SEARCH · Search PubMed

Results for “HEART DISEASE, CONGENITAL”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

SourceResearch connectionFrom yearThrough year

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,387 records · Page 77Linked to original sources

PubMed2004

Psychosocial issues affecting adults with congenital heart disease: one patient's perspective.

This article addresses a number of psychosocial issues that advance practice nurses and other health care providers should be aware of when working with patients who have congenital heart disease. If not properly addressed, particularly during adolescence, these issues can have a strong impact on a person's medical care and over-all quality of life. This article includes information from medical literature, conversations with adult patients, and my own experiences as both a patient with congenital heart disease and as a clinical social worker.

Adaptation, Psychological↗

PubMed2003

The efficacy of using pulmonary vein wedge pressure for the estimation of pulmonary artery pressure in children with congenital heart disease.

The objectives of this study were to assess the accuracy of pulmonary vein wedge pressure (PVWP) in estimating pulmonary artery pressure (PAP) in various types of congenital heart disease, including single-ventricle physiology. The systolic, diastolic, and mean values of both PAPs and PVWPs were measured in 30 patients (a total of 46 points). Pulmonary artery pressure ranged from 13 to 74 (34 +/- 15) mm Hg in systole, 5 to 25 (13 +/- 6) mm Hg in diastole, and 6 to 48 (18 +/- 10) mm Hg in mean. As a whole, good correlations between PAPs and PVWPs were observed (systole, r = 0.70; diastole, r = 0.85; mean, r = 0.82; P < 0.0001). However, with an increase in PAP, the discrepancy between PAPs and PVWPs increased. When the mean PVWP was more than 18 mm Hg, the mean PVWP in 14 of 24 (58%) underestimated the mean PAP by up to 22 mm Hg (mean difference, -1.7 +/- 5.8 mm Hg). On the other hand, all of the patients with mean PVWPs less than 18 mm Hg (n = 22) had mean PAPs less than 18 mm Hg (r = 0.86; PAP = 1.11 x PVWP - 1.41; P < 0.0001), and the mean difference was -0.2 +/- 1.8 mm Hg. The mean PVWP can accurately estimate the mean PAP in children with congenital heart disease who have a mean PVWP less than 18 mm Hg.

Cardiac Catheterization↗

PubMedDate not supplied

Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study.

The objective of this study was to ascertain the prevalence and survival rate of children born with a heart defect. A total of 816, 569 children live-born between 1980 and 1990 in Bohemia (52,478 km(2), population 6.314 million, western Czech Republic) were followed up and those with suspected heart disease referred to a center. Echocardiography was done in all of them. All dead children were autopsied. Congenital heart disease was found in 5030 of 816, 569 children (6.16 per 1000 live births). The most frequent conditions were ventricular septal defect (41.59%), atrial septal defect (8.67%), aortic (7.77%) and pulmonary (5.81%) stenoses, transposition of the great arteries (5.39%), coarctation of the aorta (5.29%) and persistent ductus arteriosus (5.07%). The first week was survived by 92.46%, the first month by 89.14%, 6 months by 82.42%, and the first year of life by 80.02%, and 77.11% (95% CI 75. 91-78.31%) survived to age 15 years. The best prognosis was found in pulmonary stenosis (15-year survival 95.55%), atrial septal defect (92.04%), persistent ductus arteriosus (90.59%), ventricular septal defect (89.37%) and aortic stenosis (88.39%). The worst results were attained in hypoplastic left heart, truncus arteriosus and pulmonary atresia with intact ventricular septum. In conclusion, the prevalence of congenital heart disease was 6.16 per 1000 live births; 77.11% of patients survived to age 15 years.

Actuarial Analysis↗

PubMed2002

New technology and methodologies for intraoperative, perioperative, and intraprocedural monitoring of surgical and catheter interventions for congenital heart disease.

We review the new technology and methods available for support of intraoperative and intraprocedural imaging in the catheterization laboratory for surgical and interventional catheterization procedures in the treatment of congenital heart disease. The methods reviewed include miniaturized probes and new ways of using them perioperatively for cardiac imaging from transesophageal, substernal, and intracardiac imaging locations. The smaller and more versatile the probes, the better adapted they will be in providing methods to improve the outcomes in babies born with serious forms of congenital heart disease.

Cardiac Surgical Procedures↗

PubMed2001

Atrioventricular reciprocating tachycardia involving twin atrioventricular nodes in patients with complex congenital heart disease.

INTRODUCTION: Histologic studies of autopsy specimens described the coexistence of two distinct AV nodes (so-called "Minckeberg sling" or "twin AV nodes") in specific congenital heart defects; however, the clinical electrophysiologic (EP) characteristics of twin AV nodes have not been characterized in detail. METHODS AND RESULTS: Since April 1993, a total of seven patients with complex congenital heart disease presented with AV reciprocating tachycardia suspected to be mediated by twin AV nodes. A common anatomic finding was AV discordance ([S,L,L] or [I,D,D]) with a malaligned complete AV canal defect in 5 of 7 patients. Intracardiac EP study was performed in five cases, and ablation was attempted in three patients with successful elimination of tachycardia inducibility by interruption or modification of 1 of the 2 AV nodes. Important EP characteristics included (1) the existence of two discrete nonpreexcited QRS morphologies, each with an associated His-bundle electrogram; (2) decremental as well as adenosine-sensitive anterograde and retrograde conduction; and (3) inducible AV reciprocating tachycardia with anterograde conduction over one AV nodal pathway and retrograde conduction over the alternate AV nodal pathway. The existence of two AV nodes was further supported in the group treated with radiofrequency ablation by the development of transient accelerated junctional rhythm during energy delivery with an identical QRS morphology to that generated by anterograde conduction over the targeted AV node. CONCLUSION: Reciprocating tachycardia mediated by twin AV nodes can be a source of recurrent supraventricular tachycardia in patients with specific forms of complex congenital heart disease. Successful treatment with catheter ablation is possible.

Atrioventricular Node↗

PubMed2006

Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease.

A member of the Tennessee state legislature recently proposed a bill that would mandate all newborn infants to undergo pulse oximetry screening for the purpose of identifying those with critical structural heart disease before discharge home. The Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Defects was convened on September 29, 2005. This group reviewed the current medical literature on this topic, as well as data obtained from the Tennessee Department of Health, and debated the merits and potential detriments of a statewide screening program. The estimated incidence of critical congenital heart disease is 170 in 100,000 live births, and of those, 60 in 100,000 infants have ductal-dependent left-sided obstructive lesions with the potential of presentation by shock or death if the diagnosis is missed. Of the latter group, the diagnosis is missed in approximately 9 in 100,000 by fetal ultrasound assessment and discharge examination and might be identified by a screening program. Identification of the missed diagnosis in these infants before discharge could spare many of them death or neurologic sequelae. Four major studies using pulse oximetry screening were analyzed, and when data were restricted to critical left-sided obstructive lesions, sensitivity values of 0% to 50% and false-positive rates of between 0.01% and 12% were found in asymptomatic populations. Because of this variability and other considerations, a meaningful cost/benefit analysis could not be performed. It was the consensus of the task force to provide a recommendation to the legislature that mandatory screening not be implemented at this time. In addition, we determined that a very large, prospective, perhaps multistate study is needed to define the sensitivity and false-positive rates of lower-limb pulse oximetry screening in the asymptomatic newborn population and that there needs to be continued partnering between the medical community, parents, and local, state, and national governments in decisions regarding mandated medical care.

Advisory Committees↗

PubMed1987

Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia.

To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95%) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5%) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.

Adolescent↗

PubMed1991

[Supraventricular paroxysmal tachycardia without congenital heart disease: clinical, therapeutic aspects and course in 65 children].

We have reviewed the records of 65 children with paroxysmal supraventricular tachycardia (PST) without congenital heart disease followed a mean of 4 years, with a total of 121 episodes. PST appeared before 6 months of age in 42 (64.6%) children. Thirteen patients (20%) had a present factor which might predispose to PST in 66.2% of the patients who were younger than 6 months of age, and in only 4.3% of those over 6 months. Wolff-Parkinson-White syndrome was present on surface ECG during sinus rhythm in 26.1% of children younger than 6 months, and in 39.1% of those over 6 months. Digoxin was the initial treatment in 84.3% of the episodes with a success rate of 75% when were employed alone and of 84.2% when were employed in combination of quinidine. PST recurred at least once in 35 children (53.8%), the 90% within three months of the first episode. All patients were alive and 63 (96.9%) doing well. One patient developed cerebral anoxia and now has hemiparesia and another patient has incessant PST. We conclude that children with PST without congenital heart disease and without delay in diagnosis had a good outcome.

Amiodarone↗

PubMed1994

Technetium-99m methoxy isobutyl isonitrile simultaneous evaluation of ventricular function and myocardial perfusion in patients with congenital heart disease.

The authors evaluated the clinical applications of Tc-99m methoxy isobutyl isonitrile (MIBI) in the simultaneous assessment of ventricular function and myocardial perfusion in patients with congenital heart disease. Global ventricular function was assessed by first-pass ECG gated study. Myocardial perfusion was evaluated on images performed 1 hour after the injection of the tracer. Regional wall motion and systolic thickening were assessed by gated study acquired after the perfusion study. Two young patients were studied after a surgical Glenn procedure. The first patient, with a history of transposition of the great vessels and univentricular heart, had an ejection fraction of 44%. The left ventricle was dilated and the right ventricle was not appreciable. The septal and inferoapical regions showed reduced perfusion and reduced systolic thickening. The second patient, with a history of pulmonary atresia, septal defect and left ventricular hypoplasia, had a right ventricular ejection fraction of 37%. Regional wall motion, systolic thickening, and myocardial perfusion were normal. The right ventricle was hypertrophic and larger than the left ventricle. Thus, a single injection of Tc-99m MIBI allows noninvasive simultaneous assessment of global and regional ventricular function and myocardial perfusion in young patients with complicated congenital heart disease.

Child, Preschool↗

PubMed2002

[Specialized tertiary care facilities for adults with congenital heart disease in North America and the United Kingdom: future directions in Japan].

Specialized tertiary care facilities have been established to care for the increasing numbers of adults with congenital heart disease. Because this cardiovascular subspecialty is relatively new, the dedicated facilities have evolved without preexisting guidelines. Therefore, a mini-review was conducted of specialized tertiary care facilities for adults with congenital heart disease in North America and the United Kingdom, to illuminate the future directions of continuous management of these patients in Japan. Specialized tertiary care was provided by integrated collaborative teams that involve medical and pediatric cardiologists, cardiac surgeons, and non-cardiac consultants. Continuing growth of this relatively new patient population and further evolution of specialized care facilities can be anticipated.

Adult↗

PubMed2005

Echocardiography in adults with congenital heart disease.

Echocardiography is a valuable tool in the investigation and follow-up of adult patients with congenital heart disease. The majority of these patients have been recognised and treated in childhood, and effective investigations in adult life depend on exact knowledge of the patient history and previous treatments/operations. The major lesions are presented and important echocardiographic features briefly discussed.

Adult↗

PubMed2000

Oxygen uptake versus exercise intensity: a new concept in assessing cardiovascular exercise function in patients with congenital heart disease.

OBJECTIVE: To assess the relation between exercise intensity and oxygen uptake during graded exercise in paediatric patients who underwent surgical repair of congenital heart disease, and to compare it with conventional measures of aerobic exercise function. DESIGN: Cross sectional study. Exercise testing was performed on a treadmill and gas exchange was measured on a breath by breath basis. PATIENTS: 29 patients who underwent an atrial switch operation for transposition of the great arteries (TGA) (mean (SD) age at testing 10.3 (2.5) years) and 30 patients who underwent total repair of tetralogy of Fallot (TF) (age 12.1 (3.3) years) performed graded exercise testing. Exercise responses were compared with data obtained in 24 normal controls (age 11.4 (2.6) years). RESULTS: The slope of oxygen uptake versus exercise intensity averaged 1.50 (0. 64) ml O(2)/min(2)/kg in the patients with TGA and 1.68 (0.75) ml O(2)/min(2)/kg after TF repair, both lower (p < 0.005) than in normal controls (2.42 (0.68) ml O(2)/min(2)/kg). The lower slope of oxygen uptake was correlated with a subnormal value for ventilatory anaerobic threshold, which averaged 78.0 (13.3)% of normal in TGA and 85.1 (10.6)% in TF. This was associated with a steeper slope (p = 0.001) of carbon dioxide output versus oxygen uptake above the ventilatory anaerobic threshold in TGA (1.26 (0.20)) and TF (1.20 (0. 18)) compared with the normal controls (1.05 (0.13)), and also a steeper slope of ventilation versus carbon dioxide in TGA (47.0 (15. 4)) and TF (41.5 (13.7)) than in the controls (30.3 (8.5)). CONCLUSIONS: Calculation of the steepness of the slope of oxygen uptake versus exercise intensity is a valid measurement of oxygen flow to the exercising tissues, which may be limited in congenital heart disease.

Adolescent↗

PubMedDate not supplied

[Prevalence of congenital heart disease in 44,985 newborns in Colombia].

OBJECTIVE: To estimate the prevalence of congenital heart defects in Colombia using the methodology of the Latin-American Collaborative Study of Congenital Malformations (ECLAMC, for its initials in Spanish) and to make an epidemiological description of the study population. MATERIAL AND METHODS: A prospective case-control study nested to a hospital-based cohort included 44,985 infants born from June 1, 2001 to April 30, 2005 in 11 Colombian hospitals. RESULTS: Fifty-five cases were reported (1.2 per 1,000); 36 (65.5%) corresponded to severe defects and 18 (32.7%) had associated extracardiac malformations. The following risk factors were identified: maternal age > or = 40 years, paternal age > or = 30 years, gestational age < or = 37 weeks, birth weight < or = 3,000 g and > or = 3 pregnancies. CONCLUSIONS: This study shows a similar prevalence of congenital heart disease to that found in Spain, Mexico, and South America. Risk factors identified emphasize the need for public health policies in a developing country undergoing an epidemiological transition.

Case-Control Studies↗

PubMed1991

[67Ga imaging in the patients with infective endocarditis after surgery for congenital heart disease].

67Ga imaging was performed in sixteen patients (age: 8 m.-18 y.) who had persistent fever and positive acute phase reactants after surgery for congenital heart disease. Abnormal uptake of 67Ga over the heart and the lungs was evaluated with a computer. Abnormal uptake of 67Ga was observed in seven patients, three of them showed it in the area of peripheral pulmonary artery and another four showed it in the area of artificial vessels for pulmonary artery reconstruction. In six patients with positive blood cultures, five showed abnormal uptake of 67Ga and in ten patients with negative blood cultures, two showed it. Vegetation was detected with 2D-echocardiography in four patients and all of them showed abnormal uptake of 67Ga, while in 12 patients without vegetation three showed it. In conclusion, 67Ga imaging was useful to detect the foci of infective endoarteritis or pulmonary embolism caused by the vegetation in infective endocarditis in the patients after surgery for congenital heart disease, especially in the peripheral pulmonary arteries and artificial vessels which could not be detected with 2D-echo.

Adolescent↗

PubMed2006

Bridging the gap between anatomy and molecular genetics for an improved understanding of congenital heart disease.

Birth defects are the leading cause of infant mortality and malformations in congenital heart disease (CHD) are among the most prevalent and fatal of all birth defects. Yet the molecular mechanisms leading to CHD are complex and the causes of the cardiac malformations observed in humans are still unclear. In recent years, the pivotal role of certain transcription factors in heart development has been demonstrated, and gene targeting of cardiac-specific transcription factor genes in animal models has provided valuable insights into heart anomalies. Nonetheless results in these models can be species specific, and in humans, germline mutations in transcription factor genes can only account for some cases of CHD. Furthermore, most patients do not have family history of CHD. There is, therefore, a need for a better understanding of the mechanisms in both normal cardiac development and the formation of malformations. The combining of expertise in cardiac anatomy, pathology, and molecular genetics is essential to adequately comprehend developmental abnormalities associated with CHD. To help elucidate genetic alterations in affected tissues of malformed hearts, we carried out genetic analysis of cardiac-specific transcription factor genes from the Leipzig collection of formalin-fixed malformed hearts. Working with this morphologically well-characterized archival material not only provided valuable genetic information associated with disease, but enabled us to put forward a hypothesis of somatic mutations as a novel molecular cause of CHD. Knowledge of cause and disease mechanism may allow for intervention that could modify the degree of cardiac malformations or development of new approaches for prevention of CHD.

Germ-Line Mutation↗

PubMed2000

Congenital heart disease among school children in Alexandria, Egypt: an overview on prevalence and relative frequencies.

A study was undertaken to determine the prevalence and relative frequencies of congenital heart diseases diagnosed by echocardiography among school children in Alexandria, Egypt. The study was conducted during a 1-year period (1 May 1995-1 May 1996) at the Students' Health Insurance Hospital, which is a referral center for all health insurance units, providing an echocardiography service. The prevalence of congenital heart diseases (CHD) among school children accounted for 1.01/1,000. There was a male predominance in cases of pulmonary stenosis and single ventricle, while in cases of patent ductus arteriosus, mitral valve prolapse, and partial atrioventricular canal there was a female predominance. The commonest cardiac defects were ventricular septal defects, pulmonary stenosis, and atrial septal defects. Pulmonary stenosis ranked the second commonest defect and is a peculiar finding in our Egyptian population. The mere presence of these cardiac defects in school children is an indicator of the poor quality of care provided to this growing childhood population.

Child↗

PubMed2005

Utility of routine follow-up defibrillator threshold testing in congenital heart disease and pediatric populations.

INTRODUCTION: Recent studies have suggested that routine defibrillation threshold (DFT) testing of implantable cardioverter defibrillators (ICDs) in adults may not be necessary. The congenital heart disease and pediatric populations are a unique group of ICD recipients having a higher incidence of lead failure. We investigated the utility of follow-up DFT testing in this population. METHODS AND RESULTS: The records of 155 ICD recipients at one center were retrospectively reviewed, and patients having one or more follow-up DFT tests were analyzed. The patients were divided into two groups. The "routine" group consisted of 58 follow-up DFT procedures in 46 patients, without known changes in ICD parameters. The "prompted" group consisted of 21 follow-up DFT procedures in 18 patients, motivated by clinical concerns about changes in ICD lead status. Of 58 "routine" DFTs performed at a mean postimplant duration of 32 +/- 23 months (range 2-78), 7 (12%) had reprogramming, and 1 required a hardware change as a result of the testing. Of the 21 "prompted" DFTs performed, 7 required device reprogramming, and 3 required hardware upgrade. Overall, 19 (24%) of 79 procedures detected clinically significant changes, requiring reprogramming or ICD system revision. No complications were seen from follow-up DFT testing. CONCLUSION: A high rate of abnormalities was found at follow-up DFT testing in this population, especially in the group of patients with clinically prompted testing. Clinically indicated DFT testing, as expected, has a high yield of important information on device function in congenital heart disease and pediatric populations.

Adolescent↗

PubMed2002

Anesthetic and perioperative outcome of teenagers and adults with congenital heart disease.

OBJECTIVE: To compare the perioperative outcome of patients >or=13 years old undergoing surgery for congenital heart disease in a children's hospital by a dedicated congenital heart surgery and anesthesia team with procedure-matched younger control patients. DESIGN: Retrospective medical record review study. From October 1997 to July 2000, medical records of all patients >12 years old requiring cardiopulmonary bypass were reviewed. A control group of patients <or=5 years old was reviewed, and 2 patients were matched to each older patient by diagnosis and surgical procedure. Data are reported as mean +/- SD. Older (study) patients were compared with younger (control) patients using t-test or chi square, with p <or= 0.05 significant. SETTING: Medical school-affiliated tertiary-care children's hospital. PARTICIPANTS: Patients undergoing congenital heart surgery. MEASUREMENTS AND MAIN RESULTS: The study group (older patients) comprised 85 patients, and the control group (younger patients) comprised 170 patients. There were no intraoperative deaths. All major complications-cardiopulmonary resuscitation, neurologic injury, massive hemorrhage with sternotomy, femoral cannulation for emergent bypass, and severe episodes of hypotension on induction of anesthesia-occurred in older patients undergoing repeat sternotomy. CONCLUSION: Mortality and major morbidity were low in both groups; however, all major intraoperative incidents occurred in older repeat sternotomy patients, suggesting increased perioperative risk for adverse outcomes in these patients.

Adolescent↗