Disease relevance of Pulmonary Valve Stenosis

• 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 [1].
• CONCLUSIONS: These data indicate that not only supravalvular pulmonary stenosis but also pulmonary valvular stenosis due to a small annulus can occur in TGA, especially in patients with a history of pulmonary artery banding and in patients with ventricular septal defect [2].
• Bacterial endocarditis in patients with pulmonary stenosis, aortic stenosis, or ventricular septal defect [3].
• Infundibular pulmonary stenosis, a subaortic large infracristal ventricular septal defect, mitral-aortic fibrous continuity and equal pressures in both the ventricles and aorta were considered mandatory for the diagnosis of tetralogy of Fallot [4].
• Outcomes in critically ill neonates with pulmonary stenosis and intact ventricular septum: a multiinstitutional study. Congenital Heart Surgeons Society [5].

Psychiatry related information on Pulmonary Valve Stenosis

• The phenotype include microcephaly, microtia with pre auricular tags, hypertelorism, epicanthus, palatal cleft, short neck, winging scapulae, hypoplasia of the distal phalanges, pulmonary stenosis and mental retardation [6].
• TGA/VSD/pulmonary stenosis was associated with lower scores than TGA and TGA/VSD in physical functioning (P=0.002), general health perceptions (P=0.012), and mental health (P=0.048) [7].

High impact information on Pulmonary Valve Stenosis

• Keutel syndrome (KS, MIM 245150) is an autosomal recessive disorder characterized by abnormal cartilage calcification, peripheral pulmonary stenosis and midfacial hypoplasia [8].
• From 1958 to 1969, 592 patients (mostly children) with pulmonary stenosis were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization [9].
• The familial association of pulmonary stenosis, atrial septal defect, and unique electrocardiographic abnormalities involving a mother and two children is reported [10].
• One patient had a sibling with pulmonary stenosis, but there was no other known familial cardiomyopathy [11].
• One-stage repair of complete atrioventricular canal, double-outlet right ventricle, left superior vena cava, cor triatriatum and pulmonary stenosis [12].

Chemical compound and disease context of Pulmonary Valve Stenosis

• Those from bis-diamine treated embryos exhibited common arterial trunk (32%), overriding aortic valve with valvar pulmonary stenosis (40%) or infundibular atresia (4%), or ventricular septal defect (24%) [13].
• The postvalvulotomy propranolol test to determine reversibility of the residual gradient in pulmonary stenosis [14].
• Pulmonary stenosis (PS) was therefore used as a model to determine its effect on lung development, which was assessed noninvasively by carbon monoxide transfer at rest and during exercise [15].
• Continuous spinal analgesia with sufentanil for labor and delivery in a parturient with severe pulmonary stenosis [16].
• Experience with 36 consecutive neonates less than 1 month of age with critical pulmonary stenosis (PS) with intact ventricular septum was analyzed to define the role of pulmonary valvotomy and of a concomitant systemic-pulmonary shunt as well as the impact of prostaglandin E1 (PGE1) therapy in the management of this disorder [17].

Biological context of Pulmonary Valve Stenosis

• This finding is supported by a recent study in the Keeshond dog demonstrating a genetic predisposition common to tetralogy of Fallot, pulmonary stenosis and ventricular septal defect [18].

Anatomical context of Pulmonary Valve Stenosis

• A case of double-outlet left ventricle [I,D,D] with a subaortic ventricular septal defect and pulmonary stenosis is reported [19].
• Both patients had associated pulmonary stenosis, ventricular septal defect, and a large tricuspid valve whose chordal attachments crossed the right ventricular outflow tract [20].
• For ventricular septal defect, pulmonary stenosis, atrial septal defect, persistent ductus arteriosus, coarctation of the aorta and tetralogy of Fallot, we have estimated the nationwide prevalence and the surgical requirements for 1995 [21].
• Seven patients underwent a second reoperation for supravalvular pulmonary stenosis (n = 3), mitral valve replacement (n = 1), ventricular septal defect closure (n = 1), and recurrent coarctation (n = 2) [22].
• Two brothers, 17 and 21 years of age, with depressed nasal bridge, prominent frontal bones, hypoplastic maxilla, mild sensorineural hearing loss, broad terminal phalanges and mild pulmonary stenosis are presented [23].

Gene context of Pulmonary Valve Stenosis

• Bullous ichthyosiform erythroderma, developmental delay, aortic and pulmonary stenosis in association with a FRA12A [24].
• Transaortic closure of ventricular septal defect in patients with corrected transposition with pulmonary stenosis or atresia [25].
• Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1) [26].
• Carcinoma of the pituitary in association with pulmonary stenosis and microcephaly [27].
• CHARGE-association with pulmonary stenosis [28].

Analytical, diagnostic and therapeutic context of Pulmonary Valve Stenosis

• We describe a case of double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis treated successfully with cardiopulmonary bypass [29].
• Eight of 45 patients (17.8%) underwent reoperation: 2 for residual ventricular septal defect, 1 for recurrent septic vegetation, and 5 for pulmonary stenosis [30].
• Echocardiography has shown moderate/severe pulmonary insufficiency in 9 patients (26.5%), moderate pulmonary stenosis in 3 patients (8.8%) and residual ventricular septal defect in 4 patients (11.8%) [31].
• These changes were more pronounced when there was complete cessation of Qp during balloon valvuloplasty in pulmonary stenosis, with a fall in Vt (9.71 (2.95) to 9.32 (2.84) ml/kg; p < 0.05) and Cdyn (0.72 (0.29) to 0.64 (0.26) ml/cm H(2)O*kg; p < 0.001), and there was also an increase in Rrs (25.1 (1. 7) to 28.8 (1.6) cm H(2)O/litre*s; p < 0.01) [32].
• METHODS: Since 1995, 12 patients ranging in age from 3 to 22 years, with transposition of the great arteries or double outlet right ventricle associated with pulmonary stenosis and ventricular septal defect, underwent the Rastelli procedure with the RVOT reconstruction utilizing cryopreserved aortic valved homograft conduits [33].

References