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PubMed1998

Increased energy expenditure in infants with cyanotic congenital heart disease.

Infants with cyanotic congenital heart disease (CCHD) often have reduced weight gain compared with infants in control groups. Our purpose was to conduct a longitudinal study of energy intake, resting energy expenditure (REE), and total energy expenditure (TEE) of a group of infants with CCHD. We hypothesized that increased REE and TEE and decreased energy intake in these infants would lead to reduced growth. Ten infants with uncorrected CCHD and 12 infants in a control group were studied at 2 weeks of age and again at 3 months. Indirect calorimetry was used to determine REE; the doubly labeled water method was used to determine TEE and intake. At 2 weeks and 3 months of age, infants with CCHD weighed significantly less than infants in the control group. No significant difference was seen in energy intake or REE between groups during either period. TEE was slightly but not statistically increased in the CCHD group at 2 weeks (72.6 +/- 17.4 vs 59.8 +/- 10.9 kcal/kg/d) and significantly increased at 3 months (93.6 +/- 23.3 vs 72.2 +/- 13.2 kcal/kg/d, P </=.03). We conclude that increased TEE but not increased REE is a primary factor in the reduced growth in infants with CCHD.

Calorimetry, Indirect↗
PubMed1999

Compression of the central airways by a dilated aorta in infants and children with congenital heart disease.

BACKGROUND: Children with congenital heart disease often experience respiratory symptoms in the preoperative and perioperative periods, which can complicate their management. An uncommon but important cause of respiratory insufficiency in such children is external airway compression. METHODS: We operated on 5 patients (median age, 6 months) with significant respiratory distress attributable to compression of the central airways by a dilated ascending aorta before or after repair of concomitant cardiovascular defects. Four of these patients had right aortic arch and 3 had pulmonary atresia with a ventricular septal defect and major aortopulmonary collaterals. In all patients, aortopexy was performed at the time of operation for the cardiovascular defects (n = 3) or after symptoms developed in the postoperative period (n = 2). The 3 patients in whom airway compression produced symptoms preoperatively also underwent reduction ascending aortoplasty. RESULTS: Symptoms resolved immediately after operation in 3 patients, whereas symptoms persisted in the other 2 patients and tracheostomy was required. At follow-up of 20 months to 5 years, all patients are alive and well, with mild or moderate respiratory symptoms in the 2 patients who required tracheostomy, both of whom were decannulated within 13 months. CONCLUSIONS: External airway compression can cause significant morbidity in patients with congenital heart defects other than vascular rings. In patients with respiratory symptoms in the context of a lesion that involves increased aortic outflow during intrauterine life and consequently, an enlarged ascending aorta, such as tetralogy of Fallot with pulmonary atresia, airway compression should be considered as a cause, especially if a right aortic arch is present or the patient also has pulmonary atresia with a ventricular septal defect and collaterals. Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved.

Airway Obstruction↗
PubMed2004

Low incidence of respiratory syncytial virus hospitalisations in haemodynamically significant congenital heart disease.

BACKGROUND: Haemodynamically significant congenital heart disease (CHD) is a risk factor for severe respiratory syncytial virus (RSV) disease in young children. Population based data on the incidence of RSV hospitalisations in CHD patients are needed to estimate the potential usefulness of RSV immunoprophylaxis using palivizumab. AIMS: (1) To obtain population based RSV hospitalisation rates in children <24 months of age with CHD. (2) To compare these rates with non-CHD patients and with previous studies. (3) To determine the number of patients needed to treat (NNT) with palivizumab to prevent one RSV hospitalisation. METHODS: Six year, longitudinal, population based study at an institution, which is the sole provider of primary to tertiary in-patient care for a precisely defined paediatric population. RESULTS: RSV hospitalisation rates (per 100 child-years) in CHD patients aged <6, <12, 12-24, and <24 months of age were 2.5 (95% CI 0.8 to 5.6), 2.0 (0.8 to 3.8), 0.5 (0.1 to 1.8), and 1.3 (0.6 to 2.3), respectively, and the relative risk (RR) in comparison with non-CHD patients was 1.4 (0.6 to 3.1), 1.6 (0.8 to 3.2), 2.7 (0.7 to 9.7), and 1.8 (1.0 to 3.3), respectively. NNT was between 80 (35 to 245) and 259 (72 to 2140) for various age groups. CONCLUSION: RSV hospitalisation rates in CHD patients were fourfold lower than reported from the USA. Based on these low rates and RR, unrestricted use of palivizumab does not appear to be justified in this study area.

Epidemiologic Methods↗
PubMed2000

Serial blood lactate measurements predict early outcome after neonatal repair or palliation for complex congenital heart disease.

OBJECTIVES: Neonates with congenital heart disease may appear hemodynamically stable after operation and then suddenly experience catastrophic decompensation. An improved means of predicting which infants will suddenly die in the early postoperative period may lead to lifesaving interventions. Studies indicate that blood lactate level is proportional to tissue oxygen debt, but information linking lactate levels with outcome in infants after operation is limited. We sought to determine whether a change in lactate level over time was predictive of a poor outcome defined as death within the first 72 hours or the need for extracorporeal membrane oxygenation. METHODS: To test this hypothesis, we studied prospectively 46 infants who were less than 1 month old and were undergoing complex cardiac surgical palliation or repair. Postoperative arterial oxygen saturation, bicarbonate, and lactate levels were recorded on admission to the intensive care unit and every 3 to 12 hours for the first 3 days. RESULTS: Thirty-seven patients had a good outcome, and 9 patients had a poor outcome. Mean initial lactate level was significantly greater in patients with a poor outcome (9.4 +/- 3.8 mmol/L) than in patients with a good outcome (5.6 +/- 2.1 mmol/L; P =.03). However, an elevated initial lactate level of more than 6 mmol/L had a low positive predictive value (38%) for poor outcome. In contrast, a change in lactate level of 0.75 mmol/L per hour or more was associated with a poor outcome (P <.0001) and predicted a poor outcome with an 89% sensitivity value, a 100% specificity value, and a 100% positive predictive value. CONCLUSIONS: Serial blood lactate level measurements may be an accurate predictor of death or the requirement for extracorporeal membrane oxygenator support for patients who undergo complex neonatal cardiac surgery.

Extracorporeal Membrane Oxygenation↗
PubMed1990

Congenital heart disease: incidence and inheritance.

Congenital heart disease occurs in approximately 1 per cent of liveborn children, but in a much higher percentage of those aborted spontaneously or stillborn. To detect as many as possible with CHD, including those with mild lesions, very intensive studies are needed. Studies that are not so intensive, especially those done before modern diagnostic techniques were in general use, considerably underestimated the incidence of CHD in liveborn children. It appears that the incidence of CHD and of the various individual lesions does not differ in different countries or at different times, providing the ascertainment of CHD is complete and accurate. The commonest form of CHD is the ventricular septal defect, which occurs in 30 to 40 per cent of all children with CHD. The risks of recurrence in siblings and of transmission to future generations depends on the exact mode of inheritance involved. Approximately 5 to 8 per cent of CHD is due to gross chromosomal abnormalities, and the recurrence risk is that of the chromosomal derangement itself. Because many children with these chromosomal lesions die in infancy or have reduced fertility, the risk to future generations is relatively low. About 3 per cent of CHD is due to classical Mendelian gene effects, with correspondingly high recurrence risks in first-degree relatives. Most CHD has lower risks of recurrence and transmission than those predicted by Mendelian single-gene action. The popular explanation for their inheritance has been the interaction of polygenic effects and the environment, but recent studies of the recurrence and transmission risks of various forms of CHD do not fit this model well. The alternative model is a single gene defect modulated by random events. The recurrence risks for future siblings are 2 to 6 per cent, and for offspring are 1 to 10 per cent, but in a few families the recurrence and transmission risks may be much higher.

Chromosome Aberrations↗
PubMed2006

Unique application of multislice computed tomography in adults with congenital heart disease.

PURPOSE: Adults with congenital heart disease (CHD) form a growing population of patients requiring coronary artery assessment. In light of their increased incidence of coronary anomalies and need for reoperations, it is important to define their coronary anatomy. The purpose of this study was to define the spectrum of coronary artery anatomies and feasibility of coronary artery delineation in adults with CHD using 16- and 64-slice computed tomography angiography. MATERIALS AND METHODS: Coronary multislice computed tomography (MSCT) examinations performed in adults with CHD between 2004 and 2005 were reviewed with institutional review board approval. Informed consent was not required. In addition to image quality, the following coronary parameters were evaluated by analysis of axial, multiplanar reformatted, and volume-rendered images: (1) relationship to great arteries, (2) ostia number and location, (3) proximal course, (4) length of vessel seen, and (5) dominance. RESULTS: Eighty-five examinations in adults with CHD adults were identified. The relationship of the great vessels, number and location of the coronary ostia, and proximal course of the coronaries could be identified in all studies. Coronary anomalies were detected in 16 (19%) subjects. The entire coronary tree was visible in 77 (91%) of subjects. CONCLUSIONS: MSCT provides detailed, noninvasive assessment of complex coronary artery anatomy in adults with CHD. MSCT should be considered a useful modality in the assessment of these patients, as it does not require arterial access and provides detailed 3-dimensional anatomic information that has important implications for long-term management.

Adult↗
PubMed2001

Prenatal diagnosis of congenital heart disease affects preoperative acidosis in the newborn patient.

OBJECTIVES: Congenital heart disease is the leading cause of death in the first year after birth. Prenatal diagnosis of the disease can optimize the preoperative condition of the patient and may help in the prevention of acidosis. In this retrospective study we compared the occurrence of metabolic acidosis in patients with and without prenatal diagnosis of a congenital heart disease. METHODS: Data of 408 patients who needed an operation for congenital heart disease within 31 days of life were analyzed retrospectively. Arterial blood gases at fixed time intervals and worst blood gas of 81 patients with and 327 patients without a prenatal diagnosis were compared, categorizing the patients on ductus dependency, anticipated univentricular or biventricular repair, and left-sided, right-sided, or no heart obstruction. RESULTS: In the overall group significant differences in lowest pH, lowest base excess, and highest lactate level were found, with metabolic acidosis more common among the patients with a postnatal diagnosis. In the group of patients with ductus-dependent congenital heart disease, the difference between patients receiving a prenatal and those receiving a postnatal diagnosis was more significant than in the group with non-ductus-dependent lesions. Analyzing patients with right-sided, left-sided, and no obstruction separately, significant differences were found in the group with left-sided heart obstruction for lowest pH and base excess and in the group with right-sided heart obstruction for lowest base excess. CONCLUSIONS: Prenatal diagnosis of congenital heart disease minimizes metabolic acidosis in patients with congenital heart disease and may be associated with improved long-term outcome and prevention of cerebral damage among this fragile group of patients, although no significant effect on direct surgical outcome was encountered.

Acidosis↗
PubMed2001

Diagnosis and management of the newborn with suspected congenital heart disease.

In critical congenital heart lesions, the ultimate outcome depends on timely and accurate diagnosis of the structural anomaly, the evaluation and resuscitation of secondary organ damage, effective lesion-specific preoperative management, and the appropriate timing and type of surgery. Crucial to this process is continuous communication among medical, surgical, and nursing disciplines.

Critical Illness↗
PubMed1989

Families with congenital heart disease.

Families with congenital heart deformities appear to have more difficulties and stress than those with other deformities. Some problems are intrinsic to the deformity but others are largely related to communication difficulties between the groups doing home support and those in supraregional centres.

Attitude to Death↗
PubMed1995

The search for genetic mechanisms of congenital heart disease.

Identification of the genetic basis for congenital heart defects has been a topic of inquiry for pediatric cardiologists for many years. Any genetic model proposed must account for three often puzzling features of congenital heart diseases, namely the high population incidence, the less than Mendelian recurrence risk, and low concordance rates of heart lesions within individual families. A multifactorial or polygenic inheritance model has been used to explain these observations. Although this model has been serviceable, it does not adequately explain the phenotypic variability within families. Further, the emphasis on teratogenic influences has not led to the elucidation of pathogenetic mechanisms. The multifactorial model should be modified to include the role of chance as originally proposed by Kurnit. Once the role of chance is acknowledged, the phenotypic variability of congenital heart diseases becomes less problematic, and the multifactorial model gives way to a model in which single genes are capable of producing congenital heart disease. Through recent work in animal models and humans, it is now clear that single congenital heart disease genes can be identified that produce phenotypic variability in families that is similar to that seen in the population as a whole. Examples demonstrating recent progress in the search for major congenital heart disease genes are discussed.

Animals↗
PubMed1999

MR imaging of adult patients with congenital heart disease.

Although the incidence of congenital heart disease remains constant among newborns, improved medical and surgical techniques have dramatically prolonged life expectancy and produced a new, growing group of patients harboring these lesions: adults with congenital heart disease. Conventional imaging techniques in these patients may be limited because of patient size, chest configuration, or poor ventricular function. MR imaging provides a noninvasive means of directly demonstrating their developmental abnormalities and the sequelae of pathophysiologic changes caused by these lesions. Combined use of spin echo and gradient reversal echo acquisition techniques allows complete evaluation of cardiac morphology and function in these patients. An new medical and surgical therapies prolong the lives of these patients, the role of MR imaging for physiologic assessment and surgical planning will expand further.

Adult↗
PubMedDate not supplied

Incidence of congenital heart disease: II. Prenatal incidence.

The incidence of congenital heart disease appears to be about 1 per 100 liveborn infants. In infants who die before term, however, there is a much higher incidence of congenital heart disease, with a tendency for an excess of complex lesions. Some but not all of these lesions are associated with gross chromosomal abnormalities, which occur frequently in first-trimester abortions. Most of these chromosomal abnormalities are associated with such maldevelopment of many organ systems that fetal death occurs in utero. Monosomy X (45, XO), has a high association with congenital heart disease. Most fetuses with this abnormality die in utero, but because the abnormality is not inevitably lethal a small increase in survival of these fetuses would cause a large increase in the total incidence of congenital heart disease.

Abortion, Spontaneous↗
PubMed2006

[Organization of care for adults with congenital heart disease].

The increasing number of adult patients with congenital heart disease and the better survival of patients with complex disease into adulthood, as a result of the success of pediatric cardiology and cardiac surgery over the last years, have increased the need for specific structures, the so-called grown-up congenital heart disease units, able to provide comprehensive care to these patients. Many of the adult patients with congenital heart disease will require, over time, further operations, urgent in-hospital admission for a wide range of complications such as arrhythmias, hemorrhage, heart failure and bacterial endocarditis. Furthermore, these patients may often experience despair due to their awareness of residual morbidities and the knowledge of possible early mortality, or limitations in their social lives and educational or occupational attainment. Provision of care for children with congenital heart disease is well established in most parts of the world. In contrast, clinical services for the adults with congenital heart disease are scarce. In this scenario, adult cardiologists are not always equipped to deal with the range ad complexity of grown-up patients with congenital heart disease, whereas pediatric cardiologists cannot be expected to manage the many acquired adult diseases in a pediatric medical environment.

Adolescent↗
PubMed2003

Vascular endothelial growth factor in children with congenital heart disease.

BACKGROUND: Children with cyanotic congenital heart disease may experience the development of abnormal vessels that become a source of significant morbidity. Abnormal vessel proliferation in these children may take several forms, including systemic-to-pulmonary collateral arteries, systemic-to-pulmonary venous collaterals, systemic venous collateral channels after bidirectional cavopulmonary anastomosis, and pulmonary arteriovenous malformations. However, no entity responsible for these abnormalities has been identified yet. This study determined whether children with cyanotic congenital heart disease have elevated serum levels of vascular endothelial growth factor (VEGF) and whether elevated VEGF correlated with these abnormal vessels. METHODS: Mean systemic room air oxygen saturation (SpO2), blood cell counts (RBC), and serum VEGF levels were measured preoperatively. Samples were obtained from 61 children with acyanotic heart disease (group N) and 102 children with cyanotic heart disease (group C) before cardiac surgery. Postoperative catheterization was performed 1-month after the operation to evaluate the abnormal vessels in group C. RESULTS: The VEGF level was significantly elevated in group C (355.0 +/- 287.1 pg/mL) compared with group N (203.0 +/- 221.6 pg/mL; p < 0.001). VEGF levels in patients with a single ventricle associated with asplenia syndrome (n = 7) in group C were significantly elevated (711.9 +/- 443.5 pg/mL) compared with other patients. There was no significant correlation between VEGF level and SpO2 or RBC. Abnormal vessels were diagnosed in 19.6% (20/102) patients in group C. There was no difference in VEGF levels between the patients with abnormal vessels (336.8 +/- 182.5 pg/mL) and the patients without abnormal vessels (359.1 +/- 306.8 pg/mL). CONCLUSIONS: Children with cyanotic heart disease have elevated systemic levels of VEGF, especially in those patients with a single ventricle associated with asplenia syndrome. There was no significant relationship in VEGF levels between the patients with abnormal vessels and without these vessels.

Adolescent↗
PubMed2003

Birth cohort effect of the mortality rate from congenital heart disease in Japan.

BACKGROUND: The mortality rate from congenital heart disease in Japan had decreased partly because the great improvement of medical treatment and care. Therefore, the patients who would have died of congenital heart disease in younger age in the past may be alive to be adult, and the number of adults with congenital heart disease might increase. If the management of such adults did not improve, the mortality rate from congenital heart disease might increase because of the increased number. METHODS: Using the Japanese vital statistics from 1960 through 1999, we observed the time series of the mortality rate from congenital heart disease by age and sex. The birth cohort consisted of those who were bone in 5-year period starting in 1960. The mortality rate was the sum of the number of death in every five years divided by the population of the center year. RESULTS: The transition of the mortality rate from congenital heart disease for age group 0 to 4 years was decreased since 1973. Other age groups showed decreased mortality rate since late 1960's. The birth cohort analyses showed that the mortality rate of each birth cohort was decreased as time passed, especially the age group 5 to 9 years old and 10 to 14 years old. CONCLUSION: Birth cohort effect of mortality from congenital heart disease exists in Japan.

Adolescent↗