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PubMed2000

The outcome of fetal congenital heart disease.

Series reporting congenital heart disease detected in utero all indicate a different spectrum and severity of disease than is seen in postnatal series, with a tendency toward more severe disease with a poorer outcome. This is largely because of the manner in which cases are selected for fetal echocardiography. As most forms of heart disease occur in otherwise normal pregnancies with no high risk features, detection of these cases is dependent on the skill of the ultrasonographer performing general obstetric scanning. Although detection of even major malformations seen in the four chamber view, is still less than perfect, it is improving. In the future, it is expected that malformations will be detected in earlier pregnancy and examination will include evaluation of great artery structure. This will give more parents the option of termination of pregnancy in complex disease. Alternatively, in continuing pregnancies, the outcome for the affected neonate will be improved by ideal perinatal care.

Decision Making↗
PubMed2004

Somatic NKX2-5 mutations as a novel mechanism of disease in complex congenital heart disease.

NKX2-5 is a pivotal transcription factor in heart development. Previous studies on lymphocytic DNA provided evidence of familial NKX2-5 gene mutations in cardiac malformations. Common mutations are rare in unrelated families. We analysed, by direct sequencing, the gene encoding NKX2-5 in the diseased heart tissues of 68 patients with complex congenital heart disease, focussing particularly on atrial, ventricular, and atrioventricular septal defects. We identified 35 non-synonymous NKX2-5 mutations in the diseased heart tissues of patients. These mutations were mainly absent in normal, for example, unaffected, heart tissue of the same patient, indicating the somatic nature and mosaicism of mutations. We also observed multiple mutations and multiple haplotypes, as well as mutations in Down's syndrome patients with cardiac malformations. Taken collectively, the above results suggest the somatic nature of NKX2-5 mutations associated with complex cardiac malformations. Somatic mutations in transcription factor genes of cardiac progenitor cells provide a novel mechanism of disease.

Base Sequence↗
PubMedDate not supplied

Adult cyanotic congenital heart disease: surgical experience.

Cyanotic congenital heart diseases constitute about 10 percent of total congenital heart disease cases in adults in the developing world. Prolonged cyanosis and old age adversely affect the outcome of surgery, thus posing a challenge to the cardiac surgeons. This study was conducted to assess the feasibility, safety and outcome of surgery in this group of patients. From January 1991 to December 1997, a total of 303 patients, aged 14 to 54 years (mean 19.8 +/- 1.5 years) with diagnosis of various cyanotic congenital heart diseases were operated at our institute. There were 210 males (69.3%). Two hundred and forty-seven patients (81.5%) had tetralogy of Fallot's physiology, 51 patients (16.8%) had single ventricle physiology and five (1.6%) had other lesions. Sixty-six patients (21.7%) had pre-operative complications such as haemoptysis, epistaxis, cerebrovascular accidents, brain abscess and infective endocarditis. Sixty patients (19.8%) had previous palliative shunts and 26 patients (8.5%) had coil embolisation of major aortopulmonary collaterals prior to surgery; 229 patients (75.5%) underwent biventricular repair, 52 (17.1%) had univentricular repair, 22 (7.7%) had palliative shunts and one patient had open ligation of a major aortopulmonary collateral in addition. In-hospital mortality was 3.3 percent. Follow-up period ranged from five months to seven years (mean 4.2 +/- 1.8 years). There were two late deaths. Of the 291 survivors, 11 were lost to follow-up. Two hundred and fifty-eight patients (92.1%) are in New York Heart Association class I. Significant residual defects warranting reoperation were present in four patients (1.3%). It is concluded that congenital heart surgery in older cyanotic patients can be performed safely with satisfactory results.

Adolescent↗
PubMed2005

The emerging role of percutaneous intervention in adults with congenital heart disease.

Adults with congenital heart disease (CHD) constitute a patient population that has grown rapidly, due to advances in diagnosis and treatment of children with CHD. Though ideally served by cardiologists with advanced training in congenital conditions, adults with CHD often receive the majority of their care from primary care physicians and general cardiologists. These patients often have unique clinical presentations. An understanding of available therapeutic modalities can assist clinicians in the timing of subspecialty referral. This review focuses on the unique challenges of cardiac catheterization and the role of percutaneous interventions in adults with CHD.

Adult↗
PubMed1993

Surgery for congenital heart disease.

Surgery for congenital heart disease has reached two important milestones. Intermediate and long-term results are available for the arterial switch operation and the modified Fontan procedure which allow us to assess their efficacy. New techniques and changes in the timing of operations have forced us to rethink older approaches and dicta. Finally, longstanding controversies continue to inspire argument as well as insightful investigation. This review highlights many of these topics.

Cardiopulmonary Bypass↗
PubMed2006

Differential duplication of an intronic region in the NFATC1 gene in patients with congenital heart disease.

Most forms of congenital heart disease (CHD) result from aberrations in cardiac morphogenesis including errors in septation, valve formation, and proper patterning of the great vessels. Transcription factors are key proteins that dictate mRNA synthesis rate and subsequent protein production in most eukaryotes. NFATC1 belongs to the Rel family of transcription factors. In mice, it is expressed in the embryonic heart and is restricted to the endocardium where it plays a major role in valve formation. To establish a role for NFATC1 in CHD, we started screening for mutations in the exons encoding the DNA-binding domain of NFATC1 in patients enrolled in our study on CHD in Lebanon. DNA was extracted from patients with pulmonary stenosis (PS), tricuspid atresia (TA) and ventricular septal defect (VSD). PCR amplification and DNA sequencing were done on the patients and their parents and (or) siblings. PCR amplification of the exon 7 region showed that 2 bands are obtained in 57% of patients with CHD (32/56) and in 45% of their healthy parents and (or) siblings. Sequencing of the 2 bands revealed that both are amplicons of the exon 7 region, and that the additional band harbors an additional 44 nucleotides segment in the intronic region. The homozygous form of this allele was only present in patients with VSD (2/21). A screen of a pool of 81 healthy, unrelated individuals showed no presence for the homozygous form of this allele, suggesting that NFATC1 is a potential VSD-susceptibility gene.

Alternative Splicing↗
PubMed2001

Treatment and outcome of serious structural congenital heart disease.

Serious structural congenital heart disease usually presents to the neonatal paediatrician, although increasingly these conditions are being diagnosed before birth. It is, therefore, important that those dealing with these fetuses and infants have some knowledge of their natural and modified history. The vast majority of lesions can either be corrected or given symptomatic palliation and this review discusses treatment options and provides up-to-date outcome information to enable fetal and neonatal staff to anticipate and to complement information given to families by paediatric cardiologists.

Arrhythmias, Cardiac↗
PubMedDate not supplied

Interventional cardiology in congenital heart disease.

Interventional procedures for congenital heart disease have evolved dramatically in the last decade. Beginning with pulmonary and aortic valvuloplasty, nowadays, stents and various devices are placed inside the heart and vessels to palliate or correct different congenital defects. The present communication summarizes the experience with interventional cardiology in congenital heart disease of the Instituto Nacional de Cardiología "Ignacio Chávez" during the last five years.

Adolescent↗
PubMedDate not supplied

Non-cardiac surgery and anaesthesia in children with congenital heart disease.

Children with Congenital Heart Disease (CHD) presenting for non-cardiac surgery have various physiological and functional abnormalities and thus pose great challenges to the anaesthetist. This one year prospective study was undertaken to determine the incidence of CHD in children presenting for non-cardiac surgery, the type of lesions and anaesthetic course. Five patients (1.5%) out of a total of 324 children aged from birth to twelve years who had surgery during the study period were found to have CHD, ventricular septal defect (VSD) being the commonest cardiac lesion. Surgery was for cataract extraction and herniorrhaphy. Although all the children had been previously treated or were on current treatment for additional medical problems, surgery under closely monitored balanced general anaesthetic technique was found to be safe.

Anesthesia, General↗
PubMed2004

Congenital heart disease and aneuploidy.

Congenital heart disease (CHD) is one of the commonest prenatal diagnoses made on routine ultrasound screening. Overall, up to 33% of CHD are associated with fetal aneuploidy. However, some specific cardiac lesions have a significantly greater association with particular chromosomal abnormalities. The majority of fetuses with CHD and aneuploidy also have extra-cardiac anomalies and are best managed by a multidisciplinary team where the management and prognosis of the cardiac defect can be discussed in the context of the baby as a whole. It is therefore important for clinicians involved in the management of fetuses with CHD to be aware of the association of aneuploidy as well as the prognosis and management of these cases, so that they can appropriately counsel the parents. In this chapter, we review the frequency and types of aneuploidy associated with the commonly diagnosed CHD and discuss their management.

Aneuploidy↗
PubMed1987

Follow-up study into early adulthood of patients with congenital heart diseases.

Patients with congenital heart diseases (CHD), who had been under our management during school days (elementary, junior high and senior high schools), were followed up into early adulthood (20-38 years in age, averaging at 27.3 years) to obtain the following results. (1) The course of mild CHD until early adulthood was favorable in terms of fatality rate. However, the incidence of complication and resultant deterioration was higher in non-operated mild CHD cases than in operated CHD cases. Representative complications in mild ventricular septal defect (VSD) cases were infectious endocarditis and aortic incompetence (often found in type I VSD cases). A chief complication in mild atrial septal defect (ASD) cases was prolapse of the mitral valve. (2) Patients with VSD or PDA (particularly mild cases) showed spontaneous recovery even in early adulthood. (3) The prognosis of non-operated CHD cases of moderate or higher severity was evidently poor compared to that of mild or operated cases. These results suggest that surgery is indicated in all CHD cases of moderate or higher severity so long as surgery is possible.

Adult↗
PubMed1984

Containing costs in the treatment of congenital heart disease.

In the Congenital Heart Program at San Diego Children's Hospital, alterations in medical practice have reduced costs without impairing quality or access. Pediatric cardiac catheterization was done in 483 consecutive elective patients without overnight hospital stay. Hospital readmission was required in one patient for psoas tendinitis. Avoiding overnight hospital stay minimized attendant risks of hospital care, lessened psychosocial trauma and reduced the average hospital bill by $493 (29%). Hospital stay was also reduced for elective surgical correction of congenital heart disease on a case-by-case basis. Review of 151 consecutive cases (1978 through 1982) showed a decrease in both preoperative days in hospital and postoperative days in an intensive care unit. The duration of the postoperative stay was shortened from 6.8 days in 1978-1979 to 4.4 days in 1982 (P <.05). No increase in morbidity and no mortality resulted from the shortened perioperative hospital stay. Financial savings from this process averaged $991 per procedure.Diagnostic tests were reassessed and many precatheterization laboratory tests were eliminated. Without change in new patients seen or surgical volume, the use of cardiac catheterization decreased from 241 procedures in 1981 to 161 in 1982 and the number of operations without catheterization increased (11 to 22, 1981 to 1982). No increase in surgical morbidity or mortality was found comparing those diagnosed only by echocardiography with those who had preoperative cardiac catheterization. The decrease of 80 catheterizations in one year resulted in a savings of $188,800.True cost containment (reducing cost without reducing quality) can be accomplished in congenital heart programs. Similar cost containments in other disciplines may also be achieved.

California↗
PubMed1996

The adult patient with congenital heart disease.

In adults with congenital heart disease who are confronted with noncardiac surgery, perioperative risks can be reduced, often appreciably, when problems inherent to this patient population are anticipated. The first necessity is to clarify the diagnosis and to be certain that appropriate information is obtained from a cardiologist with adequate knowledge of congenital heart disease in adults. Physiology and anatomy can vary significantly among patients who superficially carry identical diagnoses. Elective noncardiac surgery should be preceded by clinical assessment including review of clinical and laboratory data and securing the results of necessary diagnostic studies. Preoperative assessment should be performed far enough in advance of the anticipated date of surgery to allow critical assessment of the data and potential discussions with colleagues. Appropriate cardiovascular laboratory studies to be obtained or reviewed include electrocardiograms, chest radiographs, echocardiograms, and cardiac catheterization data, which may include specialized intracardiac electrophysiologic testing. Congenital heart disease in adults is a new and evolving area of special interest and expertise in cardiovascular medicine. Multidisciplinary centers for the care of these patients are being developed. The 22nd Bethesda Conference recommended that these centers include among their consultants anesthesiologists with special expertise in managing patients with congenital heart disease. These anesthesiologists can have the option of serving either as the attending anesthesiologists when patients require noncardiac surgery or as consultants and resource individuals to other anesthesiologists.

Adult↗
PubMed1999

Comparison of left and right atrial epicardial pacing in patients with congenital heart disease.

BACKGROUND: Complex congenital heart disease (CHD) often necessitates the use of epicardial pacing. Adequate right atrial (RA) sensing and pacing thresholds are often difficult to obtain due to suture line scarring and RA dilatation. The purpose of this study was to evaluate the placement of left atrial (LA) epicardial leads in children. METHODS: Patient demographics, pacing, and sensing data of atrial pacing systems implanted between January 1994 and January 1997 were collected. RESULTS: Forty-nine pacing systems were implanted: 14 LA epicardial, 19 RA epicardial, and 16 transvenous in the right atrium. Lead impedance, current, and energy were similar in the two epicardial groups throughout the study. Energy thresholds (ET) were lower in the LA than RA at 6 months, and 1 and 2 years (p < 0.05). Analysis of post-Fontan patients performed alone revealed a lower ET in the LA as compared with the RA. Pacing and sensing parameters from transvenous leads are presented for relative comparison. CONCLUSIONS: Transvenous leads are most efficient but often contraindicated in complex CHD. LA leads offer lower energy thresholds than RA leads with similar sensing parameters.

Adolescent↗
PubMed2003

Quality of life experienced by adults with congenital heart disease.

Adults with congenital heart disease (CHD) represent a growing population of patients. Medical and surgical advances have increased the number of CHD adult survivors, which may create quality-of-life (QOL) issues not previously considered. Quality-of-life issues pertinent to this patient population involve health and life insurance acquisition, birth control, genetic counseling, pregnancy concerns, employment, and independent living arrangements. The purpose of this study was to describe the QOL of adults with CHD. The study used a prospective cross-sectional case-control design to examine QOL using the Sickness Impact Profile (SIP). The study participants were a sample of 124 adults with CHD from an outpatient cardiology clinic in a metropolitan university-affiliated teaching hospital in the Northeast and 124 matched healthy control subjects. Between the participants and the matched control subjects, there was a significant difference in the total mean SIP score, the physical and psychosocial dimension scores, and all the category scores (P < 0.05). The areas of life the adults with CHD reported as lacking in quality involved the categories of work (SIP of 11.1, moderate disability) and sleep and rest (SIP of 9.03, mild disability). The results of this study indicate that the SIP can be used for quantitative and subjective QOL assessment of adults with CHD. It is suggested that cardiac advanced practice nurses use the results of this study to develop appropriate information, counseling, and anticipatory guidance for this patient population.

Activities of Daily Living↗
PubMed1990

[Pulse oximetry for noninvasive oxygen monitoring during dental treatment in children with congenital heart disease].

Children with congenital heart disease (CHD) are prone to present abrupt changes in cardiovascular functions which cause hypoxic spells or acute heart failure, so that oxygen monitoring during dental treatment is of great importance in cyanotic CHD patients. Monitoring of arterial oxygen saturation (SpO2) by pulse oximetry has several advantages, including rapid response time, freedom from skin injury, self-calibration and continuous readout of the SpO2 value. In these studies, SpO2 was measured in 68 pediatric patients (55 were CHD children and 13 were normal children) during dental treatment at the Pediatric Dentistry, Hokkaido University Dental Hospital. In all of the normal patients, SpO2 did not desaturate less than 95%. On the other hand, SpO2 ranged from 56% to 98% in CHD patients, and in 22% (n = 12) of these, SpO2 desaturated below 80%. All of the SpO2 less than 80% patients had been diagnosed as cyanotic CHD. Classification of CHD cases (n = 144, the total number of measurements) on the pattern of the SpO2 variation revealed that in 11% (n = 16) of the CHD cases, SpO2 decreased during dental treatment. Twelve of these 16 cases could not readily accept dental treatment. We suggest that behavior management is the most important factor in CHD patients for keeping SpO2 stable. Motion artifact of the SpO2 value, which was easily evoked when a patient bent his elbow or shook his finger, often made the pulse oximetry useless. In our studies, the SpO2 value sometimes became inaccurate in fearful infants or handicapped patients. Pulse waveform displayed on the front panel of pulse oximetry was useful to identify the motion artifact. We conclude that pulse oximetry is an appropriate apparatus for continuous arterial oxygen monitoring in pediatric patients during dental treatment as far as motion artifact is effectively eliminated.

Artifacts↗
PubMed1992

Abnormalities in von Willebrand factor and antithrombin III after cardiopulmonary bypass operations for congenital heart disease.

In patients with congenital heart disease two poorly understood postoperative complications are pulmonary hypertensive crises after repair of large atrioventricular or ventricular septal defects and right atrial and pulmonary thrombi after the Fontan operation. In this study we assessed whether cardiopulmonary bypass in these patients is associated with the release of agents that might induce platelet aggregation and vasoconstriction, such as biologically active von Willebrand factor and platelet-activating factor. In addition, we measured levels of anticoagulants such as antithrombin III and proteins C and S. Three groups of patients with congenital heart disease undergoing cardiopulmonary bypass were monitored through the perioperative period for secundum atrial septal defects, large atrioventricular or ventricular septal defects, and tricuspid atresia or univentricular heart (Fontan candidates). Control values were obtained from age-matched patients; patients requiring major noncardiac operations and those with cardiac disease not requiring cardiopulmonary bypass were also studied. After cardiopulmonary bypass in all three groups biologic activity of von Willebrand factor increased markedly in the immediate and early postoperative periods compared with preoperative values, whereas antithrombin III values were decreased. Platelet-activating factor was detected in only two patients with congenital heart disease, both in the early postoperative period. In contrast, patients who did not have cardiopulmonary bypass did not show these abnormalities. All measured parameters normalized at late follow-up (6 to 18 months after operation). Although cardiopulmonary bypass in these patients resulted in increased von Willebrand factor activity and decreased antithrombin III, changes that may predispose the patient to platelet aggregation and thrombus formation, absolute values in individual patients alone were not predictive of pulmonary hypertensive crises or detectable thrombi. This suggests that these hematologic abnormalities may contribute to but are not by themselves a cause of morbidity in the early postoperative period. Moreover, the increased von Willebrand factor biologic activity seen postoperatively in patients with congenital heart disease suggests that use of synthetic vasopressin may be ineffective and potentially detrimental.

Antithrombin III↗