Further experiences with microplethysmography in the study of congenital heart disease.
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Heart Defects, Congenital↗
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Cardiac catheterization in the diagnosis of congenital heart disease.
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Cardiac Catheterization↗
Congenital heart diseases in the feebleminded.
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Heart Defects, Congenital↗
[Transcatheter therapy of combined congenital heart diseases in children].
OBJECTIVE: To investigate the methods of interventional catheterization for combined congenital heart disease and to evaluate its efficacy in children. METHODS: From March 1994 to December 2003, 15 cases (6 boys, 9 girls) underwent transcatheter intervention for combined congenital heart diseases. The procedure of transcatheter intervention was as follows: for pulmonary stenosis (PS) and atrial septal defect (ASD) or patent ductus arteriosus (PDA), PBPV first, occlusion of ASD or PDA later; for coarctation of aorta (COA) and PDA, dilation of COA first, occlusion of PDA 4-15 months later; for aortic stenosis (AS) and PDA, PBAV first, occlusion of PDA later; for ventricular septal defect (VSD) and PDA, all occlusions with detachable coils. RESULT: Transcatheter intervention for combined congenital heart diseases was successful in all patients. There was no residual shunt after occlusion immediately apart from 2 cases of PDA which were little residual after occlusion immediately. Follow-up for (3.57 +/-2.61) years, the systolic pressure gradients across pulmonary valve and coarctation were normal by ultrasonic or transcatheter, except AS. There was 3 cases presented postoperative complications: 1 with mechanical haemolysis, 1 with fall off of coil and 1 with arterial embolism, respectively. CONCLUSION: Transcatheter intervention for combined congenital heart diseases could obtain satisfactory results with appropriate indications and procedure manipulations.
Abnormalities, Multiple↗
Antenatal diagnosis of congenital heart disease and Down's syndrome: the potential effect on the practice of paediatric cardiology.
OBJECTIVE: To predict the effect of antenatal ultrasound screening for congenital heart disease and maternal serum screening of Down's syndrome on the practice of paediatric cardiology and paediatric cardiac surgery. DESIGN: A retrospective and prospective ascertainment of all congenital heart disease diagnosed in infancy in 1985-1991. SETTING: One English health region. PATIENTS: All congenital heart disease diagnosed in infancy by echocardiography, cardiac catheterisation, surgery, or necropsy was classified as "complex", "significant", or "minor" and as "detectable" or "not detectable" on a routine antenatal ultrasound scan. RESULTS: 1347 infants had congenital heart disease which was "complex" in 13%, "significant" in 55%, and "minor" in 32%. 15% of cases were "detectable" on routine antenatal ultrasound. Assuming 20% detection and termination of 67% of affected pregnancies, liveborn congenital heart disease would be reduced by 2%, infant mortality from congenital heart disease by 5%, and paediatric cardiac surgical activity by 3%. Maternal screening for Down's syndrome, assuming 75% uptake, 60% detection, and termination of all affected pregnancies, would reduce liveborn cases of Down's syndrome by 45%, liveborn cases of congenital heart disease by 3.5%, and cardiac surgery by 2.6%. CONCLUSIONS: Screening for congenital heart disease using the four chamber view in routine obstetric examinations and maternal serum screening for Down's syndrome is likely to have only a small effect on the requirements for paediatric cardiology services and paediatric cardiac surgery.
Cardiac Surgical Procedures↗
Evolution of surgical trends in congenital heart disease: a population based study.
The aim of this study was to establish surgical trends in patients with congenital heart disease operated on between 1947 and 1997 in a population based study. All patients diagnosed as having congenital heart disease, born in Malta up to 1995 inclusive and operated for congenital heart disease up to 1997 inclusive were included. Analysis was carried out for lesions operated, age at surgery, operative centre and mortality rates, in the setting of a regional hospital providing congenital heart disease diagnostic and follow-up services for all Malta. Increasingly more operations for cardiac malformations are being carried out, with a progressively higher proportion of operations performed on complex conditions (P<0.001), at an ever younger age (P<0.001), and with a declining perioperative mortality (P<0.001). For the period 1990-1994, 4.2 operations for congenital heart disease/1000 live births were required. Factors which may increase or decrease this rate in future are discussed, along with costs of surgery. Surgery for congenital heart disease has become progressively more aggressive and safer since this method of treatment for these malformations was initiated, but this has occurred at a significant financial cost.
Adolescent↗
GATA4 haploinsufficiency in patients with interstitial deletion of chromosome region 8p23.1 and congenital heart disease.
Previous studies have shown that patients with deletion of distal human chromosome arm 8p may have congenital heart disease and other physical anomalies. The gene encoding GATA-4, a zinc finger transcription factor implicated in cardiac gene expression and development, localizes to chromosome region 8p23.1. To examine whether GATA-4 deficiency is present in patients with monosomy of 8p23.1 with congenital heart disease, we performed fluorescence in situ hybridization (FISH) with a GATA4 probe on cells from a series of patients with interstitial deletion of 8p23.1. Four individuals with del(8)(p23.1) and congenital heart disease were found to be haploinsufficient at the GATA4 locus by FISH. The GATA4 gene was not deleted in a fifth patient with del(8)(p23.1) who lacked cardiac anomalies. FISH analysis on cells from 48 individuals with congenital heart disease and normal karyotypes failed to detect any submicroscopic deletions at the GATA4 locus. We conclude that haploinsufficiency at the GATA4 locus is often seen in patients with del(8)(p23.1) and congenital heart disease. Based on these findings and recent studies showing that haploinsufficiency for other cardiac transcription factor genes (e.g., TBX5, NKX2-5) causes congenital heart disease, we postulate that GATA-4 deficiency may contribute to the phenotype of patients with monosomy of 8p23.1.
Adult↗
Diagnosis of tachycardia mechanism with cryomapping in a toddler with complex congenital heart disease.
A 2.5-year-old patient with complex congenital heart disease involving dextrocardia, atrioventricular and ventriculoarterial discordance, pulmonary stenosis, ventricular septal defect (VSD), atrial septal defect (ASD), and paroxysmal supraventricular tachycardia (SVT) underwent electrophysiological study. The tachycardia mechanism was diagnosed with cryomapping. The ability of cryomapping to have transient and reversible effect on the tissue, unlike radiofrequency (RF) ablation, helped in the establishment of diagnosis in this toddler with typical atrioventricular nodal reentrant tachycardia. Cryomapping can be an additional safe diagnostic utility in young patients with complex congenital heart disease.
Catheter Ablation↗
[Surgical treatment of newborns with congenital heart diseases].
OBJECTIVE: To summarize the experience of surgical treatment of newborns with congenital heart diseases. METHODS: The experience of surgical treatment of 8 newborns with critical congenital heart diseases, including 3 cases of D-transposition of great arteries with intact ventricular septum, 2 cases of ventricular septal defect with artrial defect (ASD), one case of complete atrioventricular canal defect, 1 case of obstructed supracardiac total anomalous pulmonary venous drainage with ASD, and 1 case of patent ductus arteriosus (PDA). RESULTS: The case of PDA underwent ligation under normothermic anesthesia and the other 7 cases were operated upon under moderate or deep hypothermic cardiopulmonary bypass. All the 8 cases were observed in ICU for 2 approximately 10 days, and were discharged 7 approximately 15 days after operation. The follow-up after discharge showed a satisfying outcome. The postoperative complications included low cardiac output, infection of mediastinum, and respiratory distress syndrome. CONCLUSION: The critical and complex congenital heart diseases should by diagnosed as early as possible and emergency operation is effective and feasible.
Cardiac Surgical Procedures↗
Is the severity of congenital heart disease associated with the quality of life and perceived health of adult patients?
OBJECTIVE: To explore whether the severity of congenital heart disease is associated with the quality of life and perceived health status of adult patients. DESIGN: Descriptive, cross sectional study. SETTING: Adult congenital heart disease programme in one tertiary care centre in Belgium. PATIENTS: 629 patients (378 men, 251 women) with a median age of 24 years. MAIN OUTCOME MEASURES: Disease severity was operationalized in terms of initial diagnosis (classification of Task Force 1 of the 32nd Bethesda Conference), illness course (disease severity index), and current functional status (New York Heart Association (NYHA) class, ability index, congenital heart disease functional index, and left ventricular ejection fraction). Quality of life was measured by a linear analogue scale, the satisfaction with life scale, and the schedule for evaluation of individual quality of life. Perceived health status was also assessed with a linear analogue scale. RESULTS: Scores derived from the disease severity classification systems were weakly negatively associated with quality of life and health status, ranging from -0.05 to -0.27. The NYHA functional class and ability index were consistently associated with quality of life and perceived health. CONCLUSIONS: This study showed that the severity of congenital heart disease is marginally associated with patients' quality of life and perceived health. Functional status was more related to patients' assessment of their quality of life than was the initial diagnosis or illness course.
Adolescent↗
Impact of antenatal screening on the presentation of infants with congenital heart disease to a cardiology unit.
AIM: Antenatal diagnosis of congenital heart disease (CHD) facilitates prenatal treatment and optimal perinatal care. This has been demonstrated to improve perinatal mortality and morbidity in neonates with CHD. Thus, antenatal diagnosis of CHD is most likely to benefit patients who require surgery in early infancy. We aimed to examine the frequency of antenatal diagnosis in neonates presenting to The Royal Children's Hospital severe CHD. METHODS: Main outcome measures were antenatal diagnosis and whether the individual lesion would have been expected to be detected on a four-chamber view or four-chamber and outflow tract view during a routine obstetric anomaly ultrasound. Poisson regression was used to estimate the average trend over the study period. RESULTS: A total of 610 patients met the inclusion criteria, of whom 164 had an antenatal diagnosis (26.8%). If routine ultrasound screening was ideal, we would have expected 63.9% of cases to be detected on four-chamber view and 83.6% on four-chamber and outflow tract view. Trend analysis demonstrated an annual rate of improvement of 9% in actual versus expected antenatal diagnosis of CHD. Malformation-specific analysis showed that antenatal detection was the highest for double inlet/outlet ventricle (51.3%, 95% confidence interval 34.8-67.6%) and the lowest for simple transposition of the great arteries (15.6%, 95% confidence interval 9.0-24.5). CONCLUSION: Despite mass screening for congenital malformations in Victoria with routine antenatal ultrasounds, a large proportion of neonates with severe congenital heart disease still present without an antenatal diagnosis.
Cardiology Service, Hospital↗
One day old infant with acyanotic congenital heart disease: critical aortic stenosis.
Congenital aortic stenosis accounts for about 5% of cardiac malformations recognized in childhood. It belongs to the category of acyanotic congenital heart disease. These lesions produce a load on the heart because of left ventricular outflow tract obstruction. Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory collapse. We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. When oxygen was given, the ductus started to close, with a worsening of the left sided output and subsequent acidosis. With the right to left shunt across the ductus, the baby was cyanotic and dependent on prostaglandin to keep the ductus open. There was minimal flow across the aortic valve because of the stenosis and extremely poor left ventricular function prior to surgery. After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis.
Adult↗
Congenital heart disease in adolescents and adults. Pregnancy and contraception.
As the detection and treatment of congenital heart disease improves, more women are reaching childbearing age. The type of congenital heart disease, whether there has been corrective surgery, and the patient's functional status determines the maternal as well as fetal outcome. The patient may present to her physician for preconceptual counseling or late in her pregnancy with little care and in severe distress. These possible situations necessitate that the physician becomes familiar with congenital heart disease and its implications in the pregnant patient.
Contraception↗
Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis.
BACKGROUND: As prenatal diagnosis of congenital heart disease has gained in popularity, the questions of whether prenatal diagnosis of congenital heart disease is beneficial for the patient and whether fetal echocardiography has improved the prognosis of congenital heart disease are arising. METHODS: We compared four patients with prenatally diagnosed hypoplastic left heart syndrome (HLHS) with 10 patients of non-prenatally diagnosed HLHS from the view points of (i) age at transfer to our Children's Hospital; (ii) whether the oxygen was inhaled during perinatal period; (iii) whether prostaglandin E1 was administered in the period of waiting before operation; (iv) whether the patient had ductal shock; (v) timing of operation; and (vi) surgical outcome. RESULTS: The timing of the transfer to our Children's Hospital was earlier in prenatally diagnosed group than in non-diagnosed group. Oxygen was not given to any of the patients in prenatally diagnosed group. In contrast, oxygen inhalation was given in two of 10 patients in the non-prenatally diagnosed group. Prostaglandin E1 was administrated in three of four patients in the prenatally diagnosed group and seven of 10 patients in the non-prenatally diagnosed group. In terms of ductal shock, none of the patients in prenatally diagnosed group had ductal shock. However, four of 10 patients had ductal shock in the non-prenatally diagnosed group. The median age at Norwood operation was 7 days in the prenatally diagnosed group; however, it was 19 days in non-prenatally diagnosed group. Surgical outcomes showed no significant changes between the two groups. CONCLUSIONS: Prenatal diagnosis of HLHS was surely beneficial for preventing ductal shock and for keeping the patients' preoperative condition good.
Echocardiography, Doppler↗
B-type natriuretic peptide levels in congenital heart disease.
The objective of this study was to evaluate the potential role of B-type natriuretic peptide (BNP) levels in children with congenital heart disease undergoing cardiac catheterization. Measurement of plasma BNP concentration has been shown to be useful in the diagnosis, risk stratification, and management of adult patients with congestive heart failure, but little is known about the role of BNP in children with structural congenital heart disease. We measured plasma BNP levels using the Triage BNP test in patients with congenital heart disease referred for diagnostic or interventional cardiac catheterization. Plasma BNP concentration was measured in 96 children and 11 adults > or = 19 years old (7.9+/-8.3 years) undergoing heart catheterization for underlying congenital heart disease. BNP levels ranged from < 5 to > 1300 pg/ml, with a median BNP concentration of 19.0 pg/ml. Baseline BNP concentrations were > 100.0 pg/ml on 19 occasions in 17 patients. The pressure difference between the left ventricle and ascending aorta was 10-110 mmHg in 21 patients. BNP concentrations for this cohort ranged from < 5.0 to 1060.0 pg/ml and correlated with the degree of left ventricular outflow obstruction (correlation coefficient, 0.661; p = 0.001). This study suggests that with additional research, BNP concentration may prove to be a useful clinical tool in managing children and adults with congenital heart disease.
Adolescent↗
Effect of premedication on arterial oxygen saturation in children with congenital heart disease.
The effect of a standardized intramuscular premedication (morphine, 0.1 mg/kg, scopolamine, 13 micrograms/kg, and secobarbital, 2.5 mg/kg) on the arterial oxygen saturation of hemoglobin was evaluated in 33 patients with congenital heart disease by use of the Nellcor pulse oximeter. Sixteen patients had noncyanotic congenital heart disease and 17 patients had cyanotic congenital heart disease. In the noncyanotic congenital heart disease group, pulse oximeter saturations decreased from 98.1% +/- 1.5% (mean +/- SD), before premedication, to 96.5% +/- 1.5% following premedication. Although this decrease was statistically significant (P less than 0.05), it was determined to not be clinically meaningful. In the patients with cyanotic congenital heart disease, oxygen saturation increased from 73.5% +/- 11.8 to 74.7% +/- 10.2 following premedication, but this change was not statistically significant. The effect of premedication on SaO2 was highly variable in patients with cyanotic heart disease; although the group mean appeared to increase, 6 of the 17 patients had decreases in saturation and the decrease exceeded 10% in saturation in 3 of them. Therefore, oxygen saturation should be monitored following premedication in patients with cyanotic heart disease and oxygen administered as needed.
Body Weight↗
The endothelin antagonist BQ123 reduces pulmonary vascular resistance after surgical intervention for congenital heart disease.
OBJECTIVE: Postoperative pulmonary hypertension in children after surgical intervention for congenital heart disease has been attributed to failure of the pulmonary endothelium to provide adequate vasodilation. Although we have shown that the impaired vasodilatory component attributable to the l-arginine-nitric oxide pathway is almost completely reversible, a nonrestorable component persists, implying an additional vasoconstrictive mechanism in postoperative pulmonary endothelial dysfunction. In this study of children after surgical intervention for congenital heart disease, we measured endothelin-1 levels and used BQ123, a selective endothelin-A receptor antagonist, together with inhaled nitric oxide to discriminate dysfunctional pulmonary endothelial vasodilation from endothelin-mediated pulmonary vasoconstriction. METHODS: All children were examined early after surgical intervention in the intensive care unit. Pulmonary vascular resistance (with respiratory mass spectrometry), as well as arterial and venous endothelin-1 levels (measured by means of a quantitative enzyme-linked immunosorbent assay), were determined in 7 children (age range, 3.3-13.7 months; median age, 6.3 months) with intracardiac shunting defects at baseline and during ventilation with a fraction of inspired oxygen of 0.65, with additional BQ123 (0.1 mg/kg infused over 20 minutes), and with inhaled nitric oxide (20 ppm). RESULTS: Pulmonary vascular resistance decreased from 7.7 +/- 3.4 at baseline to 6.1 +/- 2.8 Woods units. m(-2) (P =.022) at a fraction of inspired oxygen of 0.65 and to 4.7 +/- 2.7 Woods units. m(-2) (P =.013) during BQ123 infusion. Inhaled nitric oxide had no further effect on pulmonary vascular resistance. Left atrial endothelin-1 levels (1.35-5.12 pg/mL; mean, 2.4 pg/mL) correlated significantly with the decrease in pulmonary vascular resistance in response to BQ123 infusion (r(2) = 0.89, P =.003). CONCLUSION: Postoperative elevation of pulmonary vascular resistance in children after surgical intervention for congenital heart disease is responsive to endothelin-A blockade with BQ123. Increased levels of endothelin-1 predict the response to this therapy, which might become an important addition to the clinical armamentarium in postoperative pulmonary hypertensive disease.
Adolescent↗
Oxygen desaturation during sleep in infants and young children with congenital heart disease.
Oxygen saturation (SaO2) during sleep and pulmonary functions were evaluated in 19 infants with congenital heart disease, aged 6 +/- 4 months, and in 11 normal infants, aged 8 +/- 5 months, to determine whether infants with congenital heart disease have more frequent oxygen desaturation during sleep and, if so, its relationship to underlying pulmonary function. Infants with congenital heart disease were classified as acyanotic (n = 11) or cyanotic (n = 8) on the basis of their aortic SaO2 at the time of cardiac catheterization (greater or less than 90% SaO2). Pulmonary function tests included respiratory rate, functional residual capacity, total respiratory system compliance, and maximal flows at functional residual capacity. Significant differences were found in the values for the lowest SaO2 of each 5-minute epoch (SaO2L) averaged during the entire sleep time (normal 94% +/- 2%, acyanotic 90% +/- 3%, and cyanotic 74% +/- 4%; p less than 0.01). The three groups also differed significantly in frequency distributions of percentage of total sleep time with SaO2L less than 90% (SaO2%T) (normal 10% +/- 17%, acyanotic 36% +/- 34%, and cyanotic 97% +/- 4%; p less than 0.05). Compared with the control group, the acyanotic group had a higher respiratory rate (66 +/- 19 breaths/min vs 35 +/- 6 breaths/min; p less than 0.01), a lower tidal volume (65% +/- 29% predicted vs 105% +/- 18% predicted; p less than 0.01), and a lower total respiratory compliance (59% +/- 18% predicted vs 106% +/- 30% predicted; p less than 0.01). A negative correlation existed between SaO2%T and aortic SaO2 (R2 = 0.64; p less than 0.01). We conclude that oxygen desaturation occurs during sleep in infants with congenital heart disease; the presence of desaturation appears to be related to the initial degree of hypoxemia and the presence of abnormal pulmonary function.
Cyanosis↗