Leonard F. Fagan

Spontaneous echocardiographic contrast in the descending aorta.

The visualization of echocardiographic spontaneous contrast is a common phenomenon in patients undergoing transesophageal echocardiographic studies. Its pathophysiology is not well understood, but it has been related to the presence of a low flow state in the cardiac chambers. We report the presence of spontaneous contrast in the descending aorta of four patients, a location not previously described in the absence of aortic dissection. In two patients, spontaneous contrast was noted in both the left atrium and the descending aorta. In one patient with moderate left ventricular dysfunction, spontaneous contrast was noted in the descending aorta and in the left ventricle. In the remaining case, no cardiac or aortic abnormalities were observed and this represents the first time that spontaneous contrast has been identified in a patient with an echocardiographically normal heart. The occurrence of spontaneous contrast has been considered an abnormal echocardiographic finding, since it has always been described in patients with clinical symptoms and cardiac abnormalities. The fact that spontaneous contrast can be visualized in patients with very mild structural cardiac abnormalities or none at all, warrants further investigation, especially when therapeutic or prognostic implications are considered.

Repair of Aortic Coarctation in the First Year of Life

Twenty-five infants under 1 year of age (mean, 10.3 weeks and 4.0 kg) underwent coarctation repair. Eight had ventricular septal defect (VSD), 3 had transposition of the great arteries with VSD, and 5 had severe tubular hypoplasia. One infant required mitral valve replacement, and 1 required repair of total anomalous pulmonary venous return. Fifteen had repair by primary anastomosis. Seven underwent Dacron or subclavian aortoplasty; the advantages and technique of angioplasty are reviewed. Three patients required bypass grafts. Seventeen patients survived operation. All 5 patients who had severe tubular hypoplasia died postoperatively. The mortality for repair of coarctation with VSD by simultaneous pulmonary artery banding was high; for coarctation with VSD we currently recommend repair without banding, followed by VSD closure if indicated. Three infants have been treated successfully in this manner, with early VSD closure in 1 and regression of the VSD during follow-up in 2. The 17 survivors have been followed for a mean of 41 months with 3 late deaths. Of the 17 survivors, all of whom had a primary anastomosis, 3 have residual gradients. Of the 11 survivors who had preoperative hypertension, 6 are still hypertensive; 3 of these have a gradient between the upper and lower extremities. It is striking that 3 have persistent hypertension despite repair under the age of 1 year.

Comparison of transthoracic and transesophageal echocardiography for assessment of left-sided valvular regurgitation

To compare transthoracic and transesophageal echocardiography in the clinical assessment of left-sided valvular regurgitation, 118 patients who underwent both transesophageal and transthoracic echocardiographic studies within a 24-hour period were included in this study. Presence or absence of aortic regurgitation was identified concordantly by both techniques in 93 patients (79%). Complete agreement between both techniques was found in 88 patients (75%). Presence or absence of mitral regurgitation was identified concordantly by both techniques in 89 patients (75%). Complete agreement in grade was found in only 74 patients (63%). Twenty-nine patients (25%) had mitral regurgitation detected by transesophageal echocardiography, but not by transthoracic echocardiography. Four of these patients (14%) had significant (2 to 3+) mitral regurgitation. Differences between transesophageal and transthoracic echocardiography do not appear to be clinically important in patients with aortic regurgitation. In mitral regurgitation, significant differences exist between these 2 techniques, with transesophageal echocardiography being much more sensitive.

Aortic aneurysm in childhood: report of six instances.

Six instances of aortic aneurysm in children have been observed in the past 15 years. Four children with aortic stenosis and coarctation of the aorta had aneurysmal dilatation of the ascending aorta, and two children with coarctation of the aorta had ruptured post-stenotic dilatation of the descending aorta. Our observations suggest that aortic aneurysm can be the result of hemodynamic stress, although developmental anomaly of the aorta associated with aortic stenosis or coarctation could be responsible for the development of the aneurysm.

Prognostic value of exercise thallium scintigraphy in patients with good exercise tolerance and a normal or abnormal exercise electrocardiogram and suspected or confirmed coronary artery disease.

Exercise thallium scintigraphy is widely used to assess prognosis in patients with suspected or proven coronary artery disease. The incremental prognostic value of this technique in patients who have good exercise tolerance has not been well studied. Two hundred ninety-nine patients with known or suspected coronary artery disease without prior myocardial infarction or revascularization procedure referred for exercise myocardial perfusion imaging and able to exercise to greater than or equal to stage III of the Bruce protocol were included. After a mean follow-up of 50 +/- 10 months, there were 15 cardiac events (5%). The incidence of cardiac events was 10 versus 3% (p less than 0.001) in patients with an abnormal versus normal thallium-201 scan, and 9 versus 3% (p = 0.03) for an abnormal versus normal exercise electrocardiogram. When the 185 patients with a normal exercise electrocardiogram were examined, the incidence of cardiac events was 3% (5 of 150) in patients with a normal scan versus 0% (0 of 35) in patients with an abnormal scan. In the 114 patients with an abnormal exercise electrocardiogram, an abnormal thallium-201 scan was predictive of cardiac events (18% [8 of 44] versus 3% [2 of 70]; p = 0.006). Stepwise logistic regression analysis selected an abnormal thallium-201 scan and abnormal exercise electrocardiogram, low peak exercise heart rate, and male gender as independent variables associated with a significant increased risk of cardiac events. Thus, in patients with known or suspected coronary artery disease and good exercise tolerance, the addition of thallium-201 imaging in patients with an abnormal exercise electrocardiogram provides useful prognostic information.(ABSTRACT TRUNCATED AT 250 WORDS)

Possible X-linked congenital heart disease.

Four male members of a family are known to have congenital mitral and aortic insufficiency, a fifth male has congenital mitral insufficiency only, and a sixth male had congenital heart disease by history. The involved males comprised all males in three generations of this family. Chromosomal and dermatoglyphic studies of the two living affected males are normal.The involvement of males in this family is best explained on the basis of X-linked recessive inheritance. To our knowledge, this is the first report of possible X-linked inheritance of isolated congenital heart disease.

The Blalock-Taussig Shunt in the Neonate

With improvements in the techniques of microvascular surgery, the Blalock-Taussig shunt has been applied to smaller infants. We report our experience in 17 neonates (mean age 9 days, mean weight 3.2 kg) who underwent emergency shung operations. The early mortality was 17.6% (3 of 17), with only 1 death (7%) from renal failure and sepsis, in the last 14 patients. Three shunts were patent but inadequate and required a secondary procedure, which was successful in all 3. There were 3 patients with late shunt failures at a mean of 15 months postoperatively, while 2 are still doing well at 15 and 18 months. No patients developed congestive cardiac failure. The late mortality was high (5 of 14), but was due to late shunt failure and was preventable in only 1 patient. These results are encouraging, and we continue to perform the Blalock-Taussig shung in neonates. It is hoped that improvements in technique will reduce the incidence of inadequate shunts.

The natural and postoperative history of 252 patients with proved ventricular septal defects

Abstract In 252 patients the diagnosis of isolated ventricular septal defects was proved by cardiac catheterization and cineangiography. During a period of one to nine years, none of 163 patients with pulmonary systolic pressures below 50 mm. Hg showed any clinical evidence of progressive deterioration. Of the forty in this group undergoing serial catheterization studies, not one showed any evidence of progressive pulmonary hypertension, and two patients had spontaneous closure of the ventricular defect. It is concluded that patients with pulmonary pressures below 50 mm. Hg can be followed safely without surgical intervention, but continued close scrutiny of this group is extremely important and continued serial studies will eventually reveal the true natural history of patients in this category. The natural course of thirty-six patients with pulmonary systolic pressure above 50 mm. Hg is discouraging. Serial studies in thirteen patients showed a reduction in pulmonary pressure in only five, in two right ventricular outflow hypertrophy developed and in one a coarctation of the aorta was repaired; the rest continued to show signs of severe pulmonary hypertension. Eight patients have died. Direct surgical repair of the ventricular defect has been accomplished with varying degrees of success. The early mortality rate fluctuates with the degree of pulmonary hypertension, the changes in atrioventricular conduction damage and the competence of the surgical team. Each Center must therefore recommend surgical repair according to its own particular abilities when the hemodynamic conditions warrant intervention. The long-term results of successful surgical or spontaneous closure of the defect are still unknown, and must be followed closely to establish the eventual life expectancy.

Myocardial infarction in the perinatal period with long-term survival

A patient with documented perinatal myocardial infarction of unknown etiology with a 5 year survival is presented. Of 5 previously reported cases, all of which were fatal, 4 were unsuspected clinically and were diagnosed at postmortem examinations. The fifth patient had diagnostic electrocardiographic changes of myocardial infarction and the diagnosis was confirmed as postmortem examination. Evidence of difficult delivery was common to these 5 patients.

89 RHYTHM DISTURBANCES AFTER FONTAN PROCEDURE

Persistance of sinus rhythm is common after Fontan type operation (op) and the reported arrhythmias are infrequent. We reviewed 18 patients (pt) who survived Fontan op with regard to the occurrence of rhythm disturbances detected by ECG, Holter monitoring and stress ECG. All underwent op between 1975 and 1984 at age 3-18 years (median 11 years). Anatomical lesions were tricuspid atresia 11, univentricular heart 4, pulmonary atresia 2, transposition and pulmonary stenosis 1. Duration of follow-up was 6 months-9 years (mean 3.2 years) and all had serial ECGs. Preop rhythm disturbances were present in 8 pt (2 had short P-R intervals, 3 had ectopic atrial pacemaker or junctional rhythm and 3 had 1° A-V block). Post-op transient atrial dysrhythmias were seen in 9. Late post-op 1° A-V block developed in 1, sinus bradycardia in 2, PVCs in 3. 24 hour Holter monitor recordings were done in 12: 8 had rare PACs,, wandering pacemaker or junctional beats, 1 had short runs of supraventricular tachycardia. 8 had PVS: 6 uniform, 2 multiform. Bradycardia with junctional escape were seen in 4. Stress tests were done in 10 pt; 2 had ventricular tachycardia, and 1 also had couplets.Conclusion: Transient atrial dysrhythmias were common in immediate post-op period but prolonged atrial tachycardia was not seen. Significant ventricular dysrhythmias occurred but were rare. Sinus bradycardia developed in some. Stress test and 24 hour Holter monitoring were helpful in evaluating and detecting rhythm disturbances in post-op Fontan patients.