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Acute effects of balloon valvuloplasty and pacing on left ventricular performance in children with moderate pulmonary valve stenosis, analysed by systolic and diastolic pressure-volume relationships.

Right ventricular overload of volume and/or pressure type may affect left ventricular systolic and diastolic function. This has been shown in animal studies and has been suggested in non-invasive studies in man. Altered geometry of the left ventricle, myocardial hypertrophy and changes in contractile state may be responsible for the change in function. Balloon valvuloplasty is an effective treatment for isolated valvular pulmonary stenosis in children, and results in an immediate decrease of right ventricular systolic pressure. Whether this results in immediate changes in left ventricular performance is unknown. Eight children (age 5.2 to 13.9 years) with moderate pulmonary valve stenosis underwent pulmonary balloon valvuloplasty under general anaesthesia. Left ventricular function measurements before and after valvuloplasty were performed using a combined micromanometer-conductance catheter to obtain end-systolic (ESPVR) and end-diastolic (EDPVR) pressure-volume relationships employing inferior vena cava occlusion both at normal and pacing-induced increased heart rates. Pulmonary valvuloplasty resulted in a decrease in peak systolic right ventricular pressure from 62.8 +/- 13.5 to 34.4 +/- 7.3 mmHg (P < 0.001), without significant changes in left ventricular systolic and end-diastolic pressure, or in cardiac index. The ESPVR was fitted to a linear function to obtain the slope (Ees) and the volume intercept at 75 mmHg (V75). The EDPVR was fitted to an exponential function. At baseline, Ees was 1.68 +/- 0.99 mmHg.ml-1 and V75 was 33.6 +/- 21.8 ml. Neither valvuloplasty nor pacing, which increased mean heart rate from 81 to 112 beats.min-1 (P < 0.001), resulted in significant changes of the parameters Ees or V75.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Catheterization↗

Coarctation of the aorta associated with pulmonary valve stenosis.

A 7-year-old patient had coarctation of the aorta associated with moderate and dysplastic pulmonary valve stenosis. The child underwent corrective surgery of the coarctation. This unusual association cannot be satisfactorily explained by the theory of fetal flow patterns.

Aortic Coarctation↗

Interruption of aortic arch associated with pulmonary valve stenosis.

A newborn male is described who presented with interruption of aortic arch associated with ventricular septal defect and pulmonary valve stenosis. He underwent total corrective surgery and was discharged with good clinical results. The combination of significant right ventricular outflow obstruction and interruption of aortic arch is incompatible with fetal survival unless there are large collateral arteries which arise early in pregnancy. This is the first report of this combination of lesions.

Aorta, Thoracic↗

[Successful valvuloplasty of a calcified, high-grade pulmonary valve stenosis with removal of a right-left shunt at the atrial level].

HISTORY AND CLINICAL FINDINGS: A 68-year-old woman, with a cardiac murmur known since childhood and suffering from increasing dyspnoea for several years, was admitted to hospital after echocardiography had suggested pulmonary valve stenosis and an atrial septal defect. While her general condition was satisfactory, she had marked central cyanosis with clubbed fingers and moderate bilateral oedema of the lower legs. A rough, diamond-shaped systolic murmur was heard, loudest over the left 2nd intercostal space. In addition to a cardiac defect with right to left shunt, primary pulmonary disease with cor pulmonale was considered in the differential diagnosis. EXAMINATIONS: Haemoglobin content was 25.1 g/dl, haematocrit 72.4%, red cell count 7.44 x 10(6). Arterial oxygen partial pressure was 40 mm Hg, arterial oxygen saturation 81.8%, Echocardiography further revealed right ventricular enlargement, marked tricuspid regurgitation and a stenosed calcified pulmonary valve. At right heart catheterization a right to left interatrial shunt was calculated at 47% of systemic flow and a systolic pressure gradient between right ventricle and pulmonary artery of 131 mm Hg was measured. TREATMENT AND COURSE: Haemoglobin content was lowered to 19.4 g/dl after bloodletting. Balloon pulmonary valvuloplasty reduced the transvalvular systolic gradient to 31 mm Hg. The further course was without complication: 4 months later the patient had only grade II (NYHA) dyspnoea, the oxygen saturation was 91.3%. CONCLUSION: Severe pulmonary valve stenosis with right to left interatrial shunt can be successfully treated by balloon dilatation even in elderly patients.

Aged↗

Effects of balloon pulmonary valvuloplasty on atrial shunting--a patient with pulmonary valve stenosis and a large secundum atrial septal defect.

Percutaneous balloon valvuloplasty has been established as an effective treatment for pulmonary valve stenosis (PS) in children and adults. However, there are few reports of the use of this technique in patients with other cardiac anomalies. We report the performance of balloon pulmonary valvuloplasty in a 72-year-old woman with a large atrial septal defect (ASD). This patient had a PS with a right ventricular pressure of 108/18 mmHg and a pulmonary arterial pressure of 42/21 mmHg, corresponding to a 66 mmHg pressure gradient. The ASD measured 32 x 27 mm. Balloon pulmonary valvuloplasty reduced the pressure gradient to 5 mmHg immediately after the procedure and to 2 mmHg 40 days after the procedure. The left-to-right shunt ratio was reduced from 17% before to 12% immediately after and to 36% 40 days after the procedure. In contrast, the right-to-left shunt ratio showed a transient increase from 16% before to 28% immediately after the procedure before decreasing to 11% 40 days after the procedure. The arterial blood oxygen tension was 53 Torr before, 46 Torr immediately after and 55 Torr 40 days after the procedure. The grade of heart failure decreased from New York Heart Association class IV before to class II in the chronic stage. These findings indicated that balloon pulmonary valvuloplasty in a patient with ASD transiently increased the right-to-left shunting associated with a decrease in oxygen tension, but that the change in the chronic stage was slight. In our patient with ASD valvuloplasty was useful in relieving the symptoms associated with elevated right ventricular pressure but could not reduce the hypoxemia caused by right-to-left shunting. In conclusion, valvuloplasty in patients with ASD should be considered as a preoperative treatment with the aim of reducing the risk of surgery and to treat symptoms in patients who refuse to undergo surgery.

Aged↗

Intraoperative balloon valvuloplasty in pulmonary valve stenosis.

Balloon valvuloplasty was used in the operating room on 7 patients to visually assess the valvular changes induced by inflation of a balloon catheter. All patients had typical pulmonary valve stenosis and an associated cardiac condition which necessitated surgery. Of 23 fused commissures present, 21 were successfully opened with a single balloon inflation. In 2 patients, damage to the leaflets was observed. In 1 patient there was partial detachment and in the other a small tear was noted at the margin of one leaflet. These intraoperative results may be similar to the results obtained with percutaneous balloon valvuloplasty.

Adolescent↗

Fatal intrauterine adenoviral endomyocarditis with aortic and pulmonary valve stenosis: diagnosis by polymerase chain reaction.

We report a case of fatal hydrops fetalis owing to adenoviral endomyocarditis with aortic and pulmonary valve stenosis. A 1850-g macerated male stillborn delivered 1 week after fetal ultrasonography showed hydrops, cardiomegaly, and possible aortic valve stenosis. Autopsy confirmed hydrops and showed thickened, fibrotic semilunar valves with stenosis. The myocardium was focally fibrotic with areas of calcification. Polymerase chain reaction study of myocardial and aortic valve tissue was positive for adenovirus. Intrauterine viral myocarditis has been reported only rarely, but cases owing to Coxsackie B virus, adenovirus, and parvovirus B19 have appeared in the literature. With the exception of rubella, viral causation of significant valvular lesions in humans has received scanty support in the literature. This report suggests a broader group of causative agents. HUM PATHOL 31:1433-1435.

Adenoviridae Infections↗

Negative right ventricular diastolic pressure after operation for pulmonary valve stenosis: the phenomenon and its relation to volume load and conal contraction.

The phenomenon of negative right ventricular diastolic pressure immediately after operation for relief of isolated pulmonary valve stenosis was investigated in 11 patients. Pressures in the right ventricle and pulmonary artery were measured with a catheter tip micromanometer. One patient had a negative right ventricular diastolic pressure before operation. At the end of operation right ventricular diastolic pressure was negative in all 11 patients. The greater the right ventricular hypertrophy, the lower were these diastolic pressures. Negative right ventricular diastolic pressure is thus common in patients after pulmonary valvotomy, but fluid administration may eliminate it. Negative right ventricular diastolic pressure may be the result of hypercontraction and reduced volume of the hypertrophied right ventricle after relief of right ventricular outflow tract obstruction.

Adolescent↗

Long-term outcome of patients undergoing surgical repair of isolated pulmonary valve stenosis. Follow-up at 20-30 years.

In a group of 191 consecutive patients who underwent operation at the Mayo Clinic for isolated pulmonary valve stenosis between 1956 and 1967, late results were excellent. In patients surviving operation before the age of 21 years, long-term survival was similar to that in an age- and sex-matched control population. In the other patients, late survival and functional status were good, but survival was poorer than in the control population. This late attrition, most likely due to the sequelae of long-standing right ventricular hypertrophy, suggests the need for continued surveillance. Late sudden death occurred in only three patients, between 10 and 21 years postoperatively. The finding of normal life expectancy for such patients who survive operation before age 21 is important for actuarial purposes and for the patients' career and employment plans.

Adolescent↗

Percutaneous transluminal balloon valvuloplasty of congenital pulmonary valve stenosis, with a special report on infants and neonates.

Fifty-six percutaneous transluminal balloon valvuloplasty procedures were performed in 51 patients suffering from congenital pulmonary valve stenosis. The patients ranged in age from 1 day to 60 years (mean 6.9 years); 21 were infants less than 1 year of age, including 8 neonates. The peak systolic pressure gradient was greater than 50 mm Hg (mean 81.5) in 47 cases, and less than 50 mm Hg (mean 34.6) in 9. Valvuloplasty enabled a reduction in the mean right ventricular peak systolic pressure from 93.1 to 51.6 mm Hg (p less than 0.001), the mean transvalvular gradient from 73.4 to 27.0 mm Hg (p less than 0.001) and the mean right ventricular pressure expressed as a percent of systemic pressure from 99.5 to 52.0% (p less than 0.001). In infants and neonates, the mean right ventricular pressure expressed as a percent of systemic pressure decreased from 117.3 to 64.4% (p less than 0.001). In 23 patients, follow-up cardiac catheterization at 1 to 17 months revealed a significant change in the right ventricular systolic pressure, which decreased from 54.0 to 46.7 mm Hg (p less than 0.05), and in the peak systolic pressure gradient, which decreased from 27.3 to 22.6 mm Hg (p less than 0.05). Valvuloplasty is an effective procedure in relieving pulmonary stenosis in patients of all ages, including neonates.

Adolescent↗

The left ventricle in congenital isolated pulmonary valve stenosis. A morphological study.

The wall thickness of the left ventricle was measured in the hearts of 16 children and 2 adults who died of congenital pulmonary valve stenosis. A right-to-left shunt through a patent foramen ovale had existed in 12 cases and was excluded in the other 6. The thickness of the left ventricular wall and the interventricular septum was increased in the majority of hearts, especially in the older patients. There was a good correlation between wall thickness and histological appearance of the left ventricular myocardium; in 2 cases there was also evidence of left ventricular myocardial fibrosis. The coronary arteries appeared normal in all cases. A direct interaction between the ventricles was thought to be a possible mechanism.

Adolescent↗

Echocardiographic determinants of clinical course in infants with critical and severe pulmonary valve stenosis.

OBJECTIVES: The purposes of this study were to determine the growth pattern of the pulmonary valve (PV) annulus and right heart structures in patients with critical and severe pulmonary stenosis (PS) after balloon dilation, and to determine any morphometric or hemodynamic differences between cyanotic infants with critical PS and asymptomatic infants with severe PS that may account for their varied clinical presentations. BACKGROUND: Growth of the PV annulus and right heart structures in patients with critical PS after balloon valvuloplasty has not clearly been defined. In addition, the anatomic and hemodynamic factors that determine whether an infant with severe PS will present with cyanosis or without symptoms are not well understood. METHODS: Measurements of the PV annulus, tricuspid valve (TV) annulus and main, right and left pulmonary arteries were obtained from initial and follow-up echocardiograms, and Z values were calculated. Hemodynamic data and balloon pulmonary valvuloplasty techniques were reviewed. Right ventricular (RV) volumes were measured from angiograms. RESULTS: Fourteen patients with critical PS (mean [+/- SD] age 0.21 +/- 0.37 months) and 20 patients with severe PS (mean age 2.6 +/- 2.9 months) were evaluated at presentation and at 32 +/- 33 and 42 +/- 32 months of follow-up, respectively. Balloon pulmonary valvuloplasty was successful in 64% of patients with critical PS and in 90% of patients with severe PS. The PV, TV and pulmonary arteries increased in size after balloon pulmonary valvuloplasty in both groups at a rate that paralleled or exceeded the rate of somatic growth. The initial TV diameter and RV volume were smaller in patients with critical PS than in those with severe PS (p < 0.05 and p < 0.0008, respectively). CONCLUSIONS: After balloon pulmonary valvuloplasty in infants with critical and severe PS, right heart structures increase in size at a rate that parallels or exceeds the rate of somatic growth. The primary morphometric differences between these groups are a smaller TV diameter and RV volume in infants with critical PS. This may contribute to increased right to left atrial shunting and account for the variations in clinical presentation.

Catheterization↗

Effectiveness of an umbilical artery "snare assisted" approach for critical pulmonary valve stenosis or atresia in the neonate.

Thirteen neonates with critical pulmonary valve stenosis/atresia underwent successful transcatheter balloon valvuloplasty using an umbilical artery "snare assisted" approach. This technique simplifies the procedure and avoids femoral artery injury by using the umbilical artery, reduces fluoroscopy exposure, and eliminates the need for a gradational approach which reduces costs.

Acute Disease↗

Variability of the Doppler gradient in pulmonary valve stenosis before and after balloon dilatation.

The variability of the valve gradient measured by Doppler in pulmonary stenosis was compared with the variability of the gradient measured at catheterisation in 42 infants and children undergoing catheterisation with a view to balloon dilatation of the pulmonary valve. The maximum value measured by Doppler when the patient was unsedated was significantly higher than that measured when the patient was sedated for catheterisation, and the maximum gradient was significantly higher shortly after than several days later. In a patient with pronounced infundibular obstruction after dilatation the Doppler signal clearly showed that the obstruction was dynamic, with a superimposed lower fixed signal that correctly predicted the final low gradient. The Doppler gradient in an alert and unsedated patient may be a better measure of the true physiological value. The highest Doppler value so obtained is a more appropriate indicator of the need for balloon dilatation than a single catheter measurement. The result of dilatation is best assessed by Doppler measurement at least a day after the procedure.

Adolescent↗