OBJECTIVES:This study was designed to determine the feasibility of Doppler generation of accurate, complete right ventricular and pulmonary artery pressure curves in patients with Doppler-measurable tricuspid and pulmonary regurgitation.BACKGROUND:Doppler-derived flow velocities have been used to assess right ventricular systolic pressure; pulmonary artery systolic, diastolic and mean pressures, and left ventricular systolic and diastolic pressures. Instantaneous gradient across any area of discrete narrowing is accurately derived using the simplified Bernoulli equation (4V2). Invasive catheterization is currently the only means of generating intracardiac pressure curves. Noninvasively derived pressure curves using Doppler echocardiography would be a considerable advance in the assessment of normal and pathologic cardiac hemodynamics.METHODS:Right ventricular and pulmonary artery pressure curves were generated in 18 of 22 patients with measurable tricuspid and pulmonary valve regurgitation using superimposition of Doppler-measured tricuspid and pulmonary valve blood flow velocities on an assumed right atrial pressure. Doppler-measured right ventricular and pulmonary artery pressure curves were compared with simultaneous catheterization-measured curves.RESULTS:Doppler-derived pulmonary artery systolic pressure (Doppler PAP) correlated with simultaneous catheter-measured pulmonary artery pressure (Cath PAP) by the equation Doppler PAP = 0.92(Cath PAP) + 4.5, r = 0.98. Other Doppler-derived pressure measurements that correlated at near identity with the catheterization-measured corresponding measurement include Doppler-derived pulmonary artery mean pressure (Doppler mean PAP) [Doppler mean PAP = 0.85(Cath mean PAP) + 2.6, r = 0.97], and Doppler-derived right ventricular pressure (Doppler RVP) [Doppler RVP = 0.84(Cath measured RVP) + 7.9, r = 0.98]. Doppler-derived pulmonary, artery diastolic pressure (Doppler PAP diast) did not correspond as well in this study [Doppler PAP diast = 0.45(Cath PAP diast) + 6.6, r = 0.83].CONCLUSIONS:Clinically usable right ventricular and pulmonary artery pressure curves can be derived by superimposing Doppler-measured tricuspid and pulmonary valve blood flow velocities in patients with tricuspid and pulmonary valve regurgitation.

Eleven children who received transplants at less than 2 years of age underwent 59 echocardiograms at the time of endomyocardial biopsy for the assessment of the ability of echocardiography to predict acute rejection in the infant heart transplant recipient. Two patients died of acute rejection and autopsy findings were compared with premortum echocardiograms. Biopsy specimens were graded as no rejection (n = 46), mild rejection (cellular infiltrate, n = 5), or moderate-severe rejection (myocyte necrosis/edema, n = 8). Echocardiographic indexes measured included the following: left ventricular mass, left ventricular volume, ejection fraction, heart rate, and peak rate of posterior wall thinning. Compared with controls, patients during mild rejection had slower posterior wall diastolic thinning (p < 0.01). No significant change was noted in left ventricular mass until endomyocardial biopsy specimens showed severe rejection. No significant changes were noted in heart rate or ejection fraction in any of the groups. In conclusion, decrease in the peak rate of posterior wall diastolic thinning may be a sensitive indicator of acute rejection in the infant heart transplant recipient.

Supravalvular aortic stenosis (SVAS) is an autosomal dominant disorder characterized by abnormalities of development of the great vessels. SVAS is also commonly part of Williams syndrome. Linkage to the elastin gene on chromosome 7q11 has recently been reported in two kindreds with SVAS. Previous reports of patients with 7q11 deletions have noted great vessel abnormalities in some. We report on a family in which SVAS is cosegregating with a balanced reciprocal translocation, t(6:7) (p21.1;q11.23), providing further evidence that SVAS is the result of a mutation of elastin at 7q11.23 region. The propositus of the translocation family has some minor anomalies which occur in Williams syndrome, suggesting that elastin abnormalities may cause some of the abnormalities found in Williams syndrome.