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Results for “PULMONARY VALVE STENOSIS”

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At least 613 records · Page 34Linked to original sources
PubMed2001

Infant methylenetetrahydrofolate reductase 677TT genotype is a risk factor for congenital heart disease.

OBJECTIVE: Recently, an association between the homozygous C677T mutation in the methylenetetrahydrofolate reductase (MTHFR) gene in infants with congenital neural tube defects or congenital oral clefts has been shown. However, no data are available so far with respect to the MTHFR 677TT genotype in children with underlying structural congenital heart disease (CHD). METHODS: We investigated the MTHFR genotype in 114 Caucasian CHD patients aged newborn to 16 years (median 0.6 years; 53% male) and in 228 age- and sex-matched healthy controls. RESULTS: In childhood patients with CHD the homozygous MTHFR 677TT genotype was found in 21 out of 114 subjects (18.4%) compared with 21 out of 228 controls (9.2%; odds ratio (OR) 2.2, 95%-confidence interval (CI) 1.2-4.3; P=0.027). In patients with pulmonary valve stenosis, hypoplastic left heart syndrome, coarctation of the aorta, aortic valve stenosis or subaortic stenosis the frequency of the TT genotype varied between 38 and 67% with corresponding ORs from 6.1 (CI, 1.4-27.5; P=0.034) to 20.4 (CI, 1.8-235.0; P=0.025), whereas in other structural CHD the frequency of this genotype was not significantly different from the controls. CONCLUSIONS: With the present study we can show for the first time that the embryonal MTHFR 677TT genotype is significantly associated with the development of structural congenital heart malformations during early pregnancy. It remains to be clarified, whether this genotype is at least a risk marker or a risk factor for structural congenital heart malformations.

Adolescent↗
PubMed2006

Factors determining immediate and medium-term results after pulmonary balloon valvuloplasty.

OBJECTIVE: To determine factors influencing immediate and medium-term results of Pulmonary Balloon Valvuloplasty (PBV) for pulmonary valve stenosis. MATERIAL AND METHOD: Between 1995 and 2001, the authors carried out PBV in 25 consecutive patients. Two treatment outcomes including immediate and medium-term results were analyzed. The immediate results were classified into two groups based on the pulmonary systolic pressure gradient (PG) immediately after dilation: group 1 with a PG < or = 35 mmHg and group II with a PG > 35 mmHg. At medium-term follow-up of 6 to 60 months, echocardiographic evaluations were analyzed and divided into two groups: group A with a PG < or = 25 mmHg and group B with a PG > 25 mmHg. Demographic characteristics and hemodynamic parameters of immediate and medium-term results were compared and analyzed. RESULTS: Immediately after PBV the right ventricular systolic pressure (RVSP) decreased from 115.3 +/- 37.6 mmHg to 67.0 +/- 28.5 mmHg (p < 0.001). The pulmonary systolic pressure gradient decreased from 90.4 +/- 37.9 mmHg to 39.3 +/- 25.6 mmHg (p < 0.001). Nine of the 25 patients (group 11; 36%), with incomplete immediate relief of the obstruction, had more symptoms, a higher baseline PG, higher right atrial pressure, higher RVSP, and a higher systolic pressure ratio. Six of the 25 patients (group B; 24%), with suboptimal medium-term results, had a higher right ventricular voltage on the electrocardiogram, higher pre-dilation PG, and higher RVSP. CONCLUSION: Successful medium-term outcomes following pulmonary balloon valvuloplasty were achieved in 76% of the patients, with a greater success rate (91%) in patients with a lower right ventricular voltage electrocardiogram (R wave amplitude in V1) < or = 21 mm, a prevalvuloplasty systolic gradient < or = 90 mmHg, and right ventricular systolic pressure < or = 125 mmHg.

Adolescent↗
PubMedDate not supplied

[Balloon pulmonary valvuloplasty, 15-year experience at the Siglo XXI IMSS National Medical Center].

OBJECTIVE: To evaluate 15 years of experience with balloon pulmonary valvuloplasty in a single third level health care center. MATERIAL AND METHODS: Hundred-fifty patients underwent the procedure, 73 (48%) men and 77 (52%) women, mean age 10.5 +/- 11.3 years. RESULTS: The initial systolic gradient decreased from 86 +/- 35 to 21.67 +/- 12.20 mm Hg, p < 0.001, whereas the initial right ventricular systolic pressure decreased from 106 +/- 34.8 to 53 +/- 27 mm Hg, p < 0.0001. At the end of the follow-up, 48 +/- 44 months, the systolic gradient was 13.43 +/- 8.73 mm Hg, p < 0.001. Major complications occurred in 9 (6.4%) patients. Immediate technical success was achieved in 111 (74%) patients and failure in 39 (26%). At the end of the follow-up period, successful outcomes were achieved in 104 (89.6%); in contrast, failures were present in 12 (10.4%) patients, p < 0.001. Death occurred in 2 (1.33%) patients. The predictors for failure were age < 1.5 +/- 1-33 years (p < 0.004), dysplastic valve (p < 0.001), high initial systolic gradient (p < 0.002), and high initial systolic right ventricular pressure (p < 0.0001). CONCLUSION: Balloon pulmonary valvuloplasty is an effective, safe, and first choice treatment for congenital pulmonary valve stenosis.

Adolescent↗
PubMed1991

[Transluminal percutaneous pulmonary valvuloplasty. Follow up of 34 patients].

Thirty four children aged 2 to 11 years (means = 4.8 +/- 2.4 years) with pulmonary valve stenosis were treated by percutaneous transluminal valvuloplasty with catheter-balloon. All of them were reevaluated one month and six months after valvuloplasty, and once more year after the procedure. The evaluation consisted on clinical examination, chest Rx, ECG and Doppler 2 D-Echocardiography. We found a close correlation between transpulmonic gradients by Doppler and manometry. After pulmonary valvuloplasty we detected 12 cases of slight pulmonary insufficiency. Changes in ECG were significant since signs of right heart hypertrophy disappeared. Immediate good results got after valvuloplasty, remained and even improved throughout checking. We conclude that, faced with our results, we consider valvuloplasty as a definitive treatment of pulmonary stenosis, Doppler-two-Dimensional Echocardiography, is also useful for evaluating patients without need for a new catheterisation and EECG is an ideal complement to 2 D-Doppler Echocardiography.

Angioplasty, Balloon↗
PubMed1990

Percutaneous balloon pulmonary valvuloplasty in sickle cell anemia: a case report.

Percutaneous balloon pulmonary valvuloplasty was performed on a 19-year-old female who had moderate pulmonary valve stenosis with sickle cell anemia. The patient developed sickle cell crisis resulting in occipital infarction, but she made a good recovery. We describe this case and the methods of reducing the risk of sickle cell crisis.

Adult↗
PubMed1979

["Pedunculated tumor" of the right ventricle in an infant].

A infant is reported who had pulmonary valve stenosis with a pedunculated throbus in the right ventricle. On angiocardiography this appeared as a tumour. The origin of the thrombus and the diagnostic problems interpreting the angiocardiographic appearances are discussed.

Angiocardiography↗
PubMed1983

[Pulmonary valvulotomy or valvulectomy with simple clamping of the vena cava in infants and young children].

In infants and young children pulmonary valvulotomy or valvulectomy under simple clamping of the vena cava is the treatment of choice for pulmonary valve stenosis with normal interventricular septum. The technique, performed without cardiopulmonary bypass, is described. In 40 patients operated upon, the peri-operative mortality was 10% and short-term clinical results were satisfactory.

Constriction↗
PubMed1999

Clinical, echocardiographic, and angiographic profile of patients with double-chambered right ventricle: experience with 48 cases.

Forty-eight patients with double-chambered right ventricle (DCRV) were prospectively studied over a 3-year period. Clinical, echocardiographic, hemodynamic, and angiographic studies were done in all. Three patients were studied postoperatively also. Fifteen patients underwent surgery confirming the diagnosis. Echocardiography detected anomalous muscle bundles (AMBs) in 39/41 patients in whom this study was carried out. Angiographically AMBs were best demonstrated in anteroposterior views in a systolic frame. Common associated anomalies found with DCRV were ventricular septal defects (69%), pulmonary valve stenosis (38%), and double-outlet right ventricle (10%).

Adolescent↗
PubMed1974

[Chronic pericardial effusion and congenital cardiopathy].

Report of one case of chronic pericardial effusion combined with pulmonary valve stenosis and atrial septal defect in a man aged 31, hospitalized for congestive heart failure. The relationship between these two diseases were studied, and the diagnostic and therapeutic problems posed were studied.

Adult↗
PubMed1995

Double-chambered right ventricle: experience with 52 cases.

The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 +/- 4.4 years). Diagnosis was confirmed in 51 patients at cardiac catheterization and in other one on operation. The majority of the patients had associated cardiac anomalies: there were 33 ventricular septal defect (63%), 21 pulmonary valve stenosis (40%), nine atrial septal defect (17%), and four double-outlet right ventricle. The electrocardiograms revealed upright T waves alone in right precordial leads suggesting right ventricular hypertrophy in 33% of the patients. At cardiac catheterization, there was a pressure gradient of 20-160 mmHg between the right ventricular inflow and outflow portions. Forty patients have had surgery and four have undergone balloon pulmonary valvuloplasty. Surgical treatment was planned for two patients and other six had no indication for treatment.

Adolescent↗
PubMedDate not supplied

Hereditary vestigial pulmonary arterial trunk and related defects in rabbits.

In a population of IIIVO/J rabbits at the Jackson Laboratory a vestigial pulmonary arterial trunk is the commonest expression of a malformation involving the heart and great vessels. There appears to be a series of effects from a completely absent pulmonary trunk through a vestigial but patent pulmonary trunk and/or pulmonary valve stenosis on the one hand to a vestigial or absent ascending aorta on the other. In a few cases the pulmonary trunk is bulbous and the ductus arteriosus is vestigial or absent. In almost all cases of this syndrome, there is also a high ventricular septal defect. The animals appear perfectly normal in every other respect. In its extreme forms this mutation is lethal, but those few animals that are somewhat less severely affected may live for a short time. No effect was seen on birth weight or litter size. Inheritance of vestigial pulmonary arterial trunk appears to be due to two autosomal recessive factors both of which must be homozygous for the expression of the condition. We propose the symbols vpt-1 and vpt-2 for the genes responsible for vestigial pulmonary arterial trunk and its related abnormalities.

Animals↗
PubMed1991

Long-term results after balloon pulmonary valvuloplasty.

BACKGROUND: The objective of this study was to determine the long-term outcome of patients after percutaneous balloon pulmonary valvuloplasty (BPV) treatment of congenital pulmonary valve stenosis. METHODS AND RESULTS: This study represents a case series with duration (mean +/- SD) of follow-up of 4.6 +/- 1.9 years. Forty-six patients with a median age of 4.6 years (range, 3 months to 56 years) had BPV at one academic institution between June 1981 and December 1986. Mean peak systolic pressure gradients from the right ventricle to the pulmonary artery were as follows: before BPV, 70 +/- 36 mm Hg; immediately after BPV, 23 +/- 14 mm Hg; at intermediate follow-up by cardiac catheterization or Doppler echocardiography at less than 2 years after BPV, 23 +/- 16 mm Hg (n = 33); and at long-term follow-up by Doppler at more than 2 years after BPV, 20 +/- 13 mm Hg (n = 42). BPV acutely reduced the gradient to less than 36 mm Hg for 41 of 46 (89%) patients. Available gradients at long-term follow-up were less than 36 mm Hg for 36 of 42 (86%) patients without additional procedures. A patient age of less than 2 years at the initial BPV was a significant risk factor for gradients over 36 mm Hg at follow-up. CONCLUSIONS: BPV provides long-term relief of pulmonary valvular obstruction in the majority of patients. Close follow-up of patients who require BPV at less than 2 years of age is warranted.

Adolescent↗
PubMed1983

Regression of infundibular hypertrophy after pulmonary valvulotomy without myocardial resection.

In the period 1965-1979, 26 patients underwent valvulotomy for congenital pulmonary valve stenosis without ventricular septal defect. Infundibular stenosis was additionally present in 14 of the patients, 12 children and 2 adults, but myocardial resection in the infundibular area was not performed. During the first postoperative year the right ventricular outflow tract underwent normalization in the 12 children and the right ventricular systolic pressure normalized in all 14 patients. No relationship could be demonstrated between the R wave in the precordial lead V1 and the systolic pressure in the right ventricle, the gradient to the pulmonary artery or the extent of subvalvular hypertrophy.

Adult↗
PubMed1975

A new case of the trisomy 9P syndrome. Report of a patient with unusual chromosome findings (46,XX/47,XX, + i (9p) and a peculiar congenital heart defect.

A girl is described for whom an unusual chromosome constitution was found and who had a peculiar congenital heart defect. The girl showed the main clinical features of the trisomy 9p syndrome, such as psychomotor retardation, microcephaly and brachycephaly, enophthalmos, antimongoloid eye slant, hypertelorism, abnormal ears, a globulous nose, downward slanting mouth, hypoplasia of phalanges and abnormal palmar creases. In addition, the girl had an incomplete harelip, a cleft palate and a peculiar congenital heart defect, a ventricular septal defect with pulmonary valve stenosis and a marked hypoplasia of the pulmonary trunk, including the bifurcation. Chromosome analysis revealed a mosaicism with normal and abnormal mitoses (47 chromosomes). The extra chromosome was a metacentric E16-like chromosome, which on the basis of the G, R, Q and Giemsa-11-banding could be identified as an isochromosome of the short arm of chromosome 9 (46,XX/47,XX, +i (9) (pter leads to cen leads to pter)). The patient consequently had a partial tetrasomy of the short arm of chromosome 9.

Abnormalities, Multiple↗
PubMed1975

Evaluation of late results of surgical treatment of congenital heart disease.

Operative mortality from surgical treatment of congenital heart disease has been steadily reduced over the past 20 years. During the same period it has become clear that a proportion of survivors have residual clinical problems. Some of these are due to imperfect repair, a number being due to factors beyond present surgical control, and some are the consequence of associated cardiovascular defects. The chief problems can be elucidated by an analysis of the results of surgical treatment of six cardiac malformations: simple pulmonary valve stenosis, coarctation of the aorta, secundum atrial septal defect, isolated ventricular septal defect, tetralogy of Fallot and transposition of the great arteries. Recognition of the sequelae of preoperative hemodynamic strain and apparently minor associated malformations is important, and it is possible to anticipate such factors. Long-term follow-up of patients after operation is particularly important.

Adolescent↗
PubMed1987

Operative balloon pulmonary valvuloplasty.

Closed operative pulmonary valvulotomy for relief of critical pulmonary valve stenosis can be performed effectively by intraoperative use of a balloon catheter without inflow occlusion or hemodynamic instability and with minimal blood loss. Follow-up evaluation indicates adequate growth of the valve.

Angioplasty, Balloon↗