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PubMed2001

Arterial to end-tidal carbon dioxide tension difference in children with congenital heart disease.

In children with congenital cyanotic heart disease, right-to-left intracardiac shunting causes an obligatory difference between arterial and end-tidal carbon dioxide tension (PaCO2-PE'CO2) as venous blood, rich in carbon dioxide, is added to the arterial circulation. This obligatory PaCO2-PE'CO2 difference, which can be predicted from knowledge of oxygen saturation, haemoglobin concentration and PaCO2, increases as oxygen saturation decreases, most markedly when the haemoglobin concentration is high. A second possible cause of the PaCO2-PE'CO2 difference is the effect of pulmonary hypoperfusion caused by the shunt. We studied 60 children undergoing cardiac surgery and compared the predicted the PaCO2-PE'CO2 difference with measured values to investigate the extent to which additional factors influence the clinically observed PaCO2-PE'CO2. In many children, observed values were much greater than predicted, which is compatible with some degree of pulmonary hypoperfusion. However, this was not felt to represent the complete picture in all patients. Another cause of ventilation-perfusion mismatch was suspected in those children who showed a considerable improvement in oxygen saturation during ventilation with an increased FIO2. We believe that pulmonary congestion caused by large left-to-right shunts may further increase the PaCO2-PE'CO2 difference.

Anesthesia, General↗
PubMed2003

[Evaluation of congenital heart disease in adults].

Improvements in the diagnosis and surgical treatment of congenital heart disease during infancy and childhood have resulted in an outstanding increase in the prevalence of these entities during adulthood. Congenital heart disease in the adult represents a new diagnostic challenge to the consultant cardiologist, unfamiliar with the anatomical and functional complexities of cardiac malformations. Assessment of adult congenital heart disease with imaging techniques can be as accurate as in children. However, these techniques cannot substitute for a detailed clinical assessment. Physical examination, electrocardiography and chest x-rays remain the three main pillars of bedside diagnosis. Transthoracic echocardiography is undoubtedly the imaging technique which provides most information, and in many situations no additional studies are needed. Nevertheless, ultrasound imaging properties in adults are not as favorable as in children, and prior surgical procedures further impair image quality. Despite recent advances in ultrasound technologies such as harmonic or contrast imaging, other diagnostic procedures are sometimes required. Fortunately, transesophageal echocardiography and magnetic resonance imaging are easily performed in the adult, and do not require anaesthetic support, in contrast to pediatric patients. These techniques, together with nuclear cardiology and cardiac catheterization, complete the second tier of diagnostic techniques for congenital heart disease. To avoid unnecessary repetition of diagnostic procedures, the attending cardiologist should choose the sequence of diagnostic techniques carefully; although the information this yields is often redundant, it is also frequently complementary. This article aims to compare the diagnostic utility of different imaging techniques in adult patients with congenital heart disease, both with and without prior surgical repair.

Coronary Angiography↗
PubMed2006

Adult congenital heart disease: the patient's perspective.

This article presents the adult congenital heart disease (ACHD) patient "voice" by high-lighting issues and challenges commonly identified in peer support forums but rarely addressed in the existing literature. Representative patient quotations are provided, and relevant research on patient education and psychosocial function is referenced. Issues discussed include the provision of overly pessimistic and overly optimistic prognoses, common patient misperceptions and knowledge gaps, frustrations and dangerous encounters in the medical system, and living with invisible disabilities. Patient self-perception of congenital heart disease, the gifts of congenital heart disease, and the role of patient associations are also discussed. For each issue identified, implications for the ACHD health professional are outlined and recommendations for best practices are made.

Adult↗
PubMedDate not supplied

Prevalence of rheumatic and congenital heart disease in schoolchildren of Kathmandu valley in Nepal.

BACKGROUND: Rheumatic heart disease remains a major public health problem in developing countries with its very high prevalence. Rheumatic and congenital heart disease are significant causes of morbidity and mortality among Nepalese schoolchildren. The aim of our study was to determine the prevalence of rheumatic and congenital heart disease among schoolchildren of the Kathmandu valley in Nepal. METHODS AND RESULTS: The study included 9420 students, of whom 4466 were male and 4954 were female, with ages ranging from 5 to 18 years. A clinical survey was conducted by the examining team in selected schools, and involved answering standard questionnaires. A total of 83 children were suspected of having heart disease. Out of these 83 children, 23 were confirmed to have heart disease; 11 had rheumatic heart disease, and 12 congenital heart disease, giving a prevalence of 1.2/1000 and 1.3/1000, respectively. The commonest cardiac lesions were mitral regurgitation in the rheumatic heart disease group, and atrial septal defect in the congenital heart disease group. A higher prevalence of congenital heart disease was detected in females. CONCLUSIONS: The prevalence of rheumatic heart disease and congenital heart disease among schoolchildren of Kathmandu is 1.2/1000 and 1.3/1000, respectively, with mitral regurgitation and atrial septal defect being the commonest lesions.

Adolescent↗
PubMed1981

Structural congenital heart disease in the newborn. Its differentiation from nonstructural cardiac or pulmonary disease.

This article discusses structural congenital heart disease in the newborn. Emphasis is given to the clinical and laboratory diagnosis including the indication for cardiac catheterization and angiography. The discussion includes the group of cyanotic, noncyanotic and other structural congenital heart diseases, as well as the group of neonatal cardiopulmonary distress without structural heart disease. An outline of the general management of these entities is included in the presentation.

Angiocardiography↗
PubMed2000

Comparison of hyperventilation and inhaled nitric oxide for pulmonary hypertension after repair of congenital heart disease.

BACKGROUND: Pulmonary hypertension is associated with congenital heart lesions with increased pulmonary blood flow. Acute increases in pulmonary vascular resistance (PVR) occur in the postoperative period after repair of these defects. These increases in PVR can be ablated by inducing an alkalosis with hyperventilation (HV) or bicarbonate therapy. Studies have shown that these patients also respond to inhaled nitric oxide (iNO), but uncertainty exists over the relative merits and undesirable effects of HV and iNO. HYPOTHESIS: Alkalosis and iNO are equally effective in reducing PVR and pulmonary artery pressure (PAP) in children with pulmonary hypertension after open heart surgery. SETTING: Critical care unit of a tertiary care pediatric hospital. DESIGN: Prospective, randomized, crossover design. PATIENTS: Twelve children with a mean PAP > 25 mm Hg at normal pH after biventricular repair of congenital heart disease. INTERVENTIONS: Patients were assigned to receive iNO or HV (pH > 7.5) in random order, and the effect on hemodynamics was measured. Each treatment was administered for 30 mins with a 30-min washout period between treatments. Finally, both treatments were administered together to look for a possible additive effect. MEASUREMENTS AND MAIN RESULTS: Cardiac output and derived hemodynamic parameters using the dye dilution technique. Hyperventilation, achieved by an increase in ventilator rate without a change in mean airway pressure, decreased Pa(CO2) from a mean (SD) of 43.7+/-5.3 to 32.3+/-5.4 mm Hg and increased pH from 7.40+/-0.04 to 7.50+/-0.03. This significantly altered both pulmonary and systemic hemodynamics with a reduction in PAP, PVR, central venous pressure, and cardiac output and an increase in systemic vascular resistance. In comparison, iNO selectively reduced PAP and PVR only. The reduction in PVR was comparable between treatments, although addition of iNO to HV resulted in a small additional reduction in PVR. An additional decrease in PAP was seen when HV was added to iNO, attributable to a reduction in cardiac output rather than a further decrease in PVR. CONCLUSIONS: Inhaled NO and HV are both effective at lowering PAP and PVR in children with pulmonary hypertension after repair of congenital heart disease. The selective action of iNO on the pulmonary circulation offers advantages over HV because a decrease in cardiac output and an increase in SVR are undesirable in the postoperative period.

Administration, Inhalation↗
PubMed2005

Late occurrence of adenosine-sensitive focal junctional tachycardia in complex congenital heart disease.

BACKGROUND: Although supraventricular tachycardia in complex congenital heart disease (CHD) has been reported after surgical repair, its exact electrophysiologic identification has been limited to intraatrial reentrant tachycardia (IART). Moreover, junctional tachycardia (JT) has not previously been described as a cause of late postoperative arrhythmia. METHODS AND RESULTS: Since 1993, a total of 12 patients with congenital heart disease presented with paroxysmal focal JT. The patients with only typical immediate postoperative junctional ectopic tachycardia were excluded. Medical records, standard electrocardiography and Holter monitoring were reviewed. An intracardiac electrophysiologic (EP) study was performed in 11 patients. Ten patients were in post-Fontan status (5.7% of total Fontan survivors). Focal JT occurred more frequently in heterotaxy syndrome among the Fontan survivors (7/52 vs. 3/124; P < 0.05). The commonest anatomy of the atrioventricular (AV) junction was complete AV canal in 8 patients. EP characteristics of focal JT were as follows: (1) various tachycardia mechanisms were identified (increased automaticity or a triggered mechanism in 6/11, and focal reentry in 5/11, including one concealed nodofascicular pathway) (2) ventriculoatrial conduction during tachycardia was either dissociation (7/12) or variable (5/12) (3) All JTs were terminated by adenosine. Class III antiarrhythmic agent was effective in 5/6. His bundle ablation was performed in one Fontan patient, who already had pacemaker because of accompanying intractable IART and sinus node dysfunction. CONCLUSION: Focal JT may be a source of late term supraventricular tachycardia in patients with complex CHD. The tachycardia mechanism was either automatic/triggered or reentrant. In all patients, JT was effectively terminated by adenosine.

Adenosine↗
PubMed1999

Adolescents with congenital heart disease: psychopathological implications.

The psychopathological risks of congenital heart disease in adolescence are described. The emotional development of adolescents with cardiopathy is discussed, including distortions in body image and separation-individuation issues. The consequences of cyanotic and acyanotic cardiopathies for intellectual functioning are noted, and the impact of congenital heart disease on interactions with parents and siblings is presented. Psychopathological implications of heart transplant are also reviewed.

Adolescent↗
PubMed1996

Infective endocarditis in congenital heart disease.

The average life expectancy of patients with congenital heart disease has dramatically improved over the past four decades because of advances in medical and surgical therapy, with patients with complex lesions surviving to adolescence and adulthood. Tetralogy of Fallot, transposition of the great arteries, ventricular septal defects, patent ductus, and bicuspid aortic valves in particular are susceptible to infective endocarditis. Most operated patients are left with some form of residua or sequelae, many of which predispose to infective endocarditis. Surgical palliation, such as systemic-to-pulmonary shunts, and reparative surgery, often requiring prosthetic valve or conduit replacement, are major predisposing conditions. Accordingly, recognition, prevention, and treatment strategies for infective endocarditis assume increasing importance in adolescents and adults with congenital heart disease, operated or not.

Endocarditis, Bacterial↗
PubMed2002

Quality of life in adults with congenital heart disease.

OBJECTIVE: To examine the quality of life of adults with congenital heart disease. DESIGN AND SETTING: Observational, cross sectional study conducted at one general hospital in Birmingham, UK. PATIENTS: All 471 patients registered at the adult congenital heart disease clinic were sent the 36 item short form health survey (SF-36) to assess their quality of life. Questionnaires were completed by 276 (58.6%) patients (41.7% men; median (interquartile range) age 31.0 (26.3-36.0) years, range 16-85 years). RESULTS: Surprisingly, patients deemed surgically cured (for example, atrial septal defect repair) had significantly poorer quality of life in all domains (all p < 0.05), except for pain, than the general population, as determined from population normative data. Patients who had received palliative treatment reported quality of life scores similar to those who had never required cardiac surgery and to the general population, although both patient groups had significantly poorer physical functioning and overall general health perception than the general population (all p < 0.01). Patients with inoperable conditions had significantly poorer physical functioning (all p < 0.01) and overall general health perception (all p < 0.05) than all other patients, and significantly worse quality of life in all domains than the similarly aged general population. Patients with cyanotic conditions had significantly worse quality of life than age and sex matched acyanotic patients (all p < 0.01). CONCLUSIONS: Patients with inoperable or cyanotic conditions and, paradoxically, those deemed surgically cured, had the poorest quality of life among adults with congenital heart disease. However, all adults with congenital heart disease had significantly poorer levels of physical functioning and overall general health perception then similarly aged people in the general population.

Adolescent↗
PubMed2004

Trends and outcomes in transplantation for complex congenital heart disease: 1984 to 2004.

BACKGROUND: Cardiac transplantation for patients with complex congenital heart disease poses several anatomic and physiologic challenges for the transplant surgeon. We undertook the current single center study to evaluate surgical outcomes and lessons learned through a nearly twenty year experience with cardiac transplantation for complex congenital heart disease. METHODS: A retrospective review was performed to evaluate all patients undergoing cardiac transplantation from January 1, 1984 through January 1, 2004. Donor and recipient demographic and intraoperative and postoperative variables were acquired and correlated with perioperative (30-day) and late mortality in both univariate and multivariate analyses, and with Kaplan-Meier survival estimates. RESULTS: One hundred and six patients underwent transplantation for complex congenital heart disease and were followed for a median of 56 months. Thirty-seven (34.9%) patients died. Male gender and later year of transplantation were protective, and neonatal age and pulmonary artery reconstruction detrimental in multivariable modeling of overall mortality. Transplantation to a physiologic or anatomic single lung did not impact on survival. Patients in the study cohort had comparable survival estimates when compared with all those in the entire cohort without complex congenital heart disease. When comparing patients by era of transplantation, both cohorts demonstrated improved survival with later transplantation. CONCLUSIONS: Outcomes with transplantation for complex congenital heart disease have improved annually over the past twenty years. Transplantation to an anatomic or physiologic single lung did not impair overall survival. Pulmonary artery reconstruction imparted an increase in mortality both short and long term, a finding which merits further investigation.

Adolescent↗
PubMed2005

Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia.

Congenital heart disease (CHD) has a major impact on the survival of babies with esophageal atresia (EA). The present study assesses whether early diagnosis influences the management strategies in a large series of EA. Cases of EA treated between 1982 and 2002 were retrospectively divided into groups according to the presence or absence of CHD and to whether this was diagnosed or not prior to tracheo-esophageal fistula repair. Patients were also staged according to Spitz's classification in which major congenital heart disease (MCHD) are those associated with cyanosis and/or heart failure requiring surgery. Comparisons between groups of patients were made by standard statistical tests. Among 195 babies with EA (99 boys and 96 girls), 82 (42%) had CHD (31 boys, 39% and 51 girls, 61%, chi2 P < 0.05) and 43 out of these 82 (52%) had MCHD. Six children died without treatment. CHD was diagnosed prenatally in six cases and before EA repair in 26 cases. The diagnoses were missed in 12 instances (in five it was a MCHD). Regardless of preoperative diagnosis of right aortic arch in 3/6 cases, the oesophagus was approached from the right thorax in all cases and only in one of them the operation was not completed due to hemodynamic instability. There were 145/195 (75%) Spitz group I patients; 44/195 (22%) were group II and 6/195 (3%) group III. The mortality was respectively 9.5, 59 and 83%. We lost 15/113 (13%) babies with EA without CHD, 4/39 (10.2%) with EA and CHD and 26/43 (60%) with EA and MCHD. In children with CHD diagnosed prior to the esophageal operation 8/26 (30.7%) died and among those without diagnoses 3/12 (25%) died, (chi2 P > 0.1). 1. CHD was diagnosed after EA repair in one-third of cases. 2. Preoperative diagnosis of CHD changed the operative indication only in one case. 3. Survival after treatment for EA was not influenced by the moment or the accuracy of the diagnosis of CHD in this series. 4. With only a few exceptions, associated CHD should not change the strategies of EA repair.

Abnormalities, Multiple↗
PubMed2002

Remifentanil infusion for cardiac catheterization in children with congenital heart disease.

BACKGROUND: Cardiac catheterization of children with congenital heart disease is increasingly being performed under general anesthesia. Haemodynamic stability during anesthesia and fast and adequate recovery after the procedure is crucial in these patients. We performed a pilot study to evaluate hemodynamic stability when using remifentanil for anesthesia during cardiac catheterization. We also evaluated extubation times and recovery characteristics. METHODS: In a randomized, prospective, double-blind study 30 children (aged 1.5-20 months) received a continuous infusion of either 0.2 (group 0.2) or 0.3 microg/kg/min remifentanil (group 0.3) as part of a balanced anesthesia with 0.6 MAC sevoflurane. Heart rate, noninvasive arterial blood pressure, end tidal CO2 and pulse oxymetry were monitored throughout the procedure. Extubation times were noted, and recovery from anesthesia was evaluated using Aldrete scores. RESULTS: : Haemodynamic response to intubation was well blunted in both groups. No significant changes in hemodynamic variables were noted from induction of anesthesia until 10 min after intubation. From then on there was a decrease in HR and systolic arterial pressure, which remained significant throughout the procedure in both groups. Extubation times were similar in both groups: 7.3 min (2,1) in group 0.2 vs. 6.6 min (2,1) in group 0.3 (NS). The number of patients with an Aldrete score of nine or more was 14 (group 0.2) vs. 15 (group 0.3), 10 min after extubation (NS). CONCLUSION: Both dose regimens of remifentanil provided stable hemodynamic conditions during anesthesia for cardiac catheterization of children with congenital heart disease and allowed for rapid and adequate recovery.

Adjuvants, Anesthesia↗
PubMed1976

Genetic counseling for congenital heart disease.

The genetic knowledge of parents of children with congenital heart disease, who had received genetic counseling, was compared with that of a control noncounseled group attending the same cardiac clinic. A follow-up questionnaire showed that both groups had excellent knowledge of the nature of their children's heart lesions. The counseled group had significantly more accurate knowledge of their recurrence risks. Inasmuch as the reproductive attitudes of some of these parents were found to be influenced by genetic information, parents of children with CHD should be given a better understanding of recurrence risks for CHD than many of them possess.

Attitude to Health↗
PubMed2005

[Incidence of congenital heart disease in navarra (1989-1998)].

INTRODUCTION AND OBJECTIVES: Congenital cardiopathies are the most common forms of congenital malformation. They occur in between 5.2 and 12.5 in every thousand live births. The aim of this study was to describe the incidence and nature of congenital heart disease in the Spanish region of Navarra during a specified time period (1989-1998). PATIENTS AND METHOD: The study involved all children with congenital heart disease among the 47 783 born in the region in the specified time period. RESULTS: The incidence was 8.96 per thousand live births, with 90% having one of the 10 most common types of cardiac malformation. The accumulative percentage diagnosed was 25.3% in the first 24 hours of life, 45% in the first week, 65% in the first month, and 83.1% during the first year. Some 30.8% of cases of congenital heart disease required invasive treatment: 25.4% underwent surgery and 6.4%, cardiac catheterization. CONCLUSIONS: The incidence of congenital heart disease in Navarra falls within the range reported for developed countries. The level of care provided in this region is good, as demonstrated by existing diagnostic capabilities and treatment provision.

Female↗
PubMed2004

Parents' understanding of their child's congenital heart disease.

OBJECTIVES: To assess parents' understanding of their child's congenital heart disease in various knowledge domains and to identify significant determinants of parental knowledge. DESIGN: Cross sectional questionnaire survey SETTING: Tertiary paediatric cardiac centre. PATIENTS: 156 parents of children with relatively simple congenital heart defects were recruited from the outpatient clinic of a tertiary cardiac centre over a three month period. The questionnaire comprised 10 items of knowledge under three domains: nature of heart disease and its treatment; impact of heart disease on exercise capacity; and infective endocarditis and its prevention. The frequency distribution of the parents' knowledge in the different domains was determined. Univariate analyses and logistic regression were performed to identify significant determinants of knowledge in selected items. RESULTS: While 59% of parents correctly named their child's congenital heart disease, only 28.8% correctly indicated the heart lesion(s) diagrammatically. However, more than 80% of parents were aware of the indications and aims of previous surgical and transcatheter interventions. About half of the parents were aware of possible aetiologies and of the hereditary nature and symptoms attributable to underlying heart disease. Disappointingly, of the 56 parents whose children were taking cardiac medications, only 25 (44.6%) and 4 (7.1%) knew correctly the functions and important side effects of the medications, respectively. With regard to exercise capacity, 59% of parents indicated its level appropriate for the heart lesion. While 26.9% of parents had heard of the term "infective endocarditis", slightly more than half of the parents were aware of the need for antibiotics before dental procedures. Significant determinants of knowledge in the nature of heart disease were cardiac diagnosis, occupation of parents, and their educational level. Logistic regression failed to identify any significant determinants of parental knowledge in the other two domains. CONCLUSIONS: Parents of children with congenital heart disease have important knowledge gaps. Our findings suggest that the current educational programme is inadequate and needs to be refined to promote better parental understanding of their child's heart disease, with the ultimate aim of enabling parents to impart such knowledge accurately to their children.

Adult↗
PubMed2001

The influence of congenital heart disease on survival of infants with oesophageal atresia.

OBJECTIVE: To examine the prevalence of congenital heart disease in babies with oesophageal atresia and its influence on outcome. DESIGN: Retrospective analysis. SETTING: The resident population of one health region. RESULTS: A total of 153 babies with oesophageal atresia were identified from 509 975 live births (0.30 per 1000); 26 (17%) had cardiac defects. Survival of babies with normal hearts was 97%, 97%, and 95% at one week, one month, and one year. Survival of babies with congenital heart disease was 85%, 85%, and 67% at one week, one month, and one year, but only one of ten deaths was the result of the congenital heart disease. The remaining deaths were due to other congenital malformations, respiratory disease, or chromosome abnormalities. CONCLUSIONS: There is a high prevalence of congenital heart disease in babies with oesophageal atresia. Congenital heart disease is associated with a higher mortality in oesophageal atresia but it is not the cause of it.

Cause of Death↗
PubMed1999

Syndromes and malformations associated with congenital heart disease in a population-based study.

Congenital cardiac malformations are frequently associated with non-cardiac malformations and chromosomal anomalies. Management is therefore influenced by interventional needs for all of the various anomalies. We have studied the association of congenital heart disease with extracardiac anomalies in the relatively closed population of Malta, where echocardiographic screening of all syndromic/multiply malformed infants is routinely carried out. Malformations were classified by using the EUROCAT method, for the first time. During 1990-1994, the birth prevalence of congenital heart disease was 8.8/1000 live births (n = 231). Of these, 21 (9%) had recognised chromosomal anomalies (0.80/1000 live births; 95% CI: 0.51-1.25), four (2%) had recognised non-chromosomal syndromes and 14 (6%) had other, major, non-cardiac malformations (0.69/1000 live births; 95% CI: 0.42-1.11). The commonest non-cardiac anomalies were musculoskeletal anomalies. Down syndrome accounted for 95% of all syndromic congenital heart disease, with a birth prevalence of 0.73/1000 live births (95% CI: 0.45-1.16). Comparison of these results with earlier studies showed wide disparities between studies, and this was attributed to differences in methods. such as differing inclusion criteria for both congenital heart disease and syndromes and malformations. The commonest lesion found in association with Down syndrome was isolated ventricular septal defect, not atrioventricular septal defect, and this was attributed to our screening process which identifies small lesions which would otherwise have been clinically missed and/or closed spontaneously.

Abnormalities, Multiple↗