5 mm Gore-Tex tube in a 4½ month old infant with unilateral “absence” of the right pulmonary artery. Wide patency of the shunt was confirmed shortly after by angiography, but rarefaction of the peripheral right pulmonary arteries was seen at the age of 2½ years, when corrective surgery was performed. Postoperative hypoperfusion of the right lung, along with severe pulmonary vascular abnormalities seen in a lung biopsy, indicated failure of the two-stage correction in spite of appropriate surgery.
One-stage repair in early infancy of tetralogy of Fallot with ‘absent pulmonary valve’ syndrome
Authors: Yagihara Toshikatsu , Kishimoto Hidefumi , Yamamoto Fumio , Nishigaki Kyoichi , Matsuki Osamu , Uemura Hideki , Takahashi Osahiro , Kamiya Tetsuroh , Kawashima Yasunaru 
Published: August 2008

AbstractFive infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.
Left ventricular function in pulmonary atresia with intact ventricular septum after systemic-to-pulmonary arterial shunt
Authors: Pacileo Giuseppe , Pisacane Carlo , Russo Maria Giovanna , Calabrò Raffaele 
Published: August 2008

AbstractTo investigate the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without coronary-cardiac fistulas after a modified Blalock-Taussig shunt, 14 patients (age range 15 days-16.5 months, mean 4.03±5.6 months) and eight control subjects, matched for age, body surface area and heart rate were evaluated by cross-sectional and Doppler echocardiography. The follow-up interval after palliative procedures ranged from 12 days to 16.3 months (mean 3.67±5.6 months). Compared to controls, in the group of patients the ejection fraction was decreased (61±7% vs 68±5%, p=0.022) while the left ventricular end-diastolic volume indexed for body surface area was increased (72.7±10.8 cc/m246.1±12 cc/m2p=0.0001) with normal values of left ventricular mass indexed for body surface area (67.88±20.9 g/m2vs 76±10 g/m2p=NS). Mass-to-volume ratio was lower in patients with pulmonary atresia (0.95±0.38 vs 1.24±0.3, p=0.031). The left ventricular shape index was increased in all patients with pulmonary atresia (1.27±0.26 vs 1±0.01, p=0.009). A significant inverse correlation was noted between the ejection fraction and follow-up (r=−0.71, p=0.04). as well as between the ejection fraction and shape index (r=−0.76, p=0.048). Moreover, the patients with pulmonary atresia had decreased E/A velocity ratio (0.65±0.16 vs 1.35±0.90, p=0.009), decreased normalized peak filling rate (4.16±0.13 sv/s vs 6.88±0.68 sv/s, p=0.0001), increased peak A velocity (0.95±0.17 m/s vs 0.51±0.16 m/s, p=0.0001) and prolonged isovolumic relaxation time (46±5.4 ms vs 34±6.2 ms, p=0.0001) and deceleration time (196.4±32.2 ms vs 116±21.4 ms, p=0.0001). There was a good correlation between the normalized peak filling rate and follow-up (r=−0.80, p=0.04). These data show a progressive compromise of the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without ventriculocoronary fistulas who had undergone systemic-to-pulmonary arterial shunting. Thus, an earlier biventricular or Fontan type procedure should be recommended.
Frequency and severity of endocardial fibroelastosis in dilated hearts from children—endocardial thickness is inversely correlated to age
Authors: Aiello Vera Demarchi , de Lourdes Higuchi Maria 
Published: August 2008

SummaryDiffuse endocardial thickening in dilated hearts has been considered as a distinct pathological entity, usually being termed endocardial fibroelastosis. It is common in infants and children and rare in adults. Current opinion holds that the endocardial thickening is a non-specific reaction to stress in the ventricular wall. Our personal observations point to the most severe forms occurring in young infants. To determine the frequency and severity of endocardial fibroelastosis in children with dilated cardiomyopathy, we studied 21 necropsy hearts, both grossly and microscopically. Ages of the patients ranged from two to 175 months (mean 46.5, median 23 months). Using a graduated eyepiece, we measured the endocardial thickness in samples from three sites in the left ventricle and one from the right ventricle. The values were compared to those obtained from similar measurements of the endocardium in hearts from four children presenting with tetralogy of Fallot. All the dilated hearts presented some degree of endocardial thickening. The gross detection of fibroelastosis depended on the degree of thickening. There was a negative but linear correlation between age and the mean endocardial thickness (r=0.70; p>0.001). Our findings support the concept that endocardial cells have greater replicative and secretory potentials in young patients, and that endocardial fibroelastosis is a secondary phenomenon in dilated hearts.
Early and late results of balloon dilation for congenital mitral stenosis
Authors: Alday Luis E. , Juaneda Ernesto , Spillmann Ana , Ruiz Esteban 
Published: August 2008

AbstractBalloon dilation was attempted in seven symptomatic patients with congenital mitral stenosis aged 7.6±5.3 years. Of the five patients with typical mitral stenosis, four underwent successful dilation with decrease of the mean left atrial pressure and the gradient across the mitral valve and increases in the area of the valve of 127±43 and 85±16%, respectively, as demonstrated hemodynamically or by Doppler echocardiography. In the patient in whom the procedure was unsuccessful, a small child with recurrent pulmonary edema, death occurred during the procedure as a consequence of vasovagal reaction. In the other two patients, one with a parachute-like mitral valve and the other with Shone's syndrome, severe pulmonary hypertension and reversal of the shunt through a patent arterial duct, it was not possible to dilate the valve. In the patient with Shone's syndrome, nonetheless, palliation was achieved by balloon angioplasty of the aortic coarctation and by creation of an atrial septal defect. The patients undergoing dilation remained symptom-free during follow-up and mantained the increase in valvar area as judged by Doppler studies and, in one patient, also by cardiac catheterization. A residual atrial septal defect closed spontaneously in three patients. We conclude that balloon dilation of congenital mitral stenosis is an effective alternative to surgery in patients with typical mitral stenosis. Cross-sectional echocardiography plays an important role in defining valvar morphology and selection of patients. The procedure is not without risk, especially in very sick patients but satisfactory results are long-lasting.