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At least 1,009 records · Page 56Linked to original sources
PubMed1986

Continuous wave Doppler in the evaluation of simple and complex congenital heart disease in infants and children.

Continuous wave Doppler assessment of systolic pressure gradients was performed using a digital maximal frequency estimator in 118 consecutive infants and children with suspected ventricular outflow obstruction who were undergoing cardiac catheterisation. There was satisfactory correlation with measured systolic pressure gradients in most patients with isolated pulmonary valve stenosis, aortic stenosis or aortic coarctation. Important under-estimation of gradients, however, occurred frequently in patients with more complex lesions. In many of these, the stenotic jet was posteriorly located or obstruction to flow occurred at more than one level. Continuous wave Doppler assessment of outflow tract gradients should be interpreted with caution in complex congenital heart lesions. A low predicted gradient should not be relied upon in isolation for clinical decision making. On the other hand, demonstration of a large gradient by continuous wave Doppler ultrasound provides additional information which may obviate the need for invasive investigation.

Adolescent↗
PubMedDate not supplied

Diagnosis and follow-up of congenital heart disease in children with the use of two-dimensional Doppler echocardiography.

Two-dimensional echocardiography (2D) represents a major advance in non-invasive diagnosis of congenital heart disease (CHD) in children. Nevertheless it has diagnostic limitations in nearly all kinds of heart lesions. These can be overcome for the most part by integration of a pulsed Doppler system. This may be called two-dimensional Doppler echocardiography (2DD). Hereby blood flow information is added to the 2D image. Some common types of CHD including ventricular and atrial septal defects, persistent ductus arteriosus, pulmonic stenosis and coarctation are described with their typical 2DD findings. Non-invasive follow up of children with CHD and early recognition of typical complications can be achieved reliably using 2DD. Future prospects consist in a more quantitative diagnostic application of 2DD.

Adolescent↗
PubMedDate not supplied

Percutaneous therapies for valvular heart disease.

Balloon valvuloplasty has been in clinical use for over 20 years, but the prospect of repairing and replacing cardiac valves via catheter-based techniques represents a truly recent development. This review introduces evolving technologies and their relevance to cardiovascular pathologists.

Aortic Valve↗
PubMed1984

Clinical applications of a new type of real-time two-dimensional Doppler flow imaging system.

The clinical significance of a newly developed real-time 2-dimensional (2-D) Doppler flow imaging technique was assessed. In the instrumentation of the echocardiograph, the pulsed Doppler mechanism was incorporated in a wide-angle, phased-array system. The Doppler flow signals obtained from the cardiac chamber were processed on the basis of the autocorrelation principle. The direction, velocity and variance of the intracardiac blood flow were calculated in real time and displayed in the color-coded mode on the television screen, and were superimposed on the 2-D echocardiographic image of the heart. The technique was used in 20 healthy subjects and 100 cardiac patients. The new technique clearly visualized the whole aspect of intracardiac blood flow by the cine mode in real time; thus, the technique may be called Doppler cineangiocardiography. The mitral inflow and the aortic ejection flow were clearly demonstrated. A regurgitant jet from the valve orifices was dynamically visualized as seen in the cineangiogram. The spatial orientation and extent of the regurgitant jet were easily assessed. The jet stream through the stenotic mitral orifice was well imaged in the left ventricular cavity, showing a variety of stream directions. Intracardiac shunts in ventricular septal defect and atrial septal defect were clearly visualized. The defect could be localized on the interventricular septum on the basis of the site where the shunt flow spurted, although the echocardiographic interruption was not demonstrated in the 2-D echocardiographic image of the cardiac structure. Although some technical problems remain, our new technique greatly improves the diagnostic efficacy of ultrasound.

Adolescent↗
PubMed1987

Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy.

Right isomerism is characterized by the combination of an obstructed pulmonary outflow tract and a total anomalous pulmonary venous connection (TAPVC), which is obstructed in nearly half of the patients. Fourteen patients less than 2 years of age with right isomerism have been seen in our unit. Thirteen of them underwent palliation consisting of a systemic-pulmonary shunt (10 patients) or a combination of shunt and TAPVC repair (3). There were 7 hospital deaths (54%). The presence of obstructed pulmonary venous drainage was the major risk factor in the surgical treatment of these complex cardiac anomalies (p less than .02). Guidelines for the palliative management of right isomerism are suggested.

Echocardiography↗
PubMed1978

Concepts and practices in surgery for total anomalous pulmonary venous connection.

In the last ten years there have been extensive refinements in the surgical approach to total anomalous pulmonary venous connection (TAPVC). This communication reviews determinants of hosptal mortality and controversies in surgical management. As examples of current experience with repair of TAPVC, recent results at the University of Alabama and a combined analysis of hospital mortality for Alabama, Children's Hospital Medical Center in Boston, and Green Lane Hospital are reported. From July, 1974, to July 1977, the Alabama hospital mortality among the 17 infants with supracardiac, cardiac, and infracardiac TAPVC was 12% (2 infants).

Cardiomegaly↗
PubMed1999

Intermediate-term results in pediatric aortic valve replacement.

BACKGROUND: Aortic valve replacement (AVR) in children is now more commonly performed with human tissue valves. METHODS: The results of 100 consecutive pediatric AVRs (50 mechanical, 50 human) were reviewed. RESULTS: There were five perioperative deaths in the mechanical group and one in the human group (p = 0.2). Late complications in the mechanical group included 4 late deaths, 2 cases of endocarditis, 3 thromboembolic complications, and 10 reoperations on the aortic valve. In the human group, there were no late deaths, 2 reoperations for allograft aortic valve deterioration (both in Marfan's patients), and 1 reoperation for allograft pulmonary valve stenosis. Four-year actuarial survival was 83% in the mechanical group and 98% in the human group (p = 0.02). Four-year actuarial survival free of all valve-related complications was 61% in the mechanical group and 88% in the human group (p = 0.008). CONCLUSIONS: Human valves in children requiring AVR provide superior intermediate-term survival and freedom from valve-related complications compared to mechanical valves. Marfan's syndrome may represent a rare remaining contraindication for human AVR in children.

Actuarial Analysis↗
PubMed1997

Rare variants of divided right ventricle with sequestered apical trabecular component.

The right ventricle may be divided into two or more compartments by various structures in various ways. Rarely, the apical trabecular component may be sequestered from the rest of the right ventricle. We report 4 cases with different underlying lesions that share a common pathology of apical sequestration of the right ventricle resulting in diverse hemodynamic consequences. Case 1 had pulmonary valve stenosis. The apical sequestration of the right ventricle resulted in no significant hemodynamic consequence. Case 2 had multiple defects in the muscular ventricular septum. The volume of left-to-right shunt seemed to be reduced because of the commitment of some of the defects to the sequestered cavity. Case 3 had a large defect in the trabecular septum. As the defect involved the whole septum that was related to the sequestered right ventricular apex, the left ventricle together with the sequestered right ventricle formed a boot-shaped chamber. Hemodynamically, the muscular shelf was an interventricular septum. Case 4 had a coronary artery fistula to an isolated cavity that occupied the apical region of the right ventricle. The pathology was similar to the case that was reported as a five-chambered heart. The abnormal cavity was, in fact, the sequestered right ventricular apex.

Adolescent↗
PubMed2002

Locoregional control in infants with neuroblastoma: role of radiation therapy and late toxicity.

PURPOSE: To review patterns of failure in infants with neuroblastoma and determine late toxicity and efficacy of radiotherapy (RT) on locoregional control. MATERIALS AND METHODS: From 1955 to 1998, 53 children (35 males and 18 females) <1 year old with neuroblastoma were seen at our institution. Twenty-one (40%) were </=1 month of age (neonates). Seven congenital anomalies were seen in 4 children (atrial septal defect, pulmonary valve stenosis, and absent corpus callosum were seen in 1 patient each; the other child had atrial and ventricular septal defect, aortic arch hypoplasia, and mitral valve stenosis). Primary tumor was located in the adrenal gland in 26 (49%), abdomen/nonadrenal in 14 (26%), thorax in 9 (17%), neck in 2 (4%), and pelvis in 2 (4%). All infants were retrospectively staged according to the International Neuroblastoma Staging System (INSS); 8 had Stage 1, 7 Stage 2A, 6 Stage 2B, 15 Stage 3, 6 Stage 4, and 11 Stage 4S. All patients, except 11 with Stage 4S and 4 with Stage 4, had resection of the primary tumor. Sixteen infants had pathologic involvement of regional lymph nodes (LN+). Twenty patients received RT. Kilovoltage beams were used in 10, megavoltage photons in 9, and electrons in 1 child. Fifteen received RT to the primary site and regional nodes (postoperative 8, preoperative 7). Postoperative doses ranged from 15 to 25 Gy whereas preoperative doses ranged from 12 to 31 Gy using a median fraction size of 1.5 Gy. Chemotherapy was employed in 22 of 53 patients (42%) with the most common agents being cyclophosphamide in 22 and doxorubicin in 11. Median follow-up was 13.1 years (range, 2-41 years). RESULTS: Tumor control: The 5-year overall and freedom from progression rates were 79 +/- 9% and 81 +/- 11%; the locoregional control rate was 88 +/- 9%. INSS Stage was a prognostic factor for overall survival (p = 0.03) and freedom from progression (p = 0.035). Gender, age (</=1 month vs. >1 month), and primary site were not found to impact on survival or progression. None of the Stage 1, 2A, or 2B patients recurred. One of 15 Stage 3 and 5 of 6 Stage 4 children recurred (6 distant metastases, 4 local failure). Four of 6 (67%) LN+ patients treated with locoregional RT and 8 of 10 (80%) LN+ patients treated without RT were locally controlled. There was no isolated locoregional relapse. Two Stage 4S patients died of respiratory compromise secondary to hepatomegaly. RT toxicity: For the 20 infants who received RT, 13 are alive with long-term follow-up ranging from 9.3 to 41 years, median 23 years. The 10 and 15-year musculoskeletal toxicity rates were 38.5% and 47.3% for those receiving RT and 3.3% for no RT (p = 0.02, log-rank test). Five of 6 infants <6 months of age and 1 of 7 >/=6 months developed musculoskeletal toxicity. Musculoskeletal effects were seen in 6 RT patients and included bony hypoplasia in 6, scoliosis in 5, soft tissue hypoplasia in 3, slipped capital femoral epiphysis in 2, kyphosis in 1, and osteochondroma in 1. Three required orthopedic intervention, all receiving >/=20 Gy. One child developed bowel obstruction at 21 months and another developed a leiomyosarcoma in the treatment field 34 years after RT. CONCLUSION: Our study shows that most LN+ infants achieve locoregional control without RT. Infants <6 months receiving RT were the most susceptible to musculoskeletal abnormalities. Further studies are needed to determine if cardiovascular anomalies are more frequently seen in children with neuroblastoma.

Abdominal Neoplasms↗