1/9. Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature.Absence of the pulmonary valve occurs usually in association with tetralogy of fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.- - - - - - - - - - ranking = 1keywords = pulmonary valve, atresia, valve (Clic here for more details about this article) |
2/9. Noncompacted myocardium in Ebstein's anomaly: initial description in three patients.Exercise intolerance in Ebstein's anomaly is usually attributed to desaturation secondary to right-to-left shunting as a result of a small or distorted left ventricle (LV), significant tricuspid valve regurgitation, right ventricular dysfunction, or a combination of these. We observed one boy (age 15 years) and two women (ages 20 and 29 years) with severe Ebstein's anomaly and strikingly abnormal LV myocardium resembling the features described for LV noncompaction. LV size and systolic function were normal in the two women; the boy had a dilated LV with severely diminished ejection fraction. The LV myocardium was found to be unusually coarse and hypertrabeculated, with small intertrabecular recesses and an irregular endocardial surface. The findings in these 3 patients represent the whole spectrum of mild to severe LV noncompaction. Diastolic dysfunction was present in 2 of the 3 patients. exercise tolerance was diminished in all. There was no mitral or aortic valve disease. The 15-year-old boy underwent heart transplantation 6 months later for biventricular failure. Thus, Ebstein's anomaly does not seem to be a pathology confined to the right ventricle, but may rarely lead to LV noncompacted myocardium. This LV pathology may be an additional explanation for exercise intolerance or signs of left heart failure in patients with Ebstein's anomaly.- - - - - - - - - - ranking = 0.00060088905226521keywords = valve (Clic here for more details about this article) |
3/9. Live three-dimensional transthoracic echocardiographic assessment of anomalous origin of left coronary artery from the pulmonary artery.We describe live three-dimensional transthoracic echocardiographic (3DTTE) findings in a 52-year-old female who had previously undergone an aortopulmonary tunnel operation for anomalous origin of the left coronary artery (ACA) from the pulmonary artery. Three-dimensional transthoracic echocardiography clearly delineated the origin of the ACA from the posterolateral aspect of the main pulmonary artery just above the pulmonary valve, the surgically created tunnel, as well as a small defect in the tunnel near the aortic end communicating with the pulmonary artery.- - - - - - - - - - ranking = 0.12415410833807keywords = pulmonary valve, valve (Clic here for more details about this article) |
4/9. prenatal diagnosis of tetralogy of fallot with absent pulmonary valve using advanced dynamic flow.tetralogy of fallot with pulmonary valve absence can cause severe respiratory distress immediately after birth; therefore, a prenatal diagnosis is important. In this paper, we report a case using advanced dynamic flow to enhance its diagnosis.- - - - - - - - - - ranking = 0.62077054169034keywords = pulmonary valve, valve (Clic here for more details about this article) |
5/9. Stenting of a stenotic aortic homograft in pulmonary position after the Ross procedure.Pulmonary autograft replacement of the aortic valve (Ross procedure) has potential advantages, with favorable rates of survival and freedom from reoperation. The procedure itself, however, involves insertion of a homograft in the pulmonary position. The development of severe homograft stenosis is an uncommon but clinically important complication. We report the case of a young female who developed a symptomatic homograft stenosis a year after she underwent the Ross procedure. The lesion was stented successfully and the homograft's patency, together with a markedly improved pressure gradient, was still maintained eight months after percutaneous stenting.- - - - - - - - - - ranking = 0.0003004445261326keywords = valve (Clic here for more details about this article) |
6/9. Right-sided cardiac involvement in osteogenesis imperfecta.Skeletal manifestations are the hallmark of the osteogenesis imperfecta group of disorders. Extraskeletal involvement may, however, contribute significantly to morbidity. Structural cardiovascular anomalies reported in osteogenesis imperfecta include aortic root dilatation and aortic and mitral valve dysfunction. Herein is reported the first case of involvement of the right side of the heart in osteogenesis imperfecta.- - - - - - - - - - ranking = 0.0003004445261326keywords = valve (Clic here for more details about this article) |
7/9. Pathologic and molecular analysis in a family with rare mixed supravalvar aortic and pulmonic stenosis.Nonsyndromic supravalvar aortic stenosis (SVAS) is an obstructive vascular disorder often inherited in an autosomal dominant manner. With pulmonary artery involvement, stenotic lesions are nearly always peripheral or downstream of the pulmonic valve. In rare cases when the supravalvar pulmonic region is affected, the stenoses usually improve over time and rarely affect prognosis. We evaluated a unique family in which 10 of 14 individuals have nonsyndromic SVAS and 7 of the 10 affected family members with SVAS have the rare finding of supravalvar pulmonic stenosis (SVPS). In at least 2 of these individuals, the severity of SVPS was so significant that it led to death in early infancy. Pathologic examination of stenotic lesions in this kindred group revealed concentrically organized smooth muscle cells separated by dense elastic fibers. In contrast, the arterial pathology reported for other individuals with nonsyndromic SVAS demonstrates increased numbers of hypertrophied smooth muscle cells separated by thin, fragmented elastin fibers. Molecular analysis identified a novel ELN mutation within the donor splice site of exon 16, which may be responsible for the unique phenotype and distinct elastin histopathology found in this kindred.- - - - - - - - - - ranking = 0.0003004445261326keywords = valve (Clic here for more details about this article) |
8/9. Cor pulmonale as a complication of ventriculo-atrial shunts reviewed.The authors present the case of a nine-year-old patient with spina bifida and hydrocephalus treated with a ventriculo-atrial shunt who developed fatal pulmonary hypertension 5 1/2 years after his last valve revision; he had been well and active up to one month before his death. Details of the dramatic cardiovascular findings are given. The problems of treatment, once symptoms occur, and the difficulty of pre-symptomatic detection are discussed.- - - - - - - - - - ranking = 0.0003004445261326keywords = valve (Clic here for more details about this article) |
9/9. Doppler echocardiographic findings of indomethacin-induced occlusion of the fetal ductus arteriosus.We present an unusual case of indomethacin-induced occlusion of the fetal ductus arteriosus, which occurred in one of twins. In fetal echocardiography, the characteristic findings, a to and fro regurgitation pattern at pulmonary valve and postvalvular dilation of the main pulmonary artery, were obtained in addition to right ventricular dilation and hypertrophy, tricuspid regurgitation, right atrium dilation, and pericardial effusion. This fetus developed fetal distress and was delivered by an emergency cesarean section at 35 weeks' gestation. We suggest that these fetal echocardiographic findings may be the end-stage signs of the fetal ductal occlusion as well as the signs for emergent delivery.- - - - - - - - - - ranking = 0.12415410833807keywords = pulmonary valve, valve (Clic here for more details about this article) |