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PubMed1999

Gross and fine motor development is impaired in children with cyanotic congenital heart disease.

Many factors may intervene with the motor development of children with congenital heart disease (CHD). Children aged 5 to 14 years with various CHD were examined for disturbances of gross and fine motor development using motometric tests and compared with 30 healthy controls. The results of the Körperkoordinationstest für Kinder (KTK) (a body coordination test for children) for gross motor development were significantly lower in patients with uncorrected cyanotic CHD (motor quotient MQ 74.8 +/- 11.7, mean +/- 1 standard deviation, n = 16) and after corrective surgery (MQ 81.2 +/- 16.6, n = 25) than in controls (MQ 102.8 +/- 11.8, n = 30). No relationship between these results and the cardiopulmonary exercise capacity was found. In patients with cyanotic CHD, significant deficits in fine motor development were present before corrective surgery (e.g. Zielpunktiertest [dotting] MQ 87.7 +/- 9.9 vs. 106.5 +/- 10.8), but already two years afterwards the results reached nearly normal values (MQ 97.1 +/- 17.0). In contrast, children with acyanotic CHD demonstrated normal gross and fine motor development. These results indicate that long-standing hypoxemia in infancy must be considered as an important cause of the pronounced motor disturbances. Early neurological evaluation of these children and a specialized motor physiotherapy are recommended.

Adolescent↗
PubMed2005

Congenital heart disease: a retrospective study of their frequency.

AIM: An early diagnosis of congenital heart disease (CHD) is necessary for the obstetrical management. METHODS: One thousand five hundred and fifty-six pregnant women underwent a fetal 2-D echocardiography from 1991 to 2002. We assessed patients who had the most common risk factor (RF): family history of CHD, diabetes mellitus, teratogen drugs, polyhydramnios, abnormal fetal growth, fetal arrhythmia, maternal age over 40 years, maternal autoimmune disease, maternal disease contracted during pregnancy, oligohydramnios, only umbilical artery, uncorrected visualization in the first level 2-D fetal echocardiography. RESULTS: In 110 morphological anomalies found 54 were complex. The malformations are not equally distributed among the different RF. The most common RF is the family history of CHD where 24 patients showed complex malformations. Another high percentage of complex malformations was found in patients with no apparent presence of RF: the diagnosis was done after an uncorrected cardiac visualization in the first level 2-D echocardiography. In the minor cardiomyopathies we did not find the prevalence of any RF. After diagnosis of complex malformations we had 15% of termination of pregnancies, 37% of the newborns are alive and in good health. CONCLUSIONS: The first RF is the family history of CHD, but a correct first level fetal 2-D echocardiography is necessary because a very high percentage of complex malformations is seen in infant whose mother did not have any RF. Ninety percent of cardiac malformations where seen in a 4 chamber view, and it is of the utmost importance also for a correct diagnosis of minor cardiomyopathies where we did not find a predominant RF.

Adult↗
PubMed1997

Platelet activation in congenital heart diseases.

The research presented here investigated platelet activation in cyanotic and acyanotic congenital heart diseases (CHD). Children with cyanotic CHD are prone to both thrombosis and hemorrhage. However, patients with acyanotic CHD may also have a mild bleeding disorder. The platelet activation in CHD was investigated in support of a hypothesis that platelet activation may play a role in the hemostatic abnormalities reported in these patients. Platelet activation was determined by using flow cytometry with anti-CD62 monoclonal antibody (mAb), which has been shown to be a specific marker of platelet activation. Thirteen children with cyanotic CHD, 33 children with acyanotic CHD and 17 healthy children serving as controls were studied. Platelet activation was significantly higher in the cyanotic group and also in the acyanotic group compared with the healthy children (P = 0.0000 and P = 0.019, respectively). In the cyanotic group, platelet activation showed a direct correlation with arterial O2 saturation (SaO2) (P = 0.014). There was no correlation between platelet activation and erythrocyte related parameters in either group. Platelet activation occurs in CHD, particularly in patients with cyanotic CHD (even in patients with no evidence of clinical thrombosis) and it may play a role in the pathogenesis of thrombotic disorders seen in these patients.

Child, Preschool↗
PubMed1987

Central aorta-pulmonary artery shunts in neonates with complex cyanotic congenital heart disease.

Methods of palliating critical pulmonary oligemia in neonates with complex cyanotic congenital heart disease continue to evolve. Pulmonary artery distortion and other complications of the use of native vessels to increase pulmonary blood flow has led to the more frequent use of polytetrafluoroethylene shunts either in a central position or as a modified Blalock-Taussig shunt. Central aorta-pulmonary artery shunts have largely fallen into disfavor because of previously reported unacceptably high incidences of complications such as shunt thrombosis, congestive heart failure, and pulmonary artery distortion. This report details our experience palliating 23 neonates with pulmonary atresia or severe pulmonary stenosis by placing central aorta-pulmonary artery shunts utilizing a short segment (less than 1 cm) of polytetrafluoroethylene. Although three of the 23 died postoperatively, none of the 23 patients had evidence of shunt thrombosis. Congestive heart failure, a potential complication of any pulmonary artery shunt, was present in eight of the 20 survivors but did not require shunt takedown and was readily controlled by digoxin. Repeat catheterization was performed in 12 patients; pulmonary angiography showed good growth of both pulmonary arteries and there was no evidence of pulmonary artery hypertension. Although minor pulmonary artery distortion was present in two patients, this distortion was centrally located and easily remedied at the time of total correction. Thus we have found the central aorta-pulmonary artery shunt to be an extremely effective and reliable means of palliating pulmonary artery hypoplasia as a result of pulmonary atresia or severe pulmonary stenosis in neonates.

Angiocardiography↗
PubMedDate not supplied

A clinico-hematological study of iron deficiency anemia and its correlation with hyperviscosity symptoms in cyanotic congenital heart disease.

The present study was carried out in 33 cases of cyanotic congenital heart disease (CCHD) to determine the prevalence of iron deficiency anemia (IDA) and its correlation to hyperviscosity symptoms in terms of hematocrit levels. Furthermore, the study was aimed at assessing the response to low dose iron therapy (60 mg of elemental iron once daily) in relieving symptoms of hyperviscosity. All these cases were evaluated for presence of symptoms of hyperviscosity and later subjected to various hematological & biochemical parameters of iron deficiency anemia including hemoglobin (Hb), pack cell volume (PCV), mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), serum iron, total iron binding capacity respectively. Results showed presence of IDA in 6/33 cases (18.2%) and hyperviscosity symptoms in 10/33 cases (30.3%). Amongst the group with symptoms of hyperviscosity, in the subset having IDA these symptoms were observed at PCV levels of 0.52 L/L to 0.58 L/L in contrast to the subset not deficient in iron where the symptoms occurred at a PCV 0.68 L/L. Relief of symptoms of hyperviscosity was evident with a minimal rise of mean hemoglobin by 2.1 gm/dl. It was concluded that IDA was not an uncommon finding in CCHD cases and that it leads to symptoms of hyperviscosity at a level of PCV much lower than those known to produce these symptoms. Finally low dose iron therapy was found effective in relieving the symptoms of hyperviscosity.

Adolescent↗
PubMed2005

Prospective trial of electroanatomically guided, irrigated catheter ablation of atrial tachycardia in patients with congenital heart disease.

BACKGROUND: Ablation success rates reported for atrial tachycardia (AT) patients with congenital heart disease (CHD) is lower than the rates reported for other varieties of supraventricular tachycardia. Retrospective studies suggest these rates might be increased by the use of irrigated radiofrequency (RF) ablation. OBJECTIVES: The purpose of this study was to determine whether irrigated RF ablation increases ablation success rates in patients with CHD and AT. METHODS: Patients were studied in a prospective, randomized, nonblinded manner. The operator was limited to use of randomized therapy (standard or irrigated ablation) for the first 6 minutes of RF application to each targeted arrhythmia. Lesion characteristics were recorded, and acute ablation success was ascertained. Structured clinical follow-up was performed over a 6-month period. RESULTS: Forty-seven ATs were targeted in 26 patients; 72% of these ATs were ablated. Within the 6-minute randomization period, no difference in success rates of standard and irrigated catheters was noted. However, crossover from standard to irrigated ablation more likely was successful than vice versa (irrigated: 8 successes/8 attempts vs standard: 1 success/4 attempts, P = .018), and overall success was greater using irrigated catheters (66% vs 33%, P = .019). Mean delivered power was slightly higher in irrigated lesions (32.5 W vs 30.2 W, P = .025), and mean temperature was much lower (33.5 degrees C vs 59.3 degrees C, P < .001). A composite AT intensity score was significantly reduced compared with preablation values at 6-month follow-up of all patients. CONCLUSIONS: Ablation of ATs in patients with CHD results in symptomatic improvement over short-term follow-up. Irrigated ablation may result in higher acute success rates in these patients.

Adolescent↗
PubMed2002

Genetic counseling in congenital heart disease.

Thousands of infants are born in the United States every year with congenital heart defects. Initially, the family is saddened, angry, and concerned about treatment and survival. Eventually, however, questions will emerge about what happened and what the chances for recurrence of another affected child are. In recent years impressive progress has been made in genetic research at the chromosomal and molecular level into the underlying causes for some cardiovascular abnormalities. This challenges health care providers to keep up with new discoveries in the genetics of congenital heart disease to provide patients and families with the correct information or appropriate referrals for genetic evaluations.

Chromosome Aberrations↗
PubMed1984

[Thallium-201 myocardial imaging in patients with congenital heart disease following open-heart surgery].

Thallium-201 myocardial imaging was performed for 77 patients with congenital heart disease to evaluate myocardial damage following open-heart surgery. There were 23 patients with ventricular septal defects, 33 with tetralogy of Fallot, 12 with atrial septal defects, and nine with other diseases. The imaging results were compared with the angiographic and electrocardiographic findings. Patchy perfusion defects or images of poor thallium uptake were noted in five of 77 patients, among whom four had accompanying serious ventricular arrhythmias. This suggested a close correlation of the post-operative ventricular arrhythmias with myocardial damage. For 20 of 77 patients, vents were inserted through the left ventricle, and for the remaining 57 through the left atrium. Perfusion defects were found at the apex in three, and left ventriculography showed a paradoxical motion of the apex in five in patients whose vents were inserted through the left ventricle. Sensitivity and specificity in detecting myocardial damage of the apex were 60% and 98%, respectively. In one case, myocardial infarction was demonstrated immediately after the repair of the ventricular septal defect. Myocardial imaging was useful in evaluating the infarcted area and for follow-up study. Thus, thallium-201 myocardial imaging was regarded useful for assessing myocardial damage following open-heart surgery in congenital heart disease patients.

Adolescent↗
PubMed1988

Plasma atrial natriuretic peptide in patients with congenital heart diseases.

The plasma level of human alpha-atrial natriuretic peptide was measured in healthy children and patients, 1 month to 15 years of age, with congenital heart diseases. Significant increases were found in patients with a ventricular septal defect, tricuspid valve atresia, patent ductus arteriosus, and atrial septal defect but not in those with pulmonary valve stenosis or tetralogy of Fallot. The levels were significantly higher in children with ventricular septal defects (221 +/- 123 pg/mL) or patent ductus arteriosus (124 +/- 38 pg/mL) than in those with atrial septal defects (65 +/- 42 pg/mL) (P less than .01). The increased levels appeared to be correlated with enlargement of the left atrium (r = .85, P less than .01) but not with the right atrial size or the mean right atrial pressure. They were higher in younger than in older healthy infants, but this age difference did not affect the results. These findings indicate that human alpha-atrial natriuretic peptide is released into the circulation in response to chronic atrial expansion in patients with congenital heart disease and may have an important role in volume homeostasis.

Adolescent↗
PubMed1996

Air travel and adults with cyanotic congenital heart disease.

BACKGROUND: Concern has been expressed that a reduction of partial oxygen pressure during flight in commercial aircraft may induce dangerous hypoxemia in patients with cyanotic congenital heart disease. METHODS AND RESULTS: To evaluate the validity of this concern, the transcutaneous SaO2 was measured in 12 adults with this type of heart disease and 27 control subjects during simulated commercial flights of 1.5 and 7 hours in a hypobaric chamber. Ten of those patients and 6 control subjects also were evaluated during two actual flights of approximately 2.5 hours in a DC-10 and an A-310, respectively. During the prolonged simulated and actual flights, the capillary blood pH, gases, and lactic acid were analyzed in the patients and during one of the actual flights also in the control subjects. During the simulated flights the SaO2 was at all times lower in the patients than in the control subjects. However, the maximal mean actual percentage decrease, as compared with sea level values, did not exceed 8.8% in either patients or control subjects. During the actual flights, this maximal decrease in the patients was 6%. In-flight reduction of the capillary PO2 was considerable in the control subjects but not in the patients. It is our hypothesis that the lack of a significant decrease of the PO2 in the patients might possibly be due to a high concentration of 2.3 diphosphoglycerate in the red cells. The flights had no influence on the capillary blood pH, PCO2, bicarbonate, or lactic acid levels in either patients or control subjects. CONCLUSIONS: Atmospheric pressure changes during commercial air travel do not appear to be detrimental to patients with cyanotic congenital heart disease.

2,3-Diphosphoglycerate↗
PubMed1999

Radiographic evaluation of the neonate with congenital heart disease.

Remarkable advances in pediatric cardiology have been spurred by the explosion of technologies both in interventional and surgical techniques and the ability to manipulate the genome of experimental animals. After a brief discussion concerning the striking advances in the molecular understanding of congenital heart disease, this article focuses on clues to the diagnosis of congenital heart disease and on chest radiography and common, specific lesions of the neonate such as hypoplastic left heart, transposition of the great vessels, and severe tetralogy of Fallot. The impact of treatment protocols involving interventional cardiology in the neonate also are discussed.

Heart Defects, Congenital↗
PubMed1998

Technical considerations for valve repair in patients with congenital heart disease.

Valve repair is a critical element in the surgical treatment of congenital heart disease. Growing children require a longitudinal management strategy not commonly necessary in adult cardiac surgery. The use of valve prostheses poses unique problems in children, not only due to size limitations, but also to difficulties with chronic medical management. For these reasons, considerable effort is given to repairing even severely malformed valves in pediatric patients. This article provides a brief overview of some commonly used techniques in reparative valve surgery in children.

Aortic Valve Insufficiency↗
PubMed1990

[Hemodynamic effects of nifedipine on pulmonary hypertension secondary to congenital heart disease with shunts].

Ten patients (pts) with pulmonary hypertension (PAH) secondary to congenital heart diseases (CHD) with left to right and bidirectional shunts were studied by right heart catheterization. After getting the control hemodynamic data, pts were given nifedipine (NIF) capsule 10-20 mg sublingually and their pulmonary artery pressure (PAP), systemic blood pressure (SBP), heart rate (HR) were monitored for 60 minutes and the hemodynamic data were redetermined. The results showed that the PAP as well as SBP was decreased and the quantity index of left to right shunt (L-R QI) was also reduced by NIF in L-R shunt group (3/10). In bidirectional shunts group (7/10), 4/7 cases showed total pulmonary artery resistance index (TPRI), pulmonary artery resistance index (PARI) were decreased by 29% (P less than 0.01) and 30% (P less than 0.05) respectively without seriously increased R-L shunts. The other 3/7 pts' TPRI, PARI were increased while systemic vascular resistance index (SVRI) was lowered by 41% (P less than 0.05). The quantity index of pulmonary blood flow (Qlp) was decreased by 30 (P less than 0.05) quantity index of right to left shunt R-L QI was increased sharply by 138% (P less than 0.05) while L-R QI decreased by 76% (P less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

[Atrio-ventricular disorders following surgery for congenital heart diseases with septal defect (author's transl)].

Post-operative results in congenital heart diseases with septal defect are often darkened by the occurence of disorders in atrio-ventricular conduction. The incidence of these disorders is dependent upon the location of the septal defect, the anatomy of conduction pathways and the operative technique. Intracavitary recording of His bundle activity leads to a more precise localisation of the trouble. Such disorders are more frequent after cure of Fallot's tetralogy than after closure of an interventricular communication. They are rare after closure of an interatrial septal defect and more common after closure of the atrioventricular canal, where they are of atrial node type. The occurrence of an atrioventricular block, even transient, is of poor prognosis, and in case of proximal lesion the insertion of a pacemaker must be considered. The high incidence of conduction disorders justifies continuous per-operative recording and prolonged surveillance, if necessary by Holter's method. Locating the conductive tissue during surgery reduces the frequency.

Atrioventricular Node↗
PubMed1995

[Nutritional status of children with congenital heart disease and left-to-right shunt. The importance of the presence of pulmonary hypertension].

PURPOSE: To assess the nutritional status of children with congenital heart disease with left-to-right shunt and the nutritional disturbances related to the presence of pulmonary hypertension (PH). METHODS: In a prospective study, the nutritional status of 30 children with left-to-right shunt (16 with and 14 without PH) was determined by anthropometric parameters and plasma proteins albumin, transferrin and prealbumin. Frequency and extent of nutritional disturbances were analysed in relation to the presence of PH. A control group of 20 well-nourished children was formed, aiming to compare the values of the serum proteins. RESULTS: The nutritional classification according to Waterlow's criteria showed an overall prevalence of malnutrition of 83.3%, which was higher in those with PH (p = 0.0140). This patient's group was more wasted than the group without PH (median z score of weight for height -1.65 vs -0.78). There was a high occurrence of measurements of arm fat area below the 5th percentile, which were similar in both groups. Serum levels of albumin and prealbumin were significantly lower in children with congenital heart disease than in the control group and transferrin values were similar in both. CONCLUSION: The prevalence of malnutrition was high. The presence of PH was associated with higher nutritional disturbance. The nutritional assessment may be a good way to identify diagnostic groups at particular risk of failure to thrive. This can be useful in planning a management which ensures these patients to achieve adequate nutritional supplementation in early life.

Child↗
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↗