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Combined pulmonary stenosis and insufficiency preserves myocardial contractility in the developing heart of growing swine at midterm follow-up.

This study was conducted to determine the effects of chronic combined pulmonary stenosis and pulmonary insufficiency (PSPI) on right (RV) and left ventricular (LV) function in young, growing swine. Six pigs with combined PSPI were studied, and data were compared with previously published data of animals with isolated pulmonary insufficiency and controls. Indexes of systolic function (stroke volume, ejection fraction, and cardiac functional reserve), myocardial contractility (slope of the end-systolic pressure-volume and change in pressure over time-end-diastolic volume relationship), and diastolic compliance were assessed within 2 days of intervention and 3 mo later. Magnetic resonance imaging was used to quantify pulmonary insufficiency and ventricular volumes. The conductance catheter was used to obtain indexes of the cardiac functional reserve, diastolic compliance, and myocardial contractility from pressure-volume relations acquired at rest and under dobutamine infusion. In the PSPI group, the pulmonary regurgitant fraction was 34.3 +/- 5.8%, the pressure gradient across the site of pulmonary stenosis was 20.9 +/- 20 mmHg, and the average RV peak systolic pressure was 70% systemic at 12 wk follow-up. Biventricular resting cardiac outputs and cardiac functional reserves were significantly limited (P < 0.05), LV diastolic compliance significantly decreased (P < 0.05), but RV myocardial contractility significantly enhanced (P < 0.05) compared with control animals at 3-mo follow-up. In the young, developing heart, chronic combined PSPI impairs biventricular systolic pump function and diastolic compliance but preserves RV myocardial contractility.

Aging↗

Second natural history study of congenital heart defects. Pulmonary stenosis: echocardiography.

BACKGROUND: Two-dimensional and Doppler echocardiography can provide information about valve morphology, right ventricular size and function, and hemodynamics in patients with pulmonary stenosis. Therefore, two-dimensional and Doppler echocardiographic examinations were performed on patients with pulmonary stenosis returning for the Second Natural History Study of Congenital Heart Defects. METHODS AND RESULTS: Three hundred twenty-five patients with pulmonary stenosis underwent two-dimensional and Doppler echocardiographic examinations. Of these, 115 were in the medically treated group, and 210 had undergone a previous operation. Patients in the valvotomy group had a higher incidence of right ventricular dysfunction and a larger right ventricular diastolic dimension. The valvotomy group had a lower pulmonary valve mean gradient and a lower right ventricular systolic pressure than the medically treated group. For all patients, there was no significant correlation of the echocardiographic variables with the presence or absence of symptoms, reflecting the low incidence of patients with cardiac decompensation. CONCLUSIONS: These observations provide an objective measurement for assessing the status of the pulmonary valve and right ventricular response in patients with pulmonary stenosis. Limitations of echocardiography in this study are discussed.

Adolescent↗

Modified technique for balloon valvuloplasty of critical pulmonary stenosis in the newborn.

OBJECTIVES: We report our experience in eight consecutive neonates who underwent attempted balloon dilation as an initial therapy for critical valvular pulmonary stenosis, and we review in detail technical modifications that improved the success rate. BACKGROUND: Balloon dilation of the pulmonary valve has become the treatment of choice for valvular pulmonary stenosis in children and adults. There are few reports of its effectiveness in critical pulmonary stenosis in the newborn. In this setting, application of the technique of balloon dilation has been limited by the ability to advance the necessary guide wires and catheters across the stenotic, often near-atretic, pulmonary valve. METHODS: The pulmonary valve was crossed in all patients. When this could not be accomplished with an end-hole catheter, a soft guide wire was advanced directly across the pulmonary valve through the end-hole catheter positioned in the right ventricular outflow tract below the valve. Initial predilation was achieved in all patients by using a coronary dilation catheter in an effort to facilitate introduction of the definitive balloon dilation catheter. Definitive dilation with a balloon diameter of > or = 110% of the diameter of the pulmonary valve annulus was possible in six patients. RESULTS: Right ventricular pressure declined from a mean value of 108 +/- 32 mm Hg to a mean value of 49 +/- 11 mm Hg after balloon dilation, with no change in heart rate or aortic pressure in these six patients after definitive balloon dilation. CONCLUSIONS: The results of this small series suggest that critical valvular pulmonary stenosis in the newborn can be successfully treated by transluminal balloon valvuloplasty.

Cardiac Catheterization↗

[Indications of valvular pulmonary stenosis on plain chest films (author's transl)].

Plain chest films of 87 children aged 4-14 years with valvular pulmonary stenosis proved by cardiac catheterization and of an age matched control group of 92 healthy children were evaluated in a retrospective study. Up to 4 years the evaluation was impaired by the overlying thymus. The evidence of poststenotic dilatation of the pulmonary trunk and the left pulmonary artery branch together with a normal or increased left sided perihilar pulmonary vascularity is essential for the diagnosis of valvular pulmonary stenosis. The pulmonary trunk showed marked dilatation in 47 cases, mild dilatation in 11 cases and no dilatation in 21 cases. A cranial displacement of the pulmonary trunk which partially reached or overlapped the aortic knuckle was seen in 44 children. 50 children showed a hilar enlargement due to the poststenotic dilatation of the left pulmonary artery branch. 1/5 of the children showed extension of the poststenotic enlargement into the left sided segmental artery branches. Cardiac size, calculated by heart volume, was within age specific limits. No correlation was found concerning the severity of the stenosis and the radiographic findings. Indications of valvular pulmonary stenosis increased with the age of the child.

Adolescent↗

[Univentricular heart and pulmonary stenosis with a right-to-left shunt between the innominate and pulmonary veins after Glenn operation: a case report].

A 12-year-old girl with a univentricular heart and pulmonary stenosis, who had undergone Glenn operation at two years of age and Fontan operation at 11 years of age, had a right-to-left shunt via collaterals between the innominate and pulmonary veins. This right-to-left shunt was detected by two-dimensional contrast echocardiography. The contrast material was injected into the left antecubital vein appeared in the left atrium, and this was confirmed by innominate venography. The contrast material injected into the left innominate vein passed via the collaterals into the pulmonary veins bilaterally. No such cases have so far been reported. In our experience, in four of six cases after Glenn operation, including the present case, the abnormal collateral circulation from the superior vena cava to the atrium was detected by two-dimensional contrast echocardiography. This case is interesting embryologically and suggests that the connection between the pulmonary and systemic veins which is present in early fetal life, and subsequently disappears, was present after birth.

Adolescent↗

Creation of a one-way interatrial communication in the treatment of critical pulmonary stenosis with intact ventricular septum: a case report.

BACKGROUND: In biventricular repair of pulmonary outflow tract obstruction with intact ventricular septum, the right ventricle is loaded with total pulmonary blood flow acutely as the right-to-left shunt is abolished by closure of the atrial septal defect (ASD). METHODS: We designed a one-way interatrial communication by creation of an atrial septal flap to reduce the excessive volume load of the right ventricle. RESULTS: This procedure was successfully performed in a 3-year-old girl undergoing definitive biventricular repair for critical pulmonary stenosis associated with tricuspid stenosis and a small right ventricle. CONCLUSIONS: We believe that creation of a one-way interatrial communication might be a good alternative to adjustable ASD and/or bidirectional Glenn shunt in biventricular repair of critical pulmonary stenosis or pulmonary atresia with intact ventricular septum.

Child, Preschool↗

Congenital absence of the pulmonary valve associated with pulmonary stenosis, large ductus arteriosus and intact ventricular septum. Case report.

A six-day-old neonate was diagnosed with a severe form of the syndrome of absent pulmonary valve associated with pulmonary stenosis, aneurysmal dilatation of the pulmonary artery and rare findings including an intact ventricular septum and large ductus arteriosus. The patient underwent surgical repair by closed technique. Cardiac catheterization data, hemodynamic and clinical findings, and surgical technique are reported. Congenital absence of the pulmonary valve is a rare cardiac anomaly. An especially severe form occurs with ventricular septal defect and pulmonary stenosis. The usual findings are respiratory distress, aneurysmal dilatation of the pulmonary arteries and pulmonary stenosis.

Ductus Arteriosus, Patent↗

Surgical closed pulmonary valvotomy for critical pulmonary stenosis: implications for the balloon valvuloplasty era.

BACKGROUND: Closed pulmonary valvotomy for critical pulmonary stenosis has no apparent advantage over the percutaneous balloon technique, though it is used when balloon valvuloplasty fails. Experience of this technique at the Heart Institute, Tel Hashomer, since it was first used in 1973 has been reviewed. METHODS: Thirty eight infants up to 1 year old (25 of them neonates--that is, nil to 1 month old) with critical pulmonary stenosis were operated on from 1973 to 1989. All had a transventricular valvotomy, by a modification of the Brock method, and all underwent cardiac catheterisation before surgery. RESULTS: Five of the 25 neonates (20%) died, but none of the other infants, so that the total mortality (five out of 38) was 13%. Three of the 38 required an aortopulmonary shunt. All 38 survivors were followed up--from one month to 14 years (mean 7.5 years). All were symptom free at the last check up. Fifteen of the survivors had required further surgery; this was successful in all cases. CONCLUSIONS: For the balloon valvuloplasty era surgical pulmonary valvotomy provides a good back up for failed attempts at percutaneous valvuloplasty. Review of outcome provides data for comparison with balloon valvuloplasty in the future.

Catheterization↗

Pulmonary stenosis in recipient twins in twin-to-twin transfusion syndrome: report on 3 cases and review of literature.

This report describes 3 cases of pulmonary stenosis in the recipient twin in twin-twin transfusion syndrome. Fetal echocardiography showed cardiomegaly, tricuspid valve regurgitation, and increased reverse flow in the inferior vena cava, as signs of congestive heart failure in all 3 cases. We diagnosed 2 cases of pulmonary stenosis by fetal echocardiography prenatally and confirmed our findings in all 3 cases postnatally. Two cases underwent postnatal balloon valvuloplasty to release the pulmonary valvular stenosis in neonatal period. The third one died soon after delivery and autopsy showed a slightly thickened pulmonary valve. One of the cases was diagnosed in the early second trimester (20 weeks of pregnancy), the earliest detection of fetal pulmonary stenosis reported in literature. The presence of high peak velocity of the pulmonary artery at 20 weeks of pregnancy preceded the development of pulmonary stenosis in this case. This supports the hypothesis that alterations in fetal hemodynamics may result in structural cardiac abnormality.

Adult↗

[Phonomechanographic evaluation of the severity of pulmonary stenosis].

External phonomechanographic methods allow us to obtain a fairly precise estimate of the degree of obstruction to the pulmonary outflow, whether such an obstruction is a single entity or associated with another intracardiac malformation. The greatest precision in this field is obtained with the cases of pure pulmonary stenosis. The findings on phonomechanographic investigation of 54 cases with pulmonary stenosis have been compared with the results of the haemodynamic tests (catheterisation and angiography) as well as with the anatomical findings after a surgical cure had been effected in a certain number of cases. This study has allowed us to pick out five phonomechanographic criteria of severity of pulmonary stenosis: an increased reduplication of the second sound, lenghening of the interval between the Q wave of the electrocardiogram and the maximum portion of the systolic murmur, an increase in Furuta's ratio and also in the amplitude of the jugular venous a-wave as a function of the total height t (the a/t ratio), but most especially the "corrected" Furuta ratio as a function of the length of the ejection phase of the right ventricle, as this has been shown to possess the best correlation with the right ventricular systolic pressures.

Adolescent↗

Serial studies of pulmonary stenosis in infancy and childhood.

Thirty-five children with pulmonary stenosis were catheterized from 1 day to 9 years of age and recatheterized after 2 weeks to 15 years. Right ventricular systolic pressure rose in 24 and the increase was greater in those under than over 5 years old. Pulmonary valve orifice area per square metre of body surface area increased in 12, but did not change in 3, and fell in 17; absolute decrease in calculated orifice area was usually associated with infundibular hypertrophy. Increase in right ventricular systolic pressure with age was thus caused by failure of the valve orifice to grow fast enough to keep pace with the increase of stroke volume. In the whole group, increasingly severe pulmonary stenosis was matched by increasing electrocardiographic evidence of right ventricular hypertrophy. However, in individual patients the electrocardiogram could suggest that right ventricular pressure had decreased when in fact it had risen considerably.

Age Factors↗

Isolated subvalvular pulmonary stenosis: depiction at whole heart magnetic resonance imaging.

Isolated subvalvular pulmonary stenosis is a rare condition and its morphological evaluation is obscure. Whole heart magnetic resonance imaging (MRI) is a new, totally non-invasive technique which allows three-dimensional comprehension of the cardiac structure. We describe a patient with isolated subvalvular pulmonary stenosis, in whom whole heart MRI was useful to detect and evaluate the right ventricular outflow obstruction.

Humans↗

Structural remodeling of the renal vascular bed during experimental pulmonary stenosis and after its correction.

Renal vessels in pups with experimental pulmonary stenosis, animals with corrected defect, and control dogs were examined by histological and morphometric methods. Pulmonary stenosis was followed by remodeling of the renal vascular bed and adaptive and pathological reconstruction of veins, arteries, and glomeruli. Correction of the defect was followed by regression of pathological changes.

Animals↗

Idiopathic calcific constrictive pericarditis causing pulmonary stenosis associated with a ventricular septal defect mimicking tetralogy of Fallot.

We describe an unusual case of pulmonary stenosis caused by calcific constrictive pericarditis associated with a congenital ventricular septal defect in a 16-year-old boy who had a 2-week history of progressive dyspnea, cyanosis, fatigue, and bilateral leg edema. Echocardiographic findings led to an initial diagnosis of tetralogy of Fallot; however, findings on chest radiography and CT were suggestive of calcific constrictive pericarditis with pulmonary stenosis, which was then confirmed on cardiac catheterization. Total pericardiectomy and repair of the ventricular septal defect resulted in a satisfactory outcome. Follow-up examinations at 6 and 20 months showed that the patient was asymptomatic and considered to have class I New York Heart Association functional status. To our knowledge, this is the first reported case of calcific constrictive pericarditis with pulmonary stenosis associated with a ventricular septal defect.

Adolescent↗

Experience with balloon valvuloplasty in pulmonary stenosis.

Fourteen patients of pure valvular pulmonary stenosis of moderate to severe degree underwent balloon valvuloplasty in the Department of Cardiology, SSKM Hospital, Calcutta. Haemodynamic study revealed that immediately after valvuloplasty, right ventricular pressure dropped down from 125 +/- 17.18 mmHg. to 56.67 +/- 8.72 mmHg. (mean +/- SD). Restudy was done in each case after 4 weeks, which showed that right ventricular systolic pressure had further dropped down to 46.71 +/- 5.06 mmHg. (Mean +/- SD). Patients were further followed up for 6 to 15 months (mean 10 months). During the follow-up period, all the patients remained asymptomatic. Drop of right ventricular systolic pressure was maintained except in one case in which the peak systolic pressure gradient across the pulmonary valve was raised to 61 mmHg. from 24 mmHg., the gradient achieved immediately after valvuloplasty.

Adolescent↗

[Congenital peripheral pulmonary stenosis. Presentation of 16 cases and review of the literature].

Sixteen cases of peripheral pulmonary stenosis have been studied clinically and by cardiac catheterization. Diagnosis has been proved in all cases by manometric measurements and/or cineangiocardiography. All patients except two were below 2 years of age. Ten cases were of type I, i.e. the stenosis was localized to the pulmonary trunk or its main branches. Six patients were of type III, i.e. they had diffuse stenosis of the pulmonary arterial tree. The physical findings, which in many cases are typical and include the presence of a systolic murmur over both lung fields, should alert the physician to the diagnosis at the bedside. At cardiac catheterization the configuration of the pressure tracing in the main pulmonary artery is typical, showing an abrupt rise and fall of the systolic wave followed by a low situated dicrotic notch. There is no doubt that in the past many cases of peripheral pulmonary stenosis have been wrongly diagnosed as "essential pulmonary hypertension".

Child, Preschool↗

Balloon dilatation pulmonary valvuloplasty in pulmonary stenosis.

Balloon valvuloplasty was used to treat congenital pulmonary valve stenosis in 13 patients, 11 with isolated pulmonary stenosis, one associated with complex univentricular heart and tricuspid atresia, and one associated with tetralogy of Fallot. A balloon catheter was introduced through the pulmonary valve over a guidewire positioned in the distal pulmonary artery and inflated for 15 seconds on two or three occasions to 3 to 5 atmospheres and rapidly deflated. Marked improvement in pressure gradients was achieved in eight of the 11 patients with isolated pulmonary valve stenosis and symptoms disappeared in the only patient in this group who was symptomatic. The two patients with complex congenital malformations improved clinically. The procedure was without complication and satisfactory results have been maintained from two to twelve months later. The procedure offers effective treatment. We believe that pulmonary balloon valvuloplasty should be the initial treatment for isolated severe and moderate pulmonary valve stenosis.

Catheterization↗