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Early initiation of peritoneal dialysis after surgical repair of congenital heart disease.

The mortality rate of infants who require renal replacement therapy after surgical repair of congenital heart disease has been reported to be 30%-79%. We report our experience with early initiation of continuous manual peritoneal dialysis (CPD) to treat fluid overload in 20 consecutive critically ill children who underwent CPD post cardiotomy. CPD catheters were inserted at the discretion of the cardiothoracic surgeon. CPD was started for evidence of total body fluid overload with inadequate urine output, and stopped when negative fluid balance was achieved and urine output improved. Median age was 10 days (range 3-186 days), mean time to start CPD post-operatively was 22 h (range 5-40 h), and mean duration of CPD was 50 h (range 13-92 h). CPD resulted in mean ultrafiltration of 93 ml/kg per day (range 43-233 ml/kg per day). Net negative fluid balance was 106 ml/kg per day (range 49-273 ml/kg per day). During CPD, the mean number of inotropes decreased from 2.2 to 1.6 (P<0.05) and urine output increased from 2.2 to 3.9 ml/kg per hour (P<0.01). No patient died during CPD or had CPD discontinued due to adverse hemodynamic effects. The overall mortality rate was 20%. We conclude that early initiation of CPD can safely and effectively promote fluid removal in infants after repair of congenital heart disease, with a lower mortality rate than has previously been reported.

Adolescent↗

Pulmonary blood pressure, not flow, is associated with net endothelin-1 production in the lungs of patients with congenital heart disease and normal pulmonary vascular resistance.

OBJECTIVE: Endothelin-1 concentrations are increased in patients with increased mean pulmonary arterial pressure, pulmonary blood flow, and pulmonary vascular resistance. However, endothelin-1 concentrations have not been well characterized in patients with congenital heart disease and normal pulmonary vascular resistance. In particular, it is unclear whether pressure or flow is the key regulator of endothelin- 1 in this setting. We tested the hypothesis that pulmonary blood pressure and not flow is associated with net endothelin-1 production in patients with congenital heart disease and normal pulmonary vascular resistance. METHODS: With a commercially available immunoassay, we measured endothelin-1 concentrations in pulmonary arterial and pulmonary venous plasma of 56 consecutive patients with congenital heart disease and pulmonary vascular resistance less than 2 U. m(2) undergoing cardiac catheterization. We used multiple linear regression to analyze the effect of demographic and hemodynamic variables on pulmonary arterial and venous endothelin-1 concentrations and on the change of endothelin-1 concentration over the pulmonary vascular bed. RESULTS: Multiple linear regression revealed that of all the hemodynamic variables tested, mean pulmonary arterial pressure had the greatest effect on increasing the change of endothelin-1 concentration over the pulmonary vascular bed (P <.0001). Pulmonary blood flow did not have any effect on endothelin-1 concentrations or on the change of endothelin-1 concentration over the pulmonary vascular bed. CONCLUSIONS: This study shows that pulmonary blood pressure and not flow is associated with net endothelin-1 production in patients with congenital heart disease and normal pulmonary vascular resistance.

Blood Pressure↗

Fetal echocardiographic screening of diabetic pregnancies for congenital heart disease.

This study sought to assess pregnant diabetic women for the presence of fetal cardiac anomalies and to determine whether better diabetic control was associated with a reduced risk to the fetus. Between 1988 and 1995, pregnant type I and II diabetic women routinely underwent fetal echocardiography. Hemoglobin A1c values were used as an indicator of maternal diabetic control and any relation between congenital heart disease in the fetus and maternal hemoglobin A1c levels was sought. Cardiac defects were identified in 7 of 328 pregnancies assessed, for an incidence of congenital heart disease of 2.1% (95% confidence interval: 0.6-3.6%). A review of the postnatal cardiac database did not reveal any undetected major malformations. The mean hemoglobin A1c level was 7.6% +/- 2.0% obtained at a mean gestational age of 12 +/- 7 weeks. Hemoglobin A1c levels of mothers carrying a fetus with congenital heart disease did not significantly differ from those with a normal fetus: 8.1% +/- 3.4% versus 7.6% +/- 1.9% (p = 0.48). Mothers with an affected fetus demonstrated a wide range of HbA1c levels (4.1 to 13.7%). Thus, the incidence of significant fetal cardiac abnormalities is low and not significantly related to maternal diabetic control.

Adult↗

Oxygen saturations during breast and bottle feedings in infants with congenital heart disease.

Bottle feeding is commonly advised for infants with congenital heart disease (CHD) based on the belief that breast feeding is too difficult for them. However, studies of preterm infants have shown that greater cardiorespiratory effort occurs during bottle feeding than during breast feeding. The purpose of this study is to determine if there is a relationship between feeding method (breast vs. bottle) and oxygen saturations (SaO2) in infants with CHD. In a correlational design, pulse oximetry measured SaO2 during one breast and one bottle feeding in each of 7 infants with CHD. SaO2 during breast feeding is significantly different from SaO2 during bottle feeding (F = 59.72, p < .0001). SaO2 during breast feeding is higher on average and less variable (M = 96.3%, SD = 2.2) than SaO2 during bottle feedings (M = 92.5%, SD = 6.9), indicating that there is less cardiorespiratory stress with breast feeding. None of the infants desaturated (SaO2 < 90%) during breast feedings, whereas four infants desaturated during bottle feedings.

Bottle Feeding↗

Respiratory syncytial virus in patients with congenital heart disease: a contemporary look at epidemiology and success of preoperative screening.

Awareness of respiratory syncytial virus (RSV) as a serious pathogen in the child with congenital heart disease is increasing. We studied the impact of RSV lower respiratory tract disease on patients in a large academic pediatric cardiology practice. We found that RSV disease necessitating hospitalization occurs in congenital heart disease patients well into the second year of life. Although pulmonary hypertension remains a significant risk factor for morbidity in these patients, it does not appear to be as much of a factor as in the past. By implementing a nasopharyngeal RSV enzyme-linked immunoassay screening of young patients prior to cardiac surgery we found a reduction in community-acquired postoperative RSV disease. We postulate this will lead to a reduction in nosocomial disease in the postoperative care unit.

Analysis of Variance↗

Energy metabolism in infants with congenital heart disease.

Failure to thrive is common in children with congenital heart disease and influences the metabolic response to injury and outcome after corrective cardiac surgery. Energy imbalance is a major contributing factor. However, the published literature is difficult to interpret as studies generally involve small patient numbers with a diverse range of types and severity of cardiac lesions and genetic and/or prenatal factors. The age and time of corrective surgery affects the potential for nutritional recovery. Although the immediate postoperative period is characterized by a hypermetabolic state, low total and resting energy expenditure are reported within 24 h of surgery. After 5 d, resting energy expenditure returns to preoperative levels. Significant improvements in weight and growth occur within months after corrective surgery. However, limited postoperative recovery in nutritional status and growth occurs in infants with a low birth weight, intellectual deficit, or residual malformation. Further studies are needed to inform the timing of corrective cardiac surgery to maximize nutritional outcomes and to identify those infants who may benefit from aggressive preoperative nutrition support.

Child↗

Adults with cyanotic congenital heart disease: hematologic management.

Hematologic management of adults with cyanotic congenital heart disease has received little recent attention. The lack of practical therapeutic guidelines prompted us to consolidate our observations on 124 cyanotic adults for general physicians, cardiologists, and hematologists who care for these patients. Specific attention focused on regulation of erythrocyte mass and concepts of compensated and decompensated erythrocytosis, symptoms of deficient tissue oxygen transport, hyperviscosity and iron deficiency, the potential relation between elevated hematocrit levels and brain injury, hemostasis, urate metabolism, and renal function. Cerebral infarction was not seen in any patient. Phlebotomy is best reserved for treatment of symptomatic hyperviscosity. Iron therapy is indicated for symptomatic iron deficient erythropoiesis. Abnormal hemostatic mechanisms are the rule. Antithrombotic medications have little or no role in treatment. Hyperuricemia is the result of abnormal renal uric acid excretion not urate overproduction, and serves as a marker of abnormal renal function. Drugs that promote urate excretion are the preferred maintenance treatment in symptomatic hyperuricemic patients.

Adolescent↗

What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly?

Adults with congenital heart disease (CHD) represent a growing population of patients thanks to the medical and surgical advances which enable at least 85% of children to survive to adulthood. These advances may create quality-of-life (QoL) issues not previously considered. The purpose of this cross-sectional study of 124 adults with CHD was to describe their QoL as a basis for providing appropriate information, counseling, and anticipatory guidance. Thirteen patients had single ventricle physiology (SVP), 43 had cyanotic lesions with 2 ventricle repairs, and 68 had acyanotic CHD. On the basis of Sickness Impact Profile (SIP) scores, individuals with SVP had worse QoL than did those with cyanotic lesions (with 2 ventricle repairs) and acyanotic anomalies (SIP = 9.98 vs 4.61 and 3.76). SIP scores were statistically significantly different between those with SVP and those with acyanotic anomalies (P = .02). For all groups, the areas of life most affected were work and sleep and rest. Participants with SVP saw themselves as having the poorest QoL.

Activities of Daily Living↗

What are the responses & needs of mothers of infants diagnosed with congenital heart disease?

This article addresses the needs of families of newborns with congenital heart disease (CHD). Approximately 40,000 infants are born with a congenital heart defect each year; a large percentage survive due to technological advances in treatment, resulting in an increasing number of families who have a child living with a chronic illness. While both parents are significantly affected by a diagnosis of CHD, much of the available literature discusses the experience of mothers. The mother's experiences after learning her child's diagnosis include grief, loss of her imagined healthy child, lack of knowledge of the disease, anger, and difficulty in caregiving, among other issues. Support and encouragement at the time of diagnosis and throughout each stage of the illness are essential. This article describes various strategies that nurses can use to assist these families through their difficult journey.

Adaptation, Psychological↗

Three-dimensional, isotropic MRI: a unified approach to quantification and visualization in congenital heart disease.

PURPOSE: Current standards in magnetic resonance imaging of congenital heart disease are based mostly on anisotropic protocols to image both morphology and function. Operator-dependent acquisition planning is typically needed to obtain the desired images. We propose to instead use operator-independent, three-dimensional, isotropic imaging protocols to acquire both morphology and function (cine and flow) of the entire heart in a few standardized acquisitions. Subsequently, due to the isotropic property of the data, any desired imaging plane can be "imaged" offline by interactive planar reformatting and used for qualitative and quantitative diagnostic evaluation. MATERIALS AND METHODS: Morphological data was acquired in patients using 3D steady state free precession (SSFP) protocols, and functional data in volunteers using multislice 2D or 3D cine SSFP as well as 3D, three-component phase-contrast velocity mapping with EPI readouts. Tools to integrate morphological and functional offline image evaluation based on interactive planar reformatting, volume rendering, and corresponding quantification tools were implemented and discussed. RESULTS: We successfully acquired and integrated morphology and flow and demonstrated potential clinical applications. CONCLUSION: User independent acquisitions of morphological and functional isotropic 3D datasets with real-time, interactive planar reformatting, volume rendering, and integration of morphology and function, have the potential to replace conventional, user dependent, anisotropic 2D imaging in patients with cardiac malformations.

Adolescent↗

Case histories of neonates with congenital heart disease.

The rate of extracardiac malformation (ECM) associated with congenital heart disease (CHD) is high in neonates. 108 cases of 212 neonates with CHD had ECM (50.9%). Main ECMs were digestive system anomalies (36.1%), chromosomal aberrations (26.8%), respiratory system anomalies (21.3%), CNS anomalies (13.0%), and other (2.7%). Single lesion of left to right shunt accounted for 77.2% (61/79) of CHD with ECM from other than chromosomal aberrations. The prognosis of neonates with CHD without ECM was also studied. Total anomalous pulmonary venous connection, pure pulmonary atresia (PPA), pulmonary stenosis (PS), hypoplastic left heart syndrome are not generally associated with ECM, but the prognosis is poor. Coarctation complex which is sometimes associated with ECM has a poor prognosis in neonates. There is an increase of the survival rate in the patients with hypoxemia, such as PPA or severe PS, extreme tetralogy of Fallot, and tricuspid atresia, which can be managed with prostaglandin E1. PDA associated with respiratory distress syndrome is ideally treated with indomethacin. In recent years, mortality from PDA has decreased in neonates. The mortality rate during the neonatal period was 46.8% (51/109): 37.5% (30/80) died before surgical interventions and 72.4% (21/29) died during or after surgery. Half of neonatal deaths from CHD occurred within 3 days of admission. Thus, early detection, early diagnosis, and early treatment of neonates with CHD is most important.

Abnormalities, Multiple↗

Noninvasive diagnostics in congenital heart disease: echocardiography and magnetic resonance imaging.

The ability to noninvasively diagnose even the most complex congenital heart disease is one of the greatest advances in the care of children with cardiac defects. Initially, two-dimensional echocardiography displayed anatomy; later, Doppler allowed the quantitation of pressure and flow. Using these modalities, cardiac catheterization is unnecessary for most children with cardiac defects. Echocardiography also is routinely used for intraoperative and fetal cardiac imaging. Three-dimensional magnetic resonance imaging can now quantitate volumes of irregular shaped heart chambers (eg, the right ventricle) and blood flow (eg, as from a leaking valve). As technology moves further into the digital age, developments will continue to facilitate noninvasive diagnosis and treatment of children with congenital heart disease.

Blood Flow Velocity↗

Prediction of behavioural and emotional problems in children and adolescents with operated congenital heart disease.

AIMS: The aim of the present study was to determine which medical variables were predictors of long-term behavioural/emotional outcome after surgical correction for congenital heart disease in infancy and childhood. METHODS: The Child Behavior Checklist (CBCL) was used to predict parent-reported behavioural/emotional problems in 125 10-15 year-old congenital heart disease children from: (1) biographical status (2) medical history (3) heart surgery (4) short-term post-operative course and (5) number of heart operations and (6) extra cardiac concomitant anomalies. RESULTS: Higher CBCL total problem scores at follow-up were associated with a greater number of heart operations and deep hypothermic circulatory arrest (< 22 degrees). 'Internalizing problems' were associated with a greater number of heart operations, deep hypothermic circulatory arrest, a short gestational age, low systemic oxygen saturation, and older age at surgical repair. 'Externalizing problems' were associated with a greater number of heart operations only. CONCLUSION: Several medical variables were significant predictors and can be used to identify those congenital heart disease children who are at risk of long-term behavioural/emotional maladjustment.

Adolescent↗

[Epidemiology of congenital heart diseases in Croatia--a multicenter nationwide study, 1995-2000].

Congenital heart diseases (CHD) comprise a group of different conditions seen in 0.55-1% of live births. Tendency to classify CHD according to Clark's pathogenic classification is based on the assumption that relatively few pathogenic mechanisms cause a wide spectrum of phenotypic forms of CHD. The aim of this study was to classify CHD according to Clark's classification and to calculate the prevalence rates of CHD in the Croatian population. We formed the Registry including all children with CHD born between 1995 and 2000 treated in paediatric cardiology departments in Croatia. In the study were enrolled 276,565 live births, 2,204 of them with CHD, 1.126 males and 1.078 females. Total birth prevalence was 8.0@1000. A statistically significant difference was found between CHD prevalence rates in several counties. The prevalence of all main pathogenic groups of CHD was constant in time, except for cell death defects where a twofold increase in the prevalence of muscular ventricular septal defect (VSD) was noted (0.6@1000-1.3@1000). The most common diagnosis was perimembranous VSD (19.0%), followed by atrial septal defect (ASD) type II (14.4%), muscular VSD (11.1%) and pulmonary stenosis (8.5%). This four diagnoses were composing more than 50% of all CHD diagnoses. Prevalence and diagnostic possibilities weren't different from those presented in the literature. However, differences in prevalence between the counties warrant further investigation into the development and aetiology of CHD.

Croatia↗

Measured versus estimated oxygen consumption in ventilated patients with congenital heart disease: the validity of predictive equations.

OBJECTIVE: To determine the validity of predictive equations in calculating oxygen consumption (Vo(2)) in ventilated patients with congenital heart disease. DESIGN: Prospective study. SETTING: Cardiac catheterization laboratories and intensive care units of two university teaching hospitals. PATIENTS: A total of 126 patients with congenital heart disease were studied. Of these, 75 patients received anesthesia in the pediatric cardiac catheterization laboratory, and 51 were deeply sedated in the intensive care unit after open heart surgery. MEASUREMENTS AND MAIN RESULTS: Vo(2) was measured directly in all patients using respiratory mass spectrometry. Estimated values for absolute Vo(2) (mL/min) and indexed Vo(2) (mL.min-1.m-2) were calculated from the four predictive equations published by LaFarge and Miettinen, Lundell et al., Wessel et al., and Lindahl. The agreement between measured and estimated Vo(2) was evaluated by calculating their bias and limits of agreement. A failure of agreement between measured and estimated Vo(2) was noted in both groups of patients, irrespective the equation used, and the agreement was poorer in patients in the intensive care unit. The equation by LaFarge and Miettinen produced the closest estimation in patients at cardiac catheterization with a bias of 4.5 mL/min for absolute Vo(2) and 6.9 mL.min-1.m-2 for indexed Vo(2). A systematic error of overestimating lower and underestimating higher indexed Vo(2) mL.min-1.m-2 was introduced in both groups. CONCLUSION: Predictive equations do not accurately estimate Vo(2) in ventilated patients with congenital heart disease.

Adolescent↗

Morphometric techniques in the evaluation of pulmonary vascular changes due to congenital heart disease.

Open lung biopsy is performed in patients with congenital heart disease to determine the suitability for surgical correction. Controversy exists as to whether arterial density can be graded with certainty. We studied the influence on the grading (Heath-Edwards) of two morphometric techniques. Lung tissue from 14 controls and biopsy specimens from 80 patients with atrial septal defect (n=10) ventricular septal defect (n=27), complete atrioventricular canal (n=27), miscellaneous lesions (n=8) and tetralogy of Fallot (n=8) were analyzed with a planimetric method. Additionally, wall thickness was determined in 52 patients by distance measurements. The ratio of alveoli/arteries per area of lung tissue was measured. Medial thickness was "increased" on distance measurements in 15% of the cases where planimetric data showed normal wall thickness. The ratio of alveoli/arteries varied up to 43% from the mean. Hemodynamic data obtained at a median (range) time of 2 months (1 day to 18 months) before the operation did not correlate with morphologic findings. In 27 patients hemodynamic data were recorded at a median time of 1 year (3 days to 44 months) after the operation. Intimal fibrosis occupying more than 10% of the vessel lumen was associated with persistent high pulmonary vascular resistance. We conclude that morphometric techniques are useful to determine the degree of fibrosis in advanced vascular lesions. Arterial density cannot be determined in biopsy specimens with adequate certainty.

Adolescent↗

Risk factors in congenital heart disease.

Risk factors were studied in 801 children with congenital heart disease (CHD) coming from 105,374 consecutive births of known outcome. The incidence of CHD was 7.60%. Diagnosis was performed in 66.5% of the cases during the perinatal period. Two-hundred-fifty seven of the cases also had at least one non-cardiac malformation (multiply malformed). Ninety-two cardiac infants (11.47%) had recognized chromosomal and non-chromosomal syndromes. The most frequent noncardiac malformations were renal, digestive and limb anomalies. For each case a control was studied. The following features were screened: sex ratio, parity and previous pregnancies, parental age, residency, education, ethnic origin, length, head circumference and weight at birth, genetic and environmental factors. Odds ratio values were calculated for the risk factors. Weight, length and head circumference at birth of cardiac infants were less than those of controls. The weight of placenta was also lower than in controls. The pregnancy with CHD was more often complicated by hydramnios and threatened abortions, except in infants with isolated CHD. Oliogoamnios was more frequent in pregnancies producing multiply malformed infants and those with recognized syndromes with CHD. One out of four children with CHD had an extracardiac malformation, which is ten times the rate of incidence of malformation in our population. The incidence of CHD in first degree relatives of these infants was 3.0%. These first degree relatives also had more non-cardiac malformations than did those of the controls.

Abnormalities, Multiple↗

Outcome of children with end-stage congenital heart disease waiting for cardiac transplantation.

BACKGROUND: End-stage congenital heart disease (CHD) is a major indication for pediatric cardiac transplantation. The objective of the study was to evaluate pre-transplant outcome of children with CHD. METHODS: The clinical profile and outcome of patients with CHD <20 years of age listed for transplantation (1993 to 1999) were reviewed and patients who died waiting (Group I) were compared with survivors to transplant (Group II). RESULTS: Mean age of the patients (n = 46) was 8.3 +/- 8 years. Primary indications for transplant were ventricular dysfunction in 36 (78%), failed Fontan in 8 (18%) and severe hypoxemia in 2 (4%) patients. Thirty-two patients were Status 1 (70%), 14 were Status 2 and 5 patients were de-listed. Twenty-nine of the 41 patients that remained listed survived to transplant, 12 (29%) died waiting. Causes of death were sepsis in 2 and severe heart failure (HF) in 10 patients. Eight patients died with multi-system organ failure, including 3 on mechanical circulatory support. Mean time to death was 29 +/- 28 days and time to transplant was 94 +/- 176 days. Mean age at listing was younger in Group I (2.6 +/- 4 years) compared with Group II (9.1 +/- 7 years, p < 0.05). Mean HF duration was shorter in Group I (3.6 +/- 3.9 months) compared with Group II (25 +/- 33 months, p < 0.05). Fifty-day actuarial survival on the waiting list was lower in infants (38%) compared with older children (91%, p < 0.05). In contrast to the high mortality (71%) in infants with CHD, all infants with cardiomyopathy survived to transplant. CONCLUSIONS: Seventy-one percent of patients listed with CHD survived to transplant. Younger age at listing and rapid onset of HF were significant risk factors for pre-transplant mortality.

Actuarial Analysis↗