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At least 19 recordsLinked to original sources
PubMed1993

Cardiorespiratory responses to exercise of patients with aortic stenosis, pulmonary stenosis, and ventricular septal defect.

BACKGROUND: The purpose of this study was to determine exercise tolerance and cardiac response to exercise for a large cohort of adult subjects with aortic stenosis, pulmonary stenosis, or ventricular septal defect participating in the Second Natural History Study of Congenital Heart Defects (NHS-2). METHODS AND RESULTS: Exercise testing was performed on 134 of 235 NHS-2 full participants with aortic stenosis, 195 of 331 NHS-2 full participants with pulmonary stenosis, and 324 of 594 NHS-2 full participants with ventricular septal defect. A Bruce treadmill exercise protocol was used. Mean exercise duration for patients with aortic stenosis, pulmonary stenosis, and ventricular septal defect was 86.5%, 94.2%, and 90.8% of predicted, respectively. For patients with aortic stenosis, there was a direct relation between echocardiographic Doppler maximum transaortic gradient and ST segment change during exercise. ST segment change during exercise was very uncommon for patients with pulmonary stenosis. For patients with ventricular septal defect, there was an association between arrhythmias noted during exercise and the presence of associated aortic insufficiency. CONCLUSIONS: Although exercise duration was well preserved for patients with each of the three defects, exercise tolerance was subnormal.

Adult↗
PubMed1993

Second natural history study of congenital heart defects. Quality of life of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

BACKGROUND: Quality of life of patients with congenital heart defects is an important aspect of the assessment of outcome of medical and surgical treatment. METHODS AND RESULTS: All participants in the Second Natural History Study of Congenital Heart Defects who completed the study questionnaire were included in this analysis. The questionnaire completed by the patients included inquiries relative to medical history, marital and family life, self-perception of well-being, insurability, and employability. The following general observations were apparent. The study cohort reported a self-perception of health status that was similar to that of the general population. Second, 35-40% of patients had not had a cardiac evaluation within the previous 10 years. Third, 16% of the patients had no health insurance, and 35.7% of the patients had no life insurance. For the patients with pulmonary stenosis and ventricular septal defect, the percent who were married was less than that of the corresponding national age- and sex-specific population. For patients with aortic stenosis, the percent married was greater than that of the general population for some age groups. The proportion of divorced or separated individuals was similar to that of the general population. For all three defects, the level of educational attainment exceeded the national average. Last, unemployment rates were similar to national averages except for women with aortic stenosis, who had a significantly higher unemployment rate than did age- and sex-matched controls. CONCLUSIONS: Patients initially identified with aortic stenosis, pulmonary stenosis, or ventricular septal defect in 1959-1973 have a quality of life in the mid-1980s similar to that of the general US population.

Adult↗
PubMed1993

Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

BACKGROUND: All of the 2,401 patients with aortic stenosis (AS), pulmonary stenosis (PS), or ventricular septal defect (VSD) admitted to the First Natural History Study of Congenital Heart Defects between 1958 and 1965 were eligible for the Second Natural History Study. Most patients with severe defects were managed surgically, and most with mild defects were managed medically. Final examination in the first study was carried out 8 years after admission. METHODS AND RESULTS: For AS, the incidence rate of bacterial endocarditis (BE) was 27.1 per 10,000 person-years. The incidence rate was 15.7 per 10,000 person-years for those managed medically and 40.9 per 10,000 person-years for those managed surgically. Most patients managed surgically had severe AS, and severity was more important to the risk of BE than the method of management. For PS, only one of the 592 patients with PS experienced BE. For VSD, the incidence rate of BE was 14.5 per 10,000 person-years. Size of the VSD was not associated with risk of BE. The risk of BE before closure of the VSD was more than twice that after surgery. CONCLUSIONS: The incidence rate of BE was nearly 35-fold the population-based rate. The increased incidence in patients with AS after valvotomy was a function of severity of the defect and not a function of surgery. Presence of aortic regurgitation in patients with AS did not increase the risk of developing BE. Surgical closure of VSD lowered the risk of BE.

Adolescent↗
PubMed1993

Occurrence risk for congenital heart defects in relatives of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

BACKGROUND: Knowledge of the risk of occurrence of congenital heart defects in offspring of individuals with a congenital heart defect is important for genetic counseling and understanding the etiology of congenital heart diseases. METHODS AND RESULTS: A portion of the questionnaire mailed to all patients in the Second Natural History Study of Congenital Heart Defects (NHS-2) addressed marital status, pregnancy, miscarriage, and presence or absence of congenital heart defects or other congenital malformations in first-degree relatives. Offspring were not examined as a part of the study. For male probands with aortic stenosis (AS), three of 251 offspring had congenital heart disease, whereas one of 72 offspring of female probands with AS had congenital heart disease. For patients with pulmonary stenosis (PS), three of 176 offspring of male probands had congenital heart disease, whereas eight of 205 offspring of female probands had congenital heart disease. For male probands with ventricular septal defect (VSD), 10 of 334 offspring had congenital heart defects, and 11 of 384 offspring of female probands with VSD had offspring with congenital heart defects. The prevalence rate for noncardiac congenital anomalies in offspring of probands was 2%. CONCLUSIONS: Based on known congenital heart defects in offspring of probands in the NHS-2, occurrence rates of congenital heart disease in children of subjects with AS, PS, and VSD were 1.2% (confidence interval [CI], 0.34-3.1%), 2.8% (CI, 1.4-5.1%), and 2.9% (CI, 1.8-4.4%), respectively.

Adolescent↗
PubMed1989

Spontaneous resolution of pulmonary stenosis.

Pulmonary stenosis diagnosed in infancy usually increases in severity with age or, if it is mild, may remain so for many years. We report the case of a child in whom moderately severe pulmonary stenosis in infancy appeared to completely resolve by the age of 8 years.

Female↗
PubMed1997

Importance of right ventricular outflow tract angiography in distinguishing critical pulmonary stenosis from pulmonary atresia.

OBJECTIVE: To investigate the spectrum of pulmonary atresia and critical pulmonary stenosis using right ventricular outflow tract angiography and explore its implications for catheter interventional treatment. DESIGN: Prospective clinical study. SETTING: Two paediatric cardiology centres. SUBJECTS: 11 neonates or infants (aged 1 day to 8 months; weighing 2.3 to 7.8 kg) with pulmonary atresia or where the differentiation of pulmonary atresia from critical pulmonary stenosis was unclear on either echocardiography or angiography. METHODS: Right ventricular outflow tract angiography was performed on all patients to distinguish pulmonary atresia from critical pulmonary stenosis before opening the right ventricular outflow tract. RESULTS: Right ventricular outflow tract angiography showed that three of seven patients diagnosed as pulmonary atresia by echocardiography had pin hole jets across the pulmonary valve; another had a probe patent valve that appeared imperforate on both echocardiography and right ventricular outflow tract angiography. Three of the four patients diagnosed by echocardiography as critical pulmonary stenosis were found on right ventricular outflow tract angiography to have pulmonary atresia. The remaining patient had such a tiny orifice that a second orifice had to be created with a radiofrequency catheter. The right ventricular outflow tract was opened successfully in 10 of the 11 patients, six of whom required application of radiofrequency energy. The right ventricular to aortic systolic pressure ratio fell from 1.4 (0.9 to 1.9) to 0.6 (0.2 to 1.1) (P < 0.05). All 11 patients were alive and well with transcutaneous oxygen saturations ranging from 84% to 95% at a median follow up duration of nine months. CONCLUSIONS: Critical pulmonary stenosis and pulmonary atresia cannot always be accurately distinguished by echocardiography. Right ventricular outflow tract angiography helps to distinguish the two groups. In most cases the right ventricular outflow tract can be opened without mortality and with short to medium term survival.

Catheter Ablation↗
PubMed1993

Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring.

BACKGROUND: Arrhythmias are a significant source of morbidity and mortality in patients with congenital heart defects. METHODS AND RESULTS: As part of the Second Natural History Study of Congenital Heart Defects (NHS-2), 24-hour ambulatory ECG monitoring was performed for full participants in the study. At least 15.5 hours of monitoring was required for inclusion in the analysis. This was achieved for 755 (90.6%) of the patients. Multiform premature ventricular contractions, ventricular couplets, and ventricular tachycardia were considered "serious arrhythmias." For patients with aortic stenosis, the presence of "serious arrhythmias" was associated with higher left ventricular end-diastolic pressure, presence of aortic regurgitation, male sex, and presence of prior aortic valve replacement. For patients with pulmonary stenosis, only older age on admission to NHS-1 was significantly associated with "serious arrhythmias." For patients with ventricular septal defect, the variables associated with "serious arrhythmias" were different for medically and surgically managed patients. For medically managed patients, higher mean pulmonary artery pressure on admission to NHS-1 and older age on admission to NHS-2 were associated with the presence of serious arrhythmias. For surgically managed patients, higher New York Heart Association functional class and cardiomegaly were associated with serious arrhythmias. CONCLUSIONS: The prevalence of arrhythmias (especially ventricular arrhythmias) was higher for patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect than for an historical control population. "Serious arrhythmias" were most frequently noted in patients with aortic stenosis who also had a higher incidence of sudden death. The prevalence of "serious arrhythmias" was second highest for patients with ventricular septal defect who had the second highest incidence of sudden death.

Adult↗
PubMed1995

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum.

OBJECTIVES: This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy. BACKGROUND: Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited. METHODS: Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation. RESULTS: Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 +/- 18.7 mm Hg (mean +/- SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for > or = 2 weeks after valvotomy (n = 4). Significant differences (p < or = 0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (-1.1) versus 5.5 mm (-3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (-1.8); right ventricular volume 65 versus 29 ml/m2; and Lewis index 10.9 versus 8.9. CONCLUSIONS: Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus > 11 mm, pulmonary valve annulus > or = 7 mm and right ventricular volume > 30 ml/m2.

Catheterization↗
PubMedDate not supplied

Supravalvular aortic stenosis, pulmonary artery stenosis, and coronary artery stenosis in twins.

Supravalvular aortic stenosis (SVAS) is rare in twins. We report monozygotic twins, both of whom had SVAS, coronary ostial stenosis, and peripheral pulmonary arterial stenosis, but no other phenotypic features of Williams syndrome. One of the twins died suddenly, but the SVAS and the right and left coronary ostial stenoses were enlarged successfully at operation in the other twin.

Aortic Valve Stenosis↗