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PubMed2006

Predictive factors for long-term prognosis in adults with cyanotic congenital heart disease--Japanese multi-center study.

OBJECTIVE: Adults with cyanotic congenital heart disease (CCHD) are associated with a significant incidence of morbid events and premature deaths that may be predicted during childhood. We aimed to identify predictive factors related to long-term prognosis through a Japanese multi-center cross-sectional study. METHODS: Data were collected from 253 adults with CCHD (126 men; age 28 (18 to 56) years) from 15 participating centers between 1998 and 2003. Laboratory data such as cardiothoracic ratio (CTR), percutaneous oxygen saturation (SpO2), hematocrit levels (Ht) and platelet counts (Pl-c) at the age of 15 years were collected for predictive factor analysis for death and cardiovascular and systematic events. Predictive factors were determined by multivariate Cox regression analysis. RESULTS: After a mean follow-up of 21 (0-42) years, 23 patients died with a median age of 29 (18-54) years (heart failure in 8, sudden in 6 and systematic complications in 9). Survival since 18 years of age was 91% and 84% at 10 and 20 years, respectively. Significant predictive factor for death was Pl-c<130x10(9)/l and for renal failure (n=7) was Ht>65%. 162 patients were hospitalized and predictors for hospitalization due to heart failure (n=45) were common atrioventricular canal CTR>60% and Pl-c<100x10(9)/l and that due to arrhythmias (n=44) were systematic right ventricle and CTR>60%. CONCLUSIONS: This multi-center study provides an objective basis of assessing the long-term prognosis in patients with CCHD. These data are useful in making decisions regarding medical management and in favorably altering the non-operative course of the disease.

Adolescent↗
PubMed1997

Nitric oxide for the evaluation and treatment of pulmonary hypertension in congenital heart disease.

The use of inhaled nitric oxide as a selective pulmonary vasodilator has expanded to include patients with congenital heart disease and pulmonary hypertension. The therapeutic and diagnostic roles of inhaled nitric oxide offer additional alternatives and benefits to these patients with pulmonary hypertension, particularly in the postoperative setting. This article reviews the background, mechanism of action, toxicities, and current clinical applications of inhaled nitric oxide in the child with congenital heart disease and pulmonary hypertension.

Administration, Inhalation↗
PubMed2004

[MRI of congenital heart disease in childhood].

MRI provides a non-invasive diagnostic tool complementing echocardiography on one hand, and showing advantages over echocardiography, on the other hand, especially after corrective procedures. The multiple different MRI sequences need to be adapted to examinations of children and patients with congenital heart disease (CHD), and can be used to detect morphologic changes, blood-flow in the heart and thoracic vessels and diastolic or systolic function of myocardium. Several factors determine the success of the examination of a complex congenital heart disease or a postoperative situs. Pediatric radiologists and radiologists experienced in congenital heart diseases have to work in close cooperation. Echocardiography should be performed before MRI. The results of prior examinations and the clinical history of the patients, including possible palliative or reconstructive operations, must be available before MRI to guide the planning of the examination. With a systematic segmental approach to the situs, to the atrial and ventricular morphology, and to the visceroatrial, atrioventricular and ventriculoarterial connections, most CHDs can be diagnosed correctly. With appropriate knowledge, MRI can also be performed quite accurately after complex operations and may avoid or delay invasive diagnostic procedures. The aim of this article is to impart knowledge, to mention problems and to provide guidance in the performance of cardiac MRI in patients with CHD.

Adolescent↗
PubMed1990

Down's syndrome: different distribution of congenital heart diseases between the sexes.

We have investigated the reasons why female patients with Down's syndrome prevail in our out-patient clinic for Paediatric Cardiology compared to the higher incidence of Down's syndrome among live born male children. We reviewed 277 cases of Down's syndrome, 119 males (42.96%) and 158 females (57.04%) from 1970 to 1987. A final diagnosis of the type of the congenital heart disease was accomplished among 210 cases, 85 males (40.47%) and 125 females (59.38%). This different distribution between the sexes was significant (P less than 0.01) when compared to that of the general population with congenital heart disease (4150 patients, 2108 males and 2042 females). The dominant lesion was atrioventricular septal defect, (130 cases; 46 males [54.11%] and 84 females [67.20%]). We found an identical incidence of this lesion among patients without Down's syndrome. In the studied population, we did not find any of the congenital heart diseases usually prevalent in males, such as aortic coarctation or stenosis and complete transposition. The molecular determinants of Down's syndrome seem to influence the preponderance of atrioventricular septal defect in females, increasing its incidence, while seeming to act in a negative way concerning congenital heart diseases usually showing male prevalence.

Down Syndrome↗
PubMedDate not supplied

Assessment of cardiovascular anatomy in patients with congenital heart disease by magnetic resonance imaging.

The following discussion addresses the assessment of cardiovascular anatomy in patients with congenital heart disease by magnetic resonance (MR). The focus of this review is on the techniques of performing the MR examination. In particular, individual pulse sequences are described and illustrated with their strengths and weaknesses. Imaging strategies using the described pulse sequences are proposed. The pulse sequences described are widely available on most MR scanners. Therefore, the proposed imaging strategies are clinically proven to be simple and effective ways to perform cardiac MR examination for the assessment of cardiovascular anatomy in patients with congenital heart disease. Functional imaging, such as flow analysis and ventricular function assessment, are discussed elsewhere in this issue.

Adolescent↗
PubMed1995

Adult congenital heart disease: use of transthoracic echocardiography versus magnetic resonance imaging scanning.

There are many potential imaging modalities available for the assessment of patients with adult congenital heart disease, yet a dearth of information exists as to their comparative clinical value. Transthoracic echocardiography provides high-resolution, real-time imaging of intracardiac structures and is well suited to the investigation of these patients. It is limited by problems of acoustic penetration that vary from patient to patient, and rarely provides high-quality images of the great vessels in adults. The introduction of spectral Doppler ultrasound and color Doppler flow mapping has greatly enhanced the accuracy and quantitative ability of cardiac ultrasound in adult congenital heart disease. Magnetic resonance imaging is not subject to the problems of ultrasound but does not provide real-time imaging and it is comparatively expensive and time consuming. However, the high-resolution imaging it provides is a major advance in the investigation of patients with poor transthoracic echocardiograms or in delineating the extracardiac structures often unseen by conventional echocardiography. Transthoracic echocardiography and magnetic resonance imaging are complimentary investigations in adult congenital heart disease providing high-resolution anatomic and functional information in even the most complex forms of congenital heart lesions in this difficult and expanding population.

Adult↗
PubMed1982

Myocardial metal content in patients who expired from cyanotic congenital heart disease and acute rheumatic heart disease.

The importance of metals in normal and pathologic cardiovascular function has been recognized. Significant derangements in myocardial Ca2+, Mg2+, and Cu2+ have been reported in ischemic heart injury. We studied 3 groups of hearts: 1) fifteen specimens obtained from patients who had no heart disease, 2) nine specimens from patients who had expired from cyanotic congenital heart disease, and 3) ten specimens from patients who had expired from acute rheumatic heart disease with carditis and severe heart failure. None of the patients had undergone cardiac surgery. Left ventricular lateral wall Mg2+, Ca2+, Cu2+, and Zn2+ contents were measured by atomic absorption spectrometry. The results showed a significant decrease in myocardial Mg2- (Group I 177.06 +/- 32.71; Group II 155.66 +/- 14.79; Group III 149.00 +/- 13.29, p less than 0.05 and p less than 0.01, respectively), and Cu2+ contents (Group I 3.22 +/- 0.37; Group II 2.94 +/- 0.22; Group III 2.56 +/- 0.32, p less than 0.02 and p less than 0.001, respectively), and a rise in myocardial Ca2+ content (Group I 36.06 +/- 10.72; Group II 43.22 +/- 7.01; Group III 46.30 +/- 4.85, p = not significant, and p less than 0.01, respectively). The myocardial Zn2+ content did not change significantly (Group I 26.53 +/- 3.99; Group II 26.00 +/- 4.15; Group III 26.40 +/- 3.53). The myocardial Mg2+/Ca2+ ratio was reduced markedly in both groups (Group I 5.328 +/- 1.879; Group II 3.685 +/- 0.735; Group III 3.135 +/- 0.291, p less than 0.001 for both Groups II and III vs Group I). The latter results correlated closely with the myocardial Mg2+/Ca2+ ratios reported in experimental models in peri-infarction zones. Thus, the myocardium of patients who had expired from cyanotic congenital heart disease and acute rheumatic carditis is jeopardized by ischemia, with metal contents similar to the border areas in myocardial infarction.

Acute Disease↗
PubMed2006

[The best of congenital heart disease in 2005].

The end of 2004 and the year 2005 have seen several publications on congenital heart disease, with studies performed in the fetus, infant, child and adult. In the fetus, cardiac malformations as well as rhythm or conduction defects have been revealed, cardiomyopathies with diverse origins. In infants affected by congenital heart disease, respiratory complications due to respiratory syncitial virus can be serious, and vaccination is therefore recommended for poorly tolerated cardiopathies. Myocardial non-compaction has been the subject of several publications with an excellent review by Freedom. Coronaropathies are not confined to adults, and intravascular echography has been used in children. Aortic biscuspidy, which is more frequent in men, has also been the subject of several publications this year. They have all shown the frequency of dilatation of the ascending aorta in this anomaly, and the different consequences depending on fusion of the commissures. Residual hypertension following correction of coarctation remains a problem; the geometry of the aortic arc and arterial rigidity are responsible for it in certain cases. Late complications in the aortic wall are common. Percutaneous closure of atrial septal defects is beneficial for the anatomy and function of the heart. Articles on the foramen ovale have not been lacking, and have shown the importance of its closure in cases of transient ischaemic attacks, cerebrovascular accidents, migraines, especially with an aura, and in cases of sub-aqua diving accidents. Percutaneous implantation of intracardiac valves is becoming more and more topical, with pulmonary and even tricuspid valves in animals. Finally, there have been several publications on the long term outlook for patients undergoing Mustard, Senning or Fontan procedures.

Cardiac Catheterization↗
PubMed2002

Life expectancy and quality of life in adult patients with congenital heart disease.

The survival and quality of life of patients with congenital heart disease have significantly improved in the last 20 years. This is due to more effective medical and surgical care. The new community of grown-up congenital heart patients consists of a few natural survivors with trivial congenital lesions or very rare complex cardiac abnormalities which are naturally compensated, and of more than 75% of patients who had been submitted to cardiac surgery during infancy or childhood. Clinical follow-up is however mandatory for many of them with scheduled times and types of exams to control the effects of sequelae and late complications, and to prevent deterioration and premature death because cardiac surgery may not have resulted in normality. Moreover, these patients have many needs and even more, many questions. Not giving a correct answer to each specific question reduces the entity of surgical success.

Adult↗
PubMedDate not supplied

[Approach to congenital heart disease in adults].

After a few decades treating patients with congenital heart disease during childhood, we now face the problem of dealing with many of them as adults and few cases with disorders detected in adult life. The number of patients, with and without surgery is growing up rapidly. Conservatively speaking, there are in Mexico approximately 300 thousands patients with this condition, increasing by 15 thousands patients per year. In addition to the complexity of the congenital pathology, the knowledge of acquired diseases should be incorporated as well as the genetic advise, pregnancy care and specialized psychological support. The approach to these patients begins with stratification; diagnosis and treatment requiring a multidisciplinary, well informed and an capable to perform together medical team.

Adult↗
PubMed1985

A survey to evaluate the management of orthodontic patients with a history of rheumatic fever or congenital heart disease.

A survey concerning the orthodontic management of patients with a history of rheumatic fever or congenital heart disease was mailed to a random sample of 500 orthodontists throughout the United States. Two hundred thirty-two surveys were completed and returned. Analysis revealed that 88% of the respondents were aware that they should screen for patients with a history of rheumatic fever or congenital heart disease, only 11% used the antibiotic regimen recommended in 1977 by the American Heart Association (see Fig. 2), one half of the respondents thought it was necessary to provide antibiotic coverage during banding, and 38% deemed it necessary for debanding procedures which were likely to cause some degree of bleeding from the gingival sulcus. Only one half of the respondents would discontinue treatment if a condition of gingivitis developed which did not show signs of improvement, and only 70% provided medication for orthodontic procedures that could result in bacteremia. Furthermore, the results of this survey indicated that there was a great variation in the antibiotic regimen of orthodontic treatment for patients at risk of developing endocarditis and that there was a need to make the orthodontist more aware of the 1977 recommendations of the American Heart Association. Finally, a comparison between the year of graduation from an orthodontic program and the number of respondents who did not screen for or did not premedicate at-risk patients revealed a direct relationship. The earlier the education, the smaller the number of respondents who screened or premedicated.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Bacterial Agents↗
PubMed1983

Plain chest radiography of congenital heart disease in adults.

The plain chest radiograph can be an invaluable aid in the diagnosis of congenital heart disease in adults. Evaluation of the chest radiograph is made simpler and more accurate by the use of a systematic approach based on physiology. In many instances, a definitive diagnosis can be made; in others, only a differential diagnosis can be arrived at. The majority of adults with congenital heart disease will have some telltale abnormality on the chest radiograph that will occasionally furnish the first clue to the presence of a congenital anomaly.

Adult↗
PubMed1995

Bronchial hyper-responsiveness to inhaled histamine in children with congenital heart disease.

In order to assess bronchial responsiveness in patients with congestive heart failure secondary to congenital heart disease, we performed a histamine inhalation test while monitoring transcutaneous oxygen tension and compared the respiratory threshold to histamine with that obtained in patients with bronchial asthma. The inhalation test was performed by doubling concentrations of histamine solution for 2 min at 1 min intervals. The respiratory threshold of histamine was defined as the minimal concentration causing a drop in transcutaneous oxygen tension greater than 10% from baseline. Six of 10 patients with congenital heart disease and all of 12 patients with bronchial asthma had bronchial hyper-responsiveness to histamine. The mean of histamine concentration was 2750 micrograms/mL and 937 micrograms/mL, respectively. During the histamine inhalation test, respiratory resistance gradually increased in congenital heart disease patients. This was measured by the linear slope of transcutaneous oxygen pressure (-1.08 +/- 0.75 mmHg/min), whereas in the bronchial asthma patients it rapidly decreased at the infection point (-4.19 +/- 1.86 mmHg/min). We conclude that children with congestive heart failure had bronchial hyper-responsiveness. We suggest bronchial hyper-responsiveness to inhaled histamine in congestive heart failure was caused by the gradual increased respiratory resistance, which was different from that of bronchial asthma.

Blood Gas Monitoring, Transcutaneous↗
PubMed1996

Serum N-terminal proatrial natriuretic factor in children with congenital heart disease.

An objective and simple method of establishing and grading heart failure in children is needed. The N-terminal of the atrial natriuretic factor prohormone, called proANF, is stable in vitro, relatively easy to measure and has been demonstrated as a clinically useful marker of heart failure in adults. We measured proANF in 62 children with congenital heart disease and in 62 age-matched controls, in order to examine the relationship of proANF to different clinical and haemodynamic parameters. Echo Doppler cardiography was performed in all children, and 29 also underwent cardiac catheterization. The children were classified for volume and pressure load in each cardiac chamber, for shunt size and for signs of heart failure. In paediatric patients without cardiac or renal disease, median proANF was 384 pmol.l(-1). In children with congenital heart disease, median proANF was 904 (200-5320) pmol.l(-1) (P < 0.001). The three groups with the highest proANF levels were children with documented high atrial pressure (median proANF 1885 pmol.l(-1)), a large left to right shunt (median proANF 1565 pmol.l-(1)) and moderate or severe heart failure (median proANF 1305 pmol.l(-1)). Furthermore, the proANF level correlated negatively with age and glomerular filtration rate. We conclude that elevation of the proANF level is related to atrial pressures, heart failure and a high pulmonary to systemic flow ratio. These findings make proANF a potential new diagnostic tool in heart disease in children.

Adolescent↗
PubMed1991

Congenital heart disease.

The clinical usefulness of M-mode and two-dimensional echocardiography has been proven over the years. M-mode echocardiography allows an 'ice-pick' sampling of the heart, but in conjunction with two-dimensional echocardiography, assessments of cardiac structure and, to some extent, myocardial function can be appreciated. The limitations of these techniques include difficulty in 1) diagnosing subtle congenital heart disease and 2) estimating the severity of valvular lesions (stenosis and regurgitation). Doppler echocardiography overcomes these limitations by detecting abnormal direction, velocity, and turbulence of the blood flow (Chapter 2). This chapter will focus mainly on the two-dimensional and M-mode echocardiographic features of common congenital heart disease.

Animals↗
PubMed1994

Self expanding stents in congenital heart disease.

OBJECTIVES: To show potential indications for and clinical use of self expanding stents in patients with congenital heart disease. DESIGN: Descriptive study of selected, non-randomised patients with balloon expandable but persistent stenoses in whom the use of a balloon expandable stent was considered suboptimal or impossible. SETTING: A tertiary referral centre. PATIENTS: 10 patients, aged 15 days to 32 years. Six patients after atriopulmonary or cavopulmonary anastomosis, two with pulmonary atresia and multiple aortopulmonary collaterals, one with coarctation of the aorta, and one with congenital pulmonary venous stenosis. RESULTS: Successful, uncomplicated, placement of 10 stents with the desired haemodynamic results, even in tortuous vessels with many stenoses. CONCLUSIONS: Self expanding stents have a role in the treatment of patients with congenital heart disease. They should be considered when a smaller delivery system and flexibility would aid placement, or in the treatment of long or multiple sequential stenoses.

Adolescent↗
PubMed1994

Fungal sternal wound infection due to Curvularia lunata in a neonate with congenital heart disease: case report and review.

We describe a neonate with congenital heart disease in whom a sternal wound infection caused by the filamentous fungus Curvularia lunata developed following cardiac surgery. Despite their widespread distribution in the environment, Curvularia species rarely cause human infection. We also review the 43 cases of curvularia infection previously reported in the English-language literature; only four of these cases occurred in children. A wide spectrum of infections--including keratitis, cutaneous infections, sinusitis, allergic bronchopulmonary disease, pneumonia, chronic ambulatory peritoneal dialysis-related infections, endocarditis and disseminated infections--have been described. Curvularia is a pathogen that can cause disease in both immunocompetent and immunocompromised hosts, although more severe and disseminated disease occurs in patients with defective immune function. Surgery alone usually is successful for treating locally invasive disease, although a combination of medical and surgical therapy is necessary for treating disseminated infections.

Fatal Outcome↗
PubMed2003

Role of echocardiography versus MRI for the diagnosis of congenital heart disease.

During the last few decades, significant strides have been made in the field of noninvasive imaging for the patient with congenital heart disease. Echocardiography and MRI continue to provide improved means of anatomic and functional assessment in children and adults with congenital heart lesions. This review reports some of the recent advances in tissue Doppler, strain rate, and integrated backscatter, and highlights exciting current and future potential developments in their application. We also discuss advances in MR in evaluation of cardiac anatomy and function in congenital heart disease.

Adult↗