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PubMed1995

Serial cardiorespiratory exercise testing in patients with congenital heart disease.

Aerobic capacity of patients with different forms of congenital heart disease was serially evaluated in 79 patients and the evolution was correlated with the lesion and the level of daily activity. The patients were divided into six groups: patients with a small ventricular septal defect (VSD) with mini shunt (n = 14), mild pulmonary valve stenosis with gradient < 40 mm Hg (PS) (n = 12), mild to moderate aortic valve stenosis (gradient 36 +/- 17 mmHg) (AS) (n = 12), patients 4.7 +/- 2.1 years after repair of tetralogy of Fallot (PO-TF) (n = 16), patients 2.2 +/- 2.9 years after closure of a high flow/high gradient VSD (PO-VSD) (n = 13), and patients 2.6 +/- 1.7 years after Fontan repair (Fontan-PO) (n = 12). Aerobic capacity was assessed by determination of the ventilatory anaerobic threshold (VAT). VAT reflects the highest aerobic exercise level prior to a disproportionate increase of CO2 and ventilation relative to O2 uptake; it is independent of patient motivation. Data are expressed as percentage of normal O2 uptake at VAT, determined in 234 age/gender matched controls. The habitual level of physical activity was assessed by a standardised questionnaire. Aerobic capacity in all subgroups of patients, even with very mild defects, was at or below the lower limit of normal. Children left unrestricted from physical exercise (VSD, PS, PO-VSD) had no change over the study period. However, aerobic capacity of patients with medically imposed physical restrictions (AS) and significant residual haemodynamic lesions (PO-TF, Fontan) decreased with age.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2006

Efficacy of nitroglycerin inhalation in reducing pulmonary arterial hypertension in children with congenital heart disease.

BACKGROUND: There has been a renewed interest in nitric oxide donor drugs, such as nitroglycerin, delivered by the inhalational route for treatment of pulmonary arterial hypertension (PAH). We investigated the acute effects of inhaled nitroglycerin on pulmonary and systemic haemodynamics in children with PAH associated with congenital heart disease. METHODS: Nineteen children with acyanotic congenital heart disease and a left to right shunt with severe PAH, undergoing routine diagnostic cardiac catheterization were included in this study. Systolic, diastolic and mean systemic as well as pulmonary artery pressures, right atrial pressure and pulmonary capillary wedge pressure (PCWP) were recorded and systemic vascular resistance index (SVRI) and pulmonary vascular resistance index (PVRI) were calculated at room air, following 100% oxygen as well as after nitroglycerin inhalation in all patients. RESULTS: Systolic, diastolic and mean pulmonary artery pressure and PVRI decreased significantly, whereas heart rate, systolic, diastolic and mean systemic arterial pressure, PCWP and SVRI did not change significantly following 100% oxygen or inhalation of nitroglycerin. CONCLUSION: Inhaled nitroglycerin significantly decreases systolic, diastolic and mean pulmonary artery pressure as well as PVRI without affecting systemic haemodynamics, and thus can be used as a therapeutic modality for acute reduction of PAH in children with congenital heart disease.

Administration, Inhalation↗
PubMed1997

[Cerebral abscess and cyanotic congenital heart disease].

Cerebral abscess is a classical complication of cyanotic congenital heart disease. The authors report 7 cases of cerebral abscess diagnosed since 1982. One asymptomatic patient died of a postoperative cerebral haemorrage. The child was repatriated from Africa for complete correction of his cardiac lesion. The presentation of the other 6 cases was quite typical : headaches, pyrexia and vomiting with a neurological deficit in 4 cases : two hemiparesias and two homonymous lateral hemianopsia. These 6 patients recovered without sequeilae. Four underwent surgical drainage of the abscess with antibiotic therapy. Two recovered with antibiotic therapy alone. The causal organism was only identified in patients undergoing surgical drainage and then only in 3 cases. They were gram positive cocci, in particular the streptococcus. The association ampicillin-chloramphenicol has often been proposed as the treatment of first intention. Adaptation of antibiotic therapy then depends on clinical, biological, bacteriological (CSF, blood cultures, portal of entry) outcomes and the results of CT scanning. The association of a third generation cephalosporin and an imidazole may be proposed as treatment of second intention. The minimal duration of treatment is generally acknowledged to be 4 weeks for intravenous therapy in cases of medical therapy alone, and 2 to 3 weeks in cases with surgical drainage. The age of apparition of this complication seems to be increasing as the average age was 16 in this series (cerebral abscess is classically described as occurring between 8 and 12 years of age). This may be due to palliative surgery which reduces systemic hypoxia and polycythaemia. It also appears that neurological drainage is not systematic now because of early diagnosis of this complication. Finally, in the last few years, a new population of patients is becoming more common : patients repatriated by humanitary organisations in the third world, which should incite great vigilance in the preoperative period in this pathology.

Adolescent↗
PubMed2006

Transitional medicine: will emergency medicine physicians be ready for the growing population of adults with congenital heart disease?

BACKGROUND: Currently, approximately 85% of children with significant congenital heart problems survive to adolescence and adulthood. This survival rate represents a dramatic improvement in the medical and surgical care of congenital heart disease (CHD) during the last 35 years. Nevertheless, these patients remain at increased risk for significant cardiac problems long after primary interventions are completed. They are more likely than the general population to seek urgent medical care, often in an emergency department setting. They represent a new and growing population of emergency department patients with a specialized set of problems not traditionally part of the training for emergency medicine (EM) physicians. OBJECTIVE: We investigated the current scope and status of training for EM physicians in the immediate management of CHD patients as they grow to adolescence and adulthood. METHODS: We conducted 2 cross-sectional surveys to assess the current training environment for 2 specific groups: (1) US general EM (GEM) residency programs, and (2) US and Canadian pediatric EM (PEM) fellowship programs. Surveys were mailed to program directors during the summer of 2005. A total of 198 surveys were sent out: 134 to GEM residency directors and 64 to PEM fellowship directors. RESULTS: The response rate overall was 68%, with a 64% response rate from the GEM residency directors and 77% from the PEM fellowship directors. Across all programs, 43% (56/130 respondents) were "unsure" about the existence or location of an adult CHD (ACHD) clinic in their area. When asked to rate the importance of ACHD as a training topic, 40 (85%) of 47 PEM fellowship directors and 62 (74%) of 84 GEM residency directors ranked it as "low priority" or "unnecessary." However, 70 (55%) of 127 respondents were "unsure," "uncomfortable," or "worried" about the ACHD training their trainees receive (PEM, 59%; GEM, 53%). In addition, most program directors (75%) estimated that their trainees care for 5 or fewer ACHD patients annually. CONCLUSIONS: There seems to be a mismatch between the growing need for ACHD emergency care and the current state of this topic in both GEM residency and PEM fellowship training programs.

Adult↗
PubMed1999

Acute complications in the current era of therapeutic cardiac catheterization for congenital heart disease.

The acute complications of therapeutic cardiac catheterization for congenital heart disease as performed currently in a small unit were reviewed. In recent years, there has been a significant increase in the number of lesions thought amenable to catheter therapy. Only a few reports, however, have addressed the overall incidence of acute complications of therapeutic cardiac catheterization, all representing the experience of centres performing moderate-to-large numbers of procedures. A retrospective review was performed of 425 therapeutic catheter procedures performed at our institution between May 1993 and November 1997. Acute complications were retrieved from the database. This included all adverse events that were clinically recognized at the time of or within 2 weeks after the procedure and which, to the best of the authors' clinical judgement, were related to the catheterization and not part of the natural history of the child's illness. All patients were observed overnight following the procedure, and stayed in hospital if a complication developed. There were 49 acute complications (11.5%), of which 43 (10.1%) were deemed minor and 6 (1.4%) were considered major. The rate was low in patients with valvar pulmonary stenosis, including three neonates (3/45, 6.7%), for those undergoing angioplasty of native co-arctation (1/15, 6.7%) and pulmonary arteries (2/27, 7.4%); and for coil embolization of systemic to pulmonary collateral arteries (1/16, 6.3%). The rate was high in patients with valvar aortic stenosis, including 12 neonates (9/37, 24.3%), and for angioplasty of re-coarctation (4/23, 21.7%). There were more overall complications in neonates (25.6%) than in older patients (10.1%) (p < 0.01). Two patients died (0.5%), but no patient required emergency surgical intervention. In spite of the introduction of many new therapeutic modalities with greater intrinsic risk, and the fact that patients with more complex lesions and who are more acutely ill are being treated, the overall rate of complications remains relatively low. This probably reflects improvements in pericatheterization medical management, in selection of patients, in procedural techniques, and in the experience of operators.

Adolescent↗
PubMed2004

[Diagnostic value of color echocardiography in neonatal cyanotic congenital heart disease].

OBJECTIVE: To study the value of color echocardiography in diagnosis of neonatal cyanotic congenital heart disease (NCHD). METHOD: A total of 149 cyanotic neonates were examined by color echocardiography, of whom 25 were diagnosed having NCHD and analyzed for classification. RESULTS: Such complex deformities as transposition of the major arteries, tetralogy of Fallot, single ventricle, persistent truncus arteriosus were found in these 25 cases of NCHD, of which major artery transposition was the most common defects, followed by tetralogy of Fallot and then by single ventricle. CONCLUSION: Color echocardiography can be used routinely in diagnosis of congenital heart disease of cyanotic neonates.

Echocardiography, Doppler, Color↗
PubMed2000

Factors associated with age at operation for children with congenital heart disease.

OBJECTIVE: Previous studies have shown that children with congenital heart disease (CHD) who live in nonurban areas or who do not have private insurance are at risk for delayed referral to a pediatric cardiologist. However, the effect of these factors on the age at which cardiac surgery is performed has not been evaluated. This study is designed to evaluate the factors that influence the age at which definitive surgical repair is performed. METHODS: Data on hospital discharges for 1995 and 1996 in California were obtained from the Office of Statewide Health Planning and Development database. Children <18 years who underwent surgical repair for atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot (TOF), or atrioventricular canal (AVC) were included in the study. Age at surgery was evaluated using type of CHD, gender, race, type of insurance, surgical centers, urban or rural home location, and distance between home and surgical center as independent variables. RESULTS: In 1995-1996, 666 children underwent ASD closure (mean age: 5.1 years; median: 4.0 years), 582 VSD closure (mean age: 2.8; median: 1.1 years), 394 TOF repair (mean age: 1.7; median:.9 years), and 177 AVC repair (mean age: 1.1; median:.6 years). Comparing median and mean age at surgery, we found: AVC<TOF<VSD<ASD (< indicates younger than). A consistent trend for all 4 types of CHD was seen indicating that for median age at operation: private insurance<managed care<Medicaid. Gender or race had no effect on age at operation, although Asians tended to be older at surgery for all 4 types of CHD. There is a significant negative correlation between the case volume of surgical centers and median age at operation for ASD (r = -.37), VSD (r = -.49), TOF (r = -.63), and AVC (r = -.17). In addition, significant positive correlation was found between degree of urbanization of home locations (measured by population density) and median age at operation for ASD (r =.50), VSD (r =.77), and TOF (r =.18). No significant correlation was found between distance to surgical center and age at operation. CONCLUSIONS: Many medical and nonmedical variables play important roles in determining age for definitive repair of CHD in children. Type of insurance, a recognized surrogate for access to care, may play an important role. In addition, centers with higher surgical case volume were more likely to operate at a younger age. Finally, children in urban areas tend to be older at the time of surgery for ASD, VSD, and TOF.

Age Distribution↗
PubMed2006

Prevalence of congenital heart disease in schoolchildren of sub-Saharan Africa, Mozambique.

A survey conducted by cardiologists in Mozambique provided an opportunity for assessing the prevalence of congenital heart disease in public school children in Maputo. After randomization, a total of 2170 children were examined. Five children had clinical and echographic evidence of congenital heart disease, corresponding to a prevalence of 2.3 in 1000 (95% confidence interval: 1.0 to 5.4), 80% newly discovered during the survey. These findings confirm that congenital heart disease is at least as common in this South African black community as in Caucasians. Early detection of these cardiac diseases in children is important in order to prevent serious complications and to institute endocarditis prevention.

Adolescent↗
PubMed2000

Coronary artery imaging in grown up congenital heart disease: complementary role of magnetic resonance and x-ray coronary angiography.

BACKGROUND: There is a high incidence of anomalous coronary arteries in subjects with congenital heart disease. These abnormalities can be responsible for myocardial ischemia and sudden death or be damaged during surgical intervention. It can be difficult to define the proximal course of anomalous coronary arteries with the use of conventional x-ray coronary angiography. Magnetic resonance coronary angiography (MRCA) has been shown to be useful in the assessment of the 3-dimensional relationship between the coronary arteries and the great vessels in subjects with normal cardiac morphology but has not been used in patients with congenital heart disease. METHODS AND RESULTS: Twenty-five adults with various congenital heart abnormalities were studied. X-ray coronary angiography and respiratory-gated MRCA were performed in all subjects. Coronary artery origin and proximal course were assessed for each imaging modality by separate, blinded investigators. Images were then compared, and a consensus diagnosis was reached. With the consensus readings for both magnetic resonance and x-ray coronary angiography, it was possible to identify the origin and course of the proximal coronary arteries in all 25 subjects: 16 with coronary anomalies and 9 with normal coronary arteries. Respiratory-gated MRCA had an accuracy of 92%, a sensitivity of 88%, and a specificity of 100% for the detection of abnormal coronary arteries. The MRCA results were more likely to agree with the consensus for definition of the proximal course of the coronary arteries (P<0.02). CONCLUSIONS: For the assessment of anomalous coronary artery anatomy in patients with congenital heart disease, the use of the combination of MRCA with x-ray coronary angiography improves the definition of the proximal coronary artery course. MRCA provides correct spatial relationships, whereas x-ray angiography provides a view of the entire coronary length and its peripheral run-off. Furthermore, respiratory-gated MRCA can be performed without breath holding and with only limited subject cooperation.

Adult↗
PubMed2005

Direction, location, and size of shunt flow in congenital heart disease evaluated by ECG-gated multislice computed tomography.

OBJECTIVE: To demonstrate the utility of multislice computed tomography (MSCT) in the evaluation of direction, location, and size of shunt flow in congenital heart disease. BACKGROUND: Transthoracic and transesophageal echocardiography, magnetic resonance imaging, and conventional angiography has been used to evaluate congenital heart disease; however, some are invasive and some do not provide accurate information about the spatial relationship to other organs. METHODS: Three patients with typical presentation of ventricular septal defect, atrial septal defect, and patent ductus arteriosus were recruited. Enhanced MSCT (Light Speed Ultra 16, General Electric) was performed with a 1.25-mm slice thickness, helical pitch 3.25. Following intravenous injection of 100 ml of iodinated contrast material (350 mg I/ml) at a rate of 3 ml/s, CT scanning was performed with retrospective ECG-gated reconstruction at 5 s and 30 s after injection. RESULTS: In all cases the information of direction, location, and size of shunt flow including left-to-right and right-to-left shunt with spatial relationship to other organs could be obtained non-invasively. CONCLUSION: ECG-gated MSCT is a useful tool to evaluate congenital heart diseases with shunts.

Adult↗
PubMedDate not supplied

Heparin need of the patients with cyanotic congenital heart disease during cardiopulmonary bypass. Comparison of cyanotic, acyanotic, rheumatic and atherosclerotic subjects.

Four groups of patients were studied. Group I: Congenital cyanotic heart disease (CCHD), consisting of 24 subjects aged 5 to 28 (1.4); 18 males and 4 females. Group II: Acyanotic congenital heart disease (ACHD), consisting of 34 patients aged 5 to 42 (20.1); 17 males and 17 females. Group III: Rheumatic heart disease (RHD), consisting of 30 patients aged 11-54 (42.4); 9 males and 21 females. Group IV: Atherosclerotic heart disease (AHD), consisting of 35 patients aged 36 to 65 (49.2); 33 males and 2 females. The haematocrit value (Hct) was the highest in the CCHD group. Total amount of heparin (mg/kg) used during cardiopulmonary bypass was 5.4 in CCHD, 4.66 in ACHD, 4.8 in RHD and 4.6 in AHD group. Mean protamine values was 4.02; 4; 4.03; and 4 respectively. Although the difference of Hct value was statistically different between CCHD and RHD group (p less than 0.001), heparin need was not (p less than 0.1). One-way analysis of variance (F test) showed no difference for heparin need between the four groups (F3.119 = 0.64). Prothrombin time (PT) and activated partial thromboplastin time (aPTT) showed a positive correlation (r = 0.36 and r = 0.25) with heparin need in CCHD group but no correlation was found in RHD group.

Adult↗
PubMed1975

Incidence of congenital heart disease in Hungary.

A survey was planned for establishing the point prevalence at birth of congenital heart disease and for assessing at the same time the efficacy of a screening for such anomalies. This model included: (1) exact determination of the population under survey; (2) approximately complete ascertainment of all persons in the population studied; (3) identical principles of cardiologic screening; (4) application of modern cardiologic diagnostic methods, and (5) a sample of over 2,000 persons. 23 cases of congenital heart diseases were found among 2,259 children born 1963 in a defined Hungarian territory. This means a point prevalence of 10.2+/-2.1% live births. (An additional 6 doubtful cases were not taken into account.) From the 12 cases diagnosed in vivo, 10 were detected by the screening.

Child↗
PubMed2006

Pathogenesis of thrombocytopenia in cyanotic congenital heart disease.

Although a significant minority of patients with cyanotic congenital heart disease (CCHD) are thrombocytopenic, the pathogenesis and prevalence have not been established. This study was designed to address these 2 issues. We included 105 patients with CCHD (60 men and 45 women; aged 21 to 54 years). Systemic arterial oxygen saturations were 69% to 78%. Hematocrits were 62% to 74% with normal iron indexes. In 26 of 105 patients (25%), platelet counts were <100x10(9)/L. The diagnosis was Eisenmenger syndrome in all 26 patients with thrombocytopenia. Platelet production was determined by flow cytometric reticulated platelet counts. Megakaryocyte mass was determined indirectly by thrombopoietin levels. Disseminated intravascular coagulation was based on prothrombin time, activated partial thromboplastin time, and D-dimers. Platelet activation was determined by levels of platelet factor 4 and beta thromboglobulin. Reference ranges were derived from 20 normal acyanotic controls. A reduction in absolute reticulated platelet counts implied decreased platelet production (p<0.001). Normal thrombopoietin levels implied normal megakaryocyte mass. Normal prothrombin time, activated partial thromboplastin time, and D-dimers excluded disseminated intravascular coagulation. Normal platelet factor 4 and beta thromboglobulin indicated absent or minimal platelet activation. Twenty-five percent of the patients with CCHD were thrombocytopenic because platelet production was decreased despite normal megakaryocyte mass. We hypothesized that right-to-left shunts deliver whole megakaryocytes into the system arterial circulation, bypassing the lungs where megakaryocytic cytoplasm is fragmented into platelets, thus reducing platelet production. In conclusion, platelet counts in CCHD appear to represent a continuum beginning with low normal counts and ending with thrombocytopenia.

Adult↗
PubMed2004

Major congenital heart disease in Northern Norway: shortcomings of pre- and postnatal diagnosis.

BACKGROUND: Congenital heart disease (CHD) is a significant cause of perinatal mortality and morbidity worldwide. Prenatal detection rate of CHD remains low in most European countries and a substantial proportion of infants with serious heart disease are diagnosed only after discharge from hospital after birth. Earlier recognition of treatable abnormalities may improve the perinatal outcome. The purpose of this study was to evaluate the incidence, pre- and postnatal diagnosis and outcome of major CHD in two northern-most counties of Norway. MATERIALS AND METHODS: All cases of major CHD were identified by reviewing the records of all fetal and neonatal echocardiographies and all perinatal autopsies performed during the study period. Data were obtained on maternal age, associated risk factors, time of diagnosis, type of CHD and outcome. RESULTS: Ninety-one fetal and 52 neonatal echocardiographies and 77 perinatal autopsies were performed during the 2-year study period. There was a total of 5658 births during this period. Twenty-five cases of major CHD were identified. Six of them were diagnosed prenatally, 18 after birth, and one additional case was identified from the perinatal autopsy register. The incidence of major CHD in Northern Norway was 4.4 per 1000 births. The prenatal detection rate was 24% (6/25). In 39% (7/18) of cases diagnosed postnatally the diagnosis was made following a second admission after the baby has been initially discharged from hospital after birth. The mortality among liveborn infants with major CHD was 17.4% (4/23). CONCLUSION: Despite almost universal prenatal ultrasound screening and at least one neonatal examination before discharge, the diagnosis of major CHD is made after a relevant delay in a substantial proportion of cases.

Adult↗
PubMed2006

[Adult congenital heart diseases: physicians facing new difficulties].

Nowadays, 70 to 80% of the patients with congenital heart disease reach adulthood. Transition from pediatric cares to adult medicine is sometimes difficult resulting in loss of follow up, which can be deleterious in young adult with serious disease. Specialized units have been created all around the world and now in Europe. The needs for this population are specific (employment, contraception, pregnancy). Complex operated defects will need specialized assessment and follow up (tetralogy of Fallot, atrial switch for transposition of the great arteries). Arrhythmias are an important cause of admission. Native defect are sometimes revealed in adulthood (simple: asd, arterial duct, complex double discordance, Ebstein disease), or are not operable (mainly cyanotic disease).

Adult↗
PubMed1986

[The contribution of magnetic resonance imaging in congenital heart diseases].

The diagnostic value of magnetic resonance imaging (MRI) was assessed in 30 patients with congenital heart disease, including 7 patients with postoperative sequellae. The images obtained by synchronizing the MRI spectrometer with the electrocardiogramme were recorded in 2 or 3 different planes (sagittal, frontal and transverse) and compared to clinical, angiographic and/or echocardiographic data. The MRI provided high resolution tomographic images enabling spatial reconstitution of the heart by the use of different planes. These images were particularly useful for showing the position and dimensions of the ventricles (both chamber size and wall thickness) and their relationship to the atria and great vessels. These results confirm the value of this new non-invasive imaging technique in the diagnosis of congenital heart disease, not counting the additional information on blood flow and tissue characterisation that will soon become available.

Heart Defects, Congenital↗
PubMed1985

The thymus in congenital heart disease.

Some cases of thymus developmental defects (DiGeorge's syndrome) are associated with the development of defects of the heart and great vessels. To see if anomalies of the heart are also associated with anomalies of the thymus, a material of thymus tissue removed during operation for congenital heart disease was compared with thymus tissue from a forensic autopsy material representing the same age group, but without cardiac lesions. Among 27 cases of congenital heart disease, occurrence in the thymus of solid epithelial cords were seen in 6 and cyst- or duct-like structures were found in 7. Among 47 forensic cases, 1 showed cords and another showed duct-like structures. Of these, one had congenital anomalies in the form of hepatic hamartomas and nesidioblastosis, whereas the other had died from morbilli, raising the possibility of congenital immune deficiency. Anyhow, a striking occurrence of minor morphological deviations in the structure of the thymus was found in children with congenital heart disease. Such material therefore may be less suitable for studying the morphology and biology of the normal thymus gland.

Abnormalities, Multiple↗