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At least 199 records · Page 11Linked to original sources
PubMed1989

[Thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. A case report of successful surgical repair].

A case of thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis is reported. A cyanotic boy with omphalocele was brought to our hospital soon after his birth and was diagnosed as thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. A conservative treatment of omphalocele was performed successfully and a plastic chest shield had been made for him which protected the anterior surface of his chest. He continued to develop in a fairly satisfactory condition and operation was delayed until he was 8 years old when he had severe cyanosis and frequent episodes of headache. The operative procedures were; 1) ascending aorta to main pulmonary artery shunt with EPTFE tube; 2) to bring the heart into the thoracic and abdominal cavity by developing widely the pericardial and diaphragmatic defect behind the heart's preoperative position; 3) to reconstruct the new pericardial sac lying from thoracic to abdominal cavity by covering the heart using EPTFE sheet. This trial of making a new pericardial sac for thoracoabdominal ectopia cordis is not reported so far in the literature. It is expected that the heart has being raised to the thoracic cavity with patient's physical growth after this operation.

Child↗
PubMed1999

The effects of surgically treated pulmonary stenosis on lung growth and cardiopulmonary function in children during rest and exercise.

The effects of high pulmonary blood flow and pressure on pulmonary development are well understood, but the effect of low pulmonary flow/pressure is not. Pulmonary stenosis (PS) was therefore used as a model to determine its effect on lung development, which was assessed noninvasively by carbon monoxide transfer at rest and during exercise. One hundred and six control children (55 males, 8-16 yrs) and 11 children with isolated valvar or subvalvar PS surgically corrected > or =10 yrs prior to the study without residual stenosis/regurgitation were evaluated. Measurements of effective pulmonary blood flow, stroke volume, arteriovenous oxygen difference (AVO), transfer factor and transfer constant, alveolar ventilation and anaerobic threshold were performed using a mass spectrometer. Data from the normal children allowed calculation of z-scores for the study group matched for age, sex, pubertal stage and surface area. PS children at rest had a significantly lower forced expired volume in one second, cardiac frequency and transfer constant with a raised oxygen consumption and AVO which persisted on exercise. During exercise, the cardiac frequency was 12 beats x min(-1) slower and AVO 0.017 L greater than controls (p<0.05). A persistently mildly raised effective functional residual capacity (0.2 L x m(-2)) during exercise led to a reduced transfer constant (0.35 mmol x min(-1) x kPa(-1) x L(-1)), although the percentage rise (28%) from rest to peak exercise was normal. The percentage fall in stroke volume from the penultimate exercise stage to peak exercise stage was greater in PS children (24%, 95% confidence interval 11-37) than control children (2.4, -2-10, p<0.005). In conclusion, although the effects are small, pulmonary stenosis does affect cardiopulmonary function after surgery. This needs to be considered when contemplating the timing of treatment.

Adolescent↗
PubMed2001

Life-threatening pulmonary edema following unilateral stent implantation for bilateral branch pulmonary stenosis: recovery after contralateral stent implantation.

A 13-year-old girl, who was suffering complications with bilateral pulmonary artery stenosis after intracardiac repair for tetralogy of Fallot, suffered life-threatening left pulmonary bleeding and edema following inadvertent unilateral stent implantation for a left pulmonary stenosis. Pulmonary edema and subsequent hypoxia persisted despite intensive medical treatment; however, contralateral stent deployment resolved her symptoms quickly.

Adolescent↗
PubMed1994

Balloon pulmonary valvuloplasty for double outlet right ventricle with valvular pulmonary stenosis.

A male neonate presented with cyanosis. Echocardiograms showed a double outlet right ventricle, an absent outlet septum, a subaortic ventricular septal defect and valvular pulmonary stenosis. Balloon pulmonary valvuloplasty was performed at 19 days of age, resulting in an immediate increase of the systemic arterial oxygen saturation from 67 to 87%. As far as could be found, this is the first report of a patient with a double outlet right ventricle without the outlet septum who was clinically observed and underwent balloon pulmonary valvuloplasty.

Catheterization↗
PubMed1982

Critical pulmonary stenosis in infants less than three months of age: a reappraisal of closed transventricular pulmonary valvotomy.

Sixteen patients less than 3 months of age underwent closed transventricular pulmonary valvotomy for critical pulmonary stenosis with intact ventricular septum. There were 14 survivors; the 2 deaths were unrelated to the technique. Early and late results reveal good hemodynamics in all but 1 patient who underwent open valvotomy four years later for restenosis. We have used this technique exclusively, as it is safe, requires little preparation for operative relief in the very sick infant, and the early and late results are excellent.

Follow-Up Studies↗
PubMed1978

Natural history of valvular pulmonary stenosis in children below the age of two years. Long-term follow-up with serial heart catheterizations.

18 patients with mild and moderate valvular pulmonary stenosis diagnosed by heart catheterization before the age of 2 yr (mean 11.6 mth) were followed for 5 to 21 yr without operation. 15 cases had a repeat catheterization study after a mean interval of 10.7 yr. Of 7 cases with an initial right ventricular peak systolic pressure (RVSP) of 50 mm Hg or below, 6 had a decrease and 1 a moderate increase in RVSP. Of 8 cases with an initial RVSP of greater than 50 mm Hg 2 had a decrease, 2 had no change and 4 had a significant increase in RVSP; the latter subsequently underwent operation. Electrocardiography yielded some information, but neither roentgenography nor history was of any help in evaluating the severity of the stenosis at follow-up. As mild and moderate valvular pulmonary stenosis in infants and children below the age of 2 may progress and require operation a repeat heart catheterization is usually to be recommended later in childhood.

Adolescent↗
PubMedDate not supplied

Familial congenital valvar pulmonary stenosis: autosomal dominant inheritance.

A mother and three of her four children, one girl and two boys, who had valvar pulmonary stenosis are described. One child had an associated septum secundum atrial septal defect, and another had an associated ventricular septal defect. An autosomal dominant mode of inheritance is likely for the valvar pulmonary stenosis. The association of additional cardiac lesions in two of the three siblings raises the possibility of closely associated genetic loci.

Adult↗
PubMed1988

Pulmonary stenosis with intact ventricular septum: documentation of development of the lesion echocardiographically during fetal life.

We describe a case of severe pulmonary stenosis with intact interventricular septum diagnosed during fetal life at 34 weeks gestational age. An echocardiographic examination had been performed on the same fetus at 20 weeks, when no cardiac anomalies were observed. It is concluded that this form of pulmonary stenosis might be a lesion either acquired during fetal life or becoming progressively more severe in the prenatal period.

Diagnostic Errors↗
PubMed1989

Balloon valvuloplasty of pulmonary stenosis in infants and children--co-operative study of the German Society of Pediatric Cardiology.

Balloon valvuloplasty (BVP) of pulmonary stenosis (PST) was performed in 305 infants and children in 20 heart centres of the Federal Republic of Germany. 273 patients suffered from isolated PST. Their age ranged from 3 days to 18 years (mean = 5.85 +/- 4.82 years), their weight from 2.9 to 60 kg. The pressure gradient was measured invasively in the sedated or anaesthetized child, partly by CW Doppler. BVP was performed by mono- or trefoil catheters, mostly oversized. As a result, mean pressure gradient fell from 71.7 +/- 32 mmHg before to 31.8 +/- 25 mmHg immediately after BVP (P less than 0.0001). The gradient was reduced by less than 30% in 13% of the patients, by 30-49% in 23%, and by greater than or equal to 50% in 64%. Among 33 patients with a gradient reduction of less than 30%, nine were aged less than 1 year, nine had dysplastic valves, and nine had a short-term infundibular reaction and good late results. Gradient reduction was not improved with oversized balloons and was independent of the initial gradient. Follow-up for a mean 11.2 +/- 8 months showed a further slight improvement, probably due to regression of infundibular hypertrophy. In the whole group we observed infundibular reaction in 12%, serious complications with one late death in 4%. From the start, BVP proved to be a secure and effective tool for elimination of pulmonary stenosis.

Adolescent↗
PubMed1978

Transposition of the great arteries with ventricular septal defect and pulmonary stenosis.

A postmortem investigation was carried out of 19 heart specimens with transposition of the great arteries, ventricular septal defect, and congenital subvalvular pulmonary stenosis. Certain types of obstruction appeared to be closely related to other features of the hearts. In cases with malalignment of the infundibular septum, the obstruction was caused by this septum and the anterolateral muscle bundle of the left ventricle. If the infundibular septum was deviated considerably to the left, the pulmonary stenosis was usually severe because the infundibular septum and anterolateral muscle bundle were joined. This junction resulted in a relatively posterior position of the pulmonary orifice in the left ventricle. A less extreme deviation of the infundibular septum resulted in an obstruction by this septum and by the anterolateral muscle bundle, situated at the right and the left sides of the pulmonary orifice respectively. In some cases of paratricuspid ventricular septal defect an anomalously attached and cleft anterior leaflet of the mitral valve was found. This, together with a leftward deviation of the anterior left ventricular part of the ventricular septum, caused the obstruction.

Adolescent↗
PubMed1984

Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.

Aorta↗
PubMed2005

[Sudden transformation of pulmonary stenosis to trilogy of Fallot in a 65 year old patient].

The authors report the case of a 64 year old woman with typical valvular pulmonary stenosis in whom spontaneous and sudden reopening of the foramen ovale resulted in cyanosis. Transthoracic echocardiography with injection of contrast provides a complete diagnosis: valvular pulmonary stenosis with a mean pressure gradient of 83 mmHg and massive right-to-left interatrial shunt. The malformation was treated by interventional catheterisation in a two-stage procedure: pulmonary valvuloplasty followed by closure of the foramen ovale because of the persistence of a right-to-left interatrial shunt. The functional improvement was followed by the appearance of effort angina. Coronary angiography showed single vessel disease of the left anterior descending artery treated by stenting. The long-term outcome was satisfactory.

Cardiac Catheterization↗
PubMed1985

Valvular pulmonary stenosis. Natural history and right ventricular function in infants and children.

The purpose of this study was the analysis of natural history and right ventricular function of infants and children with valvular pulmonary stenosis. Available for assessment were the pressures in the right ventricle and pulmonary artery in 5 infants and 13 children obtained at two cardiac catheterizations, performed at intervals of 2 to 12 years (mean: 6.5 years) apart, as well as quantitative angiocardiographic data in another group of 38 children. There was no change in the pressure gradient between the right ventricle and pulmonary artery (PG) between the two catheterizations for those with an initial PG of less than 50 mmHg; but a mean increase of 8.6 mmHg year-1 occurred in those with an initial PG of more than 50 mmHg. There were good correlations between increase of PG and the first (r = 0.704) and the second (r = 0.904) catheterizations. End diastolic and stroke volume were normal while end-systolic volume was smaller (P less than 0.01) and ejection fraction greater (P less than 0.01) than normal. The natural history of children with a PG of more than 50 mmHg seems to be different from that with a PG of less than 50 mmHg. In the former group the increase of PG is rapid while PG in the latter does not change over many years. Right ventricular function is usually not impaired in patients with moderate and severe valvular pulmonary stenosis in the pediatric age group.

Angiocardiography↗