bluuebunny/crossref_metadata_2025 huggingface dataset:
Surgical treatment of acute episodes of rheumatic fever
Authors: Pomerantzeff Pablo Maria Alberto , Snitcowsky Rachel , Trevisan Isabelle Vianna , Marcial Miguel Barbero , Verginelli Geraldo , Jatene Adib D
Published: August 2008

Eight patients, four males and four females, age five to 13 years old (average: 11 years) have undergone surgery in the acute phase of rheumaticfever. The patients presented a history of rheumatic activity characterized by the presence of migratory arthritis and carditis. All patients had severe acute mitral insufficiency, while one of them had associated aortic insufficiency. Laboratory examinations revealed the presence of an acute inflammatory condition. All patients had acute heart failure and were treated initially with high doses of diuretics, peripheral vasopressor and vasodilator amines, together with cardiotonic drugs, without improvement. Surgical treatment was indicated after a period of observation between 24 hours and five days. In five patients, the Doppler echocardiogram revealed rupture and elongation of tendinous cords. Two of them had acute dilatation of the mitral ring, and one had isolated acute dilatation of the mitral ring. Five patients underwent valvar replacement and, in three, valvar repair was carried out. Two patients, who were in cardiogenic shock at the time of their referral, died in the operating room following replacement of the mitral valve. All patients who underwent repair of the mitral valve were in good condition at the last follow-up, six to 27 months after surgery.
Surgery for rheumatic lesions of the cardiac valves in the young
Authors: Lucchese Fernando A. , Sant'Anna João Ricardo M. , Kalil Renato A. K. , Sarmento Leite Rogério E. , Prates Paulo R. , Nesralla Ivo A.
Published: August 2008

Although declining in the so-called “first world,” surgery for rheumatic valvar lesions in patients less than 16 years of age is still a challenging problem in lesser developed countries. From 1970 to 1990, we operated upon and followed-up 136 children (less than 16 years) with different valvar lesions, performing various surgical procedures. The mitral valve was most frequently affected (52.9%) followed by multiple valvar disease (39.7%) and aortic (7.3%) involvement. The operations performed were valvoplasties (67), valvar replacement (49), and combined procedures (20). Hospital mortality for the overall group was 10.2%. There were 71 reinterventions in the follow-up of 20 years. Actuarial survival was 60.3% including hospital mortality. The event-free actuarial survival showed that 91% had at least one major event which required reoperation or hospital treatment, or else invasive cardiac procedures. We conclude that rheumatic valvar disease is still a challenging problem in our country, and that the severity of the illness is directly related to the younger age patients. In spite of reasonable long-term survival, very few patients had an uneventful follow-up after surgery.
Long-term observations of rheumatic carditis
Authors: Aziz Kalim-ud-Din , Cheema L. , Memon A.D.
Published: August 2008

A total of 246 consecutive patients were seen with the diagnosis of acute rheumatic fever (and/or rheumatic heart disease) and were followed for 587.7 patient years. The episode of acute rheumatic fever was the first in 64 of these patients, whereas recurrent acute rheumatic fever was seen in 26 and the other 156 patients had chronic rheumatic carditis. At presentation, those suffering an initial attack had less frequent and less severe carditis when compared to those suffering recurrent infection (p<0.05). Improvement in carditis during follow-up was noted in those having an initial attack (p<0.1), while deterioration occurred following recurrent infection (p<0.01), and no change was noted for those with chronic infection. Recurrences of acute rheumatic fever were most frequent in those presenting with their initial infection (21%) or reinfection (35%), and dropout from follow-up was highest in the group with first infection (38%) compared to those with recurrent infection (15%) and chronic carditis (25%). Non-recognition of the first episode of acute rheumatic fever and failure of secondary prophylaxis were found to be the major contributors to the observed increased pool of recurrent and chronic rheumatic heart disease. We conclude that, in the absence of programmed primary prophylaxis of acute rheumatic fever, the best chance of controlling the progression of carditis or affecting cure is to recognize the first episode ofacute rheumatic fever and then ensure strict adherence to secondary prophylaxis. Since the prognosis of recurrent carditis is poor, the best management of moderate to severe recurrent carditis is early reparative valvar surgery wherever possible.
Co-existence of congenital and rheumatic heart disease in 404 post-mortem cases—comparison with findings in 2,352 cases with isolated rheumatic disease
Authors: Zabal Carlos , Attie Fause , Buendía Alfonso , Calderón Juan , Troyo Paris , Guzzo Daniel
Published: August 2008

We reviewed 2,756 necropsy studies of patients who died because of rheumatic heart disease. The anatomic material was divided into two groups. The first group was made up of 404 cases (14.6%) having the association of rheumatic and congenital heartdisease. The second group comprised 2,352 cases with isolated rheumatic heart disease. The analysis of the site and types of congenital malformations in the first group showed 160 cases (39.6%) with aortic, 112 (27.7%) with pulmonary, 15(3.7%) with tricuspid and six (1.5%) with mitral lesions. Twenty patients (4.9%) had an atrial septal defect whilenine (2.2%) had patency of the arterial duct and four (1 %) a ventricular septal defect. The incidence of rheumatic aortic valvar disease in cases also having congenital lesions of the aortic valve (83.7%) was higher than in patients with isolated rheumatic involvement of the aortic valve (67.7%) (p<0.001). The difference in the incidence of rheumatic lesions found in patients with congenital pulmonary lesions (9.8%) and in the patients with isolated rheumatic disease (3.3%) was also significant (p<0.001). No differences were observed in rheumatic lesions to the mitral and tricuspid valves in those with or without associated congenital lesions. In patientswith an atrial septal defect, there was a higher incidence of combined mitral and tricuspid lesions (35% vs. 18%, p<0.02). Patients with patency of the arterial duct andco-existing rheumatic heart disease had a greater incidence of isolated rheumatic aortic disease (11% vs. 0.7%), and also of combined aortic and mitral lesions (33% vs. 17%), but the differences were not significant. The incidence of involvement of all four valves was 5.4% in those with associated congenital lesions and 3% in those in which rheumatic involvement was isolated (p<0.O2). We conclude that rheumatic heart disease has a tendency to involve preferentially cardiac valves that are the sites of congenital malformations.
Cot death and myocarditis
Authors: Rambaud Caroline , Cieuta Cécile , Canioni Danielle , Rouzioux Christine , Lavaud Jean , Hubert Philippe , Brousse Nicole , Rudler Michelle , Chéron Gérard
Published: August 2008

We have investigated the hearts from 153 infants found dead in their cots at ages ranging from one month to one year. The deaths occurred over a period of five years (January 1986 to December 1990) and were studied in a center for the study of the sudden infant death syndrome located in Paris. The epidemiological characteristics of this group are:male predominance (sex ratio 1.43), age less than four months (82%), and a predominance of winter deaths. These are the characteristic features ofthe sudden infant death syndrome. Ofthe 143 children studied with the permission of their parents, 24 (16.8%–12 girls and 12 boys) had histological lesions consistentwith myocarditis according to the Dallas criteria. The histological diagnosis of myocarditis is based on the association of cellular necrosis with a mononuclear or mixed inflammatory infiltrate. Cytoplasmic vacuolization, the presence of inflammatory cells in myocytes, and rupture of the cell walls of myocardial fibres are the equivalent histological signs of cellular necrosis. Myocarditis was diversely associated with respiratory, hepatic, muscular, gastrointestinal and/or neurological involvement. Twelve infants had previously been ill. Two died during the course of a serious illness. In only four cases was a viral association demonstrated. This incidence of myocardial involvement, similar to thatdescribed elsewhere in the literature, suggests that myocarditis could be a pathogenic explanation of some deaths thought on general autopsy investigation to be sudden and unexplained.
Pathophysiological mechanisms of sudden infant death syndrome
Authors: Stramba-Badiale Marco , Grancini Fabio , Porta Norberto , Schwartz Peter J
Published: August 2008

There is a consensus that sudden infant death syndrome, the leading cause of mortality in the first year of life, is multifactorial. Most of the cases are probably due to respiratory or cardiac disorders. It has been proposed that some cases of sudden infant death might result from ventricular fibrillation triggered by a sudden increase in sympathetic activity affecting the heart with reduced electrical stability. This impairment can be due to an insufficient or delayed development of cardiac vagal innervation, with the resultant lack of its protective effect that has been demonstrated to be present since the third week of life. Clinical studies suggest that some of the sudden infant death victims may have a reduced heart rate variability that could be interpreted as a decreased parasympathetic activity to the heart. On the other hand, a reduced cardiac electrical stability may be provoked by a developmental imbalance in sympathetic innervation such to create a dominance of left-sided nerves. We have demonstrated that an imbalance of this type experimentally induced in puppies increases the susceptibility to ventricular fibrillation and prolongs QT interval. In order to demonstrate a significant relationship between prolonged QT interval and sudden infant death, we performed a large prospective study on 18,832 neonates. QT interval corrected for heart rate (QTc) measured from an electrocardiogram on the fourth day of life, was within the normal limits in nine victims from known causes, while it was exceeding the mean by two standard deviations (>433 msec) in six of 12 sudden infant death victims. If these data are confirmed by a larger multicenter study started in Italy, it will be possible to identify some of the sudden infant death victims by the observation of a prolonged QT interval on the electrocardiogram. In this case a preventive strategy based on a time-limited administration of beta adrenergic blocking agents to the infants at risk, might reduce the incidence of sudden infant death.
Ventilation with external high frequency oscillation around a negative baseline increases pulmonary blood flow after the Fontan operation
Authors: Penny Daniel J. , Hayek Zamir , Rawle Peter , Rigby Michael L. , Redington Andrew N.
Published: August 2008

In this prospective study, pulmonary blood flow was measured using transesophageal Doppler echocardiography to assess whether ventilation by means of external high frequency oscillation around a negative pressure baseline can increase pulmonary blood flow, compared to intermittent positive pressure ventilation, in five patients after the Fontan operation. Pulmonary blood flow was measured when patients were ventilated by means of intermittent positive pressure ventilation and again during equivalent negative pressure ventilation using the external oscillatory technique. When compared to that with intermittent positive pressure ventilation, ventilation using external high frequency oscillation increased pulmonary blood flow by 116 ±61.5% (p=0.013). These results show that ventilation using an external oscillatory device with a mean negative chamber pressure may provide hemodynamic advantages in patients requiring assisted ventilation after the Fontan operation.
Long-term results of aortoventriculoplasty
Authors: Mitchell Ian M , Gnanapragasam James P. , Pollock James C.S. , Houston Alan B.
Published: August 2008

Obstruction of the left ventricular outflow tract continues to represent a challenging surgical problem, particularly in children. Failure to relieve the obstruction by either valvotomy or myectomy requires enlargement of the ventriculoaortic junction and outflow tract. Alternatively, the obstruction can be by passed using an apical valved veritriculo aortic conduit, althoughthis is associatedwith a high rate oflong-term complications. The use of a small xenograft in children is less than ideal because of early calcification, and the insertion of a mechanical valve may be feasible only after considerable enlargement of the ventriculoaortic junction. Aortoventriculoplasty is particularly suited for this purpose, and it is usually associated with good early results. Yet, long-term experience with this procedure, as far as we know, has not been reported previously. We describe our experience with the use of aortoventriculoplasty in four patients, three of whom have been followed-up for more than five years. Two patients have required reoperation for replacement of their prosthetic valves, one for bacterial endocarditis and the other for fracture of a continuous prolene suture which secured the prosthetic valve to the Dacron septal patch. All patients are currently in good health with continuing satisfactory relief of obstruction to their left ventricular outflow tract.
Novice experience with transcatheter closure of the arterial duct in children, adolescents and adults
Authors: Galal Omar , Wilson Neil , Fadley Fadel Al , Duran Carlos
Published: August 2008

Non-surgical occlusion of the arterial duct (ductus arteriosus) has been shown to be effective. Its limitations are yet to be fully defined. We have attempted to elucidate this issue in the setting of our early experience with the procedure, utilizing the Rashkind double disc occluder. After clinical and echocardiographic evaluation, 56 consecutive patients were studied invasively. In two patients, the patent duct was considered too large for occlusion and in one patient it could not be crossed with the diagnostic catheter. In the remaining 53 patients (95%), an attempt to implant the double disc occluder was made. The mean age of all patients was 7.9 years (range 0.16 year to 29 years). There were 35 females (63%) and 21 males. The mean weight was 24.2 kg (range 6.7 kg–85 kg). In five patients (9.5%), the procedure failed. This was due to pull through of the 17 mm device in three patients. In another patient, because of a very small duct (post surgical ligation), it was not possible to deliver the distal legs of the 12mm device. In the last patient, the device embolized to the left pulmonary artery and was retrieved to the groin. Subsequent replacement was not attempted. In 48/53 patients (90.5%) the device was successfully implanted. Nine cases of the 48 in whom the device was implanted (18.8%) showed complete occlusion at 10 minutes and 22 more (45.8%) five to 24 hours after the procedure. A total of3 1/48 patients (64.5%) were discharged home the day following the procedure with no residual shunt, as judged by color flow Doppler studies. The mean duration of the procedure in all patients was 94 minutes (range 47 to 244). The fluoroscopy time ranged from 3 to 81 minutes with a mean of 17 minutes. Follow-up data (between five days and 18 months) are available in 20/48 patients. Three patients had a clinically detectable residual shunt, with an audible continuous murmur six months after the procedure. All three have undergone complete occlusion using a second device. One more patient showed spontaneous occlusion on follow-up. So far, a total of 35/48 patients (72.9%) have had complete occlusion of the patent duct. We conclude that not all patients with an arterial duct are amenable to occlusion using the Rashkind device. The procedure appears to be safe when performed by a suitably trained novice, nonetheless, and successful implantation can be achieved in over 90% of patients.
Valvar regurgitation in normal children: Is it clinically significant?
Authors: da Silva Mattos Sandra , Severi Rossana , Cavalcanti Catarina Vasconcelos , da Fonte Freire Monica , Filho Djair Brindeiro
Published: August 2008

This study aimed to assess the prevalence of “silent” valvar regurgitation in healthy Brazilian children; to determine whether variations in the gain settings of the machine interfered with its documentation; and to compare Doppler color flow mapping with the pulsed wave Doppler. Twenty-five healthy children were selected randomly. Echocardiographic equipment used was ALOKA 870. Fixed parameters were 3.5 MHz transducer, 400 Hz filter, reject of 7/14 and aperture of 30/80. Variable parameters were Doppler color flow mapping and pulsed wave Doppler gains. A protocol for evaluation of each cardiac valve was developed. Regurgitation was noted in 93% for the pulmonary valve, 74% for the tricuspid valve and 27% for the mitral valve. In the aortic valve, jets suggestive of mild valvar regurgitation were observed with the Doppler color flow mapping technique in two children but these findings were not corroborated by pulsed wave Doppler. Only in the aortic valve were findings from Doppler color flow mapping not in agreement with those from pulsed wave Doppler. Variations in the settings for gain did not interfere significantly in the documentation of regurgitation. Inter-observer variations were not significant (two independent operators). The clinical implications of these findings are yet to be established. Labeling these regurgitant jets as “normal” may be oversimplifying their meaning and perhaps overlooking the possibility of predisposition to endocarditis or progression of the valvar abnormality. Conversely, labeling them as “abnormal” can generate a more serious problem, which is that of iatrogenic heart disease. Prospective follow-up studies should be carried out in these subjects.
Prenatal diagnosis of isomerism of the right atrial appendages
Authors: Rossi Giuseppe , Corno Antonio , Montemurro Giuseppe
Published: August 2008

A complex malformation of the heart was diagnosed by fetal echocardiography at 27 weeks of gestation. The study of the relationship of the abdominal great vessels, showing the inferior caval vein anterior and to the left of the aorta, made possible the diagnosis of right isomerism. This inferential guide to the arrangement of the thoraco abdominal organs proves to be as valid in the fetus as it is in the neonate and child. The examination of the abdominal great vessels as part of the fetal scan is of great value, enabling us to counsel mothers appropriately in the light of the poor prognosis of neonates with isomerism of atrial appendages and complex intracardiac lesions.
The potentially obstructive subaortic region and banding of the pulmonary trunk—selected observations in the patient considered for a Fontan procedure
Authors: Freedom Robert M. , Akagi Teiji , Benson Leland N.
Published: August 2008

There is a clear and present danger for the patient considered for Fontan's operation in the presence of excessive ventricular hypertrophy and/or mass. Such ventricular hypertrophy and/or mass probably reflects singly, or in combination, banding of the pulmonary trunk and subaortic stenosis, as well as other factors. While one can construct a number of therapeutic algorithms for patients seen at various ages with a restrictive ventricular septal defect and subaortic stenosis in the setting of double inlet left ventricle and discordant ventriculoarterial connections, the optimal management should be to minimize the potential risks for development of subaortic stenosis and excessive ventricular hypertrophy and/or mass.
Morphologic and hemodynamic results of staged repair of pulmonary atresia with ventricular septal defect in the presence of hypoplastic pulmonary arteries and systemic-to-pulmonary collateral arteries
Authors: Wilkinson James L. , Ng Yin Ming , Iyer Krishnan S. , Mee Roger B. B.
Published: August 2008

Follow-up cardiac catheterization and angiography was performed in 23 patients after complete, staged repair of pulmonary atresia with ventricular septal defect and multiple systemic-to-pulmonary collateral arteries. Time interval from repair varied from 0.2 to 89 months (mean 26 months). Major residual anomalies of arborization of the pulmonary arteries, with or without pulmonary vascular disease, were present in six patients, two of whom had die patch on the ventricular septal defect perforated because of systemic or suprasystemic right ventricular pressure in the early postoperative period after repair. Both of these children later died, while the other four patients were found to have moderately to severely elevated ventricular pressure ratios and pulmonary arterial pressure measurements, though they are all alive at follow-up intervals from 20 months to 6.8 years. The remaining 17 patients all had hemodynamically satisfactory or good results, with pulmonary arterial mean pressures ranging from 8 to 30 mm Hg (mean 22 mm Hg) and pressure ratios between the ventricles from 0.24 to 0.75 (mean 0.43)—the higher ratios generally reflecting gradients across the conduit valve. Angiography demonstrated that the hypoplastic pulmonary arteries increased in size to normal dimensions, during staging, in most patients, though abnormalities of arborization persisted in several. Analysis of the data from the entire cohort of 58 patients, who were accepted for staging towards repair, showed a significant association between early age at entry to the program and likelihood of complete repair being achievable.
Pulmonary vascular disease in children with congenital heart defects living at high altitude—a quantitative morphometric lung biopsy study
Authors: Balaji Seshadri , Hislop Alison A. , Levin Solomon E. , Haworth Sheila G.
Published: August 2008

Lung biopsies were taken from 30 children aged three months to 15 years (median, 11 months) who had pulmonary hypertensive congenital heart disease and were living at an altitude of 1750 meters. They had either a ventricular septal defect and/or patency of the arterial duct, atrioventricular septal defect or complete transposition with a ventricular septal defect. Biopsies were studied using quantitative morphometric light microscopic techniques. All patients with a ventricular septal defect with or without patency of the arterial duct showed a significant increase in mean percentage arterial medial thickness of both pre- and intraacinar pulmonary arteries compared with those of normal children of similar age living at sea level (p<0.001 for both pre- and intraacinar vessels) and with children with a ventricular septal defect living at sea level (p<0.001 for both pre- and intraacinar vessels). Extension of muscle to more peripheral pulmonary arteries was also greater. Intimal proliferation and fibrosis was seen in 10 patients, in three of whom it was severe. Intimal proliferation occurred more frequently than in children with a ventricular septal defect living at sea level. The findings were similar in patients with atrioventricular septal defect and complete transposition with ventricular septal defect. These findings suggest that patients with congenital heart disease who live at a relatively high altitude develop pulmonary vascular disease more rapidly than do those living at sea level.
Variability of the diameter of the pulmonary outflow tract and its relation to balloon valvoplasty for congenital pulmonary valvar stenosis
Authors: Lo Roxy N. S. , Leung Maurice P. , Yung T. C. , Chan C. H.
Published: August 2008

The diameter of the pulmonary outflow tract was measured from the right ventricular cineangiogram at different phases of the cardiac cycle prior to balloon valvoplasty in 54 patients with congenital pulmonary valvar stenosis. Measured at the level of the hinge point of the valvar leaflets, the pulmonary outflow tract was found to be largest at end-diastole and smallest at end-systole. The mean increase in diameter from systole to diastole was 25.3% (range 6–53%) for 47 patients with typical valvar stenosis, and 24.3% (range 14–41%) for seven patients with dysplastic valvar leaflets (p>0.5). Dilatation using balloons less than 1.2 times the size of the maximal end-diastolic diameter produced a mean reduction in the pressure gradient from right ventricle to pulmonary trunk of 71.1%, compared to 76.2% with bigger balloons (p>0.3). Two patients with an initial ratio between balloon and outflow tract of 0.9 required a second dilatation. The timing of measurement of the diameter of the pulmonary outflow tract should be standardized. Oversized balloons probably are not necessary for pulmonary valvoplasty.
Sectional anatomy of the ventricular septal defect in double outlet right ventricle—correlation of magnetic resonance images from autopsied hearts with anatomic sections
Authors: Yoo Shi-Joon , Ho Siew Yen , Kilner Philip J. , Seo Jeong-Wook , Anderson Robert H.
Published: August 2008

A ventricular septal defect is, almost always, an integral part of double outlet right ventricle and has been classified into the subaortic, subpulmonary, doubly committed and non-committed varieties. This study was performed to correlate the cross-sectional imaging characteristics of such ventricular septal defect in double outlet right ventricles using pathological specimens. The extent and the orientation of the outlet septum were the most important in the differentiation of the four varieties of ventricular septal defect. In the subaortic variety, the outlet septum fused with the left anterior margin of the defect, this being marked by the anterior limb of the septomarginal trabeculation. In the subpulmonary variety, the outlet septum fused with the right posterior margin of the defect, this being the posterior limb of the septomarginal trabeculation. The outlet septum was vestigial in case with doubly committed defects. In those with non-committed defects, the defect was not shown in those images or sections which demonstrated the outlet septum.
Double outlet right ventricle—an echocardiographic study
Authors: Martínez Oscar Arista-Salado , Casado José Arango , de la Cruz María V. , Díaz Francisco , Cubero Osiris
Published: August 2008

We examined the echocardiograms of 40 patients with double oudet right ventricle, proven either at autopsy or during surgery. By way of the subcostal approach, it proved possible to determine the insertion of the outlet (infundibular) septum and to categorize the malformations within the classification proposed by de la Cruz and her colleagues. It was also possible to ascertain the relationships of the atrioventricular valves with one or with both infundibulums, and these features could be correlated with infundibular interrelationships. The interrelationship of the great arteries, in contrast, was not always in accordance with the infundibular interrelationships, indicating that the arterial interrelationship may not prove to be a good parameter for surgical decision-making. Of greatest importance was the visualization and echocardiographic appraisal of the spatial position and size of the ventriculoinfundibular fold, since this anatomic feature was the key to determining the optimal surgical approach. This is so because the ventriculoinfundibular fold constitutes one of the borders of the ventricular septal defect, and separates it from the plane of the leaflets of the arterial valves. Furthermore, the fold frequendy contributes to infundibular obstruction. Our echocardiographic studies, based on a better anatomic knowledge, permitted us to choose the most suitable surgical technique in our patients.
Evidence for a quantitative genetic influence on the formation of aortic valves with two leaflets in the Syrian hamster
Authors: Sans-Coma Valentín , Cardo Manuel , Durán Ana C. , Franco Diego , Fernández Borja , Arqué José M.
Published: August 2008

We studied the morphology of the aortic valves of 1,036 Syrian hamsters belonging to two families subjected to high endogamous pressure. Most (n=955) specimens were examined using a corrosion-cast technique. In the remaining 81 specimens, valvar morphology was assessed by means of a stereomicroscope, and in 18 of these cases a histologic study was also performed. In one of the families, a high proportion (30.5%) of hamsters had aortic valves with two leaflets, the aortic sinuses being oriented ventrodorsally. The percentage of such anomalous valves was found to be positively correlated with the inbreeding coefficient of specimens. In the other family, the occurrence of an aortic valve with two leaflets was a sporadic event. Stereoscopic and histologic observations demonstrated that, in 52 of 63 aortic valves with three leaflets, the ventral commissure between the right and left leaflets was partially fused, while in 10 other cases this commissure was completely fused. In addition, in nine of 18 aortic valves with two leaflets, there was a more or less well-developed raphe located in the ventral aortic sinus, approximately where the true ventral commissure would have been. Our observations suggest the existence of a continuous spectrum of aortic valvar morphology, ranging from a valve with three leaflets and no fusion of the ventral commissure to an aortic valve with two leaflets devoid of any raphe. This spectrum seems to be the result of the interaction of genetic and intrauterine environmental factors. Results of crosses between both related and unrelated hamsters suggest that the observed morphology in individuals is the result of quantitative inheritance.
Outcome of isolated fetal pericardial effusion
Authors: Wyllie Jonathan P. , Hunter Stewart
Published: August 2008

Isolated fetal pericardial effusion is generally held to have a good prognosis. We report a study of seven cases identified from a total of 504 fetuses scanned and followed with serial fetal scans.Our results show that it was associated with a poor fetal prognosis in six of the seven pregnancies. Probably due to the high incidence of mid-trimester abortion and the association with other abnormalities only evident postnatally, this has not, hitherto, been reported. We would advocate close follow-up and a guarded prognosis when isolated pericardial effusions are found on fetal scans.
The postoperative pathology of congenital heart disease
Authors: Anderson Robert H. , Ho Siew Yen
Published: August 2008

The role of the pathologist is changing with regard to the assessment of postoperative congenital heart disease. Whereas, in the past, the access to autopsied hearts provided the opportunity for detailed description and classification, nosologic details of cardiac malformations are now largely agreed. While a detailed analysis of the lesion remains an integral part of the pathologist's task, future advances will now largely be dictated by careful analysis of the effects of cardiac surgery. Such analysis can be divided into early and late phases. When assessing the specimen from a death occurring in the operating room or in the immediate postoperative period, the pathologist must examine carefully the operative incisions, and the correctness of the surgical procedure. Proper assessment of the procedure will necessitate removal of patches and pathways constructed by the surgeon, having documented carefully the location of sutures used to secure the foreign materials. The autopsy must also include assessment of the organs in the rest of the body, particularly the lungs, and must not ignore the immediate effect of the operative procedure on myocardial status. In the late phase, while again assessing in detail the access to the heart and the procedures employed during surgical correction, the pathologist will take care to evaluate the long-term effects of the procedure on the valves of the heart, the myocardium, the pulmonary vasculature and the other organs. At this stage, care should also be taken to ensure that late scarring does not involve the conduction tissues. Particular care will be needed when assessing the efficacy and durability of prosthetic materials inserted into the heart. Most of these steps will be undertaken intuitively by the pathologist, but interest in cardiac pathology as a specialty seems to be waning. The clinician can combat this trend by ensuring that all cases dying after operative procedures are obtained for autopsy, and by encouraging the pathologist to become involved in this fascinating area.
Calcifying myocarditis mimicking an intracardiac tumor on fetal echocardiography
Authors: Oberhoffer Renate , Hillen Uwe , Lang Dieter
Published: August 2008

We 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

Two 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

Discrete 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

A 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

Thrombolysis 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

A 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.
Cardiopulmonary bypass—organ blood flow and metabolism in the pediatric patient
Authors: Lazenby W. Douglas , Krieger Karl H.
Published: August 2008

Congenital heart disease occurs in roughly 0.7% of live-births. Approximately one-half of these defects are severe, requiring surgical correction or palliation. In a large percentage of such procedures in children, moderate or profound hypothermic cardiopulmonary bypass is employed. Because, nowadays, early correction of the defect rather than a palliative procedure is more frequently pursued, the surgeon, anesthesiologist, and perfusionist are called upon to manage younger and smaller children undergoing longer and more complex operations which necessitate longer times on bypass. Due to the particular underlying heart defect, the pathway of flow and viscosity of the blood are frequently different from those encountered in the adult with atherosclerotic coronary arterial or valvar heart disease. Thus, it is important for those caring for children undergoing cardiac surgery utilizing bypass to be well informed about its possible detrimental effects on the various organs. The purpose of this review will be to focus on the effects of bypass on flow to, and metabolism of, the organs and to highlight what is known with regard to these features in children rather than adults.
Effects of cardiopulmonary bypass on the coagulation system
Authors: Edmunds L. Henry
Published: August 2008

Cardiopulmonary bypass makes a mess of homeostasis. Extracorporeal perfusion produces pulseless flow without physiologic controls, showers microemboli throughout the body, and brings blood diluted with additives to an enzymatic boil. Much of the morbidity associated with open heart surgery is due to contact of blood with the synthetic surfaces of the extracorporeal perfusion circuit. Bypass temporarily impairs function of every organ, alters concentrations of over 25 vasoactive substances within the plasma, and causes massive retention of fluid. In short, cardiopulmonary bypass makes patients sick.
Treatment of bleeding following cardiopulmonary bypass in neonates and infants
Authors: Dietrich Wulf , Mössinger Hansjörg J. , Richter Josef A.
Published: August 2008

Bleeding after cardiopulmonary bypass remains a significant problem for the cardiac surgeon. In addition to surgically-induced bleeding, the major cause of loss of blood is an acquired coagulopathy. Impaired function, produced either by the mechanical effect of cardiopulmonary bypass or by the influence of hemostatic activation of platelets during bypass, is the main cause of postoperative bleeding. The tendency towards bleeding in infants and neonates is additionally influenced by the type of operation, those with cyanotic heart disease demonstrating an increased tendency to bleeding. Most of the procedures are more complex than in adults, and many reoperations are necessary, particularly if primary correction is not possible. Furthermore, procedures for perfusion and cooling are mostly different from those used in adults. The operations are regularly performed using deep hypothermia or circulatory arrest, which may further impair the function of platelets. Yet the effect of low temperatures on hemostasis, with or without circulatory arrest, still remains unclear, since the immature system for coagulation in neonates may contribute to impaired hemostasis.
Cerebral physiology of hypothermia and hypothermic acid-base management during cardiopulmonary bypass
Authors: Hindman Bradley J.
Published: August 2008

Cerebral hypothermia is the principal means of protecting the brain during cardiac surgery, permitting periods of reduced perfusion and/or circulatory arrest. For example, at 10–20°C, circulatory arrest for 45–75 minutes is tolerated in animals without subsequent behavioral or histopathologic evidence of neurologic injury. Nevertheless, clinically, die protective effect of hypothermia is far from complete. In children undergoing hypothermic circulatory arrest under equivalent conditions, coma, seizures, choreoathetosis, and developmental retardation are recognized as potential neurologic sequels, occurring widi a combined incidence of 10-30%. Neurologic complications (stroke, neuropsychologic change) also occur with distressing frequency in adults undergoing cardiac surgery, even in the absence of circulatory arrest. An improved understanding of the physiology of cerebral hypothermia during cardiopulmonary bypass and hypothermic arrest is needed to improve upon these results. This review will address current concepts and controversies regarding flow of blood to the brain and its metabolism during hypothermic cardiopulmonary bypass and their relationship to current techniques for cerebral protection.
Cerebral blood flow following hypothermic circulatory arrest
Authors: Tam Vincent K. H. , Cameron Duke E.
Published: August 2008

Hypothermic circulatory arrest has been an important adjunct in pediatric cardiovascular surgery. Historically, use of hypothermia allowed the first intracardiac repair prior to the successful application of cardiopulmonary bypass and well before the advent of cardioplegic cardiac arrest. Today, the repair of many complex defectsis greatly facilitated by the use of hypothermic arrest. Despite the importance of the technique in the armamentarium of the congenital heart surgeon, the physiology of cerebral injury associated with hypothermic arrest has not been fully elucidated. To understand this physiology better, a number of investigators have focused on cerebral reperfusion after the event. In this review, we discuss findings from our own studies in the light of other available data on global and regional flow of blood to the brain following hypothermic arrest.
A vulnerable interval for cerebral injury—comparison of hypothermic circulatory arrest and low flow cardiopulmonary bypass
Authors: Mezrow Craig K. , Midulla Peter S. , Sadeghi Ali M. , Dapunt Otto , Gandsas Alejandro , Shiang Howard , D'Alessandro David A. , Griepp Randall B.
Published: August 2008

Over the past two decades, advances in equipment used for cardiopulmonary bypass and in operative techniques have resulted in a tremendous decrease in the mortality of patients undergoing surgical repair of congenital heart disease utilizing hypothermic circulatory arrest. Despite the widespread use of hypothermic arrest, opinion is not unanimous with regard to its safety. Previous studies which have examined neurological outcome following repair of congenital heart disease in infancy have generally agreed that when the period of arrest exceeds 60 minutes, there is increasing risk of cerebral injury.
Experimental studies of hypothermic circulatory arrest and low flow bypass
Authors: Jonas Richard A.
Published: August 2008

Although the brain is the organ most sensitive to total bodily ischemia, and probably also most sensitive to the deleterious effects of cardiopulmonary bypass, it is also the most difficult organ in which to quantitate function accurately. Clinical studies allow sensitive neuropsychometric testing, as discussed elsewhere in this issue (see Wernovsky et al, p. 308). This is not possible in studies using animals. Even testing of gross neurological integrity in animals is complicated by the associated dysfunction of other organs which accompanies the insult of cardiopulmonary bypass with or without circulatory arrest on the whole body. For example, how should one evaluate an animal undergoing neurological testing which is unable to walk or eat, perhaps because of severe cardiorespiratory or gastrointestinal dysfunction following cardiopulmonary bypass and circulatory arrest. What is the contribution of neurological dysfunction to this state? Most importantly, how is the animal scored which does not survive, even though this may be a result of multiorgan dysfunction?
Clinical neurologic and developmental studies after cardiac surgery utilizing hypothermic circulatory arrest and cardiopulmonary bypass
Authors: Wernovsky Gil , Jonas Richard A. , Hickey Paul R. , du Plessis Adré J. , Newburger Jane W.
Published: August 2008

The dramatic reduction in surgical mortality associated with repair of congenital heart anomalies in recent decades has been accompanied by a growing recognition of adverse neurologic sequels in some of the survivors. Abnormalities of the central nervous system may be a function of coexisting cerebral abnormalities or acquired events unrelated to surgical management (such as paradoxical embolus, cerebral infection, or effects of chronic cyanosis), but insults to the central nervous system appear to occur most frequently during or immediately after surgery. In particular, techniques of support used during neonatal and infant cardiac surgery—cardiopulmonary bypass, profound hypothermia and circulatory arrest—have been implicated as important causes of cerebral injury. This paper will review the effects of bypass and deep hypothermic circulatory arrest on neurodevelopmental outcome.