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At least 325 records · Page 18Linked to original sources
PubMed1986

Importance of coronary artery anomalies in operations for congenital heart disease.

A congenital coronary artery anomaly influenced operative management of 21 children (34 operations) during a recent 8-year interval. This group represented 1.3% of cardiothoracic operations performed. Survivors included 5 of 10 children having correction of the left coronary artery from the pulmonary artery and 11 children having correction of intracardiac defects associated with major coronary arteries crossing the right ventricular outflow tract. Two children survived primary repair of injured major coronary arteries. Isolated coronary artery anatomy must be considered during management of intracardiac defects. Intraoperative injury to a major coronary artery requires meticulous repair.

Adolescent↗
PubMed1987

Acute complications of catheter therapy for congenital heart disease.

Although therapeutic catheter procedures for congenital heart disease are still developing, the number of procedures being performed allows statistically relevant review of the acute complications. Between January 1, 1984, and February 1, 1987, 417 such procedures were performed at The Children's Hospital, Boston; the age range of the patients was 1 day to 51 years (median 4 years, 6 months). Catheter procedures consisted predominantly of vascular dilations (peripheral pulmonic stenosis, 97; valvular pulmonic stenosis, 67; valvular aortic stenosis, 62; recurrent coarctation, 49) and embolizations (double umbrella device, 36; steel coils, 45). Overall, there were 50 acute complications (12%); 24 (6%) were major and 26 (6%) were minor. The mortality rate was 3 of 417 (0.7%). Complication rates varied between 4% for dilation of recurrent coarctation and 40% for dilation of aortic stenosis. The age of the patients was a factor in complications of vascular access (11 patients), 8 of which occurred in patients younger than 6 months (median 5), and in cardiac arrest and ventricular fibrillation (4 patients, 3 of whom were younger than 6 months [median 3]). No statistically significant trend toward diminishing overall complication rates was discerned over the 37 months of this study.

Acute Disease↗
PubMed2005

On the use of computational models for the quantitative assessment of surgery in congenital heart disease.

The surgical repair of congenital heart disease often involves significant modifications to the circulatory tree. Resections, reconstructions, graft insertions and the deployment of implants and biomedical devices have an impact on local and systemic haemodynamics, which may be difficult to foresee or to assess quantitatively by clinical investigation alone. Mathematical models can be employed to visualise, estimate or predict events and physical quantities that are difficult to observe or measure, and can be successfully applied to the study of the pre- and post-operative physiology of cardiovascular malformations. This paper analyses the potentialities of computation fluid dynamics in this respect, outlining the method, its requirements and its limitations. Examples are given of lumped parameter models, axi-symmetric models, three-dimensional models, fluid-structure interaction simulations and multiscale computing applied to total cavo-pulmonary connection, aortic coarctation and aortic arch reconstruction.

Cardiovascular Surgical Procedures↗
PubMedDate not supplied

Haemostatic changes in children with cyanotic and acyanotic congenital heart disease.

This study was undertaken to screen children with congenital heart disease for coagulation abnormalities and to compare the groups of cyanotic and acyanotic children with congenital heart disease with respect to abnormalities of the coagulation system. Following investigations were done in all the patients: complete blood count, erythrocyte sedimentation rate, peripheral smear examination, bleeding time, prothrombin time, activated partial thromboplastin time, assay of fibrinogen, D-dimer, factors VII and VIII and antithrombin III. Red cell indices were determined in 12 control, 12 acyanotic and 20 cyanotic children. Twenty-five patients each, with echocardiographically proven cyanotic and acyanotic congenital heart disease under 12 years of age constituted the study group; as many children of the same age group were included as the control group. The results showed isolated abnormalities of laboratory tests with equal frequency (28%) in acyanotic and cyanotic groups but coexisting abnormalities of more than one test were seen in significantly larger number of cyanotic children (5/25 and 16/25, respectively). A significant association was noted between thrombocytopenia and a high haematocrit in cyanotic patients. It is concluded that laboratory abnormalities of tests of haemostasis are more common in cyanotic congenital heart disease patients. The patterns of laboratory abnormalities suggest a chronic compensated disseminated intravascular coagulation at a subclinical level, reduced synthesis of clotting factors and/or deranged platelet aggregation in different subgroups of patients.

Blood Coagulation Factors↗
PubMed2001

Is end-tidal CO2 an accurate measure of arterial CO2 during laparoscopic procedures in children and neonates with cyanotic congenital heart disease?

BACKGROUND/PURPOSE: Children with cyanotic congenital heart disease (CCHD) may require laparoscopic procedures. There are no data on the ability of capnography to predict arterial carbon dioxide concentrations (PaCO2) in patients with CCHD during pneumoperitoneum. METHODS: Seven patients (age 1 to 35 months) with CCHD undergoing laparoscopic Nissen fundoplication are presented. Standard general endotracheal anesthesia was administered. The operations were performed either by or in consultation with the cardiac anesthesia team. During each case, concomitant PaCO2 and end-tidal carbon dioxide (ETCO2) measurements were made via an arterial line and capnograph before and after insufflation of the abdomen. The PaCO2-ETCO2 gradients before and during pneumoperitoneum were then compared using a paired Student's t test. RESULTS: There was a statistically significant increase in the PaCO2-ETCO2 gradient (5.7 v 13.4) after insufflation compared with baseline (P <.015). CONCLUSIONS: These preliminary results show that ETCO2 is not a reliable monitor of PaCO2 in patients with CCHD undergoing laparoscopic procedures. The authors feel that close monitoring, including arterial blood gas measurements, and an experienced anesthesia team are necessary to perform laparoscopic procedures in patients with CCHD.

Carbon Dioxide↗
PubMed2000

Energy expenditure after surgical repair in children with cyanotic congenital heart disease.

OBJECTIVE: Infants with cyanotic congenital heart disease (CCHD) have previously been shown to have similar resting energy expenditures (REEs) and elevated total energy expenditures (TEEs) compared with age-matched healthy infants. The purpose of this investigation was to re-examine the REE and TEE of the same individuals at 5 years of age, after surgical repair of the heart defect was done, to determine whether metabolic differences persist. STUDY DESIGN: Seven children were studied approximately 2.6 years after they underwent surgical repair of CCHD along with 10 age-matched healthy children. Indirect calorimetry was used to determine REE, and the doubly labeled water method was used to determine TEE and body composition. RESULTS: Results were compared with single-factor repeated measures analysis of variance. No significant differences were found between groups in weight or body composition. No significant differences were found between groups in REE, TEE, or the energy expended in physical activity. CONCLUSION: We conclude that differences in TEE observed during infancy are no longer present in 5-year-old children after they undergo surgical repair of CCHD. Furthermore, the individual components of energy expenditure of children with CCHD after repair are indistinguishable from those of healthy age-matched children.

Analysis of Variance↗
PubMed1997

Atrial arrhythmias during chronic follow-up of surgery for complex congenital heart disease.

After surgery for complex congenital heart disease, clinically important atrial tachyarrhythmias have a higher than normal incidence if sufficiently large regions of conduction block occur within the atria, especially in the presence of hemodynamic alterations. Sinus bradycardia may result from direct damage to sinus node and its blood supply. Historical data have identified patients who have undergone the Mustard or Senning operations for dextrotransposition of the great vessels and the Fontan operation in cases of functional single ventricle as being at great risk for atrial tachyarrhythmias. These arrhythmias are especially poorly tolerated when there are co-existing hemodynamic alterations and are an important source of morbidity and mortality. Until recently, treatment strategies have been limited to antiarrhythmic drugs, bradycardia pacing, and--in suitable patients--antitachycardia pacing, often in combination. Amiodarone has been the most efficacious drug, but has only been of moderate value because of extracardiac side effects. Radiofrequency ablation of the atrial regions critical to reentrant circuits, which was discovered to be of value in patients with atrial flutter and a normal heart is being applied to this diverse group of patients. Early results are promising, but the Fontan operation patients are especially challenging because of early recurrences of apparently new reentrant circuits. Progress in this area will likely come from newer surgical techniques that prevent the milieu for atrial reentry and from multidimensional mapping systems for our current patients.

Atrial Fibrillation↗
PubMed2005

Hyperbaric treatment of cerebral air embolism in an infant with cyanotic congenital heart disease.

PURPOSE: Infants with cyanotic congenital heart disease are at risk for cerebral arterial gas embolism (CAGE) from iv infusion lines. Concern about the hazards and difficulty of caring for such patients inside a hyperbaric chamber may deter referral. We report a complex case in which a small infant was managed successfully using a modified hyperbaric oxygen treatment (HBOT) schedule. CLINICAL FEATURES: A four-month-old 6.19 kg male infant with a recent Glenn shunt for double-outlet right ventricle had a seizure and became unstable immediately after an iv drug infusion. The patient was sedated, intubated and ventilated and dobutamine was commenced. A computerized tomography (CT) scan performed ten hours later demonstrated three intracranial air bubbles. About ten hours later the patient was referred for HBOT which commenced soon afterwards in a multiplace chamber. Since the right-to-left shunt would greatly increase the risk of decompression illness from breathing hyperbaric air HBOT was modified by the use of an abbreviated schedule at reduced pressure. Two 90-min HBOT sessions were administered within 24 hr at 38 feet of sea-water pressure, equivalent to 2.15 atmospheres absolute without any air break. During treatment the infant was ventilated using an Oxford Penlon ventilator. A subsequent CT scan demonstrated the absence of air. After extubation he appeared neurologically intact except for some weakness of the left arm. CONCLUSION: Hyperbaric oxygen may be utilized to treat CAGE in small infants with right-to-left shunt and should be commenced promptly.

Embolism, Air↗
PubMed2000

Postnatal growth in southern Chinese children with symptomatic congenital heart disease.

Many Western children with congenital heart disease (CHD) show significant growth retardation. In this study postnatal growth was examined in Chinese children with symptomatic CHD in Hong Kong, in relation to their diagnosis and the time of surgery. 363 children of four years old or younger, who were admitted at Grantham Hospital, Hong Kong, in 1994 and 1995, were subdivided into six diagnostic categories and categorised into cyanotic and acyanotic groups. While a reduced birth weight SDS was present in 18% of patients, at the time of operation approximately 40% of them had subnormal weight and height values. Girls were more impaired in weight and weight-for-height than boys (-1.90 SDS vs -1.52 SDS, and -0.90 SDS vs -0.46 SDS, respectively). Children with acyanotic lesions were more affected in growth than those with cyanotic lesions, but they were also operated on at an older age than children in the latter group. Left to right shunt and common intracardiac mixing were particularly associated with wasting; transposition of the great arteries and pulmonary outflow tract obstruction with stunting; while children with left ventricular outflow obstruction revealed a proportional growth retardation in weight and height. Age at operation did not seem to have an independent effect on postnatal growth in children with CHD. As with Western children, growth retardation is a common feature in Chinese children with symptomatic cardiac defects. Haemodynamics, age at operation and nutritional influences are discussed as potential aetiologic factors.

Birth Weight↗
PubMed2005

Myocardial perfusion reserve in adults with cyanotic congenital heart disease.

In patients with cyanotic congenital heart disease (CCHD), a right-to-left shunt results in systemic hypoxemia. Systemic hypoxemia incites a compensatory erythrocytosis, which increases whole blood viscosity. We considered that these changes might adversely influence myocardial perfusion in CCHD patients. Basal and hyperemic (intravenous dipyridamole) perfusion measurements were obtained with [13N]ammonia positron emission tomographic imaging in left (LV) and right (RV) ventricular and septal myocardium in 14 adults with CCHD [age: 34.1 yr (SD 6.5)]; hematocrit: 62.2% (SD 4.8)] and 10 healthy controls [age: 34.1 yr (SD 6.5)]. In patients, basal perfusion measurements were higher in LV [0.77 (SD 0.24) vs. 0.55 ml x min(-1) x g(-1) (SD 0.09), P < 0.02], septum [0.71 (SD 0.16) vs. 0.49 ml x min(-1) x g(-1) (SD 0.09), P < 0.001], and RV [0.77 (SD 0.30) vs. 0.38 ml x min(-1) x g(-1) (SD 0.09), P < 0.001]. However, basal measurements normalized for the rate-pressure product were similar to those of controls. Calculated oxygen delivery relative to rate-pressure product was higher in the patients [2.2 (SD 0.8) vs. 1.6 (SD 0.4) x 10(-5) ml O2 x min(-1) x g tissue(-1) x (beats x mmHg)(-1) in the LV, P < 0.05, and 2.0 (SD 0.7) vs. 1.4 (SD 0.3) x 10(-5) ml O2 x min(-1) x g tissue(-1) x (beats x mmHg)(-1) in the septum, P < 0.01]. Hyperemic perfusion measurements in CCHD patients did not differ from controls [LV, 1.67 (SD 0.60) vs. 1.95 ml x min(-1) x g(-1) (SD 0.46); septum, 1.44 (SD 0.56) vs. 1.98 ml x min(-1) x g(-1) (SD 0.69); RV, 1.56 (SD 0.56) vs. 1.65 ml x min(-1) x g(-1) (SD 0.64), P = not significant], and coronary vascular resistances were comparable [LV, 55 (SD 25) vs. 48 mmHg x ml(-1) x g x min (SD 16); septum, 67 (SD 35) vs. 50 mmHg x ml(-1) x g x min (SD 21); RV, 59 (SD 26) vs. 61 mmHg x ml(-1) x g x min (SD 27), P = not significant]. These findings suggest that adult CCHD patients have remodeling of the coronary circulation to compensate for the rheologic changes attending chronic hypoxemia.

Adult↗
PubMed2006

Molecular and cellular basis of congenital heart disease.

The cellular and molecular basis of congenital heart disease (CHD) is an evolving area of rapid discovery. This article introduced the basic mechanisms underlying cardiac development and CHD in order to permit a clear understanding of current diagnostics and therapeutics and their future development. It is clear that although significant advances have been made in understanding mechanisms controlling heart formation, the direct causes of CHD remain poorly defined. Future studies tha delineate the complexity of these mechanisms are required to provide a comprehensive understanding of the etiologies of CHD. Such understanding will lead to the development of novel approaches to prevention and therapy.

Animals↗
PubMed1996

Neonates with congenital heart disease, Part II: Congenital cardiac defects with increased pulmonary blood flow.

Generally, cardiac lesions with increased pulmonary blood flow demonstrate cardiomegaly, increased pulmonary vascular markings, and pulmonary congestion on the chest x-ray. These findings occur as a result of the following: 1. A left-to-right shunt or mixing lesion in which excess volume of blood flow causes dilation of cardiac chambers, resulting in the appearance of cardiomegaly, and in which increased pulmonary artery blood flow causes increased pulmonary vascular markings 2. Obstruction of blood flow that produces pulmonary venous hypertension and resultant pulmonary edema The next article in this series will address cardiac lesions with decreased pulmonary blood flow.

Aortic Coarctation↗
PubMed1989

Fetal echocardiography in the diagnosis of congenital heart disease.

Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper reviews the accuracy and outcomes of fetal cardiac ultrasound studies at The Prince Charles Hospital over the past 4 years. A total of 43 fetuses, of gestational ages 16-39 weeks, were studied. The indications for these were: previous sibling with cardiac anomaly (16 cases); abnormal heart on obstetric ultrasound scan (USS) (13 cases); abnormalities found on obstetric USS with normal appearing heart (one case); fetal bradycardia (seven cases); fetal tachycardia (four cases); irregular fetal heart beat (two cases). At birth all fetuses assessed because of a previously affected sibling were normal. Of the 13 referred because of structural heart lesions suspected on obstetric USS, eight were abnormal, four were normal and one was terminated without autopsy. Important rhythm disturbances occurred in nine of the 11 referred because of abnormal heart rates. One false positive diagnosis of a possible coarctation was made, and in four cases an abnormality was noted on USS but the diagnosis was not completely correct. Nine of the 19 infants with abnormalities detected have died. Fetal echocardiography is an accurate and useful method of diagnosing congenital heart disease in utero. Although the mortality of affected fetuses is high, antenatal diagnosis allows planning of medical care and offers the greatest chance of a successful outcome.

Echocardiography↗
PubMed1992

The impact of pregnancy on the woman with congenital heart disease: considerations for intrapartum nursing care.

Many women with congenital heart disease can successfully tolerate the profound hemodynamic changes associated with pregnancy and birth with a collaborative team approach to management of their disease. This article provides a discussion of the interaction between pregnancy and congenital heart disease (including left-to-right shunts, obstructive lesions, and cyanotic defects), focusing on maternal risks and strategies for intrapartum hemodynamic management. Nurses providing intrapartum care for women with congenital heart disease must understand the applicable cardiac pathophysiology, the risks imposed by pregnancy and the birth process, and appropriate medical and nursing interventions to prevent complications.

Critical Care↗
PubMed1981

Dermatoglyphs in congenital heart disease.

The palmar dermatoglyphs of 800 patients with anatomically proven congenital heart disease were compared with prints from 1000 controls. A review of the previous studies revealed major technical deficiencies, and the present study failed to confirm most of the previously reported positive findings. An overall increase in the incidence of hypothenar patterns was found, probably explaining the previous suggestion of increased atd angle in congenital heart disease. A large number of statistical comparisons inevitably produced a few 'significant' results, most of which were inconsistent in various ways. Two percent of cases were found to have rare epidermal ridge malformation, ridge dissociation. The nature of the relationship between this and congenital heart disease is obscure. Claims that there are diagnostically useful dermatoglyphic changes in congenital heart disease can be disregarded.

Dermatoglyphics↗