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PubMed1987

Malformation patterns in children with congenital heart disease.

We prospectively examined 1016 infants and children with congenital heart disease (CHD) to detect additional malformation patterns. They were divided into two major groups. In the first group (n = 881), the CHD either occurred alone or was accompanied by a major extracardiac malformation (n = 68, 7.7%). In the second group (n = 135, 13.3%), the CHD was part of a malformation syndrome, embryopathy, association, or complex. In one fourth of the 56 chromosomal syndromes, the underlying cytogenetic anomaly differed from trisomy 21. Fourteen of the 30 children with non-chromosomal malformation syndromes had Noonan's syndrome. Thirteen of the 27 embryopathies were due to rubella infection, but alcohol embryopathy occurred nearly as often (n = 10). In those children with malformation associations (n = 16) seven showed cardiofacial association. Three of the five children with malformation complexes had Ivemark disease. In this study, underlying disorders were found in one of eight children with CHD, a considerably higher percentage than that reported in earlier studies.

Abnormalities, Multiple↗
PubMed1986

Congenital heart disease in supernumerary der(22),t(11;22) syndrome.

Congenital heart disease occurred in 62% of the reported cases of supernumerary der(22) syndrome. These were most commonly acyanotic lesions such as atrial septal defect, ventricular septal defect or patent ductus arteriosus. Heart disease did not, however, appear to be a major determinant of survival.

Child↗
PubMedDate not supplied

MR imaging of congenital heart diseases in adolescents and adults.

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

Adolescent↗
PubMed2000

Epidemiological characteristics of congenital heart diseases in Londrina, Paraná south Brazil.

OBJECTIVE: To determine the prevalence and other epidemiological characteristics of congenital heart diseases. METHODS: A retrospective population based study of children who were born in Londrina, from January '89 to December '98 (80,262 live births). Diagnoses were confirmed through autopsy, surgery, catheterization, or echocardiography. RESULTS: A total of 441 patients was as certain what corresponds to a prevalence of 5.494:1,000 live births. Ventricular septal defect was the commonest lesion. A small number of transpositions of the great vessels and of left ventricular hypoplasia was observed. A high propation of ventricular septal defect (28.3%) and atrioventricular septal defects (8.1%) occurred. Fifty-one (11.35%) affected children had syndromic diseases and 52 (12.01%) children had nonsyndromic anomalies. CONCLUSION: The prevalence of congenital heart diseases in Londrina is in accordance with that of other regions of the globe. This prevalence also may reflect the reality in the southern region of Brazil, because population characteristics are very similar in the 3 southernmost Brazilian states.

Brazil↗
PubMed1983

Evidence of congenital heart disease in the offspring of parents with atrioventricular defects.

Fifty-two of the 56 offspring from 90 patients with proven atrioventricular defects were examined. There were five with congenital heart disease. The defects were concordant in three and discordant in two. The mother was the affected propositus in all cases. In this relatively small sample the incidence of congenital heart disease in the offspring of parents with atrioventricular defects was 9.6%, or 14.3% if only the female propositi were considered. This is a much higher figure than that reported for the simple forms of isolated congenital heart disease.

Adult↗
PubMed2001

Three-dimensional distribution of bipolar atrial electrogram voltages in patients with congenital heart disease.

Voltage differences might be used to distinguish normal atrial tissue from abnormal atrial tissue. This study was aimed at identifying lowest voltage areas in patients with atrial tachycardia after surgical correction of congenital heart disease and to evaluate if identification of these areas in diseased hearts facilitates selection of critical conduction pathways in reentrant circuits as target sites for catheter ablation. Ten patients (four men, age 39 +/- 15 years) with normal sized atria and atrioventricular reciprocating tachycardia (control group) and ten patients (5 men, 32 +/- 7 years) with congenital heart disease and postoperative atrial tachycardia (CL 281 +/- 79 ms) referred for radiofrequency catheter ablation were studied. Mapping and ablation was guided by a three-dimensional electroanatomic mapping system (CARTO) in all patients. In the control group, voltage maps were constructed during sinus rhythm and during tachycardia to evaluate the voltage distribution. The amplitude of bipolar signals was 1.90 +/- 1.45 mV (0.11-8.12 mV, n = 660) during sinus rhythm and 1.45 +/- 1.66 mV (0.12-5.83 mV, n = 440, P < 0.05) during atrioventricular reciprocating tachycardia. In the study group, the amplitude of 1,962 bipolar signals during tachycardia was 1.01 +/- 1.19 mV (0.04-9.40 mV), which differed significantly from the control group during tachycardia (P < 0.0001). No significant difference in the tachycardia cycle length was found (P < 0.05) between the control and study groups. As the lowest voltage measured in normal hearts was 0.1 mV, this value was used as the upper limit of the lowest voltage areas in the patients with congenital heart disease. These areas were identified by detailed voltage mapping and represented by a gray color. Activation and propagation maps were then used to select critical conduction pathways as target sites for ablation. These sites were characterized by fragmented signals in all patients. Ablation resulted in termination of the tachycardia in eight (80%) of ten patients. Complications were not observed. Identification of the lowest voltage areas using a cut-off value of 0.1 mV in congenital heart disease patients with postoperative atrial reentrant tachycardia facilitated the selection of critical conduction pathways as target sites for ablation.

Adult↗
PubMed2000

Pattern of congenital heart disease and access to tertiary cardiac care in Malaysia.

This prospective, descriptive study was carried out to determine the pattern and the type of congenital heart disease seen in the Paediatric Departments in 4 government hospitals. The accessibility of surgical or transcatheter interventional treatment was also assessed. Consecutive new patients seen for suspected congenital heart disease from 1/1/95 till 31/12/95 (Group I) were registered. Records of patients seen from 1/1/95 to 31/8/95 (Group Ia) were reviewed 6 months after presentation to determine the nature of treatment received. Group II were cardiac patients who were first seen prior to the year 1995 and had undergone cardiac surgery or transcatheter interventional procedures in 1995. Of the 375 patients registered in the 4 hospitals, 250 were new patients and 125 were patients first seen prior to 1995 and had surgery or transcatheter interventional procedures in 1995. Of the 250 new patients, 83.2% had acyanotic cardiac lesions and 16.8% had cyanotic lesions. Ventricular septal defect was the commonest lesion, constituting 37.2%, followed by patent arterial duct (18.8%) and atrial septal defects (9.6%). At 6 month review, access to close-heart surgery or transcatheter interventional treatment were readily available. However, for patients with ventricular septal defects, 42.1% who required closure were still waiting for surgery 6 months after presentation. Of the 4 patients with Fallot's Tetralogy who required surgery, 2 had modified Blalock-Taussig shunt and 2 were awaiting surgery. In 1995, a total of 204 cardiac procedures were performed, there were 114 (55.9%) open heart procedures, 50 (24.5%) surgical ligation of the arterial duct, 28 (13.7%) modified Blalock-Taussig shunts, 11 (5.4%) transcatheter duct occlusion and 1 (0.5%) balloon valvuloplasty. The mean age of Fallot's Tetralogy repair was 6.4 years. No corrective surgery was performed for patients with complex cardiac lesions from the 4 hospitals in 1995. In conclusion, the pattern of congenital heart disease was similar to that seen world wide. Except for patent arterial duct, access to surgical treatment was inadequate.

Health Services Accessibility↗
PubMed1953

Congenital heart disease cyanotic children.

Cyanosis is often the only apparent symptom of congenital heart disease for which a child is brought to a physician. Some of the more common anomalies can be diagnosed from this and other symptoms by a general practitioner. Squatting after exertion is a sign of tetralogy of Fallot; severe disability with relatively mild cyanosis may indicate pure pulmonary stenosis. A brisk, short, rasping systolic murmur is characteristic of these conditions and of tricuspid atresia. Tetralogy of Fallot is further symptomatized by a boot-shaped heart, not greatly enlarged, and right axis deviation on electrocardiograms. Typically the lung fields are clear. The author's treatment of choice is aortic-pulmonary or subclavian-pulmonary anastomosis as indicated, preferably done after the child is three years old if the condition is not so severe as to require earlier operation. Pure pulmonary stenosis, which in some cases cannot be distinguished from tetralogy of Fallot except by cardiac catheterization and angiocardiography, may in more typical cases be diagnosed by convexity rather than concavity in the pulmonary segment and by differences in electrocardiograms. An expanding valvulotome is used to open the stenosed pulmonary valve, which is then dilated.A systolic murmur, a round heart and left axis deviation are usually found in tricuspid atresia. Shunt operations performed for relief of this condition may lead to later heart failure because of the devious rerouting of blood through the heart. The operations here outlined and others are statistically evaluated.

Aorta↗
PubMed1980

Lip pits, cleft lip and/or palate, and congenital heart disease.

Three individuals in whom lip pits and cleft lip and/or cleft palate were found in association with congenital heart disease are described. These individuals did not have the characteristics of previously delineated syndromes in which lip pits occur, including Vanderwoude syndrome, popliteal pterygium syndrome, and orodigitofacial dysostosis. Literature review failed to show any previously reported cases in which pits were observed in association with congenital heart disease.

Cleft Lip↗
PubMed2006

[Application of homologous conduits with valves in the treatment of complex congenital heart disease with insufficient pulmonary blood flow in children].

OBJECTIVE: To investigate the effects of homologous conduits with valves in the treatment of complex congenital heart disease with insufficient pulmonary blood flow in children. METHODS: Homologous conduits with valves were used in the right ventricular outflow tract reconstruction among 212 pediatric patients with complex congenital heart disease with insufficient pulmonary blood flow, aged 2.6 (0.5 - 15). The patients were followed up for 2.6 years (6 months to 7 years) and underwent echocardiography to assess the degree of pulmonary insufficiency and calcification of blood vessel. RESULTS: The success rate of operation was 96.5% (204/212). The ratio of right ventricle to aortic pressure was less then 0.55. During the follow-up, echocardiography showed valvular regurgitation and calcification of the blood vessel in 34 patients, 23 (11.2%) being mild, 7 (3.42%) being moderate, and 4 (1.96%) being sever. 170 patients did not show obvious pulmonary insufficiency. CONCLUSION: Application of homologous conduits with valves in the treatment of complex congenital heart disease with insufficient pulmonary blood flow in children shows excellent early results in preventing pulmonary insufficiency.

Adolescent↗
PubMed2001

Fetal diagnosis and management of congenital heart disease.

Fetal echocardiography has had a significant impact on the diagnosis and management of congenital heart disease. Although the fetal circulation is quite forgiving for many heart lesions, other flow abnormalities gravely affect fetal physiology. Serial studies have given us a window to observe the natural history of many lesions that progress during gestation. Although fetal cardiac surgery is not yet a viable alternative to neonatal repair, fetal intervention on noncardiac disorders has had limited success and the field is growing. Fetal diagnosis remains an integral part of the management of children with cardiac lesions.

Disease Progression↗
PubMed2001

Genetic assembly of the heart: implications for congenital heart disease.

More children die from congenital heart defects (CHD) each year than are diagnosed with childhood cancer, yet the causes remain unknown. The remarkable conservation of genetic pathways regulating cardiac development in species ranging from flies to humans provides an opportunity to experimentally dissect the role of critical cardiogenic factors. Utilization of model biological systems has resulted in a molecular framework in which to consider the etiology of CHD. As whole genome sequencing and single nucleotide polymorphism data become available, identification of genetic mutations predisposing to CHD may allow preventive measures by modulation of secondary genetic or environmental factors. In this review, genetic pathways regulating cardiogenesis revealed by cross-species studies are reviewed and correlated with human CHD.

Animals↗
PubMed1987

Left-to-right shunt assessed by thermodilution during surgery for congenital heart disease.

In nine children with congenital heart defects, left-to-right shunting was assessed during open-heart surgery by injecting cold 5% glucose solution through a left atrial line and detecting temperature change with a pulmonary arterial thermistor. The shunt was quantified by recording the temperature after injecting the same amount of cold glucose solution also through a central venous line. Oximetric determination of the left-to-right shunt was used as a reference. The result was expressed as shunt flow divided by pulmonary blood flow (Qshunt/Qp). The regression equation then obtained was Qshunt/Qp by thermodilution = 0.05 + 0.87 x Qshunt/Qp by oximetry. The residual standard deviation around the line of regression was 0.03. The thermodilution technique can be used for rapid assessment of the presence and magnitude of left-to-right shunting, provided that there is opportunity for the indicator to mix adequately in the main blood stream before shunting occurs.

Blood Pressure↗
PubMed1990

[Follow-up of surgically treated congenital heart disease using Doppler echocardiography].

Surgery can restore normal cardiac anatomy in some forms of congenital heart disease but in others this is not possible even after a "complete correction". In yet other forms, surgery is only palliative. Echocardiography coupled with pulsed, continuous wave and color coded Doppler should be carried out after clinical, electrocardiographic and radiological examination to assess the surgical results and detect possible complications in the short or long-term. It is therefore important to be familiar with the "normal" appearances of operated congenital heart disease in order to be able to detect postoperative abnormalities. The authors describe the normal postoperative findings for each form of congenital abnormality and the changes which may be observed during Doppler echocardiographic examination. This paper only deals with the more simple malformations: ventricular septal defect, atrial septal defect, atrioventricular canal, patent ductus arteriosus, coarctation of the aorta, aortic stenosis, tetralogy of Fallot and simple transposition of the great vessels.

Aortic Coarctation↗
PubMedDate not supplied

Myocardial dysfunction and depressed fatty acid metabolism in patients with cyanotic congenital heart disease.

BACKGROUND: Myocardial contractile dysfunction has been frequently observed in adolescents or adults with cyanotic congenital heart disease. Impaired energy metabolism may be present in such dysfunctional myocardium. METHODS AND RESULTS: To evaluate the findings of myocardial free fatty acid metabolism, and its relations to ventricular wall motion and myocardial perfusion in cyanotic congenital heart disease, we performed a combined study of iodine 123-labeled 15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) scintigraphy, thallium scintigraphy, and contrast cine-angiography in seven patients with single right or left ventricle. The results showed that wall motion was reduced in 17 of 35 ventricular segments (49%), which were mostly identical in location to decreased BMIPP uptake. The severity of BMIPP uptake deficit correlated positively with the degree of impairment of wall motion. On the other hand, thallium uptake was abnormal only in 5 of 35 segments (14%), and the severity of the perfusion defect did not correlate with the degree of wall motion abnormality. CONCLUSIONS: Contractile dysfunction in cyanotic heart disease was primarily linked to impaired free fatty acid metabolism rather than to myocardial scar as represented by perfusion defect on thallium imaging.

Adolescent↗
PubMed2005

Congenital heart disease and pregnancy: maternal and fetal risks.

Women with congenital heart disease (CHD) now comprise most patients with heart disease seen during pregnancy, accounting for 80% of all patients. In general, pregnancy is well tolerated in patients with CHD. For some women with particularly high-risk lesions and poor functional class, however, pregnancy poses significant risk for cardiovascular complications, including premature death. As result, preconception counseling and risk stratification are mandatory and should be done in all women of childbearing age with CHD.

Female↗
PubMed1998

Right-sided maze procedure for right atrial arrhythmias in congenital heart disease.

BACKGROUND: Atrial fibrillation and flutter, commonly associated with congenital heart anomalies that cause right atrial dilatation, may cause significant morbidity and reduction of quality of life, even after surgical repair of the anomalies. METHODS: In an effort to reduce the incidence of atrial tachyarrhythmias after repair of right-sided congenital heart disease, we performed a concomitant right-sided maze procedure. RESULTS: Eighteen patients with paroxysmal atrial fibrillation or flutter (n = 12) or chronic atrial fibrillation or flutter (n = 6) aged 10.9 to 68.4 years (mean 34.9 years) underwent a right-sided maze in association with repair of Ebstein's anomaly (n = 15), congenital tricuspid insufficiency (n = 2), and isolated atrial septal defect (n = 1). There were no early deaths, reoperations, or complete heart block. Discharge rhythm was sinus (n = 16) or junctional (n = 2). Follow-up was complete in all 18 patients and ranged from 3.1 to 17.2 months (mean 8.1 months); all are in New York Heart Association class I. Early postoperative arrhythmias developed in 3 patients (all were converted to sinus rhythm by antiarrhythmic drugs). There were no late deaths or reoperations. CONCLUSIONS: The inclusion of a right-sided maze procedure with cardiac repair in patients having congenital heart anomalies that cause right atrial dilatation and associated atrial tachyarrhythmias is effective in eliminating or reducing the incidence of those arrhythmias.

Adolescent↗