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Pulmonary valve incompetence. II. Application of electromagnetic flow velocity catheters in children.

During cardiac catheterization in 49 children regurgitant flow through competent and incompetent pulmonary valves was evaluated with electromagnetic flow velocity catheters using the ratio of the separately integrated backward and forward flow velocity signals (vb/vf). When the pulmonary valve was normal, vb/vf was less than or equal to 0.10. After operation for pulmonary valve stenosis or tetralogy of Fallot larger values were often found. In patients with an outflow patch across the pulmonary valve ring vb/vf values of up to 0.54 were found.

Adolescent↗

Recent advances in surgery of ongenital heart disease.

In the cyanotic group palliative procedures for transposition of the great arteries are frequently life-saving in infancy, and the definitive operations such as the atrial baffle, and the Rastelli procedure for those with ventricular septal defect and pulmonic stenosis, are now firmly established. In tetralogy of Fallot shunting procedures continue to be employed in infancy and early childhood, and the complete repair is usually done after the age of five. Corrective operations for total anomalous venous return may have to be staged, and the results are more satisfactory in older children. The various forms of endocardial cushion defects can usually be recognized accurately preoperatively, and where the normal anatomical relationships can be restored, excellent results obtained. Brilliant operative success can now be had in some forms of truncus arteriosus and double outlet right ventricle. It is quite common to find congenital heart disease in adults, frequently after many years of having been treated as rheumatic heart disease. The operative risk in this group is less than 10 percent, and in most instances such patients are restored to their normal physiological age after operation.

Arteriovenous Anastomosis↗

[Valvuloplasty of congenital aortic stenosis].

While percutaneous transluminal valvuloplasty has largely replaced open commissurotomy for congenital pulmonary valve stenosis, the experience with valvuloplasty for congenital aortic stenosis is limited. Between August, 1984 and June, 1987, a total of 27 valvuloplasties were carried out in 25 patients with congenital aortic stenosis; nine infants and 16 patients ranging in age from four to 25 years (mean age 13 +/- 6 years). The indication for valvuloplasty was established on the basis of congestive heart failure or severe stenosis in infants and in the presence of a pressure gradient in excess of 60 mm Hg in those older than one year of age. In eight infants, valvuloplasty reduced the transvalvular pressure gradient from 73 +/- 5 to 36 +/- 9 mm Hg (p less than 0.01; Figure 1). In the 16 patients older than one year of age, the pressure gradient was reduced from 93 +/- 25 to 49 +/- 15 mm Hg (p less than 0.01; Figure 2). The size of the balloon chosen was based on two-dimensional echocardiographic measurements of the aortic ring diameter from inner edge to inner edge. In infants, coronary artery dilating catheters and balloon catheters with an inflated diameter of 4.2 to 8 mm were employed; in children, the balloon diameter selected was 1 to 3 mm less than that of the valve ring diameter, in 15 cases a single-balloon catheter and in one a trefoil catheter. After puncture of the femoral artery and retrograde advancement of a guide-wire into the left ventricle, the balloon catheter was positioned via the guide-wire across the aortic valve.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Successful palliation of the "absent" pulmonary valve syndrome by banding of the pulmonary trunk.

The "absent" pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary trunk, stenosis of the ventriculo-arterial junction with or without malalignment of the outlet septum, and ventricular septal defect. When the outlet septum is malaligned, the morphology resembles that of tetralogy of Fallot. We report our experience with 4 infants with this syndrome. All were in severe respiratory distress and cardiac failure when first seen. Cardiac catheterization was performed at 0.5-4.5 months of age in 3 of them. In the other, the clinical and echocardiographic features were considered sufficient to establish the diagnosis. Banding of the pulmonary trunk was carried out at the age of 2.5-5 months. The distal pulmonary arterial pressure in 3 cases dropped to 12-19 mm Hg. These patients could be extubated within one week postoperatively. Their course 1-3 years later is excellent, with rare episodes of mild respiratory problems only and markedly diminished pulmonary insufficiency. One child, weighing 3250 g at surgery, whose pulmonary arterial pressure did not drop below 29 mm Hg, could not be weaned off the respirator. Corrective surgery was undertaken 17 days later, but the patient died of respiratory complications. Based on clinical and Doppler sonographic findings, on control catheterization data and on haemodynamic findings in 3 surviving infants and two further patients with an uneventful course who, as yet, have not undergone surgery, we conclude that the beneficial effect of banding is the combined result of reduced pulmonary arterial pressure and decreased pulmonic regurgitation.

Child, Preschool↗

[Abnormalities of the atrial septum in adults: kind, prevalence and clinical relevance].

UNLABELLED: To classify interatrial septal anomalies in adults, 24,458 Doppler-echocardiographic studies performed between 1. 1. 1987 and 31. 12. 1992 were reviewed. Patients below 16 years of age or with complex congenital heart disease, or after surgical closure of an atrial septal defect, were excluded. Additionally, all 823 transesophageal echocardiographies done between 1. 1. 1993 and 31. 12. 1994 were analyzed to see whether a patent foramen ovale was present. Among 294 patients with interatrial septal anomalies (prevalence 1.2%; male:female = 1:1.4), 298 congenital anomalies of the interatrial septum were detected. 63% of interatrial septal anomalies constituted newly detected anomalies. Age ranged from 16 to 84 (median 43) years. In 21% of the patients the left-to-right shunt was > or = 50% (QP/QS > or = 2). In 25% pulmonary hypertension was present and 25% of the patients underwent surgery. A patent foramen ovale was present in 0.16% of all transthoracal and in 5.7% of all transesophageal echocardiographies. Secundum defects constituted 46%, atrial septal aneurysms 24%, patent foramen ovale 13%, atrioventricular canals 11%, and superior sinus venosus defects with anomalous pulmonary venous return 6% of all diagnoses. Associated anomalies were mitral valve prolapse in 14%, pulmonary valve stenosis in 3%, left-sided persistent vena cava superior and Chiari network in 2% each, anomalous pulmonary venous return, ventricular septal defect, bicuspid aortic valve, and Ebstein's anomaly in 1% each. 4% of the patients showed further cardiac lesions. CONCLUSIONS: Anomalies of the interatrial septum in adults were frequent and often newly detected. In a high percentage of patients with interatrial septum the left-to-right shunt was hemodynamically relevant.

Adolescent↗

Maximal blood flow velocities--haemodynamic data obtained noninvasively with CW Doppler.

With continuous wave (CW) Doppler the maximal velocity in intracardiac jets can be recorded and simultaneous imaging makes the localization of these jets easier and unambiguous. The image may indicate the angle to the velocity, but the Doppler signal should be used to obtain the smallest angle and the highest velocity. Corrections for an assumed angle should be avoided since this may cause overestimation. With the maximal velocity a pressure drop can be calculated across obstructions and between chambers in regurgitations and shunts. The combined anatomic and haemodynamic information obtained with simultaneous two-dimensional echo and CW Doppler provides a good noninvasive assessment of most congenital and valvular lesions, including prosthetic valves.

Aortic Valve Insufficiency↗

Cardiovascular malformations among preterm infants.

OBJECTIVE: Preterm birth and cardiovascular malformations are the 2 most common causes of neonatal and infant death, but there are no published population-based reports on the relationship between them. We undertook this study to determine the prevalence and spectrum of cardiovascular malformations in a preterm population, the prevalence of prematurity among infants with cardiovascular malformations, and the influence of prematurity and cardiovascular malformations on outcomes. METHODS: We based the study on the population of the former Northern Health Region of England. We identified all live-born infants with cardiovascular malformations diagnosed in the first 1 year of life from the regional pediatric cardiology database, which includes the gestational age and details of the diagnosis. We limited ascertainment to malformations diagnosed by the age of 12 months. Infants with isolated patent ductus arteriosus or atrial septal defect were excluded, to avoid ascertainment bias. Infants with ventricular septal defect were classified according to whether they required surgery in the first 1 year. There are no population data on gestational ages for all births in our population for the era of this study; therefore, we used data published in the literature for populations similar to our own to predict that 0.4% of live births occur at <28 weeks of gestation, 0.9% at 28 to 31 weeks, and 6% at 32 to 36 weeks. Overall, 7.3% of live-born infants are preterm. RESULTS: Of 521619 live-born infants in 1987-2001, 2964 had cardiovascular malformations (prevalence: 5.7 cases per 1000 live births). Cardiovascular malformations were present at 5.1 cases per 1000 term infants and 12.5 cases per 1000 preterm infants. The odds ratio (OR) for a cardiovascular malformation in prematurity was 2.4 (95% confidence interval [CI]: 2.2-2.7). We found that 474 infants (16%) with cardiovascular malformations were born at <37 weeks of gestation, giving an OR for prematurity among infants with a cardiovascular malformation of 2.4 (95% CI: 2.2-2.7). More infants were born preterm with diagnoses of pulmonary atresia with ventricular septal defect (23%), complete atrioventricular septal defect (22%), and coarctation of the aorta, tetralogy of Fallot, and pulmonary valve stenosis (each 20%). Fewer were born preterm with diagnoses of pulmonary atresia and intact ventricular septum (7%), transposition of the great arteries (8%), and single ventricle (9%). We found that 18% of infants with ventricular septal defect requiring surgery were preterm, compared with 13% in the nonsurgical group. Preterm infants with ventricular septal defect required surgery in 30% of cases, compared with 23% of term infants with ventricular septal defect. These figures show that the excess of cardiovascular malformations among preterm infants cannot be explained by greater ascertainment of minor ventricular septal defects. In our denominator population, 646 live-born infants were recognized as having trisomy 21, and gestational age data were available for 609. Of these, 149 (25%; 95% CI: 21-28%) were preterm. Approximately two thirds of infants with complete atrioventricular septal defect have trisomy 21. Complete atrioventricular septal defect was no more common among preterm infants with trisomy 21 (16%) than among term infants with trisomy 21. However, the increased incidence of prematurity among infants with trisomy 21 probably explains some of the excess of preterm births among infants with complete atrioventricular septal defect. Only 4 (11%) of 38 infants with 22q11 deletion were born preterm. None of those infants had pulmonary atresia with ventricular septal defect; therefore, 22q11 deletion does not explain the excess of preterm births in pulmonary atresia with ventricular septal defect. The OR for death in the first 1 year in the presence of a cardiovascular malformation was 4.4 (95% CI: 3.1-5.5) overall; ORs were 1.8 at <28 weeks of gestation, 3.7 at 28 to 31 weeks, 11.0 at 32 to 36 weeks, and 35.6 at term. CONCLUSIONS: This study showed that preterm infants have more than twice as many cardiovascular malformations as do infants born at term and that 16% of all infants with cardiovascular malformations are preterm. It also showed, not surprisingly, that there is an increased mortality rate among infants born preterm with a cardiovascular malformation. The additional effect of cardiovascular malformations on mortality rates is most marked for term and near-term infants, for whom mortality rates are otherwise low. The excess of cardiovascular malformations among preterm infants is intriguing but not easy to explain. Previous studies of birth weight among infants with cardiovascular malformations reported a significant increase in the likelihood of being small for gestational age among infants with tetralogy of Fallot, complete atrioventricular septal defect, hypoplastic left heart, or large ventricular septal defect. There is an obvious relationship between birth weight and gestational age, and those studies also showed an increased prevalence of prematurity among infants with tetralogy of Fallot, pulmonary stenosis, aortic stenosis, coarctation of the aorta, complete atrioventricular septal defect, or ventricular septal defect. There is also a high prevalence of cardiovascular malformations among late stillbirths, with major differences in the number and spectrum of cardiovascular malformations, compared with those seen in postnatal life. In particular, there is a greater incidence of coarctation of the aorta, double-inlet left ventricle, hypoplastic left heart, truncus arteriosus, double-outlet right ventricle, and atrioventricular septal defect among stillbirths. This spectrum of malformations is similar to that in our study and to those in other reports. Whether the increased prevalence of cardiovascular malformations among preterm infants and the increase in stillbirths suggest clues to the cause is difficult to say. The influence of preterm birth should be taken into account in risk assessment and risk stratification for surgical repair.

Heart Defects, Congenital↗