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PubMedDate not supplied

The evolution of the treatment of isolated pulmonary valve stenosis.

In the course of studies covering 40 years, 1913 to 1953, leading to the development of cardiopulmonary bypass, many ingenious blind instrumental or digital intrusions were made into the heart chambers to treat lesions therein. Limits were defined for the arrest of circulation through part or all of the heart to permit a brief glimpse of the lesion and effect at least a partial correction. The often remarkably good results of operations performed under less than ideal operating conditions for lesions such as isolated pulmonary stenosis encouraged the interventional cardiologist and radiologist, working together, to adapt the cardiac catheter, used previously for exploration of the vascular system and diagnosing intravascular lesions, to therapeutic purposes. They positioned a catheter with uninflated balloon attached in the pulmonary artery, then either by inflating the balloon beyond the constricted orifice and pulling it through or by rapidly and precisely inflating the balloon lying across the orifice were able to disrupt the stricture and relieve the stenosis. Results matched those of early non-visual operations. Recently the cardiologist has expanded the approach to relieve other constricted orifices in the heart and great vessels and to close abnormal openings. In isolated pulmonary stenosis, the nearly complete relief of obstruction and the tolerance of the circulation to blunt disruption of valvular stenosis bodies well for the long-term success of balloon valvuloplasty in this congenital malformation of the heart.

Angioplasty, Balloon↗
PubMed1990

Tetralogy of Fallot, pulmonary valve stenosis, ventricular septal defect, and hypertrophic cardiomyopathy in WKY/NCrj rats.

We examined anatomically the hearts of 198 WKY/NCrj rats of 20 litters. There were 51 rats with moderate to severe thickening of the pulmonary valve and 19 rats with a ventricular septal defect; the two lesions occurred together in 16 rats, in 15 of which there were overriding of the aorta, stenosis of the pulmonary outflow tract, and hypertrophy of the right ventricle, fulfilling the criteria for tetralogy of Fallot in man. The papillary muscle of the conus was absent in 65 rats. The heart was abnormally heavy in 18. We analyzed the relationship between cardiac hypertrophy and valvular lesions and septal defects in these rats plus 27 selected WKY rats with abnormally heavy hearts. Of the 151 rats with neither severe valvular lesions nor septal defects, six rats had abnormally heavy hearts and 67 rats had disproportionate ventricular septal thickening. This situation in the rats is similar to hypertrophic cardiomyopathy. The occurrence of these abnormalities, isolated or in association, in rats of an established inbred strain strongly suggests that they are etiologically or genetically linked, and that the rats should be a useful animal model for these diseases in man.

Animals↗
PubMed1985

Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis.

Percutaneous balloon valvuloplasty (BV) for pulmonic valve stenosis (PS) is increasingly becoming a nonsurgical alternative in patient management. However, the mechanism by which BV dilates the obstructive lesion has not been firmly established. We have had the opportunity to examine the effects of BV in the setting of PS and present two illustrative cases documenting the morphology after BV of the stenotic bicuspid and tricuspid pulmonary valve.

Adolescent↗
PubMed1987

Pulmonary valve stenosis associated with hypertrophic cardiomyopathy.

The association of pulmonic stenosis with hypertrophic cardiomyopathy is rare in infancy. Presented here is an infant with atypical picture of pulmonic stenosis and echocardiographic evidence of hypertrophic cardiomyopathy. At eight months of age, she had a successful percutaneous balloon valvuloplasty and has subsequently been managed with propranolol.

Cardiomyopathy, Hypertrophic↗
PubMed2002

Exceptional survival of a patient with large ventricular septal defect, bidirectional shunt, and severe pulmonary valve stenosis.

A 74-year-old man has survived in good health for an exceptionally long time despite the presence of a moderate-to-large-sized membranous ventricular septal defect (VSD). He has remained acyanotic with New York Heart Association class I function. Transthoracic and transesophageal echocardiography with color flow Doppler demonstrated a membranous VSD with left-to-right and right-to-left bidirectional shunts during ventricular systole and diastole, respectively, with an right ventricular systolic pressure of 93 mm Hg, dilation of the atria and the right ventricle, and right ventricular hypertrophy. The pulmonary valve was severely stenotic with transpulmonary valve peak velocity of 6.1 m/s and a peak pressure gradient of 147 mm Hg. The pulmonary artery and inferior vena cava were mildly dilated, and the left ventricular dimension and systolic function were normal. Transesophageal echocardiography with saline solution microbubble injection demonstrated positive contrast effect in the left ventricle in diastole confirming a right-to-left shunt at the ventricular level. This man is currently the oldest survivor with a moderate-to-large-sized membranous VSD reported in the literature.

Aged↗
PubMed1995

Use of the Terumo SP catheter system for crossing the pulmonary valve in infants with critical pulmonary valve stenosis.

In short, this method (the Terumo SP hydrophilic-polymer coated microcatheter system) has proved to be safe, effective, and flexible; it has allowed us to cross critically stenotic valves when standard methods have failed, enabling us to accomplish balloon valvuloplasty with good results in patients who might have otherwise required surgical intervention because of inability to cross the pulmonary valve.

Cardiac Catheterization↗
PubMed1985

Familial pulmonary valve stenosis, atrial septal defect, and unique electrocardiogram abnormalities.

The familial association of pulmonary stenosis, atrial septal defect, and unique electrocardiographic abnormalities involving a mother and two children is reported. Familial pulmonary stenosis not occurring as part of a named syndrome or without associated multiple congenital abnormalities is rare. The constellation of pulmonary stenosis, atrial septal defect, and the particular electrocardiogram abnormalities present here is to our knowledge previously unreported. The pattern of inheritance is consistent with an autosomal dominant mode of transmission.

Cardiomyopathy, Hypertrophic↗
PubMedDate not supplied

[Rate of prenatal detection of congenital right heart defects].

Congenital right heart lesions (including tetralogy of Fallot, pulmonary valve stenosis, pulmonary atresia with intact ventricular septum, Ebstein's anomaly and dysplastic tricuspid valve) account for about 19% of congenital cardiac anomalies. We performed a retrospective study in order to assess the percentage of patients with significant right heart lesions (requiring therapy in the first year of life), which is detected prenatally and referred to a centre for perinatal treatment. From 1/1990 until 12/1997 congenital right heart lesions were diagnosed in 21 fetuses and 190 infants (211 patients. The majority of patients had tetralogy of Fallot (64%), less frequently we found critical pulmonary valve stenosis (9%), pulmonary atresia with intact ventricular septum (9%), tricuspid atresia (14%) and Ebstein's anomaly or dysplastic tricuspid valve (4%). Prenatally the cardiac anomaly was diagnosed in all 21 cases who were referred to our center (10%). The highest referral and detection rate was found among fetuses with Ebstein's anomaly or dysplastic tricuspid valve (5/8 patients = 63%) followed by fetuses with pulmonary atresia and intact ventricular septum (5/20 = 25%), critical pulmonary stenosis (4/18 = 22%) or tricuspid atresia (4/29 = 14%). The prenatal referral rate was disappointing in children with tetralogy of Fallot (3/136 = 2.2%). A higher prenatal detection rate of congenital right heart lesions can be achieved only by an improvement of prenatal screening including the 4-chamber view and the origin of the great arteries. A first step would be the inclusion of the fetal 4-chamber view into the routine examination during the 18th-20th week of pregnancy (stage 1 of a multistage concept of prenatal screening) and by assessment of the outflow tracts and the great arteries in pregnancies associated with risk factors or anomalies of the fetus (stage 2 and 3 of a multistage concept).

Echocardiography↗