The Syndrome of Pulmonary Stenosis Complicating Maternal Rubella

Auscultatory and phonocardiographic manifestations of isolated stenosis of the pulmonary artery and its branches.

“…1) and involve the main pulmonary artery as well as the proximal and distal branches, it is understandable that the systolic murmur is characterized by a wide thoracic distribution (Venables, 1965;D'Cruz et al, 1964;Delhney and Nadas, 1964;Franch and Gay, 1963). Accordingly, systolic murmurs are not only heard centrally in the vicinity of the second left and right interspaces but also peripherally in the axillae and back (Luan et al, 1960;Venables, 1965;Franch and Gay, 1963;Shafter and Bliss, 1959). Murnur in the vicinity of the second right interspace may be difficult to distinguish from supravalvular aortic stenosis which in fact may coexist (Beuren et al, 1964).…”

“…The association of these two anomalies, especially in the rubella syndrome, must be kept in mind (Venables, 1965). However, in uncomplicated stenosis of the pulmonary artery and its branches, a systolic murmur is the rule and a continuous murmur the exception.…”

“…If the intensity of pulmonary closure were influenced by the abruptness with which the leaflets seat, then the resulting sound should be increased. Though some observers have described a relatively loud pulmonary component of the second heart sound (Delaney and Nadas, 1964;Gyllensward et al, 1957), others have not found a uniform increase in intensity (Perloff, 1967;Venables, 1965;Franch and Gay, 1963;D'Cruz et al, 1964;Arvidsson et al, 1961;Bourassa and Campeau, 1963;Dighiero et al, 1957). In any event, in valvular or subvalvular pulmonary stenosis, the loudness of pulmonary closure usually varies inversely with the degree of obstruction (Perloff, 1963), whereas in pulmonary artery stenosis, the intensity is maintained but not necessarily increased as the obstruction becomes more severe.…”

“…In isolated pulmonary artery stenosis, the second heart sound may be narrowly split (Fig. 5), even in the face of appreciable obstruction (Delaney and Nadas, 1964;Venables, 1965;Perloff, 1967). It is usually found that the degree of splitting varies appropriately with respiration (Perloff, 1967) (Fig.…”

“…Pulmonary artery stenosis may be unilateral (Luan et al, 1960) or bilateral (Agustsson et al, 1962), single or multiple (D'Cruz et al, 1964); it may involve a localized arterial site with poststenotic dilatation or varying lengths of a branch from segmental narrowing to diffuse tubular hypoplasia (D'Cruz et al, 1964;Franch and Gay, 1963). Though pulmonary artery stenosis occurs as an isolated congenital malformation, it frequently coexists with other anomalies, especially valvular pulmonary stenosis (Hall et al, 1961;Franch and Gay, 1963;D'Cruz et al, 1964;Rowe, 1963), atrial septal defect (Shafter and Bliss, 1959;Franch and Gay, 1963), ventricular septal defect (D'Cruz et al, 1964), patent ductus arteriosus (Rowe, 1963;D'Cruz et al, 1964;Venables, 1965), and Fallot's tetralogy (Baum et al, 1964;D'Cruz et al, 1964). In addition, the combination of supravalvular aortic stenosis and bilateral branch stenosis of the pulmonary arteries forms a recognizable syndrome, especially when accompanied by peculiar facies, abnormal dentition, mental retardation, and infantile hypercalcaemia (Beuren et al, 1964).…”