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At least 343 records · Page 19Linked to original sources
PubMed1990

An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia.

A modified approach to the surgical management of corrected transposition of the great vessels with ventricular septal defect and pulmonary stenosis or atresia was used successfully in two patients. The procedure consisted of performing a venous switch operation, directing the blood flow from the morphologically left ventricle (right-sided chamber) into the aorta through the ventricular septal defect and inserting a valved conduit between the left-sided morphologically right ventricle and the pulmonary artery. This approach has several advantages when compared with the traditional surgical management, which consists of closure of the ventricular septal defect and a left ventricular (right-sided chamber) to pulmonary artery conduit. It uses the morphologically left ventricle as the systemic pumping chamber, thereby minimizing long-term ventricular failure. It allows closure of the defect from the right ventricular side of the septum, thus decreasing the prevalence of complete atrioventricular block. It also avoids use of the tricuspid valve as the systemic atrioventricular valve and therefore decreases the chance of postoperative valve regurgitation.

Adult↗
PubMedDate not supplied

Hypoplastic left heart syndrome and valvar pulmonary stenosis: presentation and management.

We describe the first report of an infant who was born with hypoplastic left heart syndrome and significant pulmonary stenosis. He was successfully palliated with multiple cardiac catheterization interventions while waiting for cardiac transplantation. This unusual constellation of heart defects is an entity that is rare and not well described in the literature.

Cardiac Catheterization↗
PubMed1981

Doublet-outlet right ventricle [S,D,L] with subaortic ventricular septal defect and pulmonary stenosis: successful repair using an intraventricular conduit.

An 18-year-old Japanese girl with double-outlet right ventricle (DORV) [S,D,L], subaortic ventricular septal defect and pulmonary stenosis was successfully treated under cardiopulmonary bypass by resection of the obstructing subpulmonic infundibulum, pulmonary valvulotomy and construction of an intraventricular conduit between the ventricular septal defect and the aorta. The postoperative course was uneventful, except for mild low-output syndrome, and she was discharged 7 weeks after surgery in good condition. She is at present free from all untoward cardiac symptoms.

Adolescent↗
PubMed2007

Aortic translocation for the management of transposition of the great arteries with a ventricular septal defect, pulmonary stenosis, and hypoplasia of the right ventricle.

A 2-month-old patient with transposition of the great arteries, a ventricular septal defect, pulmonary stenosis, and severe hypoplasia of the right ventricle successfully underwent a biventricular repair utilizing the aortic translocation technique. Advantages of this surgical repair over the Rastelli procedure in the management of this complex congenital heart lesion are discussed.

Aortic Valve↗
PubMed2004

Mechanisms of optimized biventricular pacing in pulmonary stenosis: effects on left ventricular geometry in swine.

We tested the hypothesis that optimized biventricular pacing (BiVP) enhances cardiac output (CO) during critical pulmonary stenosis (PS) by attenuating distortions in left ventricular (LV) geometry. Following median sternotomy in six anesthetized pigs, heart block was induced by ethanol ablation. During epicardial, DDD BiVP, atrioventricular delay (AVD) was varied from 60 ms to 180 ms in 30 ms increments. At the AVD with the highest CO right-left delay (RLD) was varied from (+) 80 ms (RV first) to (-) 80 ms (LV first) in 20 ms increments. At each pacing setting, aortic flow, ECG, and LV diameter were measured in the control state (CON) and during PS, created by snaring the pulmonary artery until CO decreased 50%. Short axis LV echocardiograms were obtained at (+) and (-) 80 ms. In CON, RLD had no effect on function or geometry. During PS optimum BiVP resulted in significant increases in CO (1.12 L/min +/- 0.13 SEM at RLD =+ 40 ms versus 0.92 +/- 0.12 at RLD = 0 and 0.73 +/- 0.08 at RLD =-80), and LV fractional shortening (8.97%+/- 0.51% at RLD =+ 40 ms versus 7.34%+/- 0.58% at RLD = 0 and 6.21%+/- 0.66% at RLD =-80). In addition, LV eccentricity with (-) RLD was significantly different versus CON at both end-diastole (0.79 +/- 0.07 vs 1.02 +/- 0.03, P = 0.011 Student's t-test) and end-systole (0.83 +/- 0.05 vs 1.00 +/- 0.02, P = 0.017). However, with (+) RLD differences versus CON were not significant at either end-diastole (0.88 +/- 0.06 vs 0.99 +/- 0.03) or end-systole (0.92 +/- 0.03 vs 1.01 +/- 0.03). In swine hearts with PS, optimized BiVP increases CO, fractional shortening, and LV symmetry. BiVP warrants further study as treatment for acute postoperative heart failure.

Animals↗
PubMed1989

Aortico-right ventricular tunnel and critical pulmonary stenosis: diagnosis by two-dimensional and Doppler echocardiography and angiography.

An infant with aortico-right ventricular (AO-RV) tunnel and critical pulmonary stenosis presented with severe distress at birth. We present the clinical, echocardiographic, and angiographic features, correlated with autopsy findings. Also we discuss the differentiation from other AO-RV communications and a theory for the embryogenesis. AO-RV tunnel should be considered in the differential diagnosis of a critically sick newborn with cyanosis, a "to and fro" murmur, and signs of right heart failure. The correct diagnosis can be made echocardiographically by demonstrating the two ends of the tunnel connecting the aorta and a dilated RV, two normal coronary arteries, and obtaining high-velocity systolic and diastolic Doppler flow signals in the tunnel. Surgical repair of this lesion is possible, and early diagnosis and a modification of the surgical procedure may help survival.

Angiography↗
PubMed1992

Changes of right ventricular size and function in neonates after valvotomy for pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Right ventricular end-diastolic and stroke volumes were calculated from orthogonal subcostal echocardiographic images in 24 neonates (mean weight +/- SD 3.4 +/- 0.4 kg) with pulmonary atresia (n = 18) or critical pulmonary stenosis (n = 6) and intact ventricular septum before and at an average of 5 days and then 19 days after pulmonary valvotomy. The preoperative echocardiographic volume determinations were compared with the respective angiographic determinations. In addition, the endocardial area outlines of the left and right ventricles were obtained by planimetry from an end-diastolic frame taken in the apical four-chamber view. End-diastolic and stroke volumes calculated by the echocardiographic method (y) correlated closely with those calculated by the angiographic method (x); the regression equations were y = 1.02 x -0.13 (r = 0.95, SEE +/- 0.45 ml) and y = 1.16 x -0.15 (r = 0.89, SEE +/- 0.38 ml), respectively. All except one infant had right ventricular hypoplasia before valvotomy with an end-diastolic volume of 16.6 +/- 6.4 ml/m2 (44.5 +/- 17.3% of normal). Right to left ventricular area ratio was 0.56 +/- 0.09 (normal 0.95). Five days after valvotomy, right ventricular end-diastolic volume decreased to 10.6 +/- 4.6 ml/m2 (p less than 0.05) and stroke volume decreased from 8.3 +/- 3.5 to 5.5 +/- 2.8 ml/m2 (p less than 0.05). Nineteen days after valvotomy, right ventricular end-diastolic volume and right to left ventricular area ratio had increased to their respective preoperative values; right ventricular stroke volume had increased further to 10.4 +/- 3.9 ml/m2 (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1980

Cystic thymoma simulating pulmonary stenosis.

A 36-year-old man presented with chest pain, an anterior mediastinal mass and a loud pulmonary systolic murmur. At operation a large, tense, well encapsulated, cystic thymoma was found to be compressing the right ventricular outflow tract and the main pulmonary trunk, producing a systolic thrill over the artery. After excision of the cyst, the thrill and the murmur disappeared and there was complete symptomatic relief. Classification, pathology, clinical presentation, diagnosis and treatment of cysts of the thymus are discussed. The syndrome of pulmonary artery compression characterized by a triad of chest pain, dyspnoea and a loud pulmonary systolic murmur, which decreases in intensity during inspiration, is described. Pertinent literature on cysts of the thymus, and acquired pulmonary stenosis due to extrinsic compression is reviewed.

Adult↗
PubMed1993

Balloon valvuloplasty for valvular pulmonary stenosis in children over 6 months of age: initial results and long-term follow-up.

Between 1984 and December 1990 balloon valvuloplasty for valvular pulmonary stenosis was planned and performed in 92 children over 6 months of age (range 0.8-17.9 years). Valvuloplasty decreased the invasively determined peak systolic gradient from 61 +/- 34 (mean +/- SD) to 27 +/- 20 mmHg (P < 0.0001). At follow-up cardiac catheterization, performed in 22 patients after 13 +/- 5 months, the gradient was 22 +/- 23 mmHg (ns). Initially the continuous wave Doppler gradient decreased from 61 +/- 23 to 26 +/- 12 mmHg (P < 0.0001). It then remained unchanged both at early follow-up after 0.6 +/- 0.3 years, being 23 +/- 12 mmHg, and at long-term follow-up after 3 +/- 1.7 years (21 +/- 10 mmHg, ns). In the 41 patients treated before the end of 1986 the residual continuous wave Doppler gradient at long-term follow-up after 5.1 +/- 0.8 years was 21 +/- 10 mmHg. Mild pulmonary regurgitation was present in 77% of the patients at early follow-up and in 83% at the last follow-up. Right ventricular hypertrophy on the electrocardiogram was present in 83% of the children before valvuloplasty. At early follow-up it had decreased to 44% with a further decrease to 24% at the last follow-up. Comparison of patients with initial invasive systolic gradients greater and smaller than 50 mmHg revealed no differences between either group with regard to the continuous wave Doppler gradient at last follow-up, the incidence of pulmonary regurgitation and the occurrence of significant complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1996

Acquired supravalvular pulmonary stenosis due to extrinsic compression by a metastatic thymic carcinoid tumor.

Acquired pulmonary artery stenosis is rare. There are two main types, firstly intrinsic disease of the pulmonary valve itself, such as carcinoid heart disease. Secondly, extrinsic compression of the pulmonary artery from a mediastinal structure. We report a case of acquired pulmonary supravalvular stenosis due to extrinsic compression by carcinoid mediastinal tumor, confirmed by echocardiographic imaging/Doppler interrogation and computerized tomography.

Carcinoid Tumor↗
PubMed1992

Hypoplastic right ventricle with mild pulmonary stenosis in an adult.

A rare cyanotic heart disease surviving to adulthood with minimal symptoms is presented. The final diagnosis was hypoplastic right ventricle with mild pulmonary stenosis, where the latter was not responsible for the right-to-left shunting across a large atrial septal defect. The differences from cases reported in the literature are highlighted.

Adult↗
PubMed1990

Natural history of supravalvular aortic stenosis and pulmonary artery stenosis.

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.

Adolescent↗
PubMed1997

[A case of modified Fontan operation for corrected transposition of great arteries (I.D.D.) with atrial septal defect, ventricular septal defect, pulmonary stenosis, mitral atresia and hypoplastic left ventricle].

A 14-year-old boy with corrected transposition of the great arteries (I.D.D.), atrial septal defect, ventricular septal defect, pulmonary stenosis, mitral atresia and hypoplastic left ventricle was successfully operated. He had undergone modified Blalock-Taussig shunt at the age of two. After atrial septal defect was closed with bovine pericardial patch, small ventricular septal defects were closed directly through left ventricular incision. Then the incisional line on the left ventricle was extended to pulmonary artery. The stenotic and thickened pulmonary valve was excised. The upper part of the incisional line on the right atrium and that on the left ventricle were anastomosed. Consequently the connection between the right atrium and the pulmonary artery was able to be made through hypoplastic left ventricle. This case is extremely rare type in terms of mitral atresia and is classified as Group II, type B according to Eliot's classification. This type of repair is similar to Björk's method, however, we could not make use of left ventricular contraction because it was weak. The merit of this procedure was to be able to reconstruct blood tract without using artificial conduits.

Adolescent↗