Korean Circ J.  1976 Jun;6(1):71-81. 10.4070/kcj.1976.6.1.71.

A Clinical Study of Congenital Pulmonary Branch Stenosis

• Shim WH
• Kim SS
• Loh WS
• Cha HD

• KMID: 1677804
• DOI: http://doi.org/10.4070/kcj.1976.6.1.71

Abstract

The congenital pulmonary artery branch stenosis (PABS) was described by Oppenheimer (1) as early as in 1938, but it had attracted very little attention until the last decade. This disease is characterized grossly by single or multiple stenosis of the pulmonary artery and branches and has been described in the literature occasionally under the various designations including supravalvular pulmonary stenosis, coarctation of pulmonary artery, multiple peripheral stenosis of the pulmonary artery and pulmonary artery branch stenosis (3~22). It is known that this defect is no longer a very rare disease and functional problem only. The clinical significance of this disease is to differentiate from other congenital heart diseases such as patent ductus arteriosus, ventricular septal defect and pulmonic stenosis and is a cause of pulmonary hypertension. Nine cases of PABS confirmed by pressure difference in pullback tracing, selective angiography and rate of rise of pulmonary artery pressure are presented. This defect occurred as an isolated cardiovascular anomaly in 3 of the 9 patients. This condition was detected usually in childhood due to exertional dyspnea, frequent upper respiratory infections and heart murmur. Clinically the most significant diagnostic feature was the wide distribution of ejection systolic or continuous murmur, which was seldom louder than grade 4/6. It is also characteristic that the upstroke of the pulmonary artery pressure tracing is similar to the right ventricular tracing with the descending limb of the curve steeper and the dicrotic notch deeper with a low diastolic plateau. This results in a wide pulse pressure. Data are presented which suggest that comparing the rate of rise prestenotic with poststenotic pressure pulse may differentiate organic from functional pressure gradient. The purpose of this paper is to present in detail the clinical features including electrocardioram, chest X-ray, cardiac catherization, angiography, rate of rise of pulmonary artery pressure and diagnostic criteria of PABS. Only two cases of PABS were repoted in Korean literature from our laboratory.

MeSH Terms

Child
Male
Female
Humans