Amnon Rosenthal

Doppler evaluation of left ventricular diastolic filling in children with systemic hypertension

To assess left ventricular (LV) diastolic function in children with systemic hypertension, 11 patients with hypertension (mean blood pressure 99 mm Hg) and 7 normal patients (mean blood pressure 78 mm Hg) underwent M-mode echocardiography and pulsed Doppler examination of the LV inflow. From a digitized trace of the LV endocardium and a simultaneous phonocardiogram, echocardiographic diastolic time intervals, peak rate of increase in LV dimension (dD/dt), and dD/dt normalized for LV end-diastolic dimension (dD/dt/D) were measured. Doppler diastolic time intervals, peak velocities at rapid filling (E velocity) and atrial contraction (A velocity), and the ratio of E and A velocities were measured. The following areas under the Doppler curve and their percent of the total area were determined: first 33% of diastole (0.33 area), first 50% of diastole, triangle under the A velocity (A area), and the triangle under the E velocity (E area). The A velocity (patients with hypertension = 0.68 +/- 0.11 m/s, normal subjects = 0.49 +/- 0.08 m/s), the 0.33 area/total area (patients with hypertension = 0.49 +/- 0.09, normal subjects = 0.58 +/- 0.08), the A area (patients with hypertension = 0.17 +/- 0.05, normal subjects = 0.12 +/- 0.03), and the A area/total area (patients with hypertension = 0.30 +/- 0.11, normal subjects = 0.20 +/- 0.07) were significantly different between groups (p less than 0.05). M-mode and Doppler time intervals, (dD/dt)/D, E velocity, and the remaining Doppler areas were not significantly different between groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic aneurysm after patch aortoplasty repair of coarctation: A prospective analysis of prevalence, screening tests and risks

Twenty-nine children were evaluated prospectively for the presence of an aortic aneurysm at the repair site 1 to 19 years after patch aortoplasty repair of coarctation of the aorta. In each child, noninvasive evaluation included a chest X-ray film, computed tomography of the chest and two-dimensional echocardiography. The presence and size of an aortic aneurysm were determined quantitatively by measuring the ratio of the diameter of the thoracic aorta at the repair site to the diameter of the aorta at the diaphragm (aortic ratio). An aortic ratio of greater than or equal to 1.5 was judged abnormal and was shown to be significantly greater than the aortic ratio of a normal control group. An aortogram was obtained in each child if any noninvasive screening test was found to be abnormal. As assessed by the aortogram, the prevalence of aortic aneurysm was 24% in this patient group. The sensitivity of echocardiography and chest computed tomography for detecting an aneurysm was 71% and 66%, and the specificity 76% and 85%, respectively. The chest X-ray film was 100% sensitive and 68% specific in determining the presence of an aneurysm. Although the data are not statistically significant, they suggest that children undergoing patch aortoplasty as the primary procedure (rather than a reoperation after earlier resection), and children in whom a Dacron patch is utilized may be at increased risk for aneurysm formation.(ABSTRACT TRUNCATED AT 250 WORDS)

Percutaneous balloon valvuloplasty for treatment of congenital pulmonary valvular stenosis in children

Percutaneous balloon pulmonary valvuloplasty was performed in seven children with moderate to severe valvular pulmonary stenosis (right ventricular to pulmonary artery pressure gradient greater than 50 mm Hg). All patients experienced a decrease in right ventricular peak systolic pressure from 108 +/- 30 to 60 +/- 5.6 mm Hg (p less than 0.001), decrease in right ventricular to pulmonary artery gradient from 90.1 +/- 30 to 38.8 +/- 5 mm Hg (p less than 0.001) and increase in pulmonary valve area from 0.33 +/- 0.06 to 0.55 +/- 0.15 cm2/m2 (p less than 0.001). In the two patients who underwent supine bicycle exercise before and after valvuloplasty, a significant decrease in both the maximal right ventricular peak systolic pressure (212 to 140 and 175 to 125 mm Hg, respectively) and in right ventricular to pulmonary artery peak pressure gradient (185 to 110 and 151 to 85 mm Hg, respectively) occurred. All patients tolerated the procedure well and no serious complications were observed. It is concluded that percutaneous balloon valvuloplasty is a safe and effective method for relief of right ventricular obstruction due to moderate or severe valvular pulmonary stenosis. However, long-term results remain unknown.

Reoperation for Coarctation of the Aorta

Between 1957 and 1980 reoperation for coarctation of the aorta was performed in 21 patients at one institution for an overall incidence rate of 7.9 percent. The incidence rate of reoperation was 38 percent for patients younger than age 3 years and 1.5 percent for patients 3 years or older at initial repair. Before reoperation 14 of the 21 patients were symptomatic, 19 had systolic hypertension of the upper limbs and 20 had a documented coarctation pressure gradient at rest (mean 42.4 mm Hg). Surgical techniques used at reoperation were patch aortoplasty in 12 patients, graft interposition in 4, end to end anastomosis in 3 and end to side left subclavian to descending aorta bypass graft in 2. There was one surgical death. The 20 survivors have been followed up a mean of 4.3 years. There has been significant symptomatic improvement (p < 0.001). Upper limb hypertension has also lessened significantly (p < 0.001) after reoperation;15 patients are no longer hypertensive and 3 have a lesser degree of hypertension. The coarctation pressure gradient at rest has significantly decreased (p < 0.001); 13 patients have no residual gradient and 7 have a mild gradient of 20 mm Hg or less. Graded treadmill exercise testing performed in five patients after reoperation documented upper limb hypertension in four and a marked increase in coarctation gradient with exercise in three. In conclusion, the incidence of reoperation is significantly increased in patients who are younger than age 3 years at initial coarctation repair. Reoperation is a safe and effective procedure. It has a low mortality rate (4.8 percent), relieves symptoms and decreases hypertension and the coarctation pressure gradient. Patch aortoplasty appears to be the operative procedure of choice. Moderate to severe hemodynamic abnormalities may persist during exercise after reoperation for coarctation of the aorta.

Results of balloon valvuloplasty in the treatment of congenital valvar pulmonary stenosis in children

Transluminal balloon valvuloplasty was used in the treatment of congenital valvar pulmonary stenosis in 19 children, aged 5 months to 18 years. The right ventricular (RV) systolic pressure and RV outflow tract gradient decreased significantly immediately after the procedure (95 +/- 29 vs 59 +/- 14 mm Hg, p less than 0.01, and 78 +/- 27 vs 38 +/- 13 mm Hg, p less than 0.01). Seven of these patients were evaluated at cardiac catheterization 1 year after balloon valvuloplasty. No significant change occurred in RV systolic pressure or RV outflow tract gradient at follow-up evaluation compared with measurements immediately after balloon valvuloplasty (60 +/- 5 mm Hg vs 56 +/- 12 mm Hg and 39 +/- 5 vs 38 +/- 10 mm Hg). In addition, follow-up evaluation was performed using noninvasive methods and included electrocardiography (n = 13), vectorcardiography (n = 11) and Doppler echocardiography (n = 11) Doppler echocardiography in 11 patients 15 +/- 9 months after balloon valvuloplasty showed a continued beneficial effect with a mild further decrease in RV outflow tract gradient. Thus, balloon valvuloplasty is effective in the relief of pulmonary stenosis.

Shone's anomaly: Operative results and late outcome☆

Shones anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation. The prognosis for patients with Shones anomaly is poor. To assess operative results and late outcome, we reviewed the records of 30 consecutive patients seen with Shones anomaly at our institution between 1966 and 1989. Anatomical diagnoses in these patients were supravalvar mitral ring (22 patients), mitral valve abnormalities including parachute mitral valve, fused chordae, or single papillary muscle (26 patients), subaortic gradients (26 patients), and coarctation (29 patients). Nineteen patients had all four lesions. Other common defects were bicuspid aortic valve (19 patients) and ventricular septal defect (20). Two patients were treated medically. The other 28 patients required 84 operative procedures with 18 patients undergoing more than one procedure. Operations included coarctation repair (28 patients), mitral valve repair or replacement (11), ventricular septal defect closure (8), subaortic resection (8), and complex left ventricular outflow tract reconstruction or bypass (4). Age at first operation ranged from 7 days to 7 years (median age, 3 months). There were no operative deaths at the first operation. However, mortality rose to 24% (4/17) after the second operation. All operative deaths were secondary to severe mitral valve disease. The survivors have been followed from 1 to 16 years (mean follow-up, 6 +/- 1 years). There were no late or sudden deaths. Morbidity has included stroke (1), gastrointestinal bleeding (2), permanent heart block (1), and persistent congestive heart failure (6).(ABSTRACT TRUNCATED AT 250 WORDS)

Use of balloon angioplasty to treat peripheral pulmonary stenosis

Balloon angioplasty was attempted in 13 children with peripheral pulmonary arterial (PA) stenosis. In 5 patients, angioplasty was successful in relieving the peripheral PA stenosis as judged by an increase in PA size of more than 75% over the predilatation size and a more than 50% reduction in the distal PA to main PA peak systolic pressure gradient. Each child has been followed for 6 to 30 months. All remain well, without signs of subsequent deterioration, and follow-up angiograms in 2 patients (at 10 and 12 months) showed persistence of anatomic and hemodynamic improvement. In 8 patients, angioplasty was unsuccessful: In 4 patients, stenosis at the site of a previous systemic-to-PA shunt could not be dilated and in 4 patients, angioplasty could not be performed because of technical difficulties. Thus, we could not dilate the stenosis in more than 60% of the patients; we also had a significant complication with the angioplasty procedure (perforation of a distal branch of the right pulmonary artery). Thus, although balloon angioplasty was not effective in all patients, it did provide significant improvement in some patients in whom traditional operative management is usually unsuccessful.

Acute hemodynamic effects of captopril in children with a congestive or restrictive cardiomyopathy.

The acute hemodynamic effects of captopril were evaluated at cardiac catheterization in 16 children (age, 0.3-18 years) with cardiomyopathy. Twelve children had congestive cardiomyopathy, whereas four had restrictive cardiomyopathy. Hemodynamic measurements were obtained 30 and 60 minutes after the oral administration of captopril (0.5 mg/kg). Blood pressures were measured in the aorta, pulmonary artery, right atrium, and pulmonary capillary wedge position; cardiac outputs were measured by the thermodilution technique. Hemodynamic data could not be obtained after the administration of captopril in one child with congestive cardiomyopathy because of an immediate, severe hypotensive response. In 11 of 12 children with congestive cardiomyopathy, cardiac index increased by 22%, from 2.3 to 2.8 l/min/m2 (p less than 0.05), and stroke volume increased by 22%, from 23 to 28 ml/m2 (p less than 0.05). Systemic vascular resistance decreased from 32 to 21 units.m2 (p less than 0.01), but the mean aortic pressure did not change significantly. In contrast, four children with restrictive cardiomyopathy had no change in cardiac output after captopril, but there was a trend toward significant arterial hypotension (mean aortic pressure decreased from 78 to 59 mm Hg). Thus, captopril acutely reduced systemic vascular resistance and increased both cardiac output and stroke volume in children with congestive cardiomyopathy. In children with restrictive cardiomyopathy, however, captopril did not affect cardiac output, but it did decrease aortic pressure. These data indicate that captopril may benefit children with a congestive cardiomyopathy but that captopril probably should not be used in children with restrictive disease.

Cardiovascular Status after Repair by Fontan Procedure

Patients surviving a Fontan operation experience dramatic symptomatic improvement, but concern remains about the long-term results of this operation. The clinical course and postoperative hemodynamic findings in 5 long-term survivors of the Fontan procedure from our institution are presented. Attention is drawn to 3 patients who required reoperation: 1 immediately for residual mild pulmonary stenosis, 1 for late onset complete heart block, and 1 for conduit valve stenosis. Review of our patients and those described in the literature reveals that all have ascites and pleural effusions as a results of high venous pressures but that this is usually a temporary problem. Late onset of obstruction to right atrial emptying has been reported in several patients in addition to ours. This raises serious concerns about the long-term fate of cloth conduits and porcine valves in this application. Although normal sinus rhythm has been thought to be essential for adequate pulmonary perfusion in these patients, it is interesting that several patients have tolerated atrial tachyarrhythmias, junctional rhythms, and even complete heart block without serious ill effects. We conclude that the Fontan procedure is extremely effective in relieving symptoms at an operative risk that is now acceptable, but these patients require very careful long-term follow-up because they are subject to a number of long-term complications.

How to Distinguish Between Innocent and Pathologic Murmurs in Childhood

While the distinction between innocent and pathologic murmurs may be readily made in most cases, the ability to clearly define and categorize the innocent murmur by precise criteria is most helpful in identifying those patients who may have more subtle or early manifestations of a pathologic condition. To this end, the author discusses the definition and classification of murmurs, how to evaluate a murmur, classification and features of innocent murmurs, differential diagnosis of the innocent murmur, features of pathologic murmurs, use of noninvasive laboratory studies in diagnosis, and management of the child.