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PubMed1995

A comparison of transesophageal and transthoracic echocardiographic assessment of left ventricular function in pediatric patients with congenital heart disease.

OBJECTIVE: To determine the quantitative utility of transesophageal echocardiographic assessments of left ventricular function in pediatric patients with congenital heart disease by evaluating the variability between observers and between echocardiographic windows. DESIGN: Retrospective, blinded analysis. SETTING: University-associated pediatric hospital. PARTICIPANTS: Transthoracic and transesophageal echocardiographic images of 25 pediatric patients with congenital heart disease were reviewed. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: End-diastolic area, end-systolic area, and fractional area change were measured from short-axis images of the left ventricle at the midpapillary level by two separate investigators. These measurements were compared by the method of Bland and Altman and Sheiner and Beal. Significant differences in measurements of end-diastolic and end-systolic area by different observers were noted, but they were systematic. A similar situation was noted for the comparison of transthoracic and transesophageal measurements of end-diastolic and end-systolic area. In the comparison of fractional area change between observers or windows, bias and absolute prediction error were lower, with 95% confidence limits of bias or absolute prediction error of 10% or less. CONCLUSIONS: The potential error in the measurement of fractional area change in 10% under optimal conditions. This would suggest that the assessment of ventricular function in the operating room or intensive care unit, under less than optimal conditions, should be viewed as a qualitative, rather quantitative, measurement. There may be significant interobserver and interwindow variability.

Bias↗
PubMed2002

[Surgical treatment of congenital heart disease complicated with infective endocarditis].

OBJECTIVE: To review the experience of surgical treatment and the diagnosis of 41 patients with congenital heart diseases complicated with infective endocarditis. METHODS: Twenty-three males and 18 females (the mean age 22.6 years) were studied. Twenty-seven patients received bacterial cultivation of blood specimens, and the results of bacterial cultivation of 11 patients were positive. Thirty-four patients underwent cardiac operations after infection was almost controlled, and 7 patients underwent operations during the infective phase. In all the cases, the infected tissues were excised, congenital cardiac was treated, and valvular dysfunctions were corrected. RESULTS: The surgical treatment results were satisfactory. One patient (2.4%) died during hospitalization. CONCLUSION: In our hospital, the incidence rate of infective endocarditis complicated with congenital heart diseases increases; echocardiography is a valuable tool in diagnosis; blood culture may be negative after previous administration of antibiotics; aggressive surgical treatment should be adopted in patients with congenital heart defects complicated with infective endocarditis.

Adolescent↗
PubMed1999

Outpatient clinics for adults with congenital heart disease: increasing workload and evolving patterns of referral.

OBJECTIVE: To examine the evolving role of specialised outpatient services for adult patients with congenital heart disease. DESIGN: A retrospective analysis of all patients attending the Toronto Congenital Cardiac Centre for Adults over three corresponding three month periods in 1987, 1992, and 1997. SETTING: A tertiary referral centre. MAIN OUTCOME MEASURES: Patient demographics, residence, medical and surgical history, type and source of referral, and investigations performed. RESULTS: In all, 570 patients were seen at the clinic during these three periods. There was a 44% and a 269% increase in workload between 1987 to 1992 and 1992 to 1997, respectively. There was a steady fall in mean age of patients seen at the clinic with time (38.5, 33.6, and 31.7 years in 1987, 1992, and 1997, respectively, p < 0.001). New referrals from community cardiologists and family physicians increased more in relative terms than did referrals from the Hospital for Sick Children, Toronto (6.7%, 15%, and 37.5%, p = 0.02). There was a steady increase in patients with previous reparative surgery (48.9%, 59.2%, and 69.2%, p < 0.002). The proportion of patients with previous reoperations also increased (2.3%, 10%, and 9.2%, p < 0.01). Echocardiography remained the predominant method of diagnosis. The diagnostic mix did not change with time. CONCLUSIONS: Over the past 10 years there has been a large increase in adults with congenital heart disease requiring and seeking specialised care in a tertiary health centre, with a concomitant evolution of referral patterns. These data may be helpful in planning of similar paediatric and adult cardiac services for this expanding population.

Adult↗
PubMed2006

Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis.

OBJECTIVE: Findings in the literature are inconsistent on the impact of congenital heart disease (CHD) on the psychological and cognitive functioning of children and adolescents. The aim of the present study was to systematically review this empirical body of literature. METHODS: We conducted a meta-analysis to review studies on behavior problems and cognitive functioning in CHD. RESULTS: Only older children and adolescents with CHD displayed an increased risk of overall, internalizing, and to a lesser extent externalizing behavior problems. In addition, patients with severe CHD exhibited lower cognitive functioning than patients with less severe CHD, specifically with respect to performance intelligence. Moreover, decreased cognitive functioning remained relatively stable across different age groups. CONCLUSIONS: Children with severe heart disease may benefit from interventions specifically targeting perceptual organizational abilities, such as visual-spatial abilities. Moreover, older children and adolescents with CHD may benefit from psychological interventions reducing anxiety symptoms and depression.

Adolescent↗
PubMed1987

Misdiagnosis of omphalocele associated with Edwards syndrome and congenital heart disease.

We present a case in which an apparent omphalocele, diagnosed at 30 weeks gestation by ultrasound, led to identification of fetal trisomy 18 and congenital heart disease. At delivery, the fetus had the features of trisomy 18 and congenital heart disease but the omphalocele was absent. We suggest that the appearances seen are easily confused with a small omphalocele and could potentially result in unnecessary further investigations being performed.

Adult↗
PubMed1978

[An epidemiological study of congenital heart disease. I. Incidence of cardiovascular malformations in 11,584 live-born babies in the District of Florence (Italy) (author's transl)].

11,584 consecutive live-born babies in the Ostetric Clinic of Florence in the years 1975 and 1976 were examined for the presence of congenital heart disease. Diagnosis was made by necropsy, cardiac catheterization and operation in 20% of cases and by clinical observations in 80%. The 74% of babies who were diagnosed as affected by congenital heart disease at birth were eventually controlled. The incidence of congenital heart disease was 7.1% in 10,789 live-born of weight over 2500 g while it was 37% in 795 under such weight. Global incidence was 9.2%. The first essentials in epidemiological study of congenital heart malformations is to define the method which is used for diagnosis since this is the primary factor responsible for wide variations in incidence. It is suggested that the institution of a Regional Register of malformations would allow more accurate estimate of overall incidence of congenital cardiopathies.

Abnormalities, Multiple↗
PubMed1998

Catheterization of the pulmonary artery using a 3 French catheter in patients with congenital heart disease.

A 3 French (3F) coaxial polyurethane catheter was utilized for catheterization of the pulmonary artery in patients with complex congenital heart disease with concomitant stenosis or atresia of the pulmonary valve, with or without Blalock-Taussig shunts. Mean aortic and atrial pressures measured with the 3F catheter were compared with those measured with 5-6F conventional catheters. The values measured with the 3F catheter were identical to those measured with 5-6F catheters. Mean pulmonary arterial pressure measured using the 3F catheter was significantly higher than that measured using the 5-6F catheters, but it was not significantly different from mean pulmonary venous wedge pressure. Of 43 patients, 5-6F conventional catheters could be inserted into the branch pulmonary artery in 22 patients, but the 3F catheter could be inserted into the pulmonary artery in all patients. These data suggest that the 3F catheter is useful for catheterization in patients with complex congenital heart disease associated with pulmonary atresia or severe pulmonary stenosis.

Adolescent↗
PubMedDate not supplied

[Changes in oxygenation indexes with the use of nitric oxide in the postoperative period of congenital heart disease surgery in patients with severe pulmonary hypertension].

UNLABELLED: In this clinical study, 7 pediatric patients with severe pulmonary artery hypertension, secondary to congenital heart disease, received inhaled nitric oxide at doses of 20 ppm in the acute postoperative management of congenital heart repair for 3.5 days. Monitoring included oxygenation index, alveoloarterial oxygen difference, pulmonary and systemic pressure, measurements were recorded previous to the NO administration; 1, 6, 12, 24, 36, 48 and 72 hours after treatment with NO, nitrous oxide (NO2) and seric methemoglobin also were monitored. RESULTS: Average age was 3.6 years. The oxygenation index before inhaling NO was 166 +/- 100, 72 hours following inhalation of NO the oxygenation index was 210 +/- 98. The alveolo-arterial baseline oxygen difference was 270 +/- 145; 72 hours after inhaling NO, it was 163 +/- 167. No decreases in pulmonary and systemic arterial pressure during NO inhalation occured. The levels of NO2 and seric methemoglobin were not toxic. CONCLUSIONS: Treatment with inhaled NO after cardiac surgery in children with severe pulmonary artery hypertension secondary to congenital heart disease may be useful to improve oxygenation with no changes in pulmonary and systemic arterial pressure. Inhaled NO at 20 ppm did not cause toxicity.

Adolescent↗
PubMed1987

Relationship between plasma atrial natriuretic polypeptide concentration and hemodynamic measurements in children with congenital heart diseases.

The plasma alpha-atrial natriuretic polypeptide (alpha-ANP) concentration in the peripheral veins of children with congenital heart diseases was measured by radioimmunoassay and compared with cardiac catheterization data. Every patient with heart failure had a higher alpha-ANP concentration (132.1 to 858.7 pg/mL) than the upper limit of the normal range (11.7 to 98.7 pg/mL), whereas more than half of the patients without heart failure had a normal alpha-ANP concentration. Although none of the 13 children with atrial septal defect had heart failure, their mean (+/- SD) plasma alpha-ANP concentration (99.4 +/- 40.7 pg/mL) was significantly higher than that in control children (44.6 +/- 22.3 pg/mL). The plasma alpha-ANP concentration was significantly correlated with the pulmonary blood flow/systemic blood flow (Qp/Qs) ratio in the children with atrial septal defect. In the 34 children with ventricular septal defect the plasma alpha-ANP concentration increased in relation to increasing size of the defect. The plasma alpha-ANP concentration was significantly correlated with the Qp/Qs ratio, pulmonary artery pressure, and left atrial pressure, estimated from mean pulmonary artery wedge pressure, in the children with ventricular septal defect. In the children with tetralogy of Fallot, the mean plasma alpha-ANP concentration was normal, and mean right and left atrial pressures were not increased. The elevated alpha-ANP concentration in the three patients with heart failure decreased after their conditions improved with various treatments. Thus the measurement of alpha-ANP concentration may be valuable in evaluating the presence or absence of heart failure and the response to treatment in children with congenital heart diseases. Distention of the right and left atrial might induce the release of alpha-ANP in patients with atrial and ventricular septal defects, respectively.

Atrial Natriuretic Factor↗
PubMed2006

[The Dutch Child Health Care guideline 'early detection of congenital heart disease in the age group 0-19 years'].

The Dutch Child Health Care guideline on the early detection of congenital heart disease was developed according to the principles for evidence-based guideline development and contains recommendations for the conduct of the examination during routine check-ups and for referral criteria. Most congenital heart diseases can be detected in the first year of life if physicians and nurses work according to this guideline. Important early times for screening are the home visit to infants at the age of two weeks by the child health nurse and the routine check-up of infants at the age of four weeks by the child health care physician. A routine cardiac screening is carried out in every child by the nurse and the physician at times specified in the basic list of professional responsibilities. A more extensive examination is carried out when indicated. Routine cardiac check-ups are recommended until the age of four, after which they should be done only when indicated on medical grounds. A feasibility study showed that physicians and nurses can apply the guideline effectively, provided that they take part in prior specific training.

Adolescent↗
PubMed1993

Plasma levels of atrial natriuretic factor (ANF) and urinary excretion of ANF, arginine vasopressin and catecholamines in children with congenital heart disease: effect of cardiac surgery.

We studied the changes in the plasma concentration of atrial natriuretic factor (ANF) and the urinary excretion of ANF, arginine vasopressin (AVP) and catecholamines in 22 children with congenital heart disease, divided into two groups. Group 1 included 11 children with congestive heart failure (CHF), treated with digitalis and diuretics. Group 2 included 11 children without CHF and without medical treatment. Each group was compared with a control group of 15 healthy, age-matched children. The plasma concentration of ANF was raised in both groups, but it was significantly higher in group 1 (235.5 +/- 82.9 pg/ml), compared to group 2 (48.4 +/- 29.4 pg/ml, P < 0.002). Urinary excretion of ANF was measurable in both groups and higher in group 1 (185.9 +/- 116.2 pg/kg per h) than in group 2 (48.5 +/- 30.7 pg/kg per h), but not significantly so. Urinary excretion of AVP and catecholamines was not different in children with congenital heart disease and healthy children. Twenty-four hours after surgery, plasma ANF diminished in group 1 (from 235.5 +/- 82.9 to 93.4 +/- 53.8 pg/ml, P < 0.003), but did not change in group 2. The urinary excretion of ANF was unchanged in both groups. In contrast, urinary excretion of AVP and catecholamines rose significantly in both groups. These data show that plasma ANF is increased in children with congenital heart disease, even in the absence of CHF. The measurement of urinary ANF is less reliable than a plasma assay. The postoperative increases in AVP and catecholamine urinary excretions could be responsible for the vasoconstriction and water retention typical of the postoperative period.

Adolescent↗
PubMed1998

Prostaglandin E1 in infants with congenital heart disease: Indian experience.

BACKGROUND: E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore and be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decade in western world, it has been introduced in India only since April, 1995. OBJECTIVE: To assess the efficacy of PGE1 at our center. SETTING: Hospital based. METHOD: 65 infants with ductus dependent congenital heart disease were included. Age at time of starting PGE1 infusion ranged from 18 hours to 39 days. Forty two of these were more than a week of age, 19 were more than 14 days, and two were above one month. PGE1 was started in an initial dose of 0.05 microgram/kg/min, decreased to 0.005-0.01 microgram/kg/min for maintenance. The indications for use of PGE1 were to increase pulmonary blood flow in 33 cases with pulmonary atresia, tricuspid atresia or critical pulmonic stenosis (Group I); to increase systemic blood flow in 15 cases with coarctation of aorta, hypoplastic left heart and interruption of aortic arch (Group II); to improve mixing in 13 cases of transposition of great arteries (Group III) and for improving the left ventricular volumes by keeping the duct open in 4 cases of transposition of great arteries with intact ventricular septum (Group IV). The efficacy of the drug was assessed by a rise on PaO2 and SaO2% determined for Group I & III, and by appearance of lower limbs pulses in Group II. Left ventricular volumes were serially measured by echocardiography in Group IV cases. RESULTS: The drug was successful in 62 of the 65 cases. There were two failures. One was a 39 days old baby with Ebstein's anomaly of tricuspid valve and pulmonary atresia and other was an eight days old baby with coarctation of aorta and renal failure. In addition, PGE1 could not be continued in another baby due to development of a linear skin rash locally. Side effects included apnea in 5 (9%) of 56 spontaneously breathing patients. Necrotizing enterocolitis, hyperpyrexia and jitteriness was sent in one case each. Six patients died. Two were related to PGE1, one due to failure, another due to its side effects. Definitive procedure were performed in 51 cases electively. PGE1 was used upto 13 days with sustained benefit. CONCLUSIONS: PGE1 is an effective drug for keeping the ductus open in infants with ductus dependent congenital heart disease. It can be used for neonates beyond the first week of life with efficacy. Apnea is a major side effect and close monitoring is essential.

Age Factors↗
PubMed2005

[Study on the effect of adrenomedulin and urotensin-II on pulmonary hypertension of patients with congenital heart disease].

OBJECTIVE: To evaluate the effect and clinical significance of adrenomedulin (ADM) and urotensin-II (UII) on pulmonary hypertension (PH), by detecting their levels of patients with congenital heart disease and pulmonary hypertension. METHODS: 52 patients with congenital heart disease who had left-to-right shunt were selected randomly. 52 patients were divided three groups according to pulmonary artery systolic pressure (PASP): group A was not pulmonary hypertension (PASP < 30 mm Hg, n = 17); group B was mild pulmonary hypertension (PASP30-49 mm Hg, n = 18); group C was moderate and severe pulmonary hypertension (PASP > or = 50 mm Hg, n = 17). The plasma levels of ADM and UII were detected at each period by radioimmunoassay (RIA) method. It was analyzed the changes of their levels within pre-operation, 20 mins and 7 days post-operation and the interrelation between them and PASP. RESULTS: (1) Following the severity degree of pulmonary hypertension, the plasma levels of ADM increase. There is positive correlation between PAP and plasma level of ADM (pre-operation r = 0.8012, P < 0.01; 20 min post-operation r = 0.6325, P < 0.01; 7 d post-operation r = 0.7126, P < 0.01). (2) Following the severity degree of pulmonary hypertension, the plasma levels of UII don't change obviously. There is no correlation between PAP and plasma level of UII (P > 0.05). (3) The plasma levels of ADM: group A (pre-operation: 33 pg/ml +/- 5 pg/ml, 20 mins post-operation: 29 pg/ml +/- 4 pg/ml, 7 d post-operation: 20 pg/ml +/- 3 pg/ml); group B (pre-operation: 44 pg/ml +/- 8 pg/ml, 20 mins post-operation: 40 pg/ml +/- 6 pg/ml, 7 d post-operation: 34 pg/ml +/- 4 pg/ml); group C (pre-operation: 60 pg/ml +/- 10 pg/ml, 20 mins post-operation: 58 pg/ml +/- 8 pg/ml, 7d post-operation: 38 pg/ml +/- 4 pg/ml). Plasma level of ADM of each group after CPB is lower than that of each group before operation. It is statistical difference only 7 days post-operation (group A q = 5.41, P < 0.01; group B q = 4.76, P < 0.01; group C q = 6.32, P < 0.01). (4) The plasma levels of UII: group A (pre-operation: 2.2 pmol/L +/- 0.5 pmol/L, 20 mins post-operation: 2.2 pmol/L +/- 0.44 pmol/L, 7 d post-operation: 2.2 pmol/L +/- 0.6 pmol/L); group B (pre-operation: 2.7 pmol/L +/- 0.6 pmol/L, 20 mins post-operation: 2.6 pmol/L +/- 0.6 pmol/L, 7 d post-operation: 2.6 pmol/L +/- 0.5 pmol/L); group C (pre-operation: 2.9 pmol/L +/- 0.6 pmol/L, 20 mins post-operation: 2.6 pmol/L +/- 0.7 pmol/L, 7 d post-operation: 2.8 pmol/L +/- 0.4 pmol/L). Compared with that of each group before operation, Plasma level of UII of each group after operation is no obvious difference (P > 0.05). CONCLUSION: (1) Following the severity degree of pulmonary hypertension, the plasma levels of ADM increase. ADM plays an important role in the formation of pulmonary hypertension and restructure. (2) Following the severity degree of pulmonary hypertension, the plasma levels of UII don't change obviously. There is no correlation between PAP and plasma level of UII, but UII may be play an important role in the formation of pulmonary hypertension and restructure. (3) Measuring the levels of ADM may be a reliable method to follow the change of pulmonary pressure and worsening of pulmonary hypertension.

Adolescent↗
PubMedDate not supplied

Intraoperative transesophageal versus epicardial ultrasound in surgery for congenital heart disease.

Twenty-eight patients (age range, 0.7 to 65 years; median age, 6.1 years) who were undergoing correction for congenital heart disease were entered into a prospective study with both intraoperative transesophageal and epicardial ultrasound to determine the relative values of these techniques before and after bypass surgery. Introduction of the transesophageal probe was successful in 26 patients (93%); children were studied with use of dedicated pediatric transducers. Epicardial studies were performed in all 28 patients. Epicardial studies allowed for higher resolution imaging and a more complete assessment before bypass surgery of the intracardiac morphological condition (ventricular septum and right ventricular outflow tract) than the assessment that was obtained by the transesophageal approach. In the period immediately after bypass surgery, the transesophageal technique allowed a more detailed insight into atrioventricular valve function (valvar regurgitation [five patients] and ventricular inflow patterns) and the continuous monitoring of left ventricular function and volume. Residual interventricular shunting (three patients) or residual outflow tract obstruction (four patients) could not be reliably documented by transesophageal studies. It is concluded that intraoperative transesophageal and epicardial ultrasound in surgery for congenital heart disease are complementary rather than alternative techniques.

Adolescent↗
PubMed2005

ECMO and congenital heart disease.

Although initially designed for respiratory failure, venoarterial extracorporeal membrane oxygenation (VA ECMO) has become a mainstay of therapy in the treatment of patients with congenital heart disease, providing preoperative and postoperative support for infants with temporary impairment of myocardial function. Postoperative support for pulmonary hypertension has allowed dramatic improvements in lesions such as total anomalous pulmonary venous connection. VA ECMO has also proven to be an acceptable bridge to cardiac transplantation in patients too small for currently available ventricular assist devices. Intraoperative elective use of ECMO has also provided a surgical environment with some specific advantages over conventional cardiopulmonary bypass for lesions such as long segment tracheal stenosis. The results of "rescue ECMO" or extracorporeal cardiopulmonary resuscitation (ECPR) has shown that many children with congenital heart disease are very salvageable despite sudden decompensation and arrest. These techniques of VA ECMO have evolved and improved over the last two decades, allowing expanded application of this life saving support.

Cardiac Surgical Procedures↗
PubMed2004

Initial experience with cardiac resynchronization therapy for ventricular dysfunction in young patients with surgically operated congenital heart disease.

Data regarding cardiac resynchronization therapy (CRT) in pediatric patients are limited. The first reported use of CRT in a young patient with congenital heart disease showed promising early results with improvement in New York Heart Association classification, aerobic capacity, and ventricular contractility 1 month after implant. Other reports have shown acute benefits of CRT in young patients with postoperative right bundle branch block after surgery for congenital heart defects. To date, however, there are no published data regarding CRT for the long-term management of systemic ventricular dysfunction in the young with associated congenital heart disease. This report describes our initial experience with CRT in these patients.

Adolescent↗
PubMed1989

The value of the sinusoidal tcPO2 pattern in the evaluation and management of cyanotic congenital heart disease with ductus dependent lesions.

A characteristic sinusoidal tcPO2 pattern was observed in 17(27%) of 62 patients with congenital heart disease manifested by cyanosis or heart failure during the neonatal period. All of these 17 patients were definitively diagnosed by cardiac catheterization, as having 8 PPA; 3 ToF; 2 TGA + VSD + PA; 2 asplenia with PA; 1 TGA + VSD + PS; and 1 TA (no TGA type). The transcutaneous oxygen pressure pattern of these patients showed a sinusoidal change with a cycle length of 6-20 min with the maximum pressure not exceeding 50 torr and the minimum pressure occasionally close to 0 torr. This sinusoidal tcPO2 pattern was associated with various types of ductus-dependent congenital heart diseases and hence may be of diagnostic value. Transcutaneous oxygen pressure monitoring is also useful in evaluating the ductal response to PG. Other possible mechanisms underlying this phenomenon including biological oscillation should be considered.

Alprostadil↗