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PubMed2005

Causative organism influences clinical profile and outcome of infective endocarditis in pediatric patients and adults with congenital heart disease.

BACKGROUND: Studies on microorganisms in a large cohort of patients with congenital heart disease (CHD) and infective endocarditis (IE) are rare. Using a nationwide survey, the relationship between causative microorganisms and clinical profiles in patients with CHD and IE was investigated. METHODS AND RESULTS: Data from 188 patients with CHD (pediatric patients (n=113), mean age, 6.2+/-4.9 years; adult patients (n=75), mean age 28.4+/-13.4 years) and IE from 60 institutions were analyzed. Causative microorganisms were Streptococcus species (94:50.0%), Staphylococcus species (68:36.2%), Haemophilus species (9:4.8%), Candida (5:2.7%), Pseudomonas species (4:2.1%) and other unclassified microorganisms (8:4.3%). Staphylococcal IE was observed significantly higher in perioperative IE (11/16), in cyanotic patients (32/73) and patients younger than 1 year old (11/16). Streptococcal IE was observed significantly higher in acyanotic patients (64/109) and patients aged 16 years or older (48/75). Total mortality was 20/188 (10.6%) and was high for candidial (2/5; 40%) and pseudomonal IE (2/4; 50%). Mortality was highly associated with younger age, especially infants (5/16), and methicillin-resistant staphylococcal IE (6/15). CONCLUSIONS: The causative microorganisms are significantly related to the clinical profile and outcome in patients with IE and CHD. These results form the basis for selecting appropriate antibiotics for prevention and management.

Adolescent↗
PubMed2005

New developments in echocardiographic methods to assess right ventricular function in congenital heart disease.

PURPOSE OF REVIEW: Evaluation of right ventricular (RV) function in patients with congenital heart disease (CHD) is an essential component of clinical management. The complex geometry of the RV makes this a challenging task and necessitates the use of alternative methods from those used to assess left ventricular (LV) function. Recent developments in echocardiographic techniques have enhanced our ability to accurately assess RV function. We focus this review on literature published since September 2003. RECENT FINDINGS: In this review we survey recent literature pertaining to advances in echocardiographic techniques used in assessing RV function. These new methods use two-dimensional echocardiography, Doppler echocardiography, tissue Doppler imaging (TDI), and strain rate imaging. Doppler techniques offer unique advantages for RV function in that they are independent of geometry and relatively independent of loading conditions. SUMMARY: Echocardiography can assess RV function in a reliable fashion in children and adults with congenital heart disease. Appropriate use of echocardiography can minimize the utilization of more invasive imaging modalities such as magnetic resonance imaging and can guide clinical decisions regarding the necessity of obtaining such imaging.

Echocardiography, Doppler↗
PubMed1985

Kabuki make-up syndrome (Niikawa-Kuroki syndrome) associated with congenital heart disease.

Kabuki make-up syndrome has been reported mainly among Japanese, so far occurring in more than 20 cases. Among these, however, only one case associated with congenital heart defect has been reported. We have treated three patients with this syndrome and of these two had congenital heart disease. We suggest the possibility that the association of congenital heart disease with Kabuki make-up syndrome may not be fortuitous.

Abnormalities, Multiple↗
PubMed2001

Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability.

OBJECTIVE: To evaluate changes in cognitive and academic functioning following cardiac surgery in children with congenital heart disease. DESIGN: A prospective cross sectional study in which patients were assessed immediately before treatment and 12 months later. PATIENTS: Three groups of children aged 3.5-17 years: a group with congenital heart disease awaiting surgery, another awaiting bone marrow transplantation, and a healthy comparison group. MAIN OUTCOME MEASURES: Intelligence quotient and measures of academic attainment, evaluated with the British Ability Scales. RESULTS: Preoperatively, children with cyanotic lesions showed deficits in comparison with those with acyanotic lesions. Postoperatively, children with cyanotic lesions showed a deterioration in performance and achieved significantly lower scores than those with acyanotic lesions. While there were significant differences between the congenital heart disease and bone marrow transplantation groups preoperatively, these were no longer apparent at follow up. CONCLUSIONS: In contrast to previously published findings, the present results suggest that cardiac surgery does not result in early postoperative improvements in cognitive function for children with congenital heart disease. The nature of the cardiac lesion continues to affect cognitive and academic performance, even after surgery.

Adolescent↗
PubMedDate not supplied

Health education needs of adolescents with congenital heart disease.

Self-concept, health beliefs, health behaviors, health information sources, and perceived health information needs were assessed in 90 patients with congenital heart disease and 54 peers without chronic illness, ages 13 to 22 years. Data revealed that patients with congenital heart disease did not differ from their healthy counterparts in overall self-concept, health beliefs, use of preventive health services, and sources of health information. Patients with congenital heart disease reported greater perceived stress in comparison to peers. Significantly more patients with heart disease needed information regarding pregnancy and contraception, and older cardiac subjects were less likely to be sexually active. For these patients who currently seem to rely largely on peers for some critical health information, sensitive and comprehensive counseling by health professionals is imperative.

Adolescent↗
PubMedDate not supplied

The adult with congenital heart disease.

There are approximately 800,000 adult survivors of congenital heart disease in the U.S., and this number continues to increase on an annual basis. It was only 50 years ago that the first intracardiac repair of a congenital heart defect was performed. Survival into adulthood has only been realistic in the last three decades. This has created a new patient population with a number of challenges such as the impact of adult onset diseases on their underlying cardiac pathology, cognitive, physical, and psychosocial development, as well as issues related to reproduction, employment and health insurance. Possibly the most significant obstacle is identifying healthcare professionals who can provide long-term care. This manuscript reviews the challenges and recommendations for the care of these patients.

Adult↗
PubMed1976

Radioactive tracers in congenital heart disease.

Radionuclide angiocardiography is a useful method in the evaluation of patients with congenital heart disease, safely and nontraumatically. Physiologic variables such as transit times, cardiac output, left ventricular ejection fraction, stroke volume, end-diastolic volume can be measured accurately with this technique. An important application of radionuclide angiocardiography in children with congenital heart disease is in the detection, localization and quantification (pulmonary to systemic flow ratio) of intracardiac shunts and shunts between the great vessels. This technique has been found useful in the evaluation of the newborn infant with cyanosis, the patient with a cardiac murmur and the patient who has had cardiovascular surgery. Newer mobile gamma camera-computer systems permit the performance of radionuclide angiocardiography in several ill premature and newborn infants and patients during the early postoperative period. Specially designed magnifying collimators and the development of ultrashort-lived radionuclides should result in an overall improvement in the diagnostic capabilities of this technique and in a further reduction in the radiation dose.

Angiocardiography↗
PubMed2001

[Role of pediatric cardiologists in the management of neonates with congenital heart disease].

Whether diagnosed before birth or suspected in a newborn, congenital heart diseases rapidly require an evaluation by the pediatric cardiologist. The role of the pediatric cardiologist is threefold: 1) to realize a careful anatomical examination using color Doppler echocardiography; 2) in many cases to perform a palliative or curative therapeutic act based upon interventional catheterization, such as Rashkind atrioseptotomy or percutaneous balloon valvuloplasty; and 3) whenever necessary, to take a rapid decision of cardiac surgery in concert with the cardiac surgeon, and to prepare the child for surgery.

Cardiac Catheterization↗
PubMed1998

Outcomes of noncardiac surgical procedures in children and adults with congenital heart disease. Mayo Perioperative Outcomes Group.

OBJECTIVE: To assess the outcomes and risk factors for morbidity associated with anesthesia and noncardiac surgical procedures in children and adults with congenital heart disease. DESIGN: We conducted a retrospective cohort study of the 6-year period from January 1987 through November 1992 at one of the Mayo-affiliated hospitals in Rochester, Minnesota. MATERIAL AND METHODS: In all children and adults 50 years of age or younger with congenital heart disease who underwent one or more noncardiac surgical or diagnostic procedures and anesthesia, we analyzed the risk factors for 30-day perioperative morbidity and mortality. RESULTS: The overall frequency of complications among the 276 patients who underwent 480 noncardiac surgical procedures and anesthesia was 5.8% (28 of 480), and only 1 patient died intraoperatively. Major risk factors univariately associated with complications for the first procedures (15 of 276 patients or 5.4%) included the presence of cyanosis (P = 0.002), current treatment for congestive heart failure (P<0.001), poor general health (P<0.001), and younger age at the time of the procedure (P = 0.027). Procedures performed on the respiratory and nervous systems also were associated with high frequencies of complications. Complications in patients undergoing ambulatory surgical procedures were infrequent (1.7%). CONCLUSION: The frequency of perioperative complications in children and adults who have congenital heart disease and undergo noncardiac surgical procedures and anesthesia is low. Patients who have pulmonary hypertension, congestive heart failure, or cyanosis and children with congenital heart disease who are younger than 2 years of age have an increased frequency of perioperative morbidity.

Adolescent↗
PubMed2001

The safety and efficacy of sevoflurane anesthesia in infants and children with congenital heart disease.

UNLABELLED: We tested the hypothesis that sevoflurane is a safer and more effective anesthetic than halothane during the induction and maintenance of anesthesia for infants and children with congenital heart disease undergoing cardiac surgery. With a background of fentanyl (5 microg/kg bolus, then 5 microg. kg(-1). h(-1)), the two inhaled anesthetics were directly compared in a randomized, double-blinded, open-label study involving 180 infants and children. Primary outcome variables included severe hypotension, bradycardia, and oxygen desaturation, defined as a 30% decrease in the resting mean arterial blood pressure or heart rate, or a 20% decrease in the resting arterial oxygen saturation, for at least 30 s. There were no differences in the incidence of these variables; however, patients receiving halothane experienced twice as many episodes of severe hypotension as those who received sevoflurane (P = 0.03). These recurrences of hypotension occurred despite an increased incidence of vasopressor use in the halothane-treated patients than in the sevoflurane-treated patients. Multivariate stepwise logistic regression demonstrated that patients less than 1 yr old were at increased risk for hypotension compared with older children (P = 0.0004), and patients with preoperative cyanosis were at increased risk for developing severe desaturation (P = 0.049). Sevoflurane may have hemodynamic advantages over halothane in infants and children with congenital heart disease. IMPLICATIONS: In infants and children with congenital heart disease, anesthesia with sevoflurane may result in fewer episodes of severe hypotension and less emergent drug use than anesthesia with halothane.

Anesthetics, Inhalation↗
PubMed2004

Transitioning the young adult with congenital heart disease for life-long medical care.

Guidelines for the successful orchestration of transitioning of the adolescent and young and older adult patient with congenital heart disease to a health care system appropriate for their long-term congenital heart disease care and counseling appear necessary to improve patient and family confidence, education, therapy, life quality, and survival outcomes. Schema for care organization and delivery for adult patients with congenital heart disease remain primitive and largely unimplemented. The presence of a strong central care oversight organization and the establishment of a multi-institutional database to assist in assessment of care outcomes and guidelines appears requisite to these needs and for the establishment of transitioning guidelines for these patients as they assume a greater and deeper shared control of their futures with their caregivers.

Adolescent↗
PubMed1984

Use of the rebreathing method in the differential diagnosis of congenital heart disease and persistent fetal circulation.

The differential diagnosis of congenital heart disease from persistent fetal circulation is clinically difficult and cardiac catheterization is often needed. The development of a safe, new technique for use of the rebreathing method has allowed the determination of effective pulmonary blood flow, lung tissue volume, lung diffusion capacity and functional residual capacity in 7 critically ill, ventilator-dependent infants at the bedside. Analysis of the data revealed highly significant differences for lung tissue volume and diffusion capacity, a minimally significant difference for effective pulmonary blood flow and no difference for functional residual capacity between the groups. Use of this method allows not only attainment of clinically useful information but also permits better insight into the pathophysiology of the disease state.

Animals↗
PubMed2005

Acquired coronary artery fistula after open heart surgery for congenital heart disease.

BACKGROUND: Most coronary artery fistulas were reported as congenital. Acquired coronary artery fistula occurring after cardiac surgery has rarely been reported. METHODS: From 1998 to 2003, 10 patients with coronary artery fistula detected by echocardiography after open heart surgery for congenital heart disease were included. Their ages ranged from 2 months to 41 years (median 4.2 years). The underlying heart disease was tetralogy of Fallot in five patients, ventricular septal defect in three, double chamber right ventricle in one, and transposition of the great arteries with ventricular septal defect in the remaining one. RESULTS: Of these 10 patients, the coronary artery fistula originated from the left coronary artery in four, right coronary artery in two, and unknown origin in the remaining four. The coronary artery fistula drained into the right ventricle in nine and into the left ventricle in the remaining one. The incidence of acquired coronary artery fistula after open heart surgery for congenital heart disease was 0.44% (8/1832). The identified risk factors for acquired coronary artery fistula were reoperation and right ventricular muscle resection in ventricular septal defect. After follow-up for 0.5-12 years (mean 4.1+/-3.3 years), the coronary artery fistula persisted, but neither symptoms nor significant left-to-right shunt was noted. CONCLUSIONS: Acquired coronary artery fistula is a rare complication after cardiac surgery. Reoperation and resection of right ventricular hypertrophic muscle increase the risk of this complication. Although shunt flow did not increase during follow-up, the significance of acquired coronary artery fistula needs further investigation.

Adolescent↗
PubMed1996

Measurement of ventricular volumes by cine magnetic resonance imaging in complex congenital heart disease with morphologically abnormal ventricles.

This study assessed the validity of cine magnetic resonance imaging (MRI) for measuring right and left ventricular volumes by using Simpson's rule in children with complex congenital heart disease. Forty-five patients with complex congenital heart disease (average age 2.6 years) and 10 controls (average age 2.3 years) were evaluated. The whole heart was encompassed by contiguous transverse sections. Ventricular volumes were calculated by adding luminal areas determined in each section at end diastole and end systole. End-diastolic and end-systolic volumes by MRI in both groups correlated well with those by ventriculography (r>0.89). Comparison of the ejection fraction in both ventricles in both groups yielded a good correlation between MRI and ventriculography (r>0.67). MRI technique in both groups had low intraobserver and interobserver variation (<6%). Cine MRI provides a suitable noninvasive means of quantifying ventricular volume in children with complex congenital heart disease.

Child↗
PubMed1983

Pulmonary artery growth during treatment with oral prostaglandin E2 in ductus dependent cyanotic congenital heart disease.

Growth of the pulmonary arteries was assessed in 11 patients with cyanotic congenital heart disease treated with oral prostaglandin E2. Each patient was dependent on patency of the ductus arteriosus for maintenance of the pulmonary circulation. Measurements were made initially from angiographic data obtained in the neonatal period. Subsequent assessments were made at the time of surgery after an interval of 2-8 months, during which time all the infants had gained weight normally. Comparisons were made with data obtained from 13 normal necropsy specimens. In only 4 of the 11 patients had the pulmonary arteries grown appreciably during the treatment period. In 2 patients no growth occurred, while in 5 patients the pulmonary arteries had actually become smaller. It had been hoped that the pulmonary arteries would grow in all 11 patients during this period, facilitating later surgical intervention. In patients with ductus-dependent cyanotic congenital heart disease, the prolonged use of oral prostaglandin E2 should be restricted to patients in whom the pulmonary arteries are too small to allow a palliative operation to be performed initially.

Body Weight↗
PubMed2006

The coronary microcirculation in cyanotic congenital heart disease.

BACKGROUND: Despite an appreciable increase in basal coronary blood flow in cyanotic congenital heart disease, flow reserve remains normal. We hypothesized that preservation of flow reserve resides in remodeling of the coronary microcirculation. Microcirculatory morphometric analyses were performed to test this hypothesis. METHODS AND RESULTS: Necropsy specimens from 4 sources were studied: (1) hearts from patients with Eisenmenger's syndrome (A; n=5), (2) structurally abnormal hearts with ventricular hypertrophy (B; n=8), (3) structurally normal hearts with ventricular hypertrophy (C; n=6), and (4) normal hearts (D; n=5). To compare responses of the microcirculation to hypoxia versus hypertrophy, sections were taken from the left ventricular free wall, which in group A, was hypoxemic but not hypertrophied; in groups B and C, was hypertrophied but not hypoxemic; and in group D, was neither hypertrophied nor hypoxemic. Coronary arterioles were immunolabeled for smooth muscle alpha-actin. Measured morphometric parameters included long and short axes, area, and perimeter. Arteriolar length, volume and surface densities were calculated. There was a significant intergroup difference for arteriolar length density (P=0.03) and diameter (P=0.03). Total length density in group A hearts was markedly lower, but mean arteriolar diameter was significantly greater (34%) compared with group B (P=0.03). Arteriolar volume density was similar to that in the other groups. CONCLUSIONS: Remodeling of the coronary microcirculation is the key mechanism for preservation of flow reserve in cyanotic congenital heart disease. The increase in short axis (diameter) compensated for lower arteriolar length density and was the principal anatomic basis for maintenance of normal flow reserve.

Adult↗
PubMed1989

The association of congenital neuroblastoma and congenital heart disease. Is there a common embryologic basis?

Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific well-known associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma.

Cell Movement↗
PubMed2005

Transcatheter palliation of congenital heart disease with reduced pulmonary blood flow.

BACKGROUND: Although surgical shunt is still considered as the best palliation of congenital heart disease with reduced pulmonary blood flow, stent implantation may be technically simpler, safer and more cost-effective than surgery in high-risk patients. This study evaluated the feasibility and results of this option in patients with duct-dependent pulmonary blood flow or systemic-to-pulmonary shunt malfunction. METHODS: Between April 2003 and July 2004, 9 patients (age 11 days-52 years, weight 2.1-52 kg) with complex congenital heart disease underwent stent implantation inside the patent ductus arteriosus (4 patients) or a stenotic surgical shunt (5 patients). RESULTS: The stenting procedure was successfully completed in all cases. The procedural time was 162 +/- 36 min (range 90-225 min). The fluoroscopy time was 33.8 +/- 6.8 min. No patient died. The morbidity rate was 22.2% (1 patient had local infection at the site of puncture and 1 had transient femoral artery pulse loss). After the procedure, the ductus/shunt diameter increased from 1.2 +/- 0.6 to 3.6 +/- 0.6 mm (p < 0.0001) and oxygen saturation improved from 74.0 +/- 6.5 to 85.2 +/- 3.3% (p < 0.01). Three patients underwent corrective surgery without technical problems after 8.0 +/- 1.0 months while oxygen saturation remained constantly >80% in patients still waiting for surgical repair (follow-up 5.3 +/- 3.1 months). CONCLUSIONS: Stent implantation is a technically feasible, safe and effective palliative option in high-risk surgical patients with congenital heart disease and reduced pulmonary blood flow. Although larger series are required to define the cost-effective clinical impact of this therapeutic option, it is reasonable to hypothesize a further extension of its indication even to elective and low-risk surgical patients.

Adolescent↗